Uro-Radiology Text and Atlass

Uroradiology
Text and Atlas

Uroradiology
Text and Atlas
Suresh M Bakle DMRD MD (Radiology)

Professor in Radiology
Nijlingappa Medical College, Bagalkot
Karnataka, India
Bipin V Daga MD DNB (Radiology)
Lecturer in Radiology
VM Govt Medical College, Solapur
Maharashtra, India
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Uroradiology: Text and Atlas

© 2010, Jaypee Brothers Medical Publishers
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First Edition: 2010
ISBN 978-81-8448-784-8
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Preface
This book intends to present complex information on Uroradiology in easily accessible formats. The notes and
images contained within this book have been structured to conform to the format needed for undergraduate and
postgraduate examinations.
In addition, the authors have, where relevant, not only tried to give complete material but also management
aspect in an attempt to provide a useful acid memory for the students.
The main use of this book as opposed to others is for pictorial review of all important cases of urology. Despite
the concise nature of the material and the inevitable omission of some of more complicated issues, we believe
that this book fulfills its purpose and will support the education of postgraduates apart from supporting the practitioners
in the glorious art and science of uroradiology.
The images in the book are unique and the most interesting part of this book mainly includes the common
and classical uroradiology cases.
Suresh M Bakle
Bipin V Daga
Contents
1. Embryology ............................................................................................................................ 1
2. Anatomy and Normal Variants .............................................................................................. 3
3. Various Imaging Modalities and Techniques in Uroradiology ............................................... 7
4. Congenital Anomalies of Urorenal Tract ............................................................................. 27
5. Cystic Disease of Kidney .................................................................................................... 71
6. Calculus Diseases of Kidney ............................................................................................... 78
7. Obstructive Diseases of Kidney .......................................................................................... 92
8. Renal Infections ................................................................................................................ 103
9. Urorenal Trauma ................................................................................................................ 120
10. Urorenal Neoplasms .......................................................................................................... 140
11. Medical Renal Disease ...................................................................................................... 161
12. Renal Vascular Diseases ................................................................................................... 168
13. Radiology of Renal Transplant .......................................................................................... 182
14. Urethral Diseases .............................................................................................................. 192
15. Urinary Bladder Diseases .................................................................................................. 199
16. Diseases of Prostate .......................................................................................................... 212
17. Urorenal Interventions ....................................................................................................... 223
18. Miscellaneous Genitourinary Conditions ........................................................................... 225
19. Recent Advances in Uroradiology ..................................................................................... 231
20. Radiological Contrast Media ............................................................................................. 234
Bibliography ................................................................................................................................... 239

Index ............................................................................................................................................... 241
Introduction and Historical Aspect
“Urology can be said to owe its existence as a specialty to inventing genius of Sir Thomas Edison and
Sir Wilhem Conrad Roentgen. The roentgen rays provided a means whereby diagnostic studies of the
entire genitourinary tract could be carried out.”
— RM NESBITT (1956)
MILESTONES IN URORADIOLOGY
• In 1896, first preoperative radiograph of a calculus was reported by John McIntyre.
• In 1897, French physician Theodore Tuffier introduced a metal stylet into a ureteral catheter to make the catheter
radiopaque and outlined the ureter.
• In 1903, Wittek first used air as a contrast agent.
• In 1905, Voelcker and von Lichtenberg devised first liquid contrast using colloidal A. They also emphasized
the value of lateral and oblique views.
• In 1923, first clinical trial of 10% sodium iodide was done by Earl Osborn, which later became the basis of
present-day IVU study.
• In 1929, Swick first described IVU.
• Smith (1994) first described the role of non-contrast spiral/helical CT in calculus disease.
Uroradiology is a discipline involving imaging techniques like plain radiography, contrast radiography, ultrasound,
computed tomography, radionuclide imaging and magnetic resonance imaging and in any given clinical situation,
the relevant information can be obtained most rapidly and efficiently by using a specific order of radiological modalities
for investigation and evaluation.
Abbreviations
ADPKD Autosomal dominant polycystic kidney disease

ARPKD Autosomal recessive polycystic kidney disease
ATN Acute tubular necrosis
CT Computed tomography
CTA CT angiography
DRC (DIC) Direct radionuclide (isotope) cystogram
DSA Digital subtraction arteriography
IRC Indirect radionuclide cystogram
IVC Inferior vena cava
IVU Intravenous urogram
MCU Micturating cystourethrogram
MRI Magnetic resonance imaging
PUJ Pelviureteric junction
PUV Posterior urethral valve
UTI Urinary tract infection
VUR Vesicoureteral reflux
MIBG Meta-iodobenzyl guanidine
RVT Renal vein thrombosis
RLQ Right lower quadrant
Embryology 1
KIDNEY
The development of the embryonic urinary system
involves three separate stages (Figure 1.1):
1. Pronephros
2. Mesonephros and
3. Metanephros.
1. The earliest structure, the pronephros, forms at about

3 weeks in utero, never functions and is reabsorbed
by the fourth week. Its caudal portion, however, plays
a role in the development of the mesonephros. The
longitudinal pronephric duct becomes the meso-
nephric or Wolffian duct, which is initially connected
to the primitive cloaca. FIGURE 1.1: Developmental sequence of urinary tract
(For color version see Plate 1)
2. Primitive excretory tubules and glomeruli develop
symmetrically from this duct to comprise the meso- the more cranial position of the mature kidney is
nephros which regresses in the latter part of the achieved through differential growth of the urinary
second month. tract relative to the trunk (Figure 1.2).
3. The metanephros or permanent kidney. It then
undergoes division into several small tubules, each BLADDER AND URETHRA
of which becomes associated with a thin part contri- The urogenital sinus is formed from the anterior part of
buted by the metanephric blastema. These caps the cloaca at the time of the descent of the urogenital
differentiate into the mature excretory tubules or septum. That portion of the urogenital sinus above the
nephrons. The presence of the ureteric bud is believed entrance of the mesonephric ducts is called the
necessary for the differentiation of tissues into mature vesicourethral canal and this becomes the bladder.
renal parenchyma. The metanephros is initially located The portion which is inferior is termed the definitive uro-
in the lower lumbar or sacral region and its shift to genital sinus.
2 Uroradiology: Text and Atlas
FIGURE 1.2: Development of collecting system of kidneys

(For color version see Plate 1)
With further development, the distal mesonephric duct
is absorbed into the urogenital sinus until the metanephric
duct (now the ureteric bud) enters separately from, and
superior to, the mesonephric duct. The latter will become FIGURE 1.3: Ascent of kidneys (For color version see Plate 1)
the Wolffian duct, precursor to the ductus deferens in
the male. At maturation it enters into that portion of the responsible for the development of the penile urethra of
definitive urogenital sinus which will become the prostatic the male does not occur and, instead, a connection with
urethra. the developing müllerian ducts gives rise to the lower
The development of the definitive urogenital sinus urethra, a small portion of the vagina and the vestibule
proceeds differently in the female. The elongation (Figure 1.3).
Anatomy and Normal Variants 3
NORMAL ANATOMY the anterior aspect of the spinal column, psoas and
• Anatomy of retroperitoneum paraspinal muscles and the superior aspect of the pelvic
• Anatomy of kidney, ureter and bladder diaphragm. The major organs and their associated fascia
• Anatomy of prostate. develop within the space defined by the transversalis
fascia (Figure 2.1).
ANATOMY OF RETROPERITONEUM During embryological development, as the kidneys
The transversalis fascia lines the inside of the abdo- ascend from the pelvis the surrounding (perinephric)
minopelvic cavity, including the inferior aspect of the fascia forms a cone, with its apex superiorly. The
diaphragm, the posterior and medial surfaces of the perinephric fascia anterior to the kidney is often referred
anterior and lateral abdominal wall muscles respectively, to as Gerota’s fascia, the posterior fascia as Zuckerkandl’s
FIGURE 2.1: Schematic representation of retroperitoneal anatomy (For color version see Plate 2)
fascia and the enclosed space as the perinephric space. adrenal gland, pelvocalyceal system, proximal ureter, and
It contains the kidney, the suprarenal gland anteromedial neurovascular and lymphatic structures. No potential
to the kidney on the left, It superomedial to the kidney communication exists between the 2 perirenal spaces.
on the right the upper ureter and the perinephric fat. There The anterior pararenal space is limited anteriorly by
is a small space between the posterior perinephric fascia the posterior parietal peritoneum and posteriorly by the
and the adjacent transversalis fascia which contains only anterior renal fascia.
fat (the posterior pararenal space). This space is of interest The contents are the first, second, and third parts of
to the interventional radiologist as a potential area in the duodenum; the pancreas; the ascending colon; the
which to kink a guide-wire and mistakenly position a drain descending colon; and the splenic, hepatic, and proximal
intended for the renal collecting system. There is a more superior mesenteric arteries. The anterior pararenal space
substantial space anterior to the perinephric spaces communicates across the midline.
between the anterior perinephric fascia and the posterior The posterior pararenal space is bounded anteriorly
layer of the peritoneum, the anterior pararenal space. by the posterior renal fascia and posteriorly by the
This contains the pancreas and duodenum centrally, with transversalis fascia. The medial extent of this space is
the ascending colon on the right and the descending on limited by the fusion of posterior renal fascia with the
the left. These organs are therefore in direct contact with fasciae of the psoas and quadratus lumborum muscles;
the anterior perinephric fascia. however, communication with the retrocrural space, and
Laterally the anterior and posterior perirenal fasciae therefore the mediastinum, is possible. Laterally, the fat
fuse with the lateroconal fascia at the fascial trifurcation. of the posterior pararenal space continues as the
The lateroconal fascia continues laterally and anteriorly properitoneal fat stripe. The posterior pararenal space
to fuse with the parietal perietal peritoneum. contains no organs. The anterior and posterior pararenal
The retroperitoneum is a large space bounded spaces communicate along their inferior margin.
anteriorly by the posterior parietal peritoneum, posteriorly
by the transversalis fascia, and superiorly by the diaphragm. STRUCTURE OF THE KIDNEY, URETER AND BLADDER
Inferiorly, it extends to the level of the pelvic brim. (FIGURE 2.2)
On either side, the retroperitoneum is divided into 1. Cortex

three compartments by coronally oriented anterior and 2. Compound calyx
posterior renal fasciae, which lie anterior and posterior 3. Minor calyx
to the kidneys, respectively. 4. Medullary pyramid
The anterior renal fascia is a thin layer of connective 5. Papilla
tissue, which is difficult to identify on images. 6. Renal sinus
The anterior and posterior renal fasciae fuse laterally 7. Renal pelvis
to form the lateroconal fascia. The lateroconal fascia 8. Infundibulum of major calyx
extends posterolaterally to the ascending and descending 9. Ureter.
colon and fuses with the parietal peritoneum. Superiorly, Ureter to the level of the ischial spine, from where
both layers of renal fasciae blend with the diaphragmatic it runs anteromedially until it enters the superolateral angle
fascia, and inferiorly, this fuses with the iliac fascia and of the bladder base. The vas deferens crosses over the
periureteric connective tissue at the level of the iliac crest. ureter, separating it from the bladder just before the ureters
Medially, the anterior renal fascia blends with the enter the bladder wall. The ureters run obliquely through
connective tissue and fat that surround the great vessels, the bladder wall for around 2 cm.
and the posterior renal fascia blends with the fascia of A fibrous capsule is closely applied to the renal cortex
the psoas and quadratus lumborum muscles. over the entire kidney apart from the hilum. The kidney
Each perirenal space, which is situated between the is surrounded by perinephric fat and lies within a space
anterior and posterior renal fasciae, contains a kidney, partly enclosed by layers of fascia, traditionally referred
Anatomy and Normal Variants 5
zonal anatomy is well delineated, with the prostatic urethra

serving as the key reference point. On T2-weighted
images, the peripheral zone demonstrates a higher signal
intensity than either the central or the transition zones.
The peripheral and central zones are consistently
distinguishable in men under 35 years old and in 35%
of older patients. The central and transition zones have
similar lower signal intensity and can be differentiated
from each other only by knowledge of their anatomical
location. In young subjects, the transition zone has a
uniformly low signal intensity, but it becomes
heterogeneous with the development of BPH. The
surgical pseudocapsule can be seen in older subjects at
the interface between the transition and peripheral zones.
The anterior fibromuscular band covering the antero-
lateral surface of the prostate gland demonstrates a low
signal intensity, allowing distinction between the prostate
and the anterior periprostatic space composed of vascular
FIGURE 2.2: The vasculature of kidney: note the main renal artery, areolar tissue. On axial T2-weighted images, the shape
the hilar branches, the lobar branches, the segmental branches and of the peripheral zone changes from the base to the apex.
finally the arcuate arteries (For color version see Plate 2)
At the base, the peripheral zone surrounds the
posterolateral aspect of the central zone. The ejaculatory
to as the perinephric space (perirenal space). The ducts can be visualized coursing through the central zone
perinephric space is one of a number of spaces within towards the verumontanum. At the apex, the peripheral
the abdomen and pelvis that are important determinants zone is nearly concentric around the urethra (with the
of the direction of disease spread. exception of the thin anterior fibromuscular band). The
apical urethra is surrounded by muscular fibers giving
MR ANATOMY OF PROSTATE low-intensity signals. The prostatic capsule is visualized
The prostate and periprostatic tissue are visualized well as a thin rim giving a low-intensity signal, surrounding
using CT. On older generation CT scanners, neither the the peripheral zone. The NVB are seen as punctate signal
zonal anatomy nor the differentiation between the voids posterolateral to the capsule at the 5 and 7 O’clock
prostatic parenchyma and the prostatic capsule were positions. The seminal vesicles look like grapes, and are
visualized. With dynamic fast-scanning CT, the contrast well seen in any plane of section.
resolution of CT has markedly improved and intrapro- Periprostatic structures can be differentiated from the
static anatomy can be demonstrated. On sequential CT prostatic parenchyma. The periprostatic venous plexus
scans, prostatic volume can be measured by using the (PVP) is seen on the anterior and lateral aspects of the
single parameters of length, width and AP diameter, or prostate gland. The levator ani muscles give a lower signal
by summation of the prostatic volumes on each slice. intensity than the peripheral zone, regardless of which
On MRI, the ability to demonstrate the zonal anatomy spin-echo TR or TE is used, though the contrast between
of the prostate gland and the distinction between the gland the peripheral zone and the levator ani muscle is enhanced
and periprostatic tissue varies with the imaging planes on T2-weighted images. Unlike CT, MRI in all three planes
and sequences used. On spin-echo (SE) T1-weighted enables the prostate to be differentiated from the
images, regardless offield strength, the prostate shows an surrounding levator ani muscle, bladder neck and lower
homogeneous intermediate signal intensity, and the zones rectum. In the assessment of prostate size using the formula,
cannot be differentiated. On T2-weighted images, the MR is more accurate than either USG or CT.
NORMAL VARIANTS (PSEUDOTUMORS) variation in renal morphology and should not be confused
• Fetal lobulations with a mass. The column of Bertin anatomically and
• Dromedary or splenic hump functionally resembles the rest of the renal cortex.
• Column of Bertin (hypertrophied) The use of different imaging modalities to
Column of Bertin is an extension of normal renal differentiate a column of Bertin from a renal tumor is
cortex into the medulla, towards the hilum. It is a normal listed below:
Modality Column of Bertin Renal neoplasm

1. Ultrasound Isoechoic to cortex Hypo- or hyperechoic relative to cortex
2. 99mTc DMSA Normal uptake Photon deficient area
3. 99mTc DTPA Normal uptake Photon deficient area
4. CT Isodense to cortex Hypodense, rarely hyperdense
Homogeneous contrast Decreased contrast enhancement
enhancement Peripheral Increase
Puddling
Nonhomogeneous
5. MRI Isointense to cortex Hypo- or hyperintense
Nonhomogeneous
Various Imaging Modalities and Techniques in Uroradiology 7
VARIOUS IMAGING MODALITIES AND Satisfactory bowel preparation is important prerequi-

TECHNIQUES site.
• 90% radiopaque calculi (calcium oxalate phosphate,
• Plain radiography (KUB film)
triple, cystine)
• Intravenous urography (IVU)
• ‘Radiologists graveyard’
• Retrograde urography (RGP)
• Antegrade urography (AGP) • Radiolucent calculi missed (uric acid, xanthine matrix,
• Nephrotomography orotic acid stones)
• Cystography and cystourethrography • Other radiopaque densities like pheloboliths, arterial
a. With voiding urethrogram calcifications, lymph nodes, masses may be mis-
b. Retrograde urethrogram leading.
c. Cystogram
d. With DC (air) THINGS TO BE SEEN ON KUBU FILM
e. Triple contrast (angiography plus pneumocysto- 1. Bowel preparation
gram plus interstitial air in wall of UB) 2. Visualized bones
f. Chain urethrogram
3. Properitoneal fat lines
g. Choke urethrogram
4. Renal outlines/shadows
• Ultrasound
5. Psoas shadows
• Color Doppler
6. Calcific density along kidney, ureter, bladder or
• Computed tomography
urethra.
• Non-contrast CT
• Contrast CT
• Magnetic resonance imaging (MRI) FEW ILLUSTRATIONS SHOWING DIAGNOSTIC USE OF
KUB RADIOGRAPHS
• Radionuclide Imaging
• Arteriography See Figures 3.1 to 3.12.
PLAIN KUBU RADIOGRAPH MIMICS OF RENAL COLIC (PLAIN KUBU FILM)

The main use of plain radiography is in calculus diseases. See Figures 3.13 to 3.18
FIGURE 3.1: Ectopia vesicae FIGURE 3.2: Prune belly syndrome FIGURE 3.3: Right pyonephrosis with
calculi
FIGURE 3.4: Nephrocalcinosis FIGURE 3.5: Vesical calculus FIGURE 3.6: Multiple calculi in right kidney
with DJ stent
RETROGRADE UROGRAPHY (RGP) give a good demonstration of the ureter, and the
Close attention to detail in performing a tailored IVU will retrograde introduction of a catheter into the upper ureter
usually result in a satisfactory demonstration of the or renal pelvis is the best method for demonstrating the
pelvicaliceal systems and ureters and retrograde pelvicaliceal system (Figure 3.18). Fluoroscopic guidance
pyelography should rarely be necessary. The latter is should be used and the study should include some oblique
indicated mainly in those patients suspected of having views of the kidney and ureter. The catheter may be left
a urothelial tumour of the upper urinary tract and in whom in a selective position for some hours to collect urine for
excretion urography is normal. Bulb ureterography will cytological examination.
FIGURE 3.7: Left putty kidney FIGURE 3.8: The lower poles of both kidneys—renal FIGURE 3.9: Seminal vesicle calcification
shadows are seen approaching towards spine
instead of divergent or laterally directed lower poles
as should be seen normally—Horse-shoe kidney)
FIGURE 3.10: Bladder-wall calcification— FIGURE 3.11: Postoperative KUB film with
schistosomiasis some prosthesis in urinary bladder
INDICATIONS ANTEGRADE UROGRAPHY (AGP)

• Mainly used for better visualization of lower ureter Antegrade pyelography is an accurate method of
and pelvi-caliceal system demonstrating precisely the site of an obstruction to the
• IVU not satisfactory upper urinary tract. The IVU with delayed films may
• Contrast reaction render IVU hazardous outline the obstruction, but the concentration of contrast
• Renal failure may be poor and the final diagnosis only made on a
• To delineate exact site of the Ureteric strictures 24 hour film. Helical CT is a more rapid and accurate
• Filling defects like: tumors, sloughed papillae method of demonstrating the site (and possible cause)
radiolucent calculus, are better seen on RGP. of obstruction.
FIGURE 3.12: Large calculus in a FIGURE 3.13: Extensive pancreatic calcification FIGURE 3.14: Worm infestation, as seen in
horse-shoe kidney on left side right lower quadrant
FIGURE 3.15: Colon studded with fecoliths FIGURE 3.16: Enteroliths
Ultrasound will rapidly confirm the presence of for cytology. Once some reduction of pressure has been
dilatation in the pelvicaliceal system and one can then achieved within the system, contrast medium is injected
proceed directly to antegrade pyelography. With the patient to outline the pelvis and ureter down to the level of
prone, a fine 20 or 22 gauge Chiba needle is inserted under obstruction and it may be necessary to tilt the patient into
ultrasound guidance or fluoroscopic control beneath the a semierect position to achieve this.
12th rib into a lower pole calyx, under local anesthesia. As
INDICATIONS
soon as the collecting system is punctured, trapped urine
will escape through the needle. This should be aspirated It gives an opportunity to perform interventional
and sent for culture and, if a urothelial tumor is suspected, procedures like:
FIGURE 3.17: Ovarian dermoid with tooth- FIGURE 3.18: Normal RGP
like calcification
• Decompression of PUJ obstruction • Longer duration required

• Drainage of pus in pyonephrosis • Radiation hazard.
• PCNL-Stone removal and Stenting An Intravenous Urogram (IVU) is an X-ray examina-
• Biopsy and balloon occlusion. tion of the kidneys, ureters and urinary bladder. Most
people are familiar with X-ray images, which produce
ADVANTAGES OF AGP
a still picture of the body’s interior by passing small, highly
• Direct site of obstruction shown (mid. 1/3 ureter) controlled amounts of radiation through the body and
• Ureteral stenting can be done capturing the resulting shadows and reflections on film.
• Brush—Biopsy from suspected lesions can be obtained An IVU study uses a contrast material (iodine) to enhance
• Stone manipulations can be attempted. the X-ray images. The contrast material is injected into
the patient’s system and its progress through the urinary
DISADVANTAGES OF AGP
tract is then recorded on a series of quickly captured images.
• To assess the prognostic value of therapeutic
The exam enables the radiologist to review the anatomy
intervention, urosurgeons demand IVU and the and the function of the kidneys and urinary tract.
functional aspect of urinary system is always better
commented and documented on IVU only EXCRETORY UROGRAPHY (INTRAVENOUS
• Invasive procedure. UROGRAPHY, IVU, IVP)
Since 1929, when the first intravenous contrast agent was
INTRAVENOUS UROGRAPHY (IVU)
developed by Swick, excretory urography has been the
INTRODUCTION primary modality for imaging the urinary tract. This
• Widely used study original agent, Uro-selectan, was the precursor of the
• Require bowel preparation numerous excellent contrast agents available at the
• IV contrast media required present time. As early as 1937, Berger made several
• Indirect signs-delayed nephrogram urogram, recommendations for improvement in technique that are
hydronephrosis, hydroureter can be absent in acute, still valid today. The elimination of the obscuring effect
partial obstruction. of abdominal gas has since been accomplished by
routine tomography. A greater renal concentration of and yet the potential for greater distention of the collecting
contrast media has been facilitated by use of higher doses system. Conversely, sodium undergoes extensive
of the safer modern contrast agents, and ureteral reabsorption by the renal tubule. While the plasma half-
compression, also advocated, is now widely practiced. life is relatively low for both meglumine and the contrast
State of the art urography is now such that with anions, that for sodium is longer because of reabsorption
“tailoring” and proper attention to detail, the renal and subsequent refiltration. The delayed excretion of
margins and parenchyma, as well as the entire collecting sodium due to reabsorption by the tubules leads to a
system including the ureters and bladder, can be visualized decreased osmotic diuresis compared with that achieved
diagnostically. Techniques have also been described for with the meglumine salts and thus results in increased
evaluating the urethra as a final step in urography. This concentrations of iodine in the urine, purportedly
procedure has now attained a high degree of safety as providing higher contrast and hence better visualization.
well as efficacy. The mortality rate of 1 in 75,000 is similar Facts such as these have led some investigators to
to that expected from the parenteral administration of recommend using only the sodium salts for urography
penicillin. because of the supposed increased contrast attainable.
Others have recommended the meglumine salts because
PHYSIOLOGY OF RADIOGRAPHIC CONTRAST of the increased distention of the collecting system that
EXCRETION
may be encountered. In fact, McClennan and Becker
Commercially available contrast agents that are approved showed that in a comparison of sodium and meglumine
for urography consist of ionic salts of benzoic acid salts, films showed no reliable difference in a
derivatives containing three iodine atoms. The two anions heterogeneous patient population or even in the same
used most widely in the United States are diatrizoate and patient. The advantage of greater collecting system filling
iothalamate. Following injection, their distribution in the as may be seen with the meglumine salts can be achieved
body and routes of excretion are identical, and their with any of the commercial preparations by application
visualization in the kidneys is equal. Assuming normal of adequate ureteral compression. Attempts to maximize
renal function, their route of excretion is greater than 99% the benefits of both cations have resulted in the production
by glomerular filtration. Other commercially available of preparations combining the two.
anions are Iodamide and Metrizoate. Cations most The quality of the urogram depends on good
commonly encountered are sodium and methylglucamine pelvocaliceal concentration of contrast media as well as
(meglumine) or a mixture of the two. sufficient distention of the collecting system. All attempts
After intravenous injection, whether by bolus or by to improve urographic quality are based on manipulation
infusion, these preparations leave the vascular system of these two variables. Elkin has found that as one goes
in two ways. First, rapid permeation of the capillary wall from small to large doses of contrast media, the urine
and equilibration with the extracellular fluid occurs. At concentration of organic iodide increases. He also found,
the same time, contrast in the bloodstream undergoes however, that there was an upper limit of such
glomerular filtration and subsequent excretion in the concentration, which, when exceeded, led to even greater
urine. As plasma contrast concentration falls as a result diuresis and subsequent dilution of iodine in the urine.
of ongoing renal excretion, there is a continual With the high doses of contrast now in common use,
redistribution of the extracellular contrast into the vascular the production of urine with a very high concentration
system. of iodine is not so much the main objective as is the
Meglumine is not metabolized and is excreted entirely duration of the high concentration of the contrast medium
by glomerular filtration. This results in a higher urinary in the urine. Thus exposures have a greater likelihood
solute output and a subsequent increase in urinary flow of being obtained at a time of peak concentration, and
rate due to an osmotic diuresis. The final result is a lower there is also a prolongation of the interval during which
concentration of urinary iodine with the meglumine salts optimal tomography can be performed. Talner states that
with large doses of contrast (30–42 g of iodine), kidneys in a rapid sequence following bolus injection of
urographic quality will be consistently good. With average contrast. The minimum series includes films at 1, 2, and
doses (17–20 g of iodine), urographic quality can be just 3 minutes post injection. The radiographic criteria for
as good if one pays attention to details of technique. In renovascular hypertension include delayed visualization
a busy department in which many urograms are of contrast in the collecting system on the affected side,
performed each day, large doses may be justifiable as decreased renal size, and delayed wash-out of contrast
routine simply because the radiologist may not have time on the later films in the urogram. A secondary finding
to monitor each study carefully. There have been no is that of notching of the proximal ureter on the involved
reports of any increase in the incidence of side-effects side due to development of collateral flow via the
with the use of larger contrast doses. periureteral plexus reconstituting the renal artery.
Thornbury and co-workers have evaluated the
INDICATIONS
hypertensive urogram with regard to its efficacy. They
By current standards of medical practice, almost anyone found the true-positive rate in 197 patients with
suspected of urologic disease will be recommended for renovascular hypertension to be only 60%. Furthermore
an intravenous urogram, although this may vary from
the predictive value for favorable surgical outcome in
hospital to hospital because of the availability and
hypertensive patients with positive hypertensive urograms
acceptance of the newer imaging techniques. Another
was only 24%. They concluded that the examination is
variable is the preference and experience of the referring
a nondiscriminatory test for renovascular hypertension.
clinician. Among the more common indications for
Their recommendation was that the suspicion of
urography, Friedland and co-workers list infections, acute
renovascular hypertension likely to be surgically
genitourinary pain, hematuria (microscopic or gross),
correctable should prompt the clinician to select renal
trauma, suspected neoplasm, renal transplantation,
arteriography and assays of peripheral and renal venous
neurogenic bladder, congenital anomalies, and
renin activity. Recently, digital subtraction angiography
investigation of complications following a surgical
has been claimed to provide good visualization of the
procedure. These investigators further stated that
renal arteries and may eventually become a satisfactory
urography often is not useful in the evaluation of acute
screening examination for renovascular hypertension.
pyelonephritis (unless obstruction is suspected), ureteral
colic in which the calculus is clearly visible on the plain The present-day urogram is not a reliable test for either
film, in the staging of lymphoma or testicular neoplasms, the presence or severity of functional impairment of the
in cases of medical renal disease, in medical hematuria, kidneys. Modern contrast media contain more iodine
in renal failure, and for the investigation of benign atoms per molecule than did the older agents and are
prostatic hypertrophy. However, clinical experience of exceedingly low toxicity. The current use of high doses
indicates that many patients with these diagnoses will results in such high plasma concentration of contrast that
eventually undergo urography at some stage of their even kidneys with significantly decreased function may
diagnosis or treatment. produce diagnostic studies.
There are several indications for immediate urography. Useful information can be gained with regard to the
These include trauma involving the genitourinary system, presence or absence of obstruction in many patients with
massive gross hematuria of unknown cause, a suspected renal failure, assuming tomography is used for better
vascular accident involving the kidneys, overwhelming visualization of the poorly opacified collecting systems.
sepsis that may possibly have its source in the urinary A better modality for evaluating such patients, however,
tract, and suspected ureteral calculus. is renal ultrasound, which is an excellent screening
In the past, indications for urography have included examination for suspected urinary tract obstruction. Its
the evaluation of hypertension. The hypertensive usefulness is based on its ability to detect hydronephrosis.
urogram involves obtaining coned-down films of the However, it must be realized that there exist a significant
number of conditions that can mimic or produce dilatation detectable increase in urographic density, but the
of the collecting system in the absence of obstruction. nephrogram is unaffected. Also to be remembered is that
Renal sonography suggestive of hydronephrosis should severe fluid restriction may occasionally result in
be followed by additional diagnostic studies to confirm inadvertent dehydration, which may be detrimental to
or exclude obstruction. the patient with multiple myeloma, diabetes, or renal
failure who is undergoing urography. The purpose of
CONTRAINDICATIONS
maintaining the patient on an NPO basis for several hours
There are no absolute contraindications to urography. prior to the examination has as its primary purpose the
There are, however, several situations in which the prevention of emesis with possible aspiration following
potential risks must be weighed against the possible injection of the contrast.
diagnostic benefits. These conditions; include combined
renal and liver failure, multiple myeloma, pregnancy, BOWEL PREPARATION
previous reactions to contrast media, history of allergy, In the past, enemas have been advocated before
infancy, thyroid disease, renal failure, and diabetes urography. However, water may be absorbed from the
mellitus. bowel, and in some cases large amounts of gas are actually
In patients with multiple myeloma or diabetes, proper introduced into the colon during the enema. Many bowel
hydration may prevent the possibility of renal damage preparation kits are available commercially. Dulcolax
from injected contrast. If it is essential to perform tablets, two to four 5 mg tablets at bedtime prior to study,
urography during pregnancy, minimal radiation exposure usually provide an adequate bowel preparation. They
can be accomplished by obtaining a plain film and a are useful in eliminating feces and gas.
30 minutes postinjection film. In patients with a history
of previous serious reactions to contrast, pretreatment with PSYCHOLOGICAL PREPARATION
cortico-steroids and the use of a different type of contrast Lalli has reported that anxiety appears to be a factor in
are recommended, although this is not a proven means the so-called idiosyncratic reactions (nausea and vomiting,
of preventing another reaction. Major life-threatening urticaria) following contrast injection, although this is an
reactions do not usually recur, whereas minor reactions unproven hypothesis. Certainly, every effort should be
tend to be repeated more often. During infancy, normal made to decrease patient anxiety during any procedure.
hydration will counteract the marked rise in plasma A gentle, relaxed approach to the patient in an
osmolarity that may occur following contrast injection. atmosphere of pleasant ambiance can do much toward
Urography should be avoided in patients with thyroid putting the patient at ease. If possible, to minimize pain,
disease who are undergoing either diagnosis or treatment larger rather than the smaller veins should be used for
of their problem, since the iodine-containing contrast may the injection.
block the thyroid uptake of radioisotopes of iodine. In
renal failure, it must be accepted that in a very few cases INFORMED CONSENT
renal function will decrease following urography. The need for informed consent prior to urography is
controversial at the present time. Certainly, the time
PATIENT PREPARATION
required to inform the patient in detail of the possible
Dehydration complications from urography as well as to obtain his
It has been well established that no significant dehydration witnessed signature delays the completion of a heavy
(change in urine osmolality) occurs with the standard schedule. It is recommended that the patient be
regimen of nothing by mouth (NPO) after midnight. questioned regarding previous reactions to contrast and
Further, it has been shown that at least 22 hours of fluid informed that he will likely feel a warm flushed feeling,
restriction is required to reliably dehydrate human a metallic taste in the mouth, and perhaps a mild wave
subjects. Effective fluid restriction may produce a slightly of nausea during and following the injection.
EXPOSURE FACTORS (THE PHYSICS OF UROGRAPHY) tending to cancel the sharper detail. Tube sweep times
Both the plain film and the urogram should be exposed of less than 1 second require generation of very high
at 66 kV(p)–70 kV(p). In general, the mean energy of current. A tube sweep time of greater than 2 seconds
polychromatic radiation is between one third and one invites patient motion and tube sway or unsmoothness.
half of its peak energy. Thus, the mean energy of the
PLAIN FILM OF THE ABDOMEN (SCOUT FILM;
recommended X-ray beam will be very close to the KIDNEYS, URETERS, BLADDER; PRELIMINARY FILM)
K-shell binding energy of iodine (33.2 keV). If the binding
A preliminar y plain film of the abdomen is an
energy of iodine and the mean of the diagnostic X-ray
beam are approximately the same, many interactions will indispensable adjunct to excretory urography (See Table
occur at the K-shell level. This allows maximum 3.1). No attempt to interpret a urogram should be made
attenuation of the beam, since attenuation is increased if a scout film is not available. Otherwise, erroneous
when the photoelectric effect predominates. As the interpretations of areas of increased contrast density such
radiation energy is increased, compton scattering comes as calculi may be made, and, conversely, when stones
more into play and cannot attenuate a beam as rapidly are present, they may be obscured by the contrast and
as the photoelectric effect. thus missed entirely.
Another advantage of the diagnostic beam energy of The most common deficiency of the preliminary film
66 kV(p)–70 kV(p) is the increased ability to image is failure to demonstrate the upper poles of the kidney
calcium-containing structures. At lower beam energies, or the region of the prostate. It has been reported that
calcium structures have a higher linear attenuation the entire urinary tract is included on a single 14 × 17
coefficient than soft tissue, resulting in greater contrast. inch film in only 17% of adult patients of average height.
As the energy of the X-ray beam increases, differential This figure seems somewhat high, but certainly, in a
attenuation between calcium structures and soft tissue significant percentage of patients, coned plain views of
is not nearly as large, resulting in reduction of image the kidneys or pubic area may be necessary to complete
contrast. Therefore, small stones that are seen easily at the preliminary evaluation. The importance of visualizing
70 kV(p) are progressively more difficult to see as the the upper renal poles and adrenal areas is self-evident
voltage increases. At 100 kV(p) many small stones cannot in that calcifications or a mass effect may be detected
be seen. on the plain film. Similarly, evaluation of the prostatic
Generally speaking, a medium-speed calcium area is necessary prior to administration of contrast to
tungstate screen/film combination is most desirable for determine the presence or absence of calcifications that
urography. If this combination is assigned a speed of 1, may suggest the diagnosis of prostatitis. In trauma
screen/film systems in the speed range of 2 to 5 offer patients, it is especially important to visualize the lower
improvement in overall quality due to diminished ribs and the entire bony pelvis for the evaluation of
respiratory motion at the cost of some loss of detail and possible fractures that could affect the urinary system.
increase in mottle. However, this trade-off is less significant If calcifications are seen on the plain film overlying
if higher-output generators are used to minimize the the renal shadows, it becomes necessary to further study
exposure time. the patient with appropriate oblique views. A right
In tomography, the motion of the arm should be linear posterior oblique is necessary for evaluation of
as the study progresses along the long axis of the kidney. calcifications questionably within the right kidney, and
A medium arc of 30º to 40º is thought to be preferable. similarly, a left posterior oblique is obtained to evaluate
There is an inverse relationship between the magnitude left-sided calcifications. If, on the oblique views, the
of the arc and the thickness of the cut obtained. The calcifications remain in constant relationship to the renal
greater the arc, the thinner the section, resulting in shadow, it can be stated with a reasonable degree of
sharpening of detail. However, more tomographic cuts assurance that they are renal in origin. On the oblique
may be needed, and, as section thickness decreases, as views, calcifications anterior to the kidney will be projected
would occur with larger arcs, contrast may also decrease, lateral to the renal shadow and calcifications posterior
Table 3.1: Intravenous urography

Radiography Modification Purpose
Plain films Additional obliques or tomograms To assist the location of potentially intrarenal opacities
Rarely required; ultrasound and other imaging maneuver usually
preferable
Nephrogram Thick slice tomogram To improve definition of the renal outlines

Omit alongwith the 5 min film and To reduce radiation dose
Take a solitary 3 min film
15 min film Second injection of contrast To improve opacification of the pelvicalyceal systems if inadequate
14 min compression Series of 1 cm thick tomograms To differentiate between overlying shadows and filling
film defects within the collecting systems
To delineate the renal outlines when inadequately seen (better done with
ultrasound)
15 min release film Additional bladder views When the bladder is poorly filled on the release film (as is often the
case) delayed films of the fuller bladder may be performed.
When equivocal filling defects are seen in the bladder area oblique films
may be performed
The above additional bladder views are rarely indicated as they increase
the radiation burden to the patient and the relevant clinical problems are
better answered by ultrasound or cystoscopy. However, occasionally a
small suspected calculus in the distal ureter may be confirmed with the
appropriate oblique
Full length post- Bladder area only If the upper tracts have already been adequately imaged then imaging
micturition film the bladder area alone with reduce the patient’s radiation burden
Omit The preceding films may have already provided all the information required
from the investigation
Prone full length film Additional view Where the renal pelvis is dilated, contrast may be slow to pass into the
ureter; this can be accelerated by positioning the patient prone when
the heavier contrast will run anteroinferiorly into the ureter, often to the
level of the obstruction. Simply asking the patient to sit or stand for a
few minutes first may improve the result.
Erect images Additional radiograph or fluoroscopy If it is difficult to determine whether or not there is a small ureteric calculus,
an erect oblique radiograph of the ureter or screening the ureter in this
position may be useful on rare occasions
Frusemide IVU Administration of 20 mg of frusemide If suspected pelviureteric junction obstruction is being

Intravenously after the 15 minute film investigated and there is no evidence of this on the
With a further film 15 minutes later Standard IVU, this maneuver can be performed. It
May provoke hydronephrosis and pain. It is rarely necessary if the patient
is to be investigated with radionuclide renography, as is often the case
in this situation.
to the kidney will be projected medial to the renal shadow. might suggest neurogenic dysfunction of the lower urinary
This allows more cogent comment as to their specific tract. If the spine is not seen clearly, lateral views are
etiology. recommended for better assessment of its integrity. If
In children, careful coning of the films is necessary tomograms are planned following injection of contrast,
to minimize radiation exposure. The preliminary films a scout tomographic section should be obtained to ensure
should include the pubic symphysis to exclude exstrophy that the tomographic series will be adequate for evaluation
and the lumbosacral spine to exclude anomalies that of the renal outlines. The midpoint for tomographic
sections (distance from table top) is easily determined tomograms and other special views at a later point in
by multiplying the thickness of the abdomen in the study.
centimeters at the level of the lower costal margin by 0.4.
The figure obtained is rounded to the nearest whole DOSAGE
number and this becomes the middle of three cuts. The concept of a routine dose is important, since there
The scout tomographic section should reveal a are no useful body nomograms of weight and surface
reasonably large portion of the renal outline as an area for every patient from which to determine an ideal
indicator that the planned section levels will encompass dose. As discussed above, doses in the range of 17 to
the entire renal outline following injection of contrast. 20 g of iodine for the average size adult will result in
In accomplishing this, it should be remembered that the diagnostic studies if careful monitoring is performed.
long axis of the kidney is slightly oblique to the long axis However, larger bolus doses of 28 to 40 g of iodine will
of the body, with the upper pole of the kidney lying more more consistently provide diagnostic urographic studies
posterior and the lower pole more anterior. in the busy radiology department. Thus, the doses in Table
The limitations of tomography are such that the 3.4 reflect the larger dose of radiographic contrast
normal routine as described above permits visualization material. Modification of these doses is recommended
of the renal margins only when they are situated tangential in patients with diabetes, multiple myeloma, renal failure,
to the X-ray beam. Distortions of the renal outline in other and congestive hear t failure. In such patients,
approximately one half the recommended normal adult
areas (anterior or posterior) may thus be missed.
dose should be sufficient to gain significant diagnostic
CONTRAST ADMINISTRATION
information about the urinary tract.
In children, weight of the patient is a more prominent
BOLUS VERSUS INFUSION factor, and recommended doses are based on this
The physiology of contrast excretion has been discussed parameter. Administration of contrast should take place
in an earlier section. There are few if any indications for through a 21 or 23 gauge scalp vein needle, whereas
choice of a specific contrast agent other than personal in the adult, a 19-gauge scalp vein needle is of adequate
preference and economic factors. caliber for bolus injection or infusion technique. In the
In the past, there has been considerable controversy adult, the needle is often left in place for the major portion
regarding the advantages of the bolus injection technique of the examination to allow a rapid access to the venous
versus the infusion technique for administration of radio- system should treatment of contrast reaction become
necessary. This is frequently difficult in the younger age-
graphic contrast agents. There are advantages to both.
groups.
In most centers, the bolus technique is currently used.
Its major advantage is that it allows the maximum plasma
FILM SEQUENCING
concentration of contrast media. An equivalent amount
GENERAL CONSIDERATIONS
of iodine given by infusion over a period of 5 to 10
minutes does not allow as high a plasma level as can The preliminary film of the abdomen and scout
be obtained following the bolus injection. tomographic section have been discussed above. If these
Infusion, on the other hand, is a somewhat more are technically and anatomically adequate, the urogram
convenient way of administering the contrast material, can proceed following injection of contrast by whatever
from the point of view of both the physician and the method has been selected. Picture of normal urogram
patient. The unpleasant flushing sensation that often can be seen in Figures 3.19 to 3.21. Table 3.2 lists a
accompanies bolus injections of a large dose of contrast recommended sequence to be followed for both the
is minimized by the longer infusion. Furthermore, infusion pediatric and adult age-groups. In the infant, bowel gas
achieves a diagnostically useful nephrogram over a longer may obscure the kidneys to a significant degree. The
time interval. This allows greater latitude in obtaining adverse effect of this gas can be minimized by allowing
the child to drink a commercial soft drink that contains allow adequate visualization of the renal outlines and
carbon dioxide. This causes distention of the stomach parenchyma.
and provides a window for visualization of the kidneys.
Alternatively, prone filming for the early view of the
kidneys will often act as a form of compression and
displace colon and stomach gas away from the renal
areas. In the adult, while abdominal bowel gas is
somewhat bothersome, routine use of tomography should
FIGURE 3.20: Normal IVU
FIGURE 3.19: Schematic diagram showing important renal structures

1. Renal cortex 2. Infundibulum 3. Minor calyx
4. Major calyx 5. Papilla 6. Infudibulopelvic junction
7. Renal pelvis 8. Infundibulum 9. Ureter
Table 3.2: Recommended film sequence
Pediatric urography
1. Plain film of abdomen-appropriate size and collimation
2. Postbolus injection
3. 1 minute full view
4. 10 minute full view
Adult urography
1. Plain film of abdomen
Full view
Coned view as necessary to include kidneys or prostatic area
Oblique views as necessary for calcifications over kidneys
Scout tomogram –0.4 × body thickness at costal margin
2. Postbolus injection FIGURE 3.21: IVU
3. 1-minute tomograms, 3–5 views as necessary
4. 5-minute full view 1. Calyx 2. Infundibulum 3. Pelvis
5. Apply compression
10-minute full view with compression in place As a general rule, all urographic exposures should be
Release compression
Full view immediately after release of compression (“release,”
made with the patient in deep expiration. This allows
“flush,” or “X” film) the kidneys to reach the upward limit of their mobility
6. Full view post voiding
with subsequent straightening of the ureter and minimizes
the possibilities of ureteral tortuosity or kinking. COMPRESSION

Furthermore, the patient should be instructed to void prior Following the 5 minute full view, many authorities
to the study to allow better opacification of the bladder recommend routine application of ureteral compression
on later films. unless contraindicated (Table 3.3). The most significant
It should be remembered that there is no single”best”
of these contraindications is that of known or suspected
method for sequencing of films on the standard urogram.
aortic aneurysm. This presumes that the preliminary film
Indeed, it would be rare to find two institutions performing
of the abdomen will have been scrutinized carefully by
exactly the same sequence of films. A ‘‘standard”
the radiologist for calcifications in the aortic wall of
urogram, as outlined in Table 3.3, can therefore be
configuration to suggest aneurysmal dilatation.
recommended only as a basic guide from which one may
While some radiographic tables have a built-in
subsequently develop his own criteria and techniques.
compression band that can be applied, this does not
TOMOGRAPHY (ADULT) appear to be as effective as the Velcro belt device with
inflatable balloons. The position of the balloons is critical
The astute radiologist should consider using tomography
in providing adequate compression of the ureter against
in virtually every adult patient undergoing an initial
the sacral promontory. The balloons should nearly touch
standard intravenous urogram. It should be noted that
in the midline when inflated, and their upper edges should
tomography of the kidneys during intravenous urography
parallel the upper level of the iliac crest. The key to success
does not bear the same connotation as the
is a tight application of the belt while the patient is in
“nephrotomography” used in the past. This latter
deep expiration prior to inflating the balloons. This allows
technique involved injection of very high doses of contrast
as much compression as possible to be obtained with
through a large-bore needle with the timing of the
the belt itself. Pain in the renal areas during or after
tomographic sections based on previous determination
application of compression usually indicates that there
of circulation time. With the high doses of contrast
currently used, entirely adequate tomographic sections has been rupture of the collecting system with pyelosinus
are obtained if exposed approximately 1 to 2 minutes extravasation. Compression should then be released
following the bolus injection of contrast or after half of immediately. This minor complication is usually of no
the contrast (150 ml) has been infused in the drip infusion consequence. However, if the patient is known to have
technique. If tomography is not to be performed, good infected urine, appropriate antibiotic coverage is
visualization of the renal outlines and full thickness of indicated.
parenchyma can be obtained by exposing a coned view After the compression cuff has been in place for
of the kidneys at 1 to 2 minutes following bolus injection approximately 4 or 5 minutes, the 10 minutes full view
of contrast. is obtained. If compression has been adequately applied,
there will be complete and adequate opacification of the
Table 3.3: Contraindications to compression upper ureters and entire intrarenal collecting system. Not
Suspected or proven aortic aneurysm (check plain film for typical infrequently there will be mild distention of the collecting
calcifications) system due to the minimal degree of obstruction caused
Evidence of obstruction on early urogram films
Recent abdominal surgery by the compression cuff. Following release of
Urinary diversion compression, a full view is exposed immediately. This
Severe hypertension
Abdominal pain on application of compression device has been termed the ‘‘flush’’, ‘‘X’’, or ‘‘release” view and
Recent acute injury (trauma) is designed to visualize the distal ureters as a result of
Renal transplant
Abdominal distention the rapid distal passage of contrast from the distended
Bowel ostomies upper systems.
junction and vice versa. Coned oblique views of the

bladder are also useful for imaging filling defects in the
bladder and irregularities of the bladder wall and for
determining the presence of extravasation in the
retrovesical region. A coned postvoid view of the bladder
is a necessity when evaluating questionable filling defects.
This view often enhances a persistent defect.
Delayed films are essential when an obstructive
nephrogram is seen on the routine early views. A
recommended sequence for these delayed views is 30
minutes, 1 hour, 2 hours, 4 hours, 8 hours, and 24 hours.
When obtaining delayed views, the patient should be
instructed to void prior to exposure of the film so that
a calculus at the level of the ureterovesical junction will
not be obscured by the full bladder. Unless the degree
of obstruction is extremely severe, at some point during
this sequence of delayed films contrast can usually be
seen columning in the ureter to the point of obstruction.
If the degree of obstruction is such that there is no
FIGURE 3.22: IVU: Postvoid film columning by 24 hours, further films are not indicated.
1. Sacrum 2. Ureter (distal) 3. VUJ 4. Bladder 5. Pubic symphysis
In such patients further imaging modalities will be
necessary to determine the anatomy of the obstructed
POSTVOID FILM (FIGURE 3.22) system. These may include antegrade or retrograde
The patient is requested to void in privacy and a full pyelography. Routine full views obtained in both
postvoiding view is obtained. It should be apparent that obliquities are a standard part of the urographic sequence
the environment in most radiology departments is not at some institutions. This will depend on the personal
conducive to normal voiding. While comment should be preference of the radiologist.
made on the radiographic report concerning the presence
LIMITED UROGRAM
and amount of postvoid residual urine, the only valid
inference that can usually be drawn from a postvoiding This study usually consists of a preliminary film of the
film will be in those patients who demonstrate an empty abdomen followed by a single full view obtained
bladder. 10 minutes after injection of a standard dose of contrast.
Indications for such studies would include, for example,
TAILORING follow-up on a patient who has recently passed a ureteral
If the study is being monitored after each exposure, calculus that was diagnosed by a standard urogram at
significant abnormalities can be noted and appropriate an earlier date. In some institutions, the limited urogram
deviations from the standard sequence requested. These is used in young female patients with signs and symptoms
may include oblique tomographic sections, coned oblique of acute pyelonephritis to exclude obstruction as a possible
views of the kidneys, or full oblique or prone views to cause. Certainly, in children, a more limited study is
hopefully provide better filling of the distal ureters. If it indicated as is noted. Most reports in the literature and
becomes necessary to better visualize the ureterovesical standard texts recommend a film shortly after injection
junctions, the right posterior oblique coned view of the followed by another full view at 5 or 10 minutes
bladder will allow visualization of the left ureterovesical postinjection.
are pregnant. The effective radiation dose from this

procedure is about 1.6 mSv, which is about the same
as the average person receives from background
radiation in six months. Radiation disadvantages are
further minimized by:
• The use of high-speed X-ray film that does not require
much radiation to produce an optimal image
• Technique standards established by national and
international guidelines that have been designed and
are continually reviewed by national and international
radiology protection councils
• Modern, state-of-the-art X-ray systems that have very
tightly controlled X-ray beams with significant filtration
and X-ray dose control methods. Thus, scatter or stray
radiation is minimized and those parts of a patient’s
body not being imaged receive minimal exposure.
LIMITATIONS OF IVU
FIGURE 3.23: Focal hydrocalicosis at right upper pole with
ureteric strictures–tubercular An IVU shows details of the inside of the urinary tract
including the kidneys, ureters and bladder. CT or MRI
may add valuable information about the functioning
tissue of the kidneys and the surface and surrounding
structures nearby the kidneys, ureters and bladder. IVU
studies are not usually indicated for pregnant women.
MODIFICATIONS OF IVU
Modern non-ionic contrast agents do not provoke an

osmotic diuresis and the degree of opacification is unlikely
to be significantly altered by dehydration. Fluid restriction
should therefore be avoided and if there is a risk that
the patient is dehydrated before the IVU this should be
corrected first.
The classical series of plain films (immediate, 5 and
15 minute, full length release and postmicturition) is
FIGURE 3.24: Black or negative pyelogram on right side
described, with mention of some of many potential
modifications. A preliminary postmicturition plain film
An Abnormal Pyelogram (KUB) is performed. This should be examined to check
See Figures 3.23 and 3.24 exposure factors, centring and obvious pathology, particu-
larly urinary tract calcification. Intravenous contrast is
DEMERITS OF IVU given relatively rapidly by hand. The standard dose is
• Contrast materials used in IVU studies can cause 50 ml of 350–370 strength water-soluble contrast. Some
adverse reactions in some people understanding of the underlying structure of water-soluble
• Women should always inform their doctor or contrast agents is desirable and a knowledge of potential
X-ray technologist if there is any possibility that they adverse reactions and their treatment is imperative. These
issues are discussed under water-soluble contrast (p. 926). distal sphincter mechanism. Water soluble contrast
At this point it is worth emphasising some safety features. medium should be introduced by means of either a clamp
Although modern contrast medium is exceptionally safe, and nozzle (Knutsson’s clamp) or a Foley balloon catheter
there is a small risk of serious reactions. The most gently inflated in the fossa naviculare just proximal to
dangerous of these are the anaphylactoid-type the urethral meatus. For a simple ascending urethrogram
hypersensitivity reactions. to demonstrate a penile or bulbar urethral stricture the
clamp is easy and quick to use, the whole investigation
CYSTOGRAPHY AND CYSTOURETHROGRAPHY taking just a few moments. As soon as the urethra is
a. With voiding urethrogram filled and contrast medium is seen to be trickling past
b. Retrograde urethrogram the distal sphincter, films are taken in two oblique
c. Cystogram projections.
d. With DC (air)
COMBINED ASCENDING URETHROGRAM AND MCU
e. Triple contrast (angiography plus pneumocystogram
When both an ascending urethrogram and a descending
plus interstitial air in wall of UB)
(micturating) study are required to show the posterior
f. Chain urethrogram
urethra, the bladder can be filled slowly using a 50 ml
g. Choke urethrogram
syringe for repeated injections. After images of the anterior
MCU urethra have been obtained in the usual way, the clamp
The MCU is the most accurate method of demonstrating or catheter is removed, the patient tilted to the upright
posterior urethra and VUR, and it is important in children position, and oblique views during micturition obtained
with UTI and reflux nephropathy. to exclude vesicoureteric reflux and to demonstrate the
It is a relatively uncomfortable investigation. posterior urethra. This combined study is of particular
The catheterization should be carried out gently but importance in demonstrating the anatomy of strictures
confidently with anesthetic jelly and an appropriately sized in the proximal and distal urethra after pelvic and perineal
catheter, using a sterile non touch technique. Dilute water- trauma. Films of the urethra obtained during micturition
soluble contrast medium (15–20%) should be used and at the end of an IVU are inadequate, the concentration
the bladder filled to capacity. After removal of the catheter of contrast medium being insufficient for this purpose.
the patient is tilted with the table to the erect position
and asked to empty the bladder into a plastic jug. Images ULTRASONOGRAPHY
during filling and during micturition should be obtained A normal kidney shows central echogenic sinus fat and
to document any vesicoureteric reflux. Micturating pelvicalyseal system and peripheral sim of cortex (normal
radiographs taken with the patient slightly oblique will thickness of cortex is 1.8–2.0 cm) with distinct CM-differ
demonstrate the posterior urethra. This part of the study entiation is corticomedullary differentiation as shown in
is especially important in children suspected of having Figure 3.25.
posterior urethral valves, which will only fill out and cause
obstruction during micturition. Contrast medium injected ADVANTAGES
retrogradely will fail to show valves. When catheterization 1. Widely used

is not possible because of a urethral stricture, the bladder 2. No hazard of ionizing radiation
can be filled in the retrograde manner using an ascending 3. Easily available
urethrogram. 4. Multiplanner capability
ASCENDING URETHROGRAM DISADVANTAGES

The ascending urethrogram gives excellent anatomical 1. Bowel gas obscures especially in mid ureter
information concerning the distal urethra as far as the 2. Obese patients
ROLE OF MDCT IN UROLOGIC DISEASES

INCLUDING THE IMAGING PROTOCOLS
INTRODUCTION
CT imaging of the urinary system has essentially replaced
conventional plain film excretory urography. CT scanning
for stones, hematuria, or for evaluating renal masses are
performed worldwide. The appropriate CT protocol will
be selected by the radiologist based on the clinical history
of the patient (see Table 3.4).
INDICATIONS
1. Differentiating cyst from tumor when ultrasound
equivocal
FIGURE 3.25: Ultrasonography: A normal kidney
2. Staging renal tumor
3. Calculus size cannot be evaluated 3. Excluding small tumors in patient with hematuria
and otherwise normal studies
4. Operator dependent
4. Renal tumor
It is a very vital modality especially in pediatric urology
5. Retroperitoneal pathology affecting the upper
practice and in screening for urorenal diseases in all age
urinary tract
groups, e.g. in obstructive diseases: 6. Retroperitoneal fibrosis
Signs of Obstruction 7. Retroperitoneal tumor
Indirect signs: 8. Differentiating uric acid calculi from urothelial tumor
1. Hydronephrosis 9. Filling defects in the pelvicaliceal system
2. Hydroureter 10. Renal trauma
3. Absent ureteric jets 11. If IVU is abnormal will show extent of injury better
4. Renomegaly than ultrasound
5. Renal blood flow changes—can be absent in early 12. Lower tract tumor
obstruction 13. Staging bladder, prostatic and testicular tumors
6. Forniceal rupture, Follow up after surgery, radiotherapy or chemo-
therapy
7. Acute renal failure
14. Calculus disease
8. Extrarenal pelvis.
15. Colic—emergency nonenhanced helical CT precise
Direct Signs localization of stones before treatment.
Calculus itself as a cause may be seen.
Table 3.4: Urinary tract imaging protocols

Stone Protocol
For detection of renal, ureteral, or bladder stones Non-contrast CT imaging from kidney to bladder.
(May be necessary infrequently to use iodinated contrast
agent to distinguish between ureteral stones and phleboliths)
Follow-up imaging with non-contrast plain film radiography
CT Urography (Hematuria Protocol)
For evaluation for common causes of persistent Non-contrast followed by contrast CT imaging from kidney
hematuria, i.e. stones, urothelial tumors, renal tumors to bladder
Renal Mass Protocol

For characterization of renal masses detected by Non-contrast followed by contrast CT imaging of kidneys
other imaging studies, e.g. ultrasound, MRI only
STONE PROTOCOL detection of stones on follow-up plain film radiographic

For detection of renal, ureteral, or bladder stones non- images since the detection rate of stones increases from
contrast CT imaging from kidney to bladder (May be 45% identified on non-contrast radiographic films alone
necessary infrequently to use iodinated contrast agent to 78% on films viewed together with 3-D reconstructions
to distinguish between ureteral stones and phleboliths). of the initial diagnostic CT images.
Follow-up imaging with non-contrast plain film Pregnant patients should be evaluated initially with
radiography. ultrasound imaging, to avoid exposure to any unnecessary
Non-contrast followed by contrast CT imaging of radiation, and MR urography is an alternative imaging
kidneys only axial image from “stone protocol” CT technique for evaluating the renal system in pregnant
showing left ureteral stone. women, children, and patients with contraindications to
contrast agents.
Urolithiasis and Nephrolithiasis
Almost all ureteral and renal stones, including those CT UROGRAPHY (HEMATURIA PROTOCOL)
containing uric acid, can be detected by non-contrast CT For evaluation for common causes of persistent
imaging. The accuracy of the technique in diagnosing hematuria, i.e. stones, urothelial tumors, renal tumors.
urolithiasis in patients presenting with acute flank pain Non-contrast followed by contrast CT imaging from
has been determined to be as high as 97%, with a kidney to bladder.
sensitivity of 95% and specificity of 98%. This compares
to sensitivities in the range of 45–58% for non-contrast RENAL MASS PROTOCOL
plain film radiography and 64–87% for plain film For characterization of renal masses detected by other
excretory urography. imaging studies, e.g. ultrasound, MRI.
However, it is occasionally difficult to distinguish Many renal lesions are incidentally detected on a
between non-obstructing distal ureteral calculi and pelvic variety of imaging tests, but cannot usually be charac-
phleboliths on non-contrast CT images. In these cases, terized at the time of detection. Currently, at this institution
it may be necessary to use intravenous contrast agent, “renal-mass protocol” CT is the gold standard for the
so that the relationship of the calculus to the opacified characterization of renal masses. This protocol acquires
ureter can be determined. Another situation in which thin section images of the kidneys before and after
intravenous contrast can be helpful is in the detection intravenous contrast administration to evaluate the
of stones in HIV positive patients on protease inhibitors important characteristic of solid lesions, the unequivocal
such as Indinovir. These calculi are typically non-radio demonstration of lesion enhancement post contrast.
opaque and may go undetected on stone protocol CT Lesions that demonstrate unequivocal enhancement
scans. The use of 3-D reconstruction techniques of require histologic diagnosis either by image-guided biopsy
contrast-enhanced pyelographic phase images can be or by surgical resection
helpful in all of these situations.
The disadvantage of “stone protocol” CT is that the ADVANTAGES OF MDCT
radiation dose is high (about 500 mrem) compared to
1. The advantages of multidetector CT urography over
that needed for plain film excretory urography (about conventional plain film excretory urography (also
150–350 mrem) and non-contrast plain film radiography known as IV urography) and ultrasound for the
(about 13 mrem). This exposure is a significant concern, evaluation of the urinary tract are numerous.
especially as urinary stones frequently affect young Dedicated CT protocols have been developed for
people. For this reason, it is better to avoid CT for follow these new high speed machines for different clinical
up studies wherever possible and to use non-contrast plain indications including “stone protocol” for the
film radiography instead. The initial CT scan and evaluation of urinary tract calculi, CT urography for
reconstruction images can be used to aid subsequent the evaluation of patients with hematuria and “renal
mass protocol” for the characterization of known renal for thorough evaluation of the renal collecting system
masses. Multidetector CT scanning is fast, taking and ureter. For this reason, intravenous saline is given
around 15 seconds for image acquisition from the at the same time as the contrast material to aid in
kidneys to the bladder during a single breath-hold. the detection of subtle filling defects and the
The images have good spatial resolution, little discrimination between urothelial neoplasms and
misregistration due to respiratory movement, and the other filling defects. Image reconstruction techniques
acquisition of multiple thin slices allows excellent two- are used to create images of the entire length of the
and three-dimensional reconstructions of the urinary system from the kidneys to the bladder. Multi-
abdominal anatomy, making it possible to detect planar 3-D reconstruction can provide the anatomic
pathologies outside the urinary tract as well as those detail required to correlate the finding with retrograde
within. Iodinated contrast agents are not usually ureterography or to perform an endoscopic evalua-
required for the detection of renal stones, thus avoiding tion. CT has been shown to detect parenchymal
the risk of adverse reactions to these agents, but are masses in the kidney with a sensitivity of 94%,
routinely used in CT urography and the renal mass compared to 67% for plain film excretory urography
protocol. and 79% for ultrasound.
2. In comparison to CT, plain film excretory urography 5. Another potential advantage of CT is that reconstruc-
offers excellent delineation of calyceal and papillary ted images can show tumors in a filled bladder
anatomy, the ureters and bladder, but it is inferior opacified with contrast agent (“virtual cytoscopy”).
to multi detector CT for imaging of the kidney 6. In comparison to CT, plain film excretory urography
parenchyma. Both CT and plain film excretory offers excellent delineation of calyceal and papillary
urography are associated with a substantial radiation anatomy, the ureters and bladder, but it is inferior
dose. Ultrasound is good for imaging the kidney to multi detector CT for imaging of the kidney
parenchyma and for detecting hydronephrosis, does parenchyma. Both CT and plain film excretory.
not require the administration of iodinated contrast, 7. Three dimensional coronal reconstruction of CT
and avoids radiation exposure. However, ultrasound urography image, showing contrast-enhanced renal
is not good for detecting urinary tract calculi, and does collecting system, ureters, and bladder. Note
not adequately image the renal collecting system or duplicated system on left side.
ureters. For these reasons, multidetector CT imaging 8. As Urography is associated with a substantial
has become the gold standard for the diagnosis of radiation dose, ultrasound is not good for detecting
urinary tract calculi, the investigation of hematuria, urinary tract calculi, and does not adequately image
and the characterization of renal masses and has the renal collecting system or ureters, multidetector
largely replaced both plain film excretory urography CT imaging has become the gold standard for the
and ultrasound examinations for these purposes. diagnosis of urinary tract calculi, the investigation of
3. The main causes of hematuria are urinary tract calculi, hematuria, and the characterization of renal masses
renal tumors, urothelial tumors, and infection. CT and has largely replaced both plain film excretory
urography is the best single diagnostic examination urography and ultrasound examinations for these
for diagnosing all of these pathologies, with the purposes.
exception of infection, which is effectively diagnosed
DISADVANTAGES OF MODALITIES IN GENERAL
in most cases by microbiological analysis of the
urine. Conventional cytoscopy remains the “gold standard” for
4. CT urography requires the use of contrast agent to the detection of tumors of the bladder, as it will detect
opacify the collecting system, the ureters, and the early mucosal lesions that do not deform the contour of
bladder. In addition to optimal opacification, the bladder wall. In addition, cytoscopy has the added
distension appears to be an important requirement capability of biopsy of suspicious lesions.
MRI administration. The typical dose is 5 mCi for adults.

MRI has as yet less to offer than CT in the imaging of Delayed planar (and pinhole in children) imaging is
the upper urinary tract, although the detection of minor acquired 2 hours after injection; in multiple projections.
venous involvement by a renal cell carcinoma may SPECT is more sensitive and may be helpful, but is usually
be better shown by MR angiography and MRI plays an not required. Split renal function as a percentage is
important role in the staging of pelvic malignancy. typically calculated.
The initial results in the staging of prostatic carcinoma The primary indication for a renal cortical scan is to
were disappointing but purpose-built, endorectal coils are assess for renal scarring or acute inflammatory changes.
producing excellent detail and may well be important This technique is superior to both ultrasound and
in demonstrating breaks in the prostatic capsule by excretory urography in both instances.
prostatic cancer. Pyelonephritis is demonstrated as a solitary defect
involving one kidney, multiple focal defects or diffuse
MR UROGRAPHY involvement of an entire kidney without volume loss (and
MR urography is being used in the diagnosis of sometimes with evidence of volume expansion).
obstruction. The coronal images using FlSP, HASTE Decreased uptake of DMSA is associated with ischemia
sequences rapidly portray a dilated collecting system and in the inflamed or scarred zones of renal parenchyma.
demonstrate the site of obstruction. It avoids irradiation An alternative hypothesis proposes failed tubular
of the patient; however, CT has the advantage of being transport secondary to paralysis from granulocyte toxic
able to show calculi which MR urography cannot by-products. Abnormalities resulting from infection are
accurately differentiate from other filling defects. transient, whereas scars result in a permanent abnor-
mality. Sequential scanning after a newly documented
RENAL SCINTIGRAPHY urinary tract infection in children should be performed
1. Dynamic renal scan 2–3 months after the initial diagnosis to assess for scarring.
2. Static renal scan The presence of scarring is indicated by photopenia and
99M
cortical contraction (volume loss).
DYNAMIC SCAN (I-123 HIPPURAN, Tc, DTPA, MAG3)
Vesicoureteral reflux (VUR) is present in 25–50% of
• Suspected obstruction kidneys with new renal scarring but the majority of
• Evaluation of surgical treatment (indirect cystography patients (63–75%) with acute inflammatory changes have
and post-renal transplant) pyelonephritis not associated with VUR. The kidney is
• With captopril for renovascular hypertension—a most susceptible to scarring from reflux in the first year
sensitive screening test. of life and a single instance of pyelonephritis may result
in scarring. Renal scars rarely develop after age 5.
STATIC SCAN (DMSA)
Infection must be present as reflux alone has not been
• To locate ectopic kidney shown to result in renal scarring.
• To diagnose intra and extrarenal arterial abnormalities Renal cortical scanning may also be used as a
• In urinary incontinence to detect occult duplication secondary means to provide information about renal
when IVU and USG are normal tumors or after renal trauma.
• For renal scarring in Chronic Pyelonephritis or UTI
with VUR ARTERIOGRAPHY (INDICATIONS)
• To estimate differential function. • Renovascular hypertension (gold standard)
• Preceding conservative surgery for Wilms’
RENAL CORTICAL SCINTIGRAPHY • In renal trauma with persistent hematuria, and
It is the most accurate evaluation for renal cortical hypertension
scarring. DMSA is bound in the renal tubular cells and • In suspected vasculitis (polyarteritis)
provides excellent visualization of the renal cortex. • Prior to interventional procedures (like embolization
Maximal uptake is reached within 3 hours of radiotracer of AVM, balloon dilatation of RA stenosis).
Congenital Anomalies of Urorenal Tract 27
INTRODUCTION Agenesis: Bilateral renal agenesis (absence of both

Congenital anomalies of the kidneys are frequently kidneys—Potter’s syndrome) is fatal. It is associated with
encountered and probably related to its complex oligohydramnios, pulmonary hypoplasia, and low-set
ears. Unilateral renal agenesis is not uncommon and
embryogenesis.
usually is accompanied by ureteral agenesis with absence
Congenital anatomic anomalies of the GU tract are
of the ipsilateral trigone and ureteral orifice. Compen-
more common than those of any other organ system.
satory hypertrophy of the solitary kidney maintains
Complications (e.g. urinary obstruction, stasis) may result
normal renal function.
in impaired renal function, infection and calculus
formation, and sexual disability or infertility. Treatment
SUPERNUMERARY KIDNEY
is often surgical.
It is extremely rare. Cleavage of the metanephric blastema
ANOMALIES OF URORENAL TRACT has been suggested as the cause. Most supernumerary
• Kidney kidneys are caudally placed and are hypoplastic. A
• Ureter separate collecting system is generally present.
• Bladder and
• Urethra ANOMALIES OF FUSION
EMBRYOLOGY
KIDNEY
The kidneys begin to form initially in the pelvis and then
• Anomalies of number rise into the upper abdomen later in development. The
• Anomalies of position so called ascent of the kidneys is caused by a
• Anomalies of fusion diminution of the body curvature as well as by the growth
• Anomalies of form of the body in the lumbar and sacral region. During this
ascent they undergo a 90º lateral rotation about a vertical
Anomalies of Number axis with the renal hilus changing from ventral to medial.
1. Agenesis As they ascend the kidneys pass through the arterial
2. Supernumerary kidney fork formed by the umbilical arteries. If one of them fails
pass completely, it remains in the pelvis close to the When ureteropelvic junction obstruction is present,
common iliac artery and is a ‘pelvic kidney’. Sometimes pyeloplasty is the treatment of choice (Figures 4.1 to 4.3).
both kidneys are pushed together during their passage
Fused pelvic kidney (pancake kidney) is a much
through the arterial fork and the lower poles become
less common fusion anomaly in which a pelvic renal mass
fused, usually between the 4th and 8th week of fetal
is served by two collecting systems and ureters. If
life. Fusion results in improper rotation and incomplete
obstruction is present, reconstruction is indicated.
ascent.
Anomalies of Position
FUSED KIDNEYS ARE DIVIDED INTO THREE GROUPS
1. Renal ectopia
DEPENDING ON THE RELATIVE HEIGHT AT THE TIME
2. Malrotation.
OF FUSION
1. Kidneys present on either side of the spine with an Renal ectopia: Renal ectopia (abnormal renal location)
isthmus across the midline joining either upper or results from a kidney that fails to ascend from its origin
lower poles are horseshoe kidneys. A central isthmus in the true pelvis or from a superiorly ascended (thoracic)
is a disk kidney and a sigmoid is an upper of one kidney. There is an increased incidence of ureteropelvic
fused to a lower of another. junction obstruction, vesicoureteral reflux, and multicystic
2. Both kidneys fused as irregular masses near the sacral
promontory, a clump kidney.
3. Both fused into one mass which lies on one side of
the spine.
Fusion anomalies (in which the kidneys are joined but
the ureters still enter the bladder on each side) increase
the risk of ureteropelvic junction obstruction, vesicoure-
teral reflux, multicystic renal dysplasia, and injury from
anterior abdominal trauma.
1. Horseshoe kidney
2. Crossed fused renal ectopia
3. Fused pelvic kidney (pancake kidney)
Horseshoe kidney is the most common fusion anomaly.
Renal parenchyma on each side of the vertebral column FIGURE 4.1: Antenatal ultrasound scan crossfused ectopia
is joined at the corresponding (usually the lower) poles,
with an isthmus of renal parenchyma or fibrous tissue
across the midline at the joined areas. The ureters course
medially and anteriorly over this isthmus and generally
drain well. Obstruction, if present, is usually secondary
to high insertion of the ureter in the renal pelvis, not
secondary to the isthmus. Pyeloplasty can be performed
without resection of the isthmus.
Crossed fused renal ectopia is the second most
common fusion anomaly. The renal parenchyma
(representing both kidneys) is on the same side of the
vertebral column. One of the ureters crosses the midline
and enters the bladder on the side opposite the kidneys. FIGURE 4.2: Ultrasound scan (postnatal)—Crossfused ectopia
Duplex kidney (double kidney) consists of a single renal

mass with more than one collecting system. An increased
risk for ureteral ectopy with or without ureterocele exists
in duplex systems; management depends on the anatomy
and function of each separately drained segment. Surgery
may be necessary to correct obstruction or vesicoureteral
reflux.
Renal dysplasias: These parenchymal abnormalities
with consequent renal dysfunction may result from
abnormal development of the renal vasculature, renal
tubules, collecting ducts, or drainage apparatus. Biopsy
may be necessary for diagnosis.
Multicystic dysplastic kidney: This nonfunctioning
renal unit consists of noncommunicating cysts with
intervening solid tissue composed of fibrosis, primitive
tubules, and foci of cartilage. Usually, there is associated
ureteral atresia. A low risk for tumor, infection, and
hypertension exists; some advocate removing these
FIGURE 4.3: Computed tomography: Coronal reconstruction–
Crossfused ectopia on left side kidneys, whereas others would observe the patient.
renal dysplasia in incompletely ascended kidneys, but Renal hypoplasia: An underdeveloped kidney is usually
not in superiorly ascended ones. Surgical correction is associated with inadequate branching of the ureteral bud.
performed when indicated. The kidney is small, with histologically normal nephrons.
Hypertension can occur with segmental hypoplasia, and
Malrotation: This usually minor anomaly of the renal
ablative surgery may be necessary (Figure 4.4).
axis can appear abnormal on radiography of the collecting
system. It should be differentiated from the effects of true
obstruction or renal masses.
Anomalies of Form
1. Duplication anomalies
2. Calyceal diverticulum
3. Renal dysplasias
4. MCDK (Multicystic dysplastic kidney)
5. Medullary sponge kidney
6. Medullary cystic disease of kidney (Juvenile nephro-
nopthesis)
7. Renal hypoplasia and Ask-up mark kidney
8. ARPKD
9. ADPKD.
Duplication anomalies: Supernumerary collecting
systems may be unilateral or bilateral and may involve
the renal pelvis and ureters (accessory renal pelvis, double FIGURE 4.4: IVU: Small sized right kidney with smooth outline
or triple pelvis and ureter), calyx, or ureteral orifice. and small pelvicalyceal system → right renal hypoplasia
Autosomal recessive polycystic kidney disease: infection. Dialysis is used as for other children with chronic
Although rare (1/10,000 births), autosomal recessive renal insufficiency.
polycystic kidney disease is the most common genetically
determined childhood cystic disease of the kidneys URETER
(involving both kidneys and the liver) and frequently Ureteral anomalies frequently occur with renal anomalies
produces renal failure in childhood. but may occur independently. Complications include
Generally, patients presenting in early childhood show obstruction, infection, and sometimes calculus formation
mainly renal-related symptoms; those presenting in from urinary stasis, as well as urinary incontinence if the
adolescence show mainly hepatic-related symptoms. ureter bypasses the bladder and terminates in the urethra,
These differences probably reflect phenotypic variation perineum, or vagina in the female.
of the same genetic disorder. 1. Peliv-ureteric Junction (PUJ) Obstruction
2. Duplication anomalies
Severely affected newborns commonly have
3. Ectopic orifices
pulmonary hypoplasia secondary to the in utero effects
4. Ureteral stenosis
of renal dysfunction and associated oligohydramnios. Less
5. Ureterocele
severely affected newborns have a protuberant abdomen
6. VUR
with huge, firm, smooth-surfaced, symmetric kidneys. The 7. Megapolycalicosis and megaureter
enlarged liver is abnormal with periportal fibrosis, bile 8. Retrocaval ureter.
duct proliferation, and rare cysts; the remainder of the
hepatic parenchyma is normal. These pathologic findings CONGENITAL PUJ OBSTRUCTION
are responsible for perisinusoidal portal hypertension with
INTRODUCTION
minimal or absent hepatic dysfunction. Ultrasonography
is the best diagnostic tool and, in late pregnancy, usually It is the most common congenital anomaly of the urinary
allows presumptive in utero diagnosis. tract.
In patients aged 5 to 10 years, signs of portal hyper- It is also the most common cause of an abdominal
tension appear, such as esophageal and gastric varices mass in a neonate.
and hypersplenism (leukopenia, thrombocytopenia). If
INCIDENCE
the patient presents in adolescence, nephromegaly is less
marked. Renal insufficiency may be mild to moderate. Male to female ratio is 2:1. The left side is more commonly
The major symptoms are related to progressive hepatic affected. Bilateral PUJ obstruction is present in 10–40%
of cases. A familial tendency has been reported.
fibrosis (portal hypertension, gastric and esophageal
varices, hepatic insufficiency, hypersplenism). Diagnosis PATHOPHYSIOLOGY
is difficult, especially without a positive family history.
The intrinsic form is responsible for 80% of cases and
Ultrasonography may demonstrate renal or hepatic cysts;
it thought to be due to a defect in the circular muscle
definitive diagnosis may require biopsy.
bundle of the renal pelvis that results in an inability to
Many newborns die in the first few days or weeks
transmit normal peristalsis. The extrinsic form accounts
of life from pulmonary insufficiency. Most who survive
for no more than 15–20% of cases and is usually caused
the first few years have progressive renal failure. Those
with less renal involvement develop progressive portal by aberrant renal vessels that cross anterior to the pelvis
hypertension. Portacaval or splenorenal shunts reduce or proximal ureter.
morbidity but not mortality. Experience with transplan- Some authors have postulated less widely held views
tation in these patients is limited. If transplantation is regarding the causation of the intrinsic form of PUJ
performed, hypersplenism must be controlled to prevent obstruction, such as the presence of abnormal folds or
immunosuppression; otherwise, hypersplenism may a kink of the PUJ, or that the lesion represents an
induce leukopenia, increasing the risk of systemic exaggerated from of extrarenal pelvis.
CLINICAL FEATURES Also, percutaneous nephrostomy followed by balloon

It may be clinically silent until adulthood, when symptoms pyeloplasty or endoscopic endopyelotomy may be
of flank pain, fever or hypertension (rarely) may occur. performed. These may also be performed as secondary
However, increasingly, many cases are discovered prior procedures when surgery has failed. Success rates of 85%
to birth by obstetric ultrasound. In some cases, a sustained have been reported. The procedures should not be utilized
diuresis may be needed to provoke symptoms (“beer- in small infants.
drinker’s hydronephrosis”). Such cases may be due If obstruction secondary to an aberrant vessel is
to a mild PUJ obstruction that is compensated in the suspected, the above mentioned procedure should not
absence of diuresis. be performed until the aberrant vessel has been excluded
by prior angiography.
ROLE OF RADIOLOGY AND IMAGING
Duplication Anomalies
Urography demonstrates a dilated renal pelvis and
calyces. Slow opacification of the affected side is seen. Duplication of PC system only with normal ureter is
Delayed radiographs are needed to demonstrate that the common and can otherwise be considered as a normal
PUJ is the point of obstruction. With high-grade/long- variant. Partial or complete duplication of one or both
standing obstruction, the renal pelvis may be markedly ureters may occur with duplication of the ipsilateral renal
dilated (>10 cm in diameter) with a ballooned appea- pelvis. The ureter from the upper pole of the kidney opens
rance. The kidney may be virtually non-functional. If there at a more caudal location than the orifice of the lower
is kinking of the PUJ, obstruction secondary to an aberrant pole ureter. Ectopia or stenosis of one or both orifices,
vessel should be considered. vesicoureteral reflux into the lower or both ureters, or
A diuretic renogram or the Whitaker procedure ureterocele may occur. Surgery may be necessary to
performed after acute symptoms are brought on may be correct vesicoureteral reflux, obstruction, or urinary
helpful to determine whether functionally significant incontinence (Figures 4.5 and 4.6).
obstruction is present, in patients with equivocal PUJ Ectopic Orifices
obstruction or if there is a discrepancy between clinical
These malpositioned openings of single or duplicated
symptoms and radiologic findings.
ureters may occur on the lateral bladder wall, distally along
Antegrade or retrograde pyelography may help
the trigone, in the bladder neck, in the female urethra
better define the ureter and thereby establish the
distal to the sphincter (leading to continuous inconti-
diagnosis. The antegrade procedure may be performed
nence), in the genital system (prostate and seminal vesicle
by percutaneous nephrostomy. The retrograde procedure
in the male, uterus or vagina in the female), or externally.
may be useful to confirm the diagnosis prior to surgery.
Lateral ectopic orifices frequently are associated with
A voiding cystourethrogram is recommended to exclude
vesicoureteral reflux, whereas distal ectopic orifices more
vesicoureteral reflux as the cause of the renal pelvic often are associated with obstruction and incontinence.
dilatation. Surgery is indicated for obstruction and incontinence and
Ultrasound will demonstrate a very dilated renal is sometimes necessary for vesicoureteral reflux.
pelvis and caliectasis but will not be able to demonstrate
a dilated ureter. Stenosis
Narrowing may occur at any location in the ureter, most
DIFFERENTIAL DIAGNOSIS
commonly at the ureteropelvic junction and less com-
A large extrarenal pelvis may be distinguished by the fact monly at the ureterovesical junction (primary mega-
that the calyces are not dilated. ureter). Stenoses often improve with time and growth,
but ureteral tapering and reimplantation may be necessary
TREATMENT
when there is increasing dilatation and infection. Any
It may include pyeloplasty. obstruction is also an indication for surgery.
FIGURE 4.5: Ultrasound scan: Bifid pelvicalyceal system
FIGURE 4.6: CT scan: Coronal reconstruction—Bifid pelvicalyceal

Ureterocele
system on left side
Ballooning of the lower end of the ureter into the bladder
may produce progressive obstructing dilatation, leading Bacteria in the lower urinary tract can be easily
to ureterectasis, hydronephrosis, infection, occasional transmitted by reflux to the upper tract, leading to
calculus formation, and potential loss of renal function. parenchymal infection with potential scarring and loss
Treatment includes endoscopic transurethral incision or of renal function. Chronically elevated bladder storage
open repair. When a ureterocele involves the upper pole and emptying pressures (> 40 cm H2O) have resulted
of duplex ureters, treatment may depend on function in in progressive hydrostatic damage to the kidney in most
that renal segment because of the significant incidence patients, even without infection or reflux.
of renal dysplasia. Removal of the affected renal segment Abdominal or flank pain, persistent or recurrent UTI,
and the affected ureter may be preferable to repair of dysuria or flank pain with voiding, frequency and urgency,
the obstruction if no segmental renal function is found or signs of renal insufficiency may be secondary to
or if significant renal dysplasia is suspected. vesicoureteral reflux. Pyuria, hematuria, proteinuria, and
bacteriuria may be present.
Vesicoureteral Reflux Filling and voiding cystourethrograms demonstrate reflux
Reflux of urine from the bladder into the ureter may result and are the preferred means to diagnose bladder outlet
in damage to the upper urinary tract by bacterial infection obstruction, which can be surgically repaired. IVU may show
and occasionally by increased hydrostatic pressure. It most calyceal dilatation, ureteral “ribboning,” and ureterectasis
often is due to congenital anomalous development of with dilatation of the upper collecting system. Reflux may
the ureterovesical junction. Incomplete development of also be demonstrated by direct (catheter) radioisotope
the intramural ureteral tunnel causes a failure of the flap cystogram. Renal cortical involvement with acute infection
valve action at the ureterovesical junction and permits or scarring is precisely delineated with succimer
backwash of bladder urine into the ureter and renal pelvis. (dimercaptosuccinic acid) nuclear scans, when indicated.
Reflux can occur even in the presence of an ordinarily Vesicoureteral reflux is usually mild to moderate (with
sufficient tunnel when there is bladder outlet obstruction little or no calyceal dilatation) and often resolves
with increased intravesical pressures or neurogenic vesical spontaneously over months to several years while daily
dysfunction. antibacterial prophylaxis is maintained. Infection despite
prophylaxis, or significant and progressive renal scarring

is best managed by ureteral reimplantation. When such
reflux is accompanied by high-pressure storage or
emptying of urine in the bladder, the approach is to lower
bladder pressures by pharmacotherapy and/or behavioral
means. Reflux may sometimes resolve with this
management; if not, reimplantation would be appropriate.
This approach almost always eliminates reflux and
minimizes future pyelonephritis, with reduced morbidity
and mortality from renal disease secondary to reflux and
infection.
Retrocaval Ureter
Anomalous development of the vena cava (pre-ureteric
vena cava) allows the infrarenal vena cava to form
anterior to the ureter. A retrocaval ureter on the left is
seen only with persistence of the left cardinal vein
system or with complete situs inversus. It can cause FIGURE 4.7: IVU: Pubic diastasis with small hypoplastic urinary
ureteral obstruction. For significant ureteral obstruction, bladder in a case of bladder exstrophy
surgery consists of division of the ureter with uretero-

ureteral anastomosis anterior to the vena cava or iliac fails to expand sufficiently or has sphincter insufficiency.
vessel. Reconstruction of the genitalia is required (Figure 4.7).
BLADDER Congenital Bladder Diverticula
Congenital anomalies of the urinary bladder include Bladder diverticula predispose to UTI and may
exstrophy; agenesis; duplication; persistent urachus; be associated with reflux. Diagnosis is made by cysto-
megacystis syndrome, which may be a primary graphy and cystoscopy. Surgical removal of diverti-
myoneural defect and diverticula. cula and reconstruction of the bladder wall may be
1. Exstrophy of bladder indicated.
2. Congenital bladder diverticuli.
PENIS AND URETHRA
Exstrophy
The penis in the male and the urethra in the male or
In this easily detectable and serious major anomaly, the female may be congenitally absent. Other anomalies
bladder is open (unroofed) in the suprapubic region with include hypospadias; epispadias; double penis; congenital
urine dripping from the ureteral orifices. The bladder penile curvature or malrotation; microphallus; urethral
mucosa is continuous with the abdominal skin, and the valves, stricture, stenosis, or duplication; meatal stenosis;
pubic bones are separated. The prognosis for main- and phimosis and paraphimosis.
tenance of normal renal function is good. The bladder 1. Hypospadias
can almost always be reconstructed and returned to the 2. Epispadias
pelvis, although vesicoureteral reflux is invariably present. 3. Congenital urethral stricture
Ureterosigmoidostomy or other types of continent urinary 4. Urethral meatal stenosis
diversion may be used to treat a bladder reservoir that 5. Urethral diverticulum
6. Duplication of urethra Urethral Stricture

7. Phimosis and paraphimosis. Although urethral stricture in the male is most commonly
acquired, typically from a crush injury after straddle
Hypospadias
trauma, it may be congenital. Acquired stricture usually
A displaced urethral opening is caused by failure of presents with postvoid bloody urethral discharge and may
tubularization and fusion of the urethral groove. In female heal spontaneously or progress to true stricture. Initial
hypospadias, the urethra opens into the vaginal introitus. management is endoscopic urethrotomy.
In the male, the foreskin fails to become circumferential
and appears as a dorsal hood. The urethral opening may Urethral Meatal Stenosis
be located on the underside of the penile shaft, at the Urethral meatal stenosis associated with hypospadias is
penoscrotal junction, between the scrotal folds, or in the the most common form of congenital urethral stenosis,
perineum. It is often associated with chordee (ventral although it is more commonly an acquired condition
curvature of the penis, most apparent on erection), caused resulting from healed irritation of the meatus in boys who
by fibrous tissue along the usual course of the corpus are circumcised while still in diapers. Meatotomy is
spongiosum. Prognosis for functional and cosmetic indicated for a significantly deflected stream or for a
correction is good. During the first year of life, the chordee pinpoint stream.
can be repaired and a neourethra constructed using penile
Duplication of Urethra
shaft skin or foreskin.
It may be complete or incomplete. It occurs in sagittal
Epispadias plane.
Three types known:
This dorsal fusion defect of the urethra can be partial
1. Epispadic (dorsal) type usually abortive
(in 15% of cases) or complete, the most severe form being
2. Hypospadic (Ventral) type is more functional
associated with exstrophy of the urinary bladder. It is more
3. Other types: forked
common in males. In partial epispadias, urinary control
Perneal is a rare variant.
can be satisfactory. Reconstruction of the penis alone may
be associated with persistent incontinence. Bladder outlet Phimosis and Paraphimosis
reconstruction is often required to achieve complete Phimosis is congenital or acquired (inflammatory)
urinary control. constriction of the foreskin, which cannot be retracted.
Paraphimosis is inability of the retracted constricting
Posterior Urethral Valves foreskin to be reduced distally over the glans. When either
Congenital folds of the urethra act as valves that occur condition is present, surgical circumcision is indicated.
in the prostatic urethra (posterior urethral valves). A preliminary dorsal slit may be required. The prognosis
Complications are due to obstruction, which may be is excellent.
severe and may lead to myogenic bladder malfunction, Few important anomalies are discussed in details in
following pages.
massive upper tract damage, and renal insufficiency.
Symptoms and signs include weak and dribbling urinary
stream, overflow incontinence, and UTI. Diagnosis is RENAL AGENESIS
established by voiding cystourethrography. Initial treat- INTRODUCTION
ment is endoscopy. Early surgery may prevent progressive True renal agenesis is defined as the complete congenital
renal deterioration. A different entity, diverticulum of the absence of renal tissue. Acquired forms of agenesis are
anterior urethra, may act as a valve (anterior urethral characterized by the development of renal tissue which
valve) and is also treated endoscopically. atrophies either during development or during childhood.
INCIDENCE
It is between 1:500 and 1:1500 births.
PATHOPHYSIOLOGY
Failure of formation of the ureteral bud or because of

an inherent deficiency of the metanephric blastema.
BILATERAL RENAL AGENESIS
This is extremely rare and is incompatible with life. Males

are affected in three-fourths of cases. Infants demonstrate
characteristic features of Potter’s syndrome including low-
set ears and a prominent palpebral fold.
Bilateral renal agenesis is uncommon and
incompatible life. Newborns that die from chronic renal
failure do so that the end of the first week of life and
those who die in hours or day of life usually do so from FIGURE 4.8: IVU: Left renal agenesis
respiratory. Bilateral renal agenesis is almost always
accompanied by oligohydramnios and there is a high
RENAL ECTOPY
incidence of associate monary hypoplasia. Following
INTRODUCTION
delivery, many affected have a spontaneous pneumo-
thorax or pneumomediastinum they also have a typical It refers to an abnormal position of the kidney.
facies with low-set ears - the so~called ‘Potter facies’. Renal ectopia (abnormal renal location) results from
a kidney that fails to ascend from its origin in the true
ASSOCIATED ANOMALIES pelvis or from a superiorly ascended (thoracic) kidney.
Most commonly, it takes the form of a pelvic kidney,
The ipsilateral adrenal gland is absent in 8–10% of cases.
in which the kidney is located in the true pelvis (pelvic
Some investigators report a two-fold increase in the
kidney) or adjacent to the sacrum (sacral kidney).
incidence of congenital anomalies of the contralateral
Occasionally, the kidney may lie at the level of the iliac
kidney. Associated genital abnormalities in males may
crest (abdominal ectopy). Rarely (1:15,000 births), the
include cysts of the ipsilateral seminal vesicle, absence
kidney ascends to a position higher than the second
of the ipsilateral vas deferens, hypoplasia or agenesis of
lumbar vertebra, and enters the thorax, presumably via
the testicle, and hypospadias. In females, unicornuate
a diaphragmatic aperture (intrathoracic kidney). This
or bicornuate uterus, absence or hypoplasia of the uterus,
anomaly is more common in males and on the left side.
and absence or aplasia of the vagina (Rokitansky-Kuster-
Blood supply to an intrathoracic kidney arises from the
Hauser syndrome) may be present.
abdominal aorta in its normal location.
ROLE OF RADIOLOGY AND IMAGING INCIDENCE
In true agenesis, a hemitrigone is found in the bladder 1:500 to 1:1200.
on cystoscopy. No renal artery is present. The colon
occupies the renal fossa on the affected side, and this PATHOPHYSIOLOGY
may suggest the diagnosis on plain films or barium enema. As the fetal kidneys ascend from their pelvic position to
IVU is helpful (Figure 4.8). meet the adrenal glands, each kidney acquires blood
supply from neighboring vessels. The initial supply from If the ectopic kidney is small and no hydronephrosis is
the external and internal iliac vessels is lost and blood present, the kidney may be obscured by the bony pelvis.
supply is acquired directly from the aorta around the 8th In such cases, tomography is useful (Figure 4.9).
week of development. Renal ectopy occurs if there is any
abnormality in acquiring such blood supply or there is Ultrasound
an associated abnormality of the spine which prevents
It is the modality of choice to demonstrate a small ectopic
such cephalic migration from occurring.
kidney not visualized on urography. A reniform mass with
Left-sided ectopia is more common, and men are
affected three times more often than women. It is usually a characteristic pattern of renal sinus echoes will be
unilateral, but may be bilateral Thoracic kidney has been identified in the pelvis (Figure 4.10).
recognized prenatally, and it has also appeared after a
previously normal chest radiograph. High ectopia is
usually not associated with functional abnormality and
is more commonly an incidental finding on chest X-ray,
however, there is an increased incidence of ureteropelvic
junction obstruction, vesicoureteral reflux, and multicystic
renal dysplasia in incompletely ascended kidneys, but
not in superiorly ascended ones. Malrotation may be
present with the renal pelvis anterior to the kidney. The
ureter is elongated. The origin of the renal artery can
be at the level of the opposite renal artery, or it may be
higher. The adrenal gland may accompany the kidney,
and one instance in which ipsilateral superior splenic
ectopia occurred has been reported.
CLINICAL FEATURES
Often asymptomatic.
In nearly 50% of cases, a pelvic kidney is associated with
hydronephrosis or vesicoureteral reflux, leading to
obstruction, infection and related pain. FIGURE 4.9: IVU: Ectopic right kidney with bilateral VUR
ASSOCIATED ANOMALIES
Other GU malformations are commonly present in
patients with pelvic kidneys. These include: Ureteropelvic
junction obstruction; cryptorchidism; hypospadias
(males); and absence of the vagina (females). Also, non-
GU anomalies may be present, such as vertebral and
rib (skeletal) anomalies; septal defects (heart anomalies);
malrotation, imperforate anus (GI anomalies); and, in
nearly 50% of patients with unilateral renal ectopy, an
abnormality of the normally positioned kidney is also
present.
Urography
Findings depend on the degree of renal function in the
pelvic kidney, and presence of associated anomalies. FIGURE 4.10: Ultrasonography: Ectopic right kidney
CT
A functional mass of renal parenchyma can usually be
identified.
Angiography
Prior to any surgical procedure contemplated on a pelvic
kidney, angiography is performed to investigate the highly
variable nature of its blood supply.
INTRATHORACIC KIDNEY
Thoracic kidney or superior renal ectopia is the rarest FIGURE 4.11: IVU: Bilateral FIGURE 4.12: IVU: Left intra-
intrathoracic kidneys thoracic kidney-Retrocardiac
of the renal ectopies. The adrenal gland and the spleen opacity
may accompany the kidney. Simple chest film combined
with sonography and confirmed by enhanced CT scan
after or excretory urography readily allows differentiation
from other masses in the chest thus avoiding unnecessary
and even hazardous biopsy or surgery . There is no need
to treat the malformation. If there is an associated
diaphragmatic hernia or eventration containing bowel
or stomach, the diaphragm is repaired.
Intrathoracic kidneys have a reported prevalence of
less than 1 in 10,000 and represent only 5% of ectopic
kidneys. It is a rare form of renal ectopia. Four forms
of intrathoracic ectopy have been reported. Most
commonly, as was found in our patient, a thin membrane
of diaphragm will cover the kidney, thus making it
subdiaphragmatic yet intrathoracic in position. This is also
FIGURE 4.13: CECT (Axial): Left intrathoracic kidney
referred to as a diaphragmatic eventration. The
embryologic origin is debatable; various authors have
CROSSED FUSED RENAL ECTOPIA (CFE)
proposed either an abnormality in pleuroperitoneal
INTRODUCTION
membrane fusion or abnormally high migration of the
kidney due to delayed mesonephric involution. Traumatic It is the fusion of both kidneys, with at least one kidney
rupture of the diaphragm, Bochdalek’s hernia, and on the side opposite its normal location. Originally, this
supradiaphragmatic ectopic kidney without herniation are condition was diagnosed at autopsy; currently, it is
less common causes of an intrathoracic kidney. Anatomi- identified with various imaging studies.
cally, four features are commonly found: Rotational Crossed fused ectopia is seen in 1/1000 to 1/1500
abnormality, in which the hilum is anterior; elongated autopsies. In 85–90% of patients with ectopic kidney will
ureter; high renal vessel origin; and medial deviation of be fused. CFE is believed to occur when either there is
the lower pole. The adrenal gland may or may not be a failure of nephrogenic cells to separate or fusion of 2
ectopic. The possibility of an intrathoracic kidney exists blastemas during abdominal ascent. Typically the lower
with all chest masses, though its likelihood is low (Figures kidney is malrotated and both pelves point toward
4.11 to 4.13). midline. The ureter of the ectopic kidney crosses midline
and enters the bladder on the contralateral side. There varies depending on the secondary symptoms and comp-
are no known congenital anomalies associated with lications. In the absence of associated complications and
CFE. symptoms, the condition may be incidentally discovered
Typically CFE is asymptomatic and is an incidental on images obtained for reasons other than the evaluation
finding. There can be an higher risk of injury the ectopic of crossed fused kidneys. Many cases of crossed fused
renal ectopia remain undiagnosed, although the exact
kidney if it overlies the spine.
number is unknown.
PATHOPHYSIOLOGY
Crossed fused renal ectopia often is clinically silent
unless it is detected during imaging for the evaluation
The fusion of the two kidneys is believed to result from of urinary tract infection or abdominal symptoms (e.g.
either of the two events: abdominal pain).
1. Failure of the primitive nephrogenic cell masses to A urinary tract infection may cause dysuria, fever, or
separate or nonspecific symptoms (e.g. failure to thrive), especially
2. Fusion of the two blastemas during their abdominal in the young child. An associated urinary tract calculus
ascent. may present with hematuria or flank pain.
Either component of the fused kidneys can have
Anatomical Basis
associated renal abnormalities such as a Wilms’ tumor,
The ectopic kidney, located entirely or primarily on the
hydronephrosis, multicystic dysplasia, and ectopic
opposite side of the abdomen, typically forms the lower
ureterocele. These abnormalities can occur in unfused
portion of the renal fusion mass. Usually, the lower kidney
nonectopic kidneys or in conjunction with renal ectopia.
is malrotated, and both pelves point toward the midline.
Crossed fused renal ectopia is not known to be associated
The ureter of the upper renal component descends on
with abnormalities of nongenitourinary systems.
the ipsilateral side into the bladder, while the ureter from
Incidence: Crossed fused ectopy occurs in approxi- the crossed ectopic kidney crosses the midline to enter
mately 1:1000 births. The anomaly is more common in the bladder on the contralateral side. Multicystic dysplasia
males (2:1). Left-to-right ectopy is three times more of the crossed renal unit is reported.
common than right-to-left ectopy.
PREFERRED INVESTIGATIONS
Etiology: An abnormally situated umbilical artery
IVU Figure 4.14A.
prevents normal cephalic migration from occurring. As
a consequence, the developing kidney takes the path of
least resistance and crosses to the opposite side, where
cephalic migration resumes. An alternative hypothesis
postulates that the abnormality occurs when the ureteral
bud crosses to the opposite side where it induces nephron
formation in the contralateral metanephric blastema.
Clinical symptoms: rare. These patients generally present
in adulthood with abdominal pain, pyuria, or urinary tract
infection. There is a slightly higher incidence of urinary
tract calculi, probably related to stasis.
CLINICAL FEATURES
Age
Crossed fused renal ectopia is a congenital malformation
that is present at birth. The patient’s age at diagnosis FIGURE 4.14A: IVU: Thyroid kidney
Ultrasonography (USG) Accuracy is high with MRI, primarily as a result of

It is the preferred radiologic modality for detecting the its excellent anatomic delineation and the absence of
condition because it is noninvasive, involves no ionizing image degradation due to bowel gas or osseous structures,
radiation, usually is readily available, and is less expensive unlike with US.
than either CT or MRI. Nuclear Medicine Studies
Features: Crossed fused renal ectopia is not definable It demonstrates a single focus of isotope excretion or
on plain radiographs. Usually, crossed fused renal ectopia localization by a kidney, with no contralateral isotope
is apparent on intravenous urograms (IVUs), which excretion or localization.
demonstrate an atypical renal mass, 2 collecting systems,
and 2 ureters. Ultrasonograms demonstrate an atypical MORTALITY/MORBIDITY
or complex appearance of the renal parenchyma, with No mortality or morbidity is observed unless crossed
no definable contralateral kidney. The overall renal fused renal ectopia is associated with renal failure that
dimensions should exceed the normal range for the results from obstruction or chronic pyelonephritis. Patients
patient’s age. with ectopic kidneys have an increased risk for
Usually, fusion occurs along the longitudinal aspect, complications such as hydronephrosis, infection, and
with the kidneys side by side. The result is a dysmorphic calculus formation.
appearance, with a sigmoid or S shape on the ultrasono-
gram. The fused lower pole unit is positioned medially SUMMARY
and extends anterior to the spine (Figure 4.14B).
Crossed ectopy is defined as a kidney that is located on
the opposite side of the midline from its ureter. The crossed
kidney usually lies below the normally situated kidney.
In 90% of cases, at least partial fusion between the kidneys
is present (called crossed fused ectopy). In the remainder,
two discrete kidneys are present on the same side (crossed
unfused ectopy). Other variations include solitary crossed
ectopy and bilateral crossed ectopy.
HORSESHOE KIDNEY
INTRODUCTION
Horseshoe kidney is probably the most common fusion

FIGURE 4.14B: Ultrasonography: crossfused ectopia right kidney anomaly.
fused to lower pole of left kidney
The term “horseshoe kidney” refers to the appearance
A multicystic mass of variable size that is contiguous of the fused kidney, which results from fusion at one pole.
with the lower pole of the upper renal unit is present in In more than 90% of cases, fusion occurs along the lower
patients with multicystic dysplasia. pole. Technically, the term horseshoe kidney is reserved
Limitations: On ultrasonograms, overlying abdominal for cases in which most of each kidney lies on one side
gas may obscure a portion of the fused kidneys, making of the spine. It includes:
precise diagnosis difficult. This diagnosis should be • Symmetric horseshoe kidney (midline fusion) or
considered when 2 separate kidneys cannot be identified. • Asymmetric horseshoe kidney (L-shaped kidney)
when the fused part, or the isthmus, lies slightly lateral
MRI to the midline (lateral fusion).
MRI findings include fused renal masses with two Horseshoe kidney is generally differentiated from
collecting systems. crossed fused ectopia in which both fused kidneys lie on
one side of the spine and the ureter of the crossed kidney Horseshoe Kidney with Associated Anomalies
crosses the midline to enter the bladder. About one-third of cases of horseshoe kidney are
associated with other congenital anomalies, which include
PATHOPHYSIOLOGY
anomalies of the urogenital, gastrointestinal, neurologic,
The development of the normal kidney depends on the and skeletal systems, as well as some chromosomal
union of ureteric buds from the mesonephric ducts with abnormalities. These anomalies include multisystem
the nephrogenic cords in the embryo. It is believed to abnormalities such as urogenital anomalies (e.g. uretero-
occur around the fourth gestational week. This union pelvic obstruction, vesicoureteric reflux, ureteral duplica-
normally occurs at the level of the first or second sacral tion, hypospadiasis, undescended testis, ectopic ureter,
vertebral segment. Subsequent straightening of the hind retrocaval ureter, bicornuate and/or septate uterus).
end of the embryo, along with differential growth of the GI abnormalities include anorectal malformations
developing pelvic structures, leads to the ascent of both such as imperforate anus, malrotation, and Meckel
kidneys to their normal dorsolumbar regions between the diverticulum. CNS anomalies such as neural tube defects
fourth and ninth weeks of gestation. may be seen. Skeletal anomalies include rib defects,
The abnormal fusion probably occurs at the 5 to 12 clubfoot, or congenital hip dislocation. Cardiovascular
mm embryonic stage when the kidneys are in the true
abnormalities, such as a ventricular septal defect (VSD),
pelvis and the renal capsule has not yet matured. One
may occur in some patients.
embryologic explanation regarding midline fusion is that,
Horseshoe kidney has also been found in association
at that stage, abnormal variation in growth, ventral flexion
with some chromosomal abnormalities such as Turner’s
of the hind end of the embryo, or other variations in the
syndrome and trisomy 18.
growth of pelvic structures may bring the metanephric
The clinical course largely depends on the nature of
blastemas (developing kidneys) abnormally close together
the anomalies, because horseshoe kidney itself is relatively
for a longer period; this proximity can lead to fusion.
asymptomatic.
The explanation for lateral fusion is that, during early
embryonic life, lateral flexion of the lumbosacral spine
Isolated Horseshoe Kidney
may push one of the developing kidneys toward the
midline. This positioning can lead to asymmetric fusion. In the pediatric clinical setting, about 90% of patients
In later embryonic life, the ascent of the fused kidney are asymptomatic, and the most common presentation
is limited at the level of the inferior mesenteric artery, is UTI.
when the isthmus of the horseshoe kidney gets trapped When symptoms are present, they are usually related
under it. Consequently, the horseshoe kidney always lies to hydronephrosis, infection, stone formation, or hema-
at a position that is lower than normal. However, whether turia. The most common symptom is vague abdominal
the cause is abnormal fusion, abnormal migration of the pain, which may radiate to the back. Occasionally, nausea
posterior nephrogenic areas, or another teratogenic factor and vomiting may be reported. Also, the so-called Rovsing
is not yet clear. sign (nausea, vomiting, and abdominal pain with hyper-
extension of the spine) may be positive in some patients.
INCIDENCE
A small percentage of patients may have a palpable
In general population, the incidence of horseshoe kidney lump in the abdomen.
is about 1 case in 400 persons worldwide, and it is the
Horseshoe kidney has been reported to be associated
most common renal fusion anomaly.
with increased risk for renal neoplasms such as Wilms’
CLINICAL FEATURES tumors, renal carcinoids, and transitional cell carcinoma.
Clinically, horseshoe kidney can be divided into two Sex: Renal fusion anomalies occur predominantly in
groups: one with associated anomalies and the isolated males. The male-to-female ratio is approximately 2:1 for
horseshoe kidney without any associated anomaly. horseshoe kidney and 6:1 for crossed fused ectopia.
Age: Clinically, this congenital anomaly is diagnosed in 3. Recurrent stone formation related to PUJ obstruction
individuals of all ages, from fetuses to the elderly. or infection may occur.
However, because of its association with other congenital 4. An increased risk of trauma to the isthmus exists
anomalies, horseshoe kidney is more commonly because of its position anterior to the spine.
diagnosed in children. 5. Horseshoe kidney may pose problems for the surgeons
during abdominal surgery for other abdominal
ANATOMICAL BASIS problems.
6. Some evidence suggests an increased incidence of
In horseshoe kidney, fusion occurs at the lower poles in
certain renal tumors in horseshoe kidney.
about 95% cases. This region of fusion, called the isthmus,
7. Horseshoe kidney may occur as an isolated anomaly
is usually composed of renal parenchymal tissue.
or in association with other congenital anomalies. The
However, in many cases, it may consist of fibrous tissue. morbidity and mortality rates largely depend on
The isthmus may be wide or narrow, depending on the whether it is associated with other anomalies.
degree of fusion. The isthmus usually lies anterior to the
aorta and inferior vena cava (IVC) and posterior to the PREFERRED INVESTIGATIONS
inferior mesenteric artery. However, rarely, the isthmus
Plain Radiographs
may pass between the aorta and IVC or even posterior
Plain KUB films may show low-lying renal outlines with
to these vessels. The ureters usually pass anterior to the
an altered renal axis. Usually, the kidneys follow the axis
isthmus, and they may have a high insertion point in
of the psoas muscles, with the lower poles lying at a more
the renal pelvis. The renal pelves are usually malrotated
lateral position than the upper poles. In horseshoe kidney,
and lie anteriorly or laterally. this axis is reversed, and the lower poles lie closer to the
In the midline fusion variety, the kidneys are spine (Figure 4.15).
symmetric, with lower poles converging toward the
midline. In the lateral fusion variety, one kidney is more IVU
vertical, while the other kidney is more horizontal; the For the purpose of diagnosis, IVU is usually the first-
isthmus lies slightly toward one side. In rare cases, the line investigation, followed by CT or scintigraphy in
upper poles may fuse, reversing the appearance of cases with doubtful findings.
horseshoe. Also rarely, both the poles may fuse; the result
is a ringlike mass termed disc kidney, doughnut kidney,
or pancake kidney.
Blood supply of the horseshoe kidney may vary. In
about 30% cases, it consists of one renal artery to each
kidney. Other variants include supply from 2 or 3 renal
arteries to one or both kidneys. The blood supply of the
isthmus also varies. It may come from the renal artery,
or it may directly arise from the aorta above or below
the isthmus. Occasionally, it arises from the common iliac,
external iliac, or inferior mesenteric arteries.
COMPLICATIONS OF HORSESHOE KIDNEY

1. Pelviureteric junction (PUJ) obstruction is a common
complication, possibly because of the high insertion
of the ureter.
2. Recurrent infections occur because of urine stasis and FIGURE 4.15: Plain KUB film showing the altered axes of renal
associated vesicoureteric reflux. shadows with lower poles lying closer to spine→horseshoe kidney
Findings:
IVU usually reveals the classical findings associated with
horseshoe kidney. Findings on the initial tomogram may
be deceptive because of the exclusion of the anteriorly
lying isthmus. Renal axis abnormalities are confirmed,
as seen on the plain radiographs. In midline fusion, the
kidneys are symmetric, with the lower pole calyces lying
closer to or actually overlying the spine. The lower calyces
are usually medially rotated, and they may actually lie
medial to the ureters. Some degree of malrotation of the
kidneys is usually present. A renal pelvis is often extrarenal
and large.
The degree of malrotation has been associated with
the degree of fusion. If the isthmus is narrow, the kidneys
are usually less malrotated, with pelvis lying antero-
medially in its near normal position. In cases of a wide
isthmus, the renal pelves lie anteriorly or laterally. FIGURE 4.16: IVU: Altered axes of both kidney, lower poles lying
closer to spine and calyces seen end-on→Horseshoe kidney
Associated UPJ obstruction may be present because of
the higher ureteric insertion point that leads to delayed
pelvic emptying. Ureters may have the so-called flower- in which the continuity of the poles with the isthmus
vase appearance in which the upper ureters diverge cannot be clearly demonstrated.
laterally over the isthmus and then converge inferiorly. Findings: Ultrasonography can be useful for diagnosing
The lateral fusion variety with an L-shaped kidney this anomaly. The most important feature in establishing
can also be readily appreciated on IVUs. In this variety, the diagnosis on the basis of sonographic findings is the
one kidney has a relatively vertical position while the other isthmus and its continuity with the lower poles. Other
is relatively horizontal. features, such as malrotation and an altered renal axis,
Limitations: may be difficult to assess at ultrasonography. In cases
1. Most of the time, IVU cannot be used to differentiate in which the isthmus is composed of only a thin fibrous
between a fibrous isthmus and a parenchymal isthmus. band, this midline soft tissue may not be seen.
Also, in many cases, the diagnosis of a horseshoe Findings such as a curved configuration of the lower
kidney is difficult on the basis of only IVU findings. poles, elongation of the lower poles, and poorly defined
In these cases, CT or scintigraphy may be helpful. lower poles, suggest the presence of this anomaly. Other
2. On IVUs, a malrotated or ectopic kidneys may associated findings, such as stones, hydronephrosis, and
sometimes be confused with a horseshoe kidney. cortical scarring, are reliably depicted on sonograms.
3. Gibbous deformity of the spine may alter the renal Ultrasonography has also been useful in the diagnosis
axis, which may then resemble horseshoe kidney of this anomaly in utero.
(Figure 4.16).
Ultrasonography CECT
Utility of USG depends on the visualization of the isthmus Contrast-enhanced CT has a high degree of accuracy
and the proof of its continuity with both the lower poles. in defining the structural abnormalities such as the
In many patients, especially patients with a large body degree and site of fusion, degree of malrotation,
habitus, overlying bowel gas makes the acquisition of associated renal parenchymal changes (e.g. scarring,
adequate scans difficult, for technical reasons. In cases cystic disease), and collecting system abnormalities (e.g.
duplex system, hydronephrosis). It can also be used to RNI

differentiate a fibrous isthmus from a parenchymal A diuretic renal scintigram is helpful in differentiating
isthmus and show its relation to the surrounding obstructed and nonobstructed dilated collecting
structures.
systems.
Although routine CT may show the variant arterial
supply, this is better defined with CT angiography with Findings: Scintigraphy demonstrates the fusion best if the
3D reconstruction and volume rendering. The use of 3D isthmus consists of functioning parenchymal tissue,
multisection helical CT has also been advocated in cases because this imaging modality depends not only on the
of neoplasm associated with horseshoe kidney, because structure but also on the function of the tissue. 99mTc–
it further clarifies the structural details. labeled dimercaptosuccinic acid (DMSA) can be used to
define the fused segments, as well as the altered axis of
Limitations: Figure 4.17.
both kidneys.
MRI This condition is incidentally diagnosed on bone scans,
Although MRI accurately reveals the anatomy, it is not
99m
Tc-labeled RBC studies, or other nuclear medicine
generally used for diagnosis because of its high cost. MR studies obtained for reasons other than the evaluation
angiography provides additional information about the of horseshoe kidney. The use of mercaptoacetyltriglycine
vascular anatomy. A voiding cystourethrogram is usually (MAG-3) with diuresis is helpful in differentiating
required to evaluate associated vesicoureteric reflux. nonobstructed and obstructed parts of the collecting
systems.
Findings: MRI has an advantage in depicting the structural
Horseshoe kidney can be confidently diagnosed with
details because of its ability to permit multiplanar imaging,
scintigraphy, which reveals the functioning parenchymal
but it is more costly than other examinations. However,
an added advantage may be obtained by using MR isthmus (Figure 4.18).
angiography to delineate the vascular anatomy. MRI is
probably the best modality to use in evaluating the extent
of renal tumors associated with horseshoe kidney.
With MRI diagnosis, as well as defining other
associated structural findings is better. However, asso-
ciated small stones may be missed on MRIs.
FIGURE 4.17: CECT–Axial section: Enhancing/functioning FIGURE 4.18: Renal scintigraphy: altered axis of both kidneys with
parenchyma isthmus seen joining the two kidneys functioning isthmus seen joining lower poles of kidneys
Angiography 6 (PKHD1) as the genetic locus. The frequency of the

It is usually reserved for presurgical planning to fully heterozygous state is estimated to be one in 70. The
evaluate the arterial supply pattern. PKHD1 gene is expressed at high levels in the fetal and
adult kidney and at lower levels in the liver, which
CTA: CT angiography with 3-dimensional (3D) corresponds to the principle sites of disease.
reconstruction may also reveal the vascular anatomy and ARPKD is characterized by pathologic changes in the
collecting system for presurgical planning. kidney and/or liver. In the kidney, epithelial hyperplasia
Angiography is not normally performed to diagnose occurs along the collecting duct of the nephron. The
this condition, but it is performed to evaluate the vascular hyperplastic cells undergo a functional change from being
anatomy and its variations in a presurgical setting. resorptive to becoming secretory. The fluid secreted from
Angiograms may show 1, 2, or 3 renal arteries on either these abnormal cells is rich in epithelial growth factors,
side and a large variation in the blood supply of the which further stimulate epithelial proliferation. The
isthmus. However, in cases of associated renal tumors, combination of epithelial hyperplasia and fluid secretion
angiography is used to evaluate tumor vascularity. results in significant ductal ectasia. Approximately
Angiography is occasionally performed to check renal 10-90% of the ducts may be affected, resulting in a
artery stenosis in hypertensive patients who have this wide variability of renal dysfunction. Depending on
anomaly. the number of ducts involved, the kidneys may be
massively enlarged. Examination of the kidney reveals
INTERVENTION multiple small subcapsular cystic spaces that correspond
Image-guided percutaneous nephrostomy can be histologically with radially oriented, ectatic collecting
performed to relieve hydronephrosis associated with ducts.
horseshoe kidney. Percutaneous stone removal may also Liver disease is present in every patient with ARPKD,
be achieved with image guidance with the manifestations varying according to the patient’s
age at presentation. The chief pathologic hallmarks of
AUTOSOMAL RECESSIVE POLYCYSTIC liver disease are periportal fibrosis and biliary duct ectasia.
Significant liver involvement is referred to as congenital
KIDNEY DISEASE (ARPKD)
hepatic fibrosis. Although the mechanism is not clearly
INTRODUCTION
defined, the most common clinical manifestation of
It is the most common heritable cystic renal disease congenital hepatic fibrosis is portal hypertension.
occurring in infancy and childhood. It is distinct from
autosomal dominant polycystic kidney disease (ADPKD), CLASSIFICATION OF ARPKD
which tends to occur in an older population. The clinical Blyth and Ockenden initially classified ARPKD into four
spectrum shows a wide variability, ranging from perinatal groups:
death to a milder progressive form, which may not be 1. Perinatal
diagnosed until adolescence. 2. Neonatal
3. Infantile
PATHOPHYSIOLOGY
4. Juvenile
ARPKD follows an autosomal recessive inheritance The four categories are based on the individual’s age
pattern, with siblings of either sex having a 25% chance and the onset of clinical manifestations; however, the
of developing disease while the parents are unaffected. disease is expressed as a part of a spectrum of findings
The disease has variable expression, such that siblings rather than fitting into clearly defined subcategories.
may manifest different degrees of disease. Despite the 1. Category 1 is perinatal ARPKD. Patients with the
clinical variability of ARPKD, it appears that a single perinatal form are born with a markedly enlarged
unidentified gene is responsible for all forms of the disease. abdomen due to nephromegaly, which may interfere
Linkage studies have localized an area on chromosome with delivery. Approximately 90% of the collecting
ducts are dilated with minimal liver involvement. INCIDENCE

Severe renal impairment in utero leads to oligo- ARPKD has an incidence of 1 in 6,000 to 1 in 55,000
hydramnios and subsequent pulmonary hypoplasia. live births.
Other clinical findings may include sequelae of Notably in Finland, the incidence is reported to be
oligohydramnios, such as Potter facies and clubfoot. higher (1 in 1000).
Most of these infants do not survive beyond the first
CLINICAL FEATURES
week of life. Unfortunately, approximately 75% of all
cases of ARPKD is this severe. Race: No information has shown a racial predominance.
2. Category 2 is neonatal ARPKD. Patients with the Sex: Both sexes are affected equally.
neonatal form have palpable kidneys at birth.
Age: ARPKD may initially occur anytime between
Approximately 60% of the kidney is affected and there
perinatal period to five years of age depending on
is mild liver disease. Pulmonary involvement is less classification.
of a factor in this form because renal impairment is Two constant features of the disease are kidney and
often less severe in utero. Progressive renal failure is liver involvement of variable severity. Generally, renal
the dominant feature of this form, resulting in death and hepatic disease manifest opposite degrees of severity.
within a few months. Patients that develop severe kidney disease early in life
3. Category 3 is infantile ARPKD. The infantile form tend to succumb to renal failure before significant hepatic
of the disease tends to present after a few months disease can develop. On the other hand, patients with
of life. Approximately 25% of renal collecting ducts a milder form of kidney disease tend to develop severe
are dilated, with moderate hepatic periportal fibrosis. hepatic complications later on in life. The main
characteristic of kidney involvement is dilatation of the
Clinical presentation includes large kidneys and
collecting system resulting in multiple cysts, which
hepatosplenomegaly. Patient often develops chronic
manifests as progressive renal failure. Disease in the liver
renal failure and/or portal and systemic hypertension. is typically diffuse, presenting as portal and interlobular
The disease often progresses to end-stage renal failure fibrosis, dilatation and hyperplasia of bile ducts, or a
by adolescence, which is the predominant cause of combination of both. Liver disease ultimately results in
mortality. portal hypertension.
4. Category 4 is juvenile ARPKD. The hallmark of the
juvenile form is pronounced hepatic involvement. ANATOMICAL BASIS
Renal insufficiency is generally absent or mild, with ARPKD results in bilaterally generally symmetrically
less than 10% of the kidneys affected. The disease enlarged kidneys that maintain their reniform shape.
has a wide range of age presentations, from 6 months Beneath the capsule are scattered opalescent cysts from
to 5 years. The presentation is characterized by dilated collecting ducts, usually 1–2 mm in diameter, but
variable renal enlargement and hepatosplenomegaly. sometimes larger. On sections, the renal parenchyma
resembles a sponge with ectatic, nonobstructed, radially
Significant liver involvement results in portal
oriented collecting tubules that have areas of hyperplastic
hypertension. Morbidity and mortality are often
cuboidal or low columnar lining epithelium. Interstitial
secondary to the sequelae of portal hypertension, fibrosis develops but the glomeruli remain normal.
including variceal bleeding and thrombocytopenia ARPKD results in dilated bile ducts with protrusions
or anemia secondary to hypersplenism. Mortality for from the walls and bridging tissue between the duct walls.
this type is lowest among the 4 categories, with There are often increased numbers of ducts. There is
approximately 80% of patients surviving beyond the congenital hepatic fibrosis with increased connective tissue
age of 15 years. in the enlarged portal tracts.
Generally, there is a reciprocal relationship between PATHOPHYSIOLOGY

the degree of renal and hepatic involvement in individual ADPKD is an inherited condition comprising at least 3
patients. Those with more severe renal involvement have phenotypically indistinguishable but genetically distinct
less severe hepatic disease.
entities caused by mutations in 3 genes: PKD1, PKD2,
PREFERRED INVESTIGATIONS and PKD3. The gene is located on chromosome arm
16p and chromosome 4 in 90% of patients and is related
IVU It helps to differentiate ARPKD from ADPKD,
to spontaneous mutation in 10%. ADPKD is transmitted
Glomerulocystic disease and renal dysplasia by demon-
strating bilateral and symmetrical enlargement of the as an autosomal dominant trait, with almost 100%
kidneys and ‘streaky/ chaotic/ Swiss-cheese’ nephrogram penetrance if patients live long enough. Because of the
as the contrast clears in the ectatic collecting ducts. variable expressivity and spontaneous mutation, a family
history is not found in nearly one half of patients.
Ultrasonography Histologically, ADPKD is characterized by an
It is the primary modality for the evaluation of ARPKD, abnormal rate of tubule divisions, with hypoplasia of
especially during the perinatal and neonatal periods, portions of tubules left behind as the ureteral bud
which characteristically shows bilateral renomegaly with advances. Cystic dilatation occurs in the Bowman’s
uniformly increased reflectivity. capsule, loop of Henle, and proximal convoluted tubule
interspersed with normal renal tissue. Thus, in distinction
CT and MRI
to simple renal cysts in which the contents are
In older children, CT and MRI are often used to evaluate biochemically similar to plasma, the biochemical features
liver disease. of the fluid content of cysts in ADPKD is closer to those
MORTALITY/MORBIDITY
of urine, particularly when samples are taken from distal
nephrogenic cysts. Cysts in ADPKD are lined by flattened
ARPKD accounts for 1.5% of children in renal replace-
or cuboidal epithelium. Stromal changes are nonspecific
ment therapy prior to the age of 15 and 0.6% of patients
and are those of renal failure; dystrophic calcification is
treated because of end-stage real failure before the age
common.
of 20 years.
With minimal disease, the kidneys are normal in size
and are smooth, and the cysts are discovered only on
ADULT POLYCYSTIC KIDNEY DISEASE (ADPKD) cut pathologic specimens. As the size of cysts increases,
INTRODUCTION the kidneys enlarge, often asymmetrically, and the kidneys
It is transmitted as an autosomal dominant trait and affects may become bosselated and lose their reniform shape.
approximately 1 in 1000 people. Cysts arise from the The age of patients at onset of cyst formation varies; 54%
nephrons and the collecting tubules. Islands of normal appear by the first decade of life, 72% occur by the second
parenchymal renal tissue are interspaced between the decade, and 86% occur by the third decade. By the age
cysts. Microdissection reveals that the cysts communicate of 80 years, evidence of cyst formation exists in all persons
directly with the nephrons and collecting tubules. who have the gene.
Patients present with hypertension and progressive True unilateral disease is rare because most genetic
renal failure after their third decade of life. Uncommonly, diseases involving paired organs are bilateral. Segmental
autosomal dominant polycystic kidney disease (ADPKD) ADPKD also occurs rarely, although some doubt the
appears in children, and it is rarely seen in neonates. existence of segmental disease and suggest that it should
Of patients with ADPKD, 25–50% have associated not be considered a forme fruste of ADPKD. Segmental
hepatic cysts, 9% have associated pancreatic cysts, and disease is not inherited and not associated with renal
5% have associated splenic cysts; pulmonary cysts occur failure. Rarely, ADPKD may be detected in utero, usually
uncommonly. These extrarenal manifestations are not in the third trimester, although the earliest diagnosis
found in neonates and children. recorded was at 14 weeks gestation.
With small cysts, ADPKD can be confused with CLINICAL FEATURES

autosomal recessive polycystic kidney disease (ARPKD) Race: No race predilection exists.
because the kidneys may be enlarged and echogenic.
Sometimes, the cortical cysts are large enough to be Sex: No sex preponderance exists.
demonstrated on ultrasonographic images, which can Age: Patients of any age can be affected, but the mean
confirm the diagnosis when cysts are demonstrated in age at diagnosis is 43 years. In rare cases, renal cysts
the fetus of a parent with the disease. are incidentally discovered in people aged 70–90 years.
With progression of the disease, impaired renal Although the disease probably begins in utero in most
function ensues. Hypertension precedes renal failure. patients, symptoms are unusual until the fourth or fifth
Extrarenal manifestations include liver cysts in 25–50% decade. With increasing use of cross-sectional imaging,
of patients, pancreatic cysts in 9%, and splenic cysts in ADPKD is discovered incidentally in asymptomatic
5% of patients. Other cysts reported include cysts of the patients in their 80s. However, the most common
thyroid, parathyroid, lung, brain, pituitary gland, pineal
presentation is a palpable mass, hypertension, abdominal
gland, ovary, uterus, testis, seminal vesicles, epididymis,
pain, and hematuria. Hypertension often predates renal
bladder, and the peritoneum. Aneurysms of cerebral
failure.
arteries (berry aneurysms) have been reported in 3–50%
Renal failure ultimately affects most patients by the
of patients. A variety of cardiac and aortic abnormalities
time they are aged 60 years. Patients may present with
have been associated with ADPKD, including aortic root
fever, dysuria, and leukocytosis due to urinary tract
dilatation, aortic regurgitation, bicuspid aortic valves,
infections. Renal and/or ureteric colic from calculi is a
coarctation of the aorta, mitral regurgitation, and
known complication. Hemorrhage, which may be
abdominal aortic aneurysm.
intracystic or retroperitoneal, may present with hematuria,
Cysts vary in size from those barely visible to those
abdominal pain and, rarely, massive hemorrhagic shock
that are several centimeters in diameter. Cysts usually
or anemia. Polycythemia is a rare but known association
contain clear straw-colored fluid, but hemorrhage into
1 or more cysts is common, which may change the gross, secondary to increased erythropoietin production. Rarely,
biochemical, and histologic character of the fluid. Cysts intracystic hemorrhage within a liver cyst may cause acute
may become infected, and aspiration of the fluid may abdominal pain, mimicking an acute cholecystitis.
reveal purulent contents. Incidence of renal cell carcinoma Urinalysis may reveal proteinuria and hematuria.
is only slightly increased in patients with ADPKD; a greater
PREFERRED INVESTIGATION
increased incidence is associated with cystic disease of
dialysis. Plain radiographs offer limited information. Plain
Approximately 50 cases of renal cell carcinoma have radiographic findings are normal in the early stages of
been reported in association with polycystic kidneys; some ADPKD, but with enlargement of the kidneys, soft-tissue
of the cases were associated with von Hippel-Lindau masses displace the normal intra-abdominal organs.
disease and tuberous sclerosis. No correlation exists
IVU when multiple cysts are readily visible on USG the
between the severity of renal disease and the number
IVU will show compression and displacement of calyces
of liver cysts. Liver function usually remains normal, but
by the intrarenal cysts. There is difficulty in identifying
with longer survival of patients with ADPKD, liver function
the renal outline. Puddling of contrast medium on
abnormalities may occur, particularly in patients with
IVU is differentiating feature of ADPKD from that of
portal hypertension. An association between ADPKD and
ARPKD, especially in infants.
congenital hepatic fibrosis has been described.
Ultrasonography is the modality of choice in the workup
INCIDENCE of patients with ADPKD, and it is an ideal modality
One in 1000 people carry the ADPKD trait. ADPKD is for screening the family of patients. In earlier studies
the most common genetically linked renal disorder. in young children, intravenous urography and
nephrotomography were shown to be slightly more Radionuclide studies have a complementary role in
sensitive than ultrasonography. the assessment of renal function in ADPKD. These studies
CT scanning is as sensitive as ultrasound in the detection do not have the added hazard of an exposure to iodinated
of cystic disease, although problems may arise with contrast material.
smaller cysts. CT appears to be more specific than
Limitations of above Modalities in General
sonography in differentiating an obstructed renal pelvis
from a parapelvic cyst. CT scans also appear superior On plain radiographs, nephromegaly may occur because
to ultrasonographic images in helping assess of causes other than ADPKD. Similarly, curvilinear
retroperitoneal rupture of a cyst and perinephric extension calcification is not specific for ADPKD and may be found
of blood or pus from an infected cyst. in other types of cysts and in tumors and granulomas.
MRI is especially useful in patients who are allergic to Cysts similar to those of ADPKD can occur in nongenetic-
iodinated contrast media and in patients with related simple renal cysts and in von Hippel-Lindau
compromised renal function who are at risk for iodinated disease. These findings apply to intravenous urography,
contrast-induced renal failure. MRI also has advantages ultrasonography, CT scanning, MRI, and angiography.
in patients in whom hemorrhagic cysts are considered. Cysts associated with ADPKD cannot always be diffe-
MRI is probably superior to other modalities in charac- rentiated from multiple simple cysts and cysts associated
terizing complicated cysts (Figure 4.19). with von Hippel-Lindau disease or tuberous sclerosis.
RNI: A 99mTc DMSA scan will give info regarding the
MORTALITY/MORBIDITY
differential function and will show photon-deficient
areas occupied by the cysts with uptake in the intervening Renal failure affects most patients with ADPKD by the
normal renal parenchyma. The kidneys have lost their time they are aged 60 years.
normal reniform appearances and the appearances can Hypertension predates renal failure. Complications
be quite misleading for the unwary. from hypertension secondary to ADPKD are similar to
essential hypertension. Patients who are normotensive
Angiography: Its role in the diagnosis of ADPKD is
at presentation have a better prognosis in terms of
limited. Although angiography has a high degree of
survival. Infections, hemorrhage, cyst rupture, and renal
accuracy in the diagnosis of ADPKD, its specificity is low.
calculus disease are recognized complications of ADPKD.
Results of experimental studies have suggested that cystic
kidneys become infected more easily than noncystic
kidneys. The urinary tract carries a particular risk of
serious infections in ADPKD, which adds considerably
to morbidity and mortality. Rarely, massive intracystic or
retroperitoneal hemorrhage can occur; these require
nephrectomy. Before the availability of renal dialysis and
renal transplantation, most patients died within 10 years
after the onset of symptoms. Liver and other extrarenal
cysts seldom cause symptoms; however, with longer
survival of patients with ADPKD, liver impairment may
cause increased morbidity and mortality rates. Rare
complications of hepatic cystic disease include cyst
hemorrhage, infection, portal hypertension, biliary obstru-
ction from cystic mass effect, and cholangiocarcinoma.
FIGURE 4.19: Coronal T2WMR: Bilateral polycystic The variety of cardiac and aortic problems associated
kidneys–ADPKD with ADPKD may add to morbidity and mortality.
Approximately 10% of patients with ADPKD die from • Tend to involute with time, cysts shrink and residual
a ruptured intracranial berry aneurysm. tissue does not have a reniform shape, typically not
surgically removed.
MULTICYSTIC DYSPLASTIC KIDNEY (MCDK) • A few reports of Wilms’ tumor occurring in MCDK
may change management.
DEFINITION
• Up to 40% of patients with MCDK have contralateral
A non-functional kidney, replaced by multiple cysts and abnormality-PUJ obstruction and vesicoureteral reflux
dysplastic tissue, can vary in size from .10-15 cm to only are most common.
1–2 cm. It is the second most common abdominal mass • Can be segmental in duplicated kidneys.
in a neonate (Figure 4.20).
It probably due to atresia of ureter. Equal incidence INCIDENCE
in males and female. Gross pathologic-surgical features
0.03% in autopsy series.
include Walls of cysts vary in thickness fibrotic, renal
stroma, may be quite large and bizarre. IMAGING FINDINGS
By classic imaging appearance: 2 forms are generally
recognized: Sonography to document cystic nature and exclude
1. Pelvoinfundibular MCDK more common type—results enlarging mass over time (potential for Wilms’ tumor)
from atresia of ureter or renal pelvis, cysts are remnants Nuclear scintigraphy to document non-function and
of dilated calyces confirm normal drainage of contralateral kidney.
2. Hydronephrotic type of MCDK occurs less frequently
Nuclear Scintigraphy
—results from atretic segment of ureter, cysts are the
entire pelvocalyceal system. Best imaging clue: Nuclear scintigraphy documents
lack of renal function. If some excretion is present,
consider poorly functioning hydronephrosis. Initial blood
flow images show perfusion of the MCDK, but sequential
images document lack of any excretory function. Note
that 99mTc DMSA may localize to the renal cortex in MCDK
due to the presence of tubular cells, but this is different
than true excretion of radiopharmaceutical.
a. Longitudinal sonogram of MCDK showing cysts of
varying sizes and echogenic intervening fibrous
stroma.
b. Posterior images from 99mTc MAG3 scan showing
normal drainage of left kidney and absent function
on the right in a toddler with right-sided MCDK.
MAG3, DTPA, Glucoheptonate are typically agents
of choice.
CT/MR Findings
Typically an incidental finding on CT or MRI scans, cystic
kidney with some enhancement possible in solid
FIGURE 4.20: Multicystic dysplastic kidney components, but no true excretion of contrast, cortex
(For color version see Plate 3) replaced by cysts.
Ultrasound PATHOPHYSIOLOGY
Multiple anechoic cysts of varying size, largest cyst is not The pathogenesis of MSK is unknown. Most authors
generally centrally located, echogenic fibrous tissue in believe that MSK is a developmental defect affecting the
intervening septa. formation of the collecting tubules. Some authors believe
that MSK is a progressive degeneration of the collecting
INTRAVENOUS UROGRAPHY (IVP)
tubules that occurs later in life. According to Osathanondh
It will also confirm non-function, but seldom used in and Potter, the primary abnormality is hyperplasia of part
pediatrics. of the medullary collecting tubules. The size of the kidney
may be normal or slightly enlarged. On pathologic
RETROGRADE URETEROGRAM (RUG)
sections, multiple cysts representing dilated terminal
It will show blind ending ureter, different from rapid collecting tubules are seen, measuring from 1–7 mm. The
change in caliber of ureter and communication with cysts usually communicate proximally with collecting
calyces seen in UPJ obstruction or other causes of hydro- tubules and distally with papillary ducts or calyx.
nephrosis. Intercommunicating and noncommunicating cysts
are seen occasionally. Calculi may be seen within the
DIFFERENTIAL DIAGNOSIS cysts.
1. Hydronephrosis: Calyces should communicate with
INCIDENCE
each other in hydronephrosis, look for connections
on ultrasound. The frequency of MSK in the general population has
2. Congenital mesoblastic nephroma: Solid tumor been estimated to be 1 case per 5,000–10,000
of infancy. population, and MSK is seen in approximately 0.5% of
3. Wilms’ tumor: Can be difficult to separate by USG, patients examined by using intravenous urography (IVU)
absent excretory function is key. for various reasons.
PROGNOSIS CLINICAL FEATURES
Excellent when uncomplicated. Sex: The sex predilection varies.

Sometimes, the frequency is reported to be higher
MEDULLARY SPONGE KIDNEY (MSK) in males than in females, and sometimes, it is higher in
females.
INTRODUCTION
Age: MSK usually is diagnosed in persons aged 10–30
It is a developmental abnormality occurring in the
years, although MSK has been reported in children as
medullary pyramids of the kidney. MSK is characterized
by cystic dilatation of the collecting tubules in 1 or more young as 2 years.
renal pyramids in 1 or both kidneys. Most patients with MSK remain asymptomatic
Lenarduzzi first described MSK in 1939. throughout life, and the condition is discovered
The etiology is unknown. incidentally when IVU is performed for reasons other than
Most patients remain asymptomatic, and MSK is the assessment of MSK. Patients with MSK are usually
detected incidentally on urograms unless it is complicated asymptomatic, although acidification or impaired concen-
by infection, stone formation, or hematuria. Most cases tration of urine has been documented. Complications
are sporadic, but a few hereditary cases have been such as infection, hematuria, and stone formation may
reported. be the presenting complaint in approximately 10% of
MSK also has been documented in siblings and in patients.
several generations of families. The disease occurs in The frequency of calculus disease is increased and
persons of all ages and is more common in males than manifested by hematuria, renal colic, flank pain, fever,
in females. and dysuria.
ASSOCIATED SYNDROMES
MSK has been associated with hemihypertrophy, Ehlers-

Danlos syndrome, adult polycystic disease, congenital
hypertrophic pyloric stenosis, Caroli disease, parathyroid
adenoma, anodontia, Beckwith-Wiedemann syndrome,
distal renal tubular acidosis, horseshoe kidney, Marfan’s
syndrome, renal artery stenosis, pyeloureteritis cystica,
and ureteral duplication.
When associated with MSK, Beckwith-Wiedemann
syndrome has a high rate of tumors, including Wilms’
tumor and hepatoblastoma. MSK has been reported to
cause growth failure in children due to incomplete renal
tubular acidosis type 1, and it accounts for hypertension
in pregnancy in 1% of patients.
PREFERRED EXAMINATION
Plain Radiograph FIGURE 4.21: Plain KUB X-ray: Nephrocalcinosis
It may demonstrate nephrocalcinosis. Although MSK

deformities with multiple cyst and cystlike cavities of
cannot be diagnosed by using plain radiographic findings
various sizes with beaded or striated cavities extending
alone, the presence of linear and rounded medullary
through the pyramid from tip to base.
calcifications may suggest the diagnosis. The specific In patients with full-blown MSK, calyces tend to be
diagnosis of MSK cannot always be made with plain broad, shallow, distorted, and widely cupped. If calculi
radiographic findings alone because MSK is one cause are present, they tend to be arranged in groups around
of nephrocalcinosis, which has a wide differential
a calyx, similar to a cluster of grapes or a bunch of flowers.
diagnosis.
Renal function decreases with subsequent poor depiction
Findings: Plain radiograph findings may be normal or of the kidney. In view of the high incidence of nephro-
may demonstrate nephrocalcinosis. Nephrocalcinosis is lithiasis in MSK, many patients have ureteral calculi. In
characteristic of MSK, with several discrete pyramidal these patients, the excretory urogram may show
medullary calcifications occurring in clusters. When obstruction, calyceal distortion or destruction, and
passed into the collecting systems, calculi may be seen evidence of urinary tract infection.
in the renal pelvis, ureter, or bladder. Renal size is usually
normal, but the kidneys can be enlarged if the condition USG
is associated with polycystic kidney disease (Figure 4.21). Ultrasonographic (USG) and CT findings are more
IVU sensitive than plain radiographic findings in showing
medullary calcifications, but they are less specific than
The principal method for diagnosing MSK is IVU, in which
IVU findings. The sonographic appearances of MSK are
discrete linear papillary densities, characteristic of MSK,
also nonspecific because hyperechoic medulla with or
are seen. IVU appearances depend on the type of tubular
without shadowing has been documented in a large
changes present. Changes form a spectrum that ranges
variety of conditions.
from mild dilatation of the renal collecting tubules (often
called renal tubular ectasia), which shows discrete linear Findings: USG findings demonstrate echogenic medullary
opacities in 1 or more papillae through increasing severity pyramids in patients with MSK, irrespective of the
of tubular dilatation and cystic changes, to gross presence of medullary nephrocalcinosis. The echogenic
medulla may cast acoustic shadowing. The increased

echogenicity is seen in particular at the periphery of
each pyramid between the interlobar cortices. US
findings can demonstrate complications related to calculus
disease.
CT
Findings: CT can be helpful in confirming the presence
of nephrocalcinosis, when it is suggested on USG images,
and CT scans can demonstrate tubular ectasia. In MSK,
unenhanced CT scan findings may be normal or
demonstrate medullary nephrocalcinosis.
Enhanced scans may demonstrate contrast FIGURE 4.22: CECT: MSK with medullary calcification
accumulation within the papillae. is marked by contrast
CT scans readily depict obstructive changes and help RNI
identify complications such as interstitial infection and
The role of radionuclide scans in MSK is limited to the
abscess formation.
assessment of renal function and to the identification of
Ill-defined areas of low attenuation representing
a focus of renal infection.
interstitial infection can readily be distinguished from
normally enhancing renal parenchyma. INTERVENTION
CT scans also can help in differentiating interstitial Radiologic intervention is seldom required; however,
infection from abscess formation because abscesses percutaneous nephrostomy may occasionally be useful
appear as sharply defined low-attenuation areas with thick in treating a ureteric obstruction due to a calculus.
walls. Although urinary tract infection occurs in approxi-
CT scans can also help in assessing the perinephric mately one-third of symptomatic patients with MSK, renal
extension of abscesses, and they can guide percutaneous abscess is a rare complication of the disorder. The
drainage. diagnosis of renal abscess should be suspected in patients
Although CT has a limited role in evaluating patients with MSK and acute pyelonephritis which do not respond
with MSK, it plays a significant role in evaluating to appropriate antibiotic therapy. CT scans may reveal
complications such as infection or abscess formation, and large abscesses that require percutaneous or open surgical
CT can be used to guide percutaneous drainage of these drainage or small abscesses that require prolonged high-
collections (Figure 4.22). dose antibiotic therapy.
MRI MORTALITY/MORBIDITY
MRI is insensitive in detecting calcification. Morbidity associated with MSK appears to be higher in
The role of radioisotope uptake imaging is to assess women than in men.
renal function and to show the site of renal parenchymal In the vast majority of patients, MSK is associated
scarring. CT appearances of MSK are nonspecific. with a normal life expectancy.
MRI has a complementary role and is a useful A few cases do progress, with deterioration of renal
alternative in patients who are allergic to iodinated function and eventual renal failure.
contrast media.
CALYCEAL DIVERTICULUM
MRI is poor in depicting calcification. The role of MRI
in MSK is yet to be defined, but MRI may provide an INTRODUCTION
alternative to IVU in patients who are allergic to Calyceal diverticulum, also known as pylogenic cysts, is
radiographic iodinated contrast medium. typically a symptomatic and incidentally found on IVUs.
They are found in both males and females of all ages.

Calyceal diverticulums are composed of uroepithelium
lined pouches that extend from the collecting system into
the renal parenchyma.
TYPES
They can be of three types:
1. Type I originates from the minor calyx,
2. Type II originates from the calyceal infundibulum and
3. Type III from the renal pelvis.
These diverticuli may lead to urinary stasis, which
may predispose the patient to stone formation or urinary
tract infections. During an intravenous urogram, the
calyceal diverticulum will appear opaque later in the
pylographic phase. It typically remains radiopaque after
the remainder of the kidney has drained due to the slow
exchange of urine caused by a narrow communicating
neck. The diverticulum should have a round, smooth-
walled appearance. It should not be confused with a hydro
calyx that develops from an infundibular stricture and FIGURE 4.23: Retrocard ureter
has a squared off appearance.
A calyceal diverticulum is a lesion that results from TREATMENT
an out pouching of a portion of the collecting system
Management of symptomatic stone disease associated
that protrudes into the corticomedullary region. They can
arise in any part of the collecting system from a fornix with calyceal diverticula has changed from an open
to the renal pelvis. Size varies anywhere from a few surgical approach to include ESWL, percutaneous,
millimeters to several centimeters in diameter. They are laparoscopic or ureteroscopic techniques. The choice of
uroepithelial-lined cavities that communicate via a narrow therapy depends largely on the anatomic location of the
channel to a nearby calyx. They may be congenital, or diverticulum.
acquired lesions (Figure 4.23).
It is not uncommon to see calcified stones COMPLETE URINARY COLLECTING SYSTEM
characteristically layering in the dependent portion of the DUPLICATION
diverticulum. The stones that form within the diverticulum
INTRODUCTION
may pass and cause symptomatic renal colic but they
are typically confined to the diverticulum due to its narrow It can be complicated by upper pole moiety obstruction
neck connection to the distal collecting system. and lower pole moiety vesicoureteral reflux.
They are regions of urinary stasis and the dependent
sediment that eventually develops in a calyceal PATHOPHYSIOLOGY
diverticulum is referred to as “milk of calcium”. Larger
Partial duplication is caused by branching of the ureteral
stones may form that are confined to the diverticulum
bud prior to its connection with the metanephric blastema
and may be a source of chronic pain.
(“Y” ureter). The two ureters may join between the kidney
On CT, the diagnosis is made with delayed imaging
showing the diverticulum fill with contrast. Alternatively and the bladder or one will end in a blind pouch.
if stones are present, the patient can be rescanned in the When two separate buds arise from the mesonephric duct,
opposite position. If the stones settle dependently, and complete duplication occurs as each ureteric bud
are confined to the lesion, the diagnosis can be made separately joins the metanephric blastema. This forms
in the absence of delayed imaging. both upper and lower pole “moieties”.
The Weigert-Meyer rule specifies the upper pole Duplications are familial. Incomplete duplication
moeity ureter enters the bladder inferior and medial to results in no increased incidence of disease. Complete
the insertion of the lower pole moiety ureter. While duplication results in higher incidence of UTI, VUR,
uncomplicated duplex kidney is considered a normal parenchymal scarring and obstruction. If no reflux or
obstruction, considered a normal variant.
variant, there are potential complications as in this case:
a. Ectopic ureterocele of ureter draining upper pole may
CLINICAL FEATURES
result in upper pole hydroureteronephrosis.
b. Vesicoureteral reflux into lower pole due to abnormal Frequently discovered during evaluation of a urinary tract
development of lower pole ureter-bladder wall valve infection in a child or prenatally as hydronephrosis.
Results in variable to normal function with malfunction
mechanism.
due to both dysplasia of upper pole cortex due to obstruc-
Less commonly, the ectopic upper pole ureter may
tion and/or lower pole reflux nephropathy.
insert distal to the urethral sphincter causing continuous
urinary dribbling/incontinence or an ectopic ureter may
reflux.
On sonography, a duplex collecting system is seen as two
VARIETIES central echogenic renal sinuses with intervening bridging
hypoechoic renal parenchyma.
Duplex kidney refers to two ureters draining one
On CT, the two ureters can be followed on sequential
kidney. Most common (1 in 160) anomaly of the upper
contrast-enhanced images as they exit the renal sinus and
collecting system. Spectrum from bifid renal pelvis to
extend to join each other or the bladder. With obstructed
complete duplication. Incomplete form with bifurcation
systems, the hydronephrotic segment of the kidney and
of the ureter high (bifid renal pelvis), anywhere along
the nonenhancing dilated ureter can be followed to its
the ureter (bifid mid-ureter) or near the bladder (low bifid
point of termination. MRI is useful in that the ureteral
ureter).
insertion can be display in multiple planes. Sonography,
Incomplete duplication more common than complete
duplication. Weigert-Meyer rule applies if the duplication especially in children, is ususally sufficient for diagnosis.
is complete: Ureteral orifice of the upper pole moiety
inserts ectopically into the bladder medial and inferior
to its normal location and to the ureter draining the lower Renal ectopia, congenital ureteropelvic junction obs-
renal segment. With complete duplication, the ureter truction, megaureter.
draining the lower pole has a more perpendicular course
through the bladder wall making it more prone to reflux. TREATMENT
The ectopic ureter from the upper pole is prone to Endoscopic ureterocele incision (upper pole moeity’s
obstruction, reflux or both. If large, a ureterocele may block ureteral herniation into bladder) - relieves hydonephrosis
the contralateral ureteral orifice and/or the urethral orfice AND re-implantation of lower pole moiety ureter into a
at the bladder neck. Ureterocele treatment is surgical. more oblique course through bladder wall eliminating
Ectopic ureterocele is a cyst like protrusion into the continued reflux into lower pole.
bladder lumen of the dilated submucosal distal portion
of an ectopic ureter. Associated with duplication, usually DUPLICATED COLLECTING SYSTEMS
with the upper pole moiety. Upper pole of the duplex
INTRODUCTION
kidney is dilated and connects with a dilated, tortuous
ureter. There may be dilatation of the lower moiety due Duplicated collecting systems can be defined as renal units
to VUR or extrinsic PUJ obstruction of the lower pole containing two pyelocalyceal systems associated with a
ureter by the crossing dilated upper pole ureter. At the single ureter or with double ureters. The two ureters empty
level of the bladder, the hydroureter terminates in a round, separately into the bladder or fuse to form a single ureteral
thin-walled anechoic intravesical ureterocele. orifice.
Duplicated collecting systems can be unilateral or Rarely, a single proximal ureter divides distally to form
bilateral and occur in 15% of the population. Duplicated an inverted Y appearance. Usually, 2 ureteral orifices are
systems can be associated with a variety of congenital observed on the same side; rarely, one of the ureters can
genitourinary tract abnormalities. Most patients are be ectopic. Infrequently, the middle third of the ureter
asymptomatic and genitourinary tract abnormalities are may be duplicated, with a single proximal third and a
detected incidentally on imaging studies performed for single distal third. Double ureters remain completely
other reasons. Symptomatic patients usually have separated and in approximately 85% of patients, the
complete ureteric duplication in which the ureters are upper pole ureter drains below and medial to the lower
prone to developing obstruction, reflux, and infection. pole ureter (Weigert-Meyer rule).
Ureteropelvic obstruction is more common when a duplex Certain potential abnormalities result from or are
kidney exists and can be inherited in an autosomal related to duplicated systems. The following are well-
dominant pattern. known examples:
• Upper pole hydronephrosis from stenosis of the upper
PATHOPHYSIOLOGY
pole ureteral orifice
During embryogenesis, if a single ureteral bud bifurcates • Ectopic insertion of the upper pole ureter
before bifurcation of the ampulla, a duplex kidney results • Ectopic ureterocele of the upper pole ureter
with bifid pelvis or bifid ureter (Figure 4.24). If two ureteral • Reflux involving the lower pole from maldevelopment
buds arise from the Wolffian duct, a duplex kidney results of the valve mechanism.
with complete ureteral duplication. The ureteral bud
associated with the future lower pole separates first from INCIDENCE
the Wolffian duct and the orifice progresses superiorly
The incidence of duplex kidney appears to be 12-15%
and laterally as a result of growth of the urogenital sinus.
in the general population.
The common excretory duct, with the remaining ureter
still attached, is taken up in to the urogenital sinus. The
CLINICAL FEATURES
orifice of the ureter draining the upper pole opens medial
and inferior to the orifice draining the lower pole. Race: No racial predilection has been recorded.
A duplex kidney may be drained by a single ureter Sex: No sex predilection is found among patients with
or by two ureters that unite to form a single ureter or bifid collecting systems and partial ureteric duplication.
drain separately. Usually, the lower pole system is The presence of double ureters appears to be 10 times
dominant; a large renal pelvis drains the lower pole more common in females. Duplex kidney with uterus
through many calices. The upper pole pyelocalyceal didelphys has been reported in identical twins.
system may have only a single calyx and a single Age: A duplicated collecting system is a developmental
infundibulum and drain directly into the ureter. anomaly and patient age at presentation varies depending
Bifid ureters draining a duplex kidney join to form on the type of abnormality. Patients with duplex kidney
a single ureter, which can be extravesical (common; are usually asymptomatic and duplex kidney is detected
Y- shaped) or intravesical (V-shaped) and usually empties incidentally on imaging studies performed for other
into the bladder. reasons, unless complications arise.
Presentation in patients with duplicated collecting
systems varies depending on the type of anomaly. Patients
with duplex kidney are usually asymptomatic.
Ureteropelvic junction obstruction is more common when
duplex kidney exists. Giant hydronephrosis in a duplex
kidney can manifest as a huge abdominal and
FIGURE 4.24: Development of complete ureteral duplication retroperitoneal mass and, rarely, can cause hypertension.
The incidence of vesicoureteric reflux, urinary tract 6. Upper and lower pole ureters: The upper pole ureter
infection, and parenchymal scarring is increased in drains the upper pole of a duplex kidney while the lower
patients with duplicated collecting systems; patients can pole ureter drains the lower pole of a duplex kidney.
present with pyrexia and dysuria. Prolapsed ureterocele
associated with a duplicated ureter can cause urethral PREFERRED INVESTIGATIONS
obstruction in males and females. Patients with ectopic Plain Radiography
insertion of the ureter with completely duplicated ureters
can present with urinary incontinence, particularly in Plain radiography makes no major contribution but a
females. Males are always “dry” since the insertion is renal mass may be apparent because the duplex kidney
proximal to the sphincter. is almost always longer than the nonduplex kidney.
Because of the widespread use of antenatal Hydronephrotic upper or lower pole moiety in a duplicated
ultrasound, duplicated collecting systems are diagnosed collecting system can also be observed as a renal mass.
in utero. Features: The duplex kidney is almost always longer than
An ectopic ureter observed in a patient with complete the nonduplex kidney. In a duplicated collecting system,
ureteral duplication can present in the early stage, hydronephrotic upper or lower pole moiety can be
especially in female patients, since the ectopic ureter may observed as a renal mass.
insert below the sphincter or outside of the urinary tract
(e.g. vagina). Limitations: Plain radiographs can demonstrate a renal
Duplicated ureter complicated by transitional cell mass, which is a nonspecific finding.
carcinoma occurs in the elderly population.
IVU/ Excretory Urography
ANATOMICAL BASIS IVU findings are almost always diagnostic in most patients.
When a single ureteral bud bifurcates before the ampulla Difficulty may arise when function is poor or absent in
bifurcates, a duplex kidney with a bifid renal pelvis or one of the moieties.
bifid ureter results. If two ureteral buds arise from the Features: A duplex kidney is usually longer than the
Wolffian duct, a duplex kidney with complete ureteral nonduplex kidney.
duplication ensues. Parenchymal thickness of one of the poles of the
TERMS RELEVANT TO DUPLEX COLLECTING SYSTEMS duplex kidney is less than the thickness of the other pole.
The calyces are asymmetric.
1. Duplex kidney: The duplex kidney has a single renal
An ectopic upper pole ureteric insertion can cause
parenchyma drained by two pyelocalyceal systems.
a nonopacified segment. This mass effect results in the
2. Upper or lower pole: The poles represent one
“drooping lily” sign with the depression of the lower
component of a duplex kidney.
3. Duplex system: The kidney has two pyelocalyceal pole pelvicaliceal system (Figure 4.25).
systems and is associated with single or bifid ureters If the lower pole of the duplex kidney is poorly
(partial duplication) or two ureters (double ureters) functioning or nonfunctioning, the lower pole collecting
that drain separately into the urinary bladder system may not opacify and no discernible parenchyma
(complete duplication). surrounds it (nubbin sign). This may resemble a non-
4. Bifid system: Two pyelocalyceal systems join at the duplicated kidney with a lower polar mass or renal infarct.
ureteropelvic junction (bifid pelvis) or the 2 ureters Reduction in the number of calyces, depiction of a
join before draining into the urinary bladder (bifid portion of the collecting system, and the presence of a
ureters). straight inferior border help differentiate a duplicate
5. Double ureters: Two ureters open separately into the collecting system from a renal mass.
renal pelvis superiorly and drain separately into the Anomalies of the ureter, such as partial or complete
bladder or genital tract. ureteral duplication, may be demonstrated.
Ultrasound findings provide excellent anatomic

information but do not necessarily differentiate a bifid
renal pelvis from a bifid ureter or two complete ureters.
A large Bertin column can mimic a duplex collecting
system on CT. It is valuable in evaluating an intravesical
ureterocele, either orthotopic or ectopic.
Features: The duplex kidney appears as two central echo
complexes with intervening renal parenchyma.
Hydronephrosis of one pole is suggestive of a duplex
kidney. Although hydronephrosis can occur in either pole,
it is more common in the upper pole. Occasionally, two
distinct collecting systems and ureters can be observed
on ultrasound images.
CT with Contrast
CT with contrast is superior to ultrasound and excretory
urography in diagnosing the nubbin.
FIGURE 4.25: Complete duplication of right PC system Features: The intervening renal parenchyma in a duplex
hydronephrosis of lower pole calyces kidney lacks a collecting system and major vessels and
is termed a faceless kidney. CT can help determine
Limitations: On excretory urography, an obstructed
whether an obstruction exists and can help assess the
nonfunctioning upper or lower pole may mimic a renal
renal parenchyma. CT can help determine whether
mass.
insertion of the duplicated ureter is intravesical or
MCU/VCUG extravesical. CT can demonstrate the collecting system
Ectopic ureter of a nonfunctioning moiety can be in the nubbin or mass effect of tissue at the pole.
demonstrated best using voiding cystourethrogram if CT scanning is superior to ultrasound and IVU in
vesicoureteral reflux exists. diagnosing the lower pole nubbin. CT is helpful when
function is poor or absent.
Features: The intravesical ectopic ureter of a nonvisualized
moiety is demonstrated better using voiding cystourethro- Magnetic Resonance (MR) Urography
gram. Magnetic resonance (MR) urography may be used as a
Antegrade Pyelography primary diagnostic method in assessing a duplicated
ectopic ureter and the complications associated with
Antegrade pyelography is useful in patients with
duplex kidneys. Spatial resolution is a limiting factor.
hydronephrosis to demonstrate the presence of a second
ureter and to determine the level of ureteric termination. Features: MR urography can provide information similar
to that of excretory urography when renal function is poor
Features: Antegrade pyelography: Antegrade pyelography
or absent. An ectopic ureter extending from a poorly
is useful in patients with hydronephrosis for demonstrating
functioning moiety of a duplex kidney invisible on other
the presence of a second ureter and to determine the
imaging may be observed with MR urography.
level of termination.
MR urography may be used as a primary diagnostic
Ultrasound method in assessing a duplicated ectopic ureter and
Ultrasound is a noninvasive and extremely useful complications associated with duplex kidneys.
examination, particularly in children. The sonographic Limitations: Availability of MRI is limited, the procedure
appearance of a duplex kidney is specific but not sensitive. is expensive, and it requires sedation of patients with
claustrophobia. However, MR urography is an extremely ectopia, a hemitrigone is found in the bladder. With
useful technique in patients who have the probability of bilateral single ectopia (very rare), there is no trigone;
an adverse reaction to radiopaque contrast media. the bladder fails to completely develop.
Scintigraphy INCIDENCE
Scintigraphy is useful in the assessment of relative renal Females to males 6:1 ratio.
function and detection of renal scars.
EMBRYOLOGY
Features: Duplex kidneys appear as two separate The ureteral bud fails to separate from the mesonephric
collecting systems on the same side of the body. duct. Its orifice opens in a urogenital sinus or Wolffian
Scintigraphy may demonstrate reflux up the ureter in a duct structures, such as the bladder neck, urethra, seminal
nonfunctioning duplex kidney with ureteral duplication. vesicles, vas deferens, or ejaculatory duct in males, and
Limitations: Scintigraphy can reveal differential bladder neck, vestibule, or urethra in females. Ureteral
functioning. However, if functioning is markedly ectopy into the uterus, vagina or cervix may also be seen.
depressed, imaging is limited. CLINICAL FEATURES
Arteriography In males, urinary infection and obstruction but not
Arteriography is an invasive procedure and is no longer incontinence may be experienced, since the the ureter
used to diagnose duplex collecting systems but may opens above the urogential diaphragm. By contrast,
occasionally be useful in planning nephron-sparing urinary incontinence may be seen in females. In many
surgery. cases, the diagnosis may not be made until adulthood,
especially if incontinence is absent.
Findings: Invariably, two separate arteries arise, mostly
independently from the aorta. Hydronephrosis of a IMAGING
moiety appears as a filling defect displacing arterial Urography will demonstrate upper moiety obstruction due
branches. to a stenotic ectopic ureteral orifice in cases associated
Arteriography is an invasive procedure and is no with duplication. Cystoscopy and retrograde pyelography
longer used to diagnose a duplex collecting system. may be used to confirm the diagnosis if the ectopic orifice
terminates within the urinary tract. Otherwise, ultrasound
MORTALITY/MORBIDITY
or CT may be useful. If a single ureter is present, a
Most cases of duplicated collecting system are detected nonfunctioning dysplastic kidney will be seen on the side
incidentally; however, the incidence of ureteropelvic associated with the ureteral ectopia.
obstruction is increased with duplex kidney, hydronep-
hrosis, and pyelonephritis in patients with complete
URETEROCELE
ureteral duplication.
INTRODUCTION
ECTOPIC URETER A ureterocele is a submucosal cystic dilation of the

terminal segment of the ureter.
INTRODUCTION
A ureter that does not terminate at the trigone of the CLASSIFICATIONS

bladder, which is its normal location. The term is used A ureterocele may be classified most easily as:
to describe a ureter that opens outside the bladder. 1. Intravesical, defined by its presence entirely within
the bladder
ASSOCIATED ANOMALIES 2. Extravesical, defined by the permanent presence of
Eighty percent of ectopic ureters are associated with some portion of the ureterocele at the bladder neck
complete ureteral duplication. With unilateral single or urethra.
Other classification systems for ureteroceles are based functional studies. Vesicoureteral reflux is present in 50%
on the location of insertion of the ureter into the bladder: of ipsilateral lower-pole moieties in duplex-systems and
1. Simple (orthotopic) 10–25% of contralateral moieties in single and duplex
2. Ectopic systems.
3. Cecoureterocele
PATHOPHYSIOLOGY
4. Pseudoureterocele.
According to their morphology, ureteroceles may be: The true etiology of ureteroceles is not known. A common
1. Stenotic (obstructive ureteral orifice within the bladder) etiology is unlikely in simple and ectopic ureteroceles.
2. Sphincteric (ureteral orifice at the bladder neck), or Several postulated theories for the etiology of ureteroceles
3. Sphincteric stenotic (obstructive ureteral orifice at the include the incomplete dissolution of the Chwalla
bladder neck) membrane during development, altered development of
4. In addition, they can also be cecoureteroceles the ureteral bud, inadequate muscularization, Schisto-
(extension of the ureterocele past the bladder neck soma hematobium infection, and trauma.
into the urethra and may cause bladder outlet Histologically, ureteroceles contain abnormal mus-
obstructions). culature, with an incomplete patchy muscular coat.
Electron microscopy shows a paucity of muscle
Ureteroceles are most commonly found in association bundles and smaller myocytes and an absence of thick
with complete ureteral duplication (80%), but they can myofibrils.
also be seen at the terminus of a single system. The
association of a fluid-filled structure within the bladder CLINICAL FEATURES
leading to an ectopic dilated ureter and a hydronephrotic Sex: Females are affected more commonly than males,
upper pole of a kidney is the sine qua non of a with a female-to-male ratio of 6:1. Interestingly, ectopic
ureterocele associated with a duplicated system. The single system ureteroceles are more common in males
function of the upper pole segment is variable depending and associated with additional congenital anomalies.
on the relative degree of obstruction caused by the
Age: Two distinct groups of patients present with
ureterocele.
ureteroceles. Infants and children most commonly (60%)
A ureterocele associated with the upper pole moiety
present with ectopic ureterocele associated with a duplex
is located medially and inferiorly to the lower pole ureter
system. Large ureteroceles are commonly seen in very
inserting in a more superolateral location (Weigert-
young infants. On the other hand, adults are most likely
Meyer rule).
to present with a simple single-system ureterocele.
Aphorism: UU-LR, i.e. upper moiety is associated with Ureteroceles are associated with obstructive uropathy.
ureterocele and obstruction and the lower moiety with Detrusor hypertrophy is often seen if the ureterocele is
reflux, in which case the draining moiety is dysplastic. associated with bladder outlet obstruction. Vesicoureteral
Intravesical single-system ureteroceles are typically reflux is present in 50% of ipsilateral moieties in duplex
associated with good renal function (>80% with excretory systems and 10–25% of contralateral moieties in single
function), whereas extravesical single-system ureteroceles and duplex systems. Renal dysplasia is seen in 40% of
are seen with poor renal function (<30% with excretory resected duplex systems, with hydronephrotic atrophy and
function), dysplastic kidneys, and bladder outlet renal scarring also commonly seen.
obstruction. Ectopic drainage (outside of the trigone) in females
Increasingly, ureteroceles are detected on antenatal may result in incontinence because the ureter drains
sonograms. Initial findings often include hydronephrosis beyond the bladder neck into the urethra, vagina, or
and a fluid-filled structure within the bladder. Antenatal uterus. However, incontinence is not seen in males
findings must be correlated with results of postnatal because the ectopic ureter always inserts proximal to the
studies, including ultrasonography, cystography, and external sphincter.
INCIDENCE of choice for evaluating and grading the vesicoureteral

The incidence varies from 1 case per 500 population to reflux. It also helps in therapeutic decision making.
1 case per 4000 population. A predilection for ureteroceles
is well documented in Caucasians. Ultrasound
ROLE OF RADIOLOGY AND IMAGING Radiologic evaluation of an ureterocele in an adult usually

includes a kidney-bladder ultrasonography. Ultrasono-
Functional studies of the renal system includes:
graphy can be the initial study performed, and it can
1. Intravenous pyelography
be used to detect prenatal ureteroceles. Ultrasonography
2. CT scanning, and
noninvasively depicts anatomic changes in the kidney
3. Renal scanning.
and bladder. Sonography should be performed with the
These contrast-enhanced studies help in delineating
bladder empty and with it filled to eliminate
the relevant renal anatomy, especially with regard to
nonvisualization of ureteroceles due to either compression
potential renal duplications. They are also useful in
evaluating the differential renal function. Contrast- of the bladder or the ureterocele. Ultrasonography is the
enhanced studies help in identifying ureteroceles within most sensitive test and often the only radiologic evaluation
the bladder as a thin nonenhancing rim around contrast required for the diagnosis of ureteroceles.
enhancement within the ureterocele. These functional Limitations of techniques: Ultrasonography is the most
studies often add supplemental information useful in the sensitive test for the detection of ureteroceles.
treatment of patients with ureteroceles, but they are Ultrasonography may cause ureteroceles to be missed
typically not used as first-line imaging modalities for the if the patient’s bladder is empty or fully distended, if the
diagnosis of suspected ureteroceles. ureteroceles are small, or if the patient’s body habitus
CT scans, intravenous pyelograms, and renal scans precludes proper examination. Ultrasonographic findings
are less sensitive for ureteroceles, but they help in more are relatively specific for ureteroceles because the other
clearly delineating the functional anatomy of the kidneys. etiologies of a cyst within a cyst are relatively rare.
Voiding Cystourethrography
Antenatal Ultrasonography
VCUG/MCU should be performed in all newborns with
On antenatal sonograms, the typical presentation is that
fetal hydronephrosis. Early imaging prior to complete
opacification of the bladder with contrast material may of an enlarged hydronephrotic fetal kidney. Renal
show the ureterocele as a filling defect. VCUG defines duplication and/or ureteral duplication may be seen.
the degree of vesicoureteral reflux in both ipsilateral and Intravesical cystic dilation or septa within the bladder
contralateral systems and also possible inferior displace- (which represent ureterocele walls) suggest that the
ment of the lower pole as a large obstructed upper pole. etiology of the hydronephrosis may be an ureterocele.
The drooping lily sign is a classic description of moderate– Such antenatal ultrasonographic findings warrant
to–high-grade reflux into a displaced lower pole. comprehensive postnatal evaluation and confirmation of
In addition, VCUG may be performed to evaluate the diagnosis.
the size, position, tension, degree of detrusor backing, Antenatal ultrasonography is increasingly becoming
and compressibility of ureteroceles. Eversion of uretero- the diagnostic modality for diagnosing prenatal
celes on VCUGs may be seen as protrusions outside the ureteroceles. Findings of a cyst within the bladder and
urethral or vesical wall. hydronephrosis are virtually diagnostic of a ureterocele.
VCUGs are used routinely to visualize the bladder However, any finding on antenatal sonography must be
and evaluate the accompanying reflux. Larger uretero- confirmed with comprehensive postnatal ultrasonogra-
celes are clearly identifiable as areas of cystic lucency phic evaluation. Hydronephrosis may be associated with
within the bladder. VCUG is the diagnostic procedure ureteroceles, but it is not pathognomonic.
Renal/bladder Ultrasonography ureteroceles are often seen as opacities on the scout

The initial study performed for the evaluation of a neonate image. On the other hand, radiolucent stones are often
with fetal hydronephrosis with or without a suspected seen as filling defects within the ureterocele during the
excretory phase of the urogram.
ureterocele should be renal and/or bladder ultrasono-
IVUs are not typically obtained in infants because the
graphy. During every postnatal ultrasonographic
procedure is more invasive and the results are less
evaluation of antenatal hydronephrosis, examining the
informative. The reason is the failure of infant kidneys
bladder is imperative to avoid missing associated
to concentrate the contrast agent. IVUs are more
ureteroceles. The sonographic finding of a well-defined
commonly performed in adults, in whom ureteroceles
cystic intravesical mass within the posterior bladder wall
may be serendipitously depicted.
is suggestive of a ureterocele.
The classic description is that of a cyst within a cyst CT
(see Figures 4.1 to 4.3). Occasionally, the dilated Widespread use of CT for diagnostic imaging has allowed
intramucosal section of the ureter may be visualized as the functional and anatomic identification of ureteroceles
it inserts into the bladder and terminates in the ureterocele in a single study. CT scans without and with intravenous
(see Figures 4.4 to 4.6). Ultrasonography also defines
contrast enhancement can be used to define the size,
the degree of hydronephrosis, and it possibly depicts renal
shape, and location of the ureterocele (see Figures 4.11
dysplasia as cortical thinning. With their distinct echogenic
to 4.14). Ureteroceles may be seen as cobra-head
renal pelves, duplex renal systems may also be identified
deformities (see Figure 4.14). In addition, CT scans enable
on the initial ultrasonographic examination. This finding
identification of duplicated renal systems, the renal and
should exclude any solid periureteric orifice mass in the
ureteral contours, the degree of hydronephrosis, the
bladder, such as a pseudoureterocele.
cortical thickness of each moiety, and the functional ability
Renal and/or bladder ultrasonography is highly
of the kidneys to excrete contrast material, as well as other
sensitive for the detection of ureteroceles and widely used.
anatomic anomalies.
Similar to all other imaging modalities, smaller uretero-
CT scans offer greater anatomic resolution of the entire
celes may be compressed and thus missed on sonograms.
urinary tract, especially in complicated cases. Ureteroceles
can be identified accurately and consistently by using CT
Intravenous Urogram
scans.
The classic finding on an intravenous urogram (IVU) is
a round radiopacity in the bladder surrounded by a MRI
radiolucent rim. It is not usually applicable, but it should be as effective
If function of the affected kidney is poor, the upper as the IVU (as with magnetic resonance urography with
pole system may not be seen. Very few calices may be or without contrast enhancement) and CT scanning.
noted in the lower pole system, with a downward and
RNI
laterally displaced lower-pole moiety (drooping lily
sign). The lower-pole ureter may be displaced laterally Radionuclide renal scans obtained with dimethylsuccinic
or looped around the dilated upper pole ureter near the acid, diethylenetriamine pentaacetic acid, or mercapto-
sacrum. triglycylglycine may be useful in evaluating baseline renal
Defects in the lower urinary tract are more evident function in the ipsilateral and contralateral units. These
in single-system ureteroceles subtending well-functioning scans commonly demonstrate minimal or no function in
kidneys. The contrast agent–filled ureterocele may be the upper-pole moiety of the duplicated system, with
separated from contrast medium in the bladder by a thin ureteral obstruction at the point of the ureterocele. A single
lucent halo of the ureterocele wall (spring-onion or intravesical system is typically associated with good renal
cobra-head deformity). Radiopaque stones within function.
Renal radionuclide scanning is not used to detect bladder outlet obstruction, (3) preservation of urinary
ureteroceles, but it may aid in therapeutic decision continence mechanisms, and (4) protection of renal
making. Evaluation of the function of the affected renal function.
units may help in deciding whether partial or radical In infants with symptomatic ureteroceles, antibiotics
nephrectomy is indicated. should be administered to treat urinary tract infections.
Antibiotic prophylaxis is associated with a low incidence
of urinary tract infections, and it may be used to delay
Pseudoureteroceles, bladder diverticula, and mesonephric surgical intervention until the bladder matures. Small
duct cysts may all resemble the ureterocele as a cystic asymptomatic ureteroceles may be observed with careful
lucency on the radiologic imaging, and they may serial physical and ultrasonographic examinations.
contribute to false-positive findings. A pseudoureterocele Surgical options include endoscopic ureterocele
is so named because it can mimic the appearance of a incision, and, depending on renal function, percutaneous
ureterocele on VCUGs or IVUs. A pseudoureterocele can diversion, ureteropyelostomy, partial or total nephroure-
be caused by etiologies such as edema due to impaction terectomy, or complete reconstruction.
or passage of calculi, radiation cystitis, and transitional In the endoscopic approach, a small endoscopic
cell carcinoma. Because ureteroceles are compressible, incision is made inferiorly and medially on the anterior
they can be missed when the patient’s bladder is full. wall of the ureterocele above its base at the bladder neck.
Small (<1 cm) ureteroceles may also be missed. This minimally invasive method is associated with low
With IVU, bladder diverticula and mesonephric duct morbidity rates and represents an effective method of
cysts may all resemble ureteroceles as a cystic lucency decompression in infants. The endoscopic approach is
on the radiologic imaging, and they may contribute to highly successful for small, single-system intravesical
false-positives findings. Pseudoureteroceles can either ureteroceles. With this procedure, the reported incidence
mimic an obstructed ureterocele (lucent filling defect) or of iatrogenic reflux and incontinence (<10%) is low, and
a non-obstructed ureterocele (lucent halo surrounding the secondary procedures are often not needed (10–15%).
The endoscopic approach represents a good first-line
ureteral orifice), depending on the size of the abnormality
method for the acute management of symptomatic
and degree of the obstruction. Rarely, a focal bladder
ureteroceles.
tumor can cause a similar appearance. Because
Radiologic interventions for ureteroceles are
ureteroceles may change the size and shape, depending
primarily temporizing maneuvers rather than definitive
on the degree of filling, they may not be visible in all
procedures. Percutaneous nephrostomy drainage allows
phases of the urogram. Finally, because the IVU depends
decompression of a dilated renal pelvis prior to definitive
on kidney function for optimal visualization, the use of
treatment of the ureterocele.
IVU for diagnosing ureteroceles may be compromised.
MORTALITY/MORBIDITY
INTERVENTION The morbidity of ureteroceles may be largely attributed
Each patient with a ureterocele is clinically unique to their ability to cause obstructive uropathy. The primary
regarding anatomy, pathophysiology, and renal function. obstruction is at the level of the ureterovesical junction.
As such, treatment options must be individualized. Ipsilateral (50%) and contralateral (25%) vesicoureteral
Treatment often involves surgical intervention. Prior to reflux is common. As a consequence, urinary tract infec-
surgical intervention, the anatomy of the urinary tract tions are a common finding in infants with ureteroceles.
must be delineated as clearly as possible. Ipsilateral and Pyelonephritis and frank pustulant urosepsis may be
contralateral renal function must be assessed, and present. Hydronephrosis is a common finding in both
treatment should be initiated expeditiously. The 4 goals ipsilateral and contralateral renal units.
of intervention include (1) control and elimination of Untreated, ureteroceles may progress to renal scarring,
infection, (2) minimization of vesicoureteral reflux and renal failure if both kidneys are affected with the sub-
sequent need for dialysis and kidney allograft via the ureters. Although sterile VUR may cause renal
transplantation. scarring, most studies indicate that the appearance of
Prolapsed ureteroceles also may cause a functional renal scarring or the extension of established renal scars
bladder outlet obstruction. requires infection (Escherichia coli is the common
pathogen). The higher the grade of reflux, the greater
OTHER BLADDER ABNORMALITIES CAUSING the likelihood of development of new or progressive
URETERIC OBSTRUCTION
scarring in association with infection.
1. Cystitis Intrarenal reflux, or the extension of VUR into the
2. Hemorrhagic, bullous, eosinophilic, radiation, collecting tubules of the nephrons that allows urinary
tuberculous, schistosomiasis, lupus, Wegener’s, microorganisms access to the renal parenchyma, is
small fibrotic bladder believed to be particularly important in the development
3. TCC, SCC, rhabdomyosarcoma, mesodermal of renal scarring.
tumors, invasion or metastasis from gynecological Radiologic evidence of renal scarring is noted in
cancer, prostatic cancer 30–60% of children with VUR, and VUR is present in
4. BPH almost all children with severe renal scarring. A direct
5. Neuropathic bladder correlation between the prevalence of scarring and the
6. Congenital or secondary to outlet obstruction grade of VUR has been demonstrated.
Following ureteric catheterization, transurethral or Although VUR occurs at a similar rate in boys and
open procedures on bladder, including fulguration girls, girls are at greater risk of developing reflux nephro-
7. Foley balloon pathy because of increased incidence of urinary tract
8. Tumor infection (UTI). In the absence of reflux and UTI, abnor-
9. Muscular hypertrophy, bladder diverticulum malities of organ systems other than the genitourinary
Iatrogenic trauma tract do not place a child at increased risk for developing
10. Miscellaneous. reflux nephropathy.
VESICOURETERAL REFLUX (VUR) INCIDENCE

INTRODUCTION The incidence of renal scarring in children resulting from
Hodson initially noted the association of vesicoureteral VUR is unknown. The prevalence of VUR in asympto-
reflux (VUR) with renal parenchymal scarring. matic children is less than 0.5%, but VUR is present in
Normally the ureter enters the superolateral angles 29–50% of children with UTIs.
of the trigone, penetrating the bladder wall at an oblique
angle, giving rise to an oval ureteric orifice. ANATOMICAL BASIS
This acts as a valve preventing urine refluxing from Most VUR is considered primary because of incompetence
the bladder into the ureter during micturation, the rise of the ureterovesical junction (UVJ), and it is not
in intravesical pressure tending to close the orifice. secondary to either obstruction or infection. As the UVJ
In some children the ureter takes more direct course matures to assume its adult 1.5 cm oblique path through
in the bladder wall leading to a short intramural portion the bladder wall, VUR tends to decrease in severity and
and a round ureteric orifice, which predisposes to reflux. eventually disappears just before puberty. An exception
Renal cortical scarring and dilatation of calyces may is in patients with an anatomic abnormality, such as a
eventually result if untreated. bladder diverticulum, into which the refluxing ureter
enters. Incompetence of the vesicoureteral junction
PATHOPHYSIOLOGY resulting from a lack of maturation is the most common
Reflux nephropathy is based on persistent reflux of sterile cause of vesicoureteral reflux in children. Obstruction and
or infected urine from the bladder to one or both kidneys infection rarely cause reflux. With maturation of the
incompetent vesicoureteral junction, reflux usually

eventually resolves.
A small subgroup of children who have a
perpendicular ureteric insertion at the bladder wall with
a resultant golf-hole ureteric orifice. In these patients,
surgical correction is necessary to eliminate VUR. A
decision to perform a repair, either a ureteroneocysto-
stomy or a collagen periurethral injection is dependent
upon the appearance of the ureteric orifice. Nevertheless,
all patient are covered with appropriate antibiotics to await
the maturation of the intramural portion of the ureter
or undergo surgery to stop the reflux.
FIGURE 4.26: Grades of VUR (For color version see Plate 3)
CLINICAL FEATURES
Sex: Reflux occurs at a similar rate in boys and girls; anomalies, masses, calcification, hydronephrosis, and size
however, the much higher incidence of UTI in girls places and can identify renal scarring if moderate to severe in
them at greater risk for reflux nephropathy. degree. VCUG helps in evaluating for vesicoureteric reflux
and the anatomy of the lower urinary tract (bladder and
Age: In children, the occurrence of VUR decreases with
increasing age. The vesicoureteral insertion matures just urethra).
prior to puberty. The mature bladder wall intramural
VCUG
portion of the ureter attains its 1.5 cm length.
Children with reflux nephropathy may be asymp- Plain images offer no diagnostic information regarding
tomatic, they may present with nonspecific symptoms VUR and reflux nephropathy, although they can be used
(e.g. failure to thrive, fever, poor food intake), they may to evaluate spinal anomalies and potential urinary tract
be acutely ill in association with acute pyelonephritis, or calculi, which rarely occur in this population. VCUG is
they may present with renal failure with advanced renal essential in evaluating lower urinary tract anatomy and
scarring associated with reflux nephropathy. in identifying or excluding VUR.
With voiding cystourethrography, contrast material is
GRADES OF VUR
instilled in the urinary bladder via transurethral catheter
Vesicoureteric reflux is graded on a scale of 0–5. and demonstrates increased opacity in the ureters and
Grade 0 indicates no reflux; pelvicaliceal systems when VUR is present. VUR grading
Grade 1 indicates reflux into the ureter but not reaching (grades 0–5) is based on the distribution and degree of
the renal pelvis; grade 2 indicates reflux into the renal associated upper urinary tract dilatation demonstrated
pelvis and calices but without any dilatation; and on VCUG.
Grades 3, 4, and 5 indicate reflux to the renal pelvis and Intravenous Urography (IVU)
calices with mild, moderate, and severe dilatation,
respectively (Figure 4.26). It can demonstrate changes reflecting reflux nephropathy
and renal scarring, including the following:
ROLE OF RADIOLOGY AND IMAGING 1. Dilatation and deformity of one or more renal calyces
Renal ultrasonography (RUS) and voiding cystourethro- 2. Loss of parenchymal thickness of a region of the
graphy (VCUG) are the preferred radiologic methods. kidney (especially upper and lower poles)
The two procedures provide complementary information. 3. Focal contour indentation typically adjacent to a
RUS helps in evaluating the upper urinary tract (kidneys calyx, representing renal scarring (Figures 4.27A and
and proximal portion of the collecting system) for 4.27B).
FIGURE 4.27B: USG pelvis: Dilated left lower

ureter due to VUR
FIGURE 4.27A: MCU: Bilateral highgrade VUR
With VCUG, rate of detecting VUR is high but

detecting chronic pyelonephritic scarring rate is low. With
IVU, sensitivity is moderate for detecting chronic
pyelonephritic scarring.
Ultrasonography
Findings: Alteration of renal contour and reduced
thickness to the renal parenchyma are the primary findings
of reflux nephropathy. Ultrasonography is excellent for
demonstrating upper urinary tract dilatation.
Echo-enhanced cystosonography performed by using
ultrasonographic contrast media is a new diagnostic
procedure with a major attribute in the exclusion of
ionizing radiation. Echo-enhanced cystosonography, in
limited experience, is reported to be as accurate as
radiographic VCU in the identification of reflux
(Fig. 4.27B).
CT scanning is not indicated in the evaluation of FIGURE 4.28: MRU: Bilateral high grade VUR
reflux nephropathy because of radiation dose, the need
for intravenously administered contrast material, and the RNI
potential need for patient sedation. However, CT scans 99m
Tc dimercaptosuccinic acid and 99mTc glucoheptonate
can demonstrate renal scarring and hydronephrosis but are scintigraphic agents with a high affinity for the renal
not VUR. cortex because they are concentrated by renal tubular
MRI is not routinely indicated in the evaluation of cells. As a result, they produce a relatively high level of
reflux nephropathy because of cost of the procedure and renal detail. Concern for acute pyelonephritis, the
the potential need for patient sedation. However, MRI assessment of renal cortical scarring, and the identification
can demonstrate renal scarring and hydronephrosis but of a malpositioned kidney are among the indications for
not VUR (Figures 4.28 and 4.29). cortical scintigraphy. The use of single-photon emission
Imaging with renal scanning may require sedation in

younger children. Sedation is increasingly requested,
although rarely necessary, for children undergoing VCUG.
MORTALITY/MORBIDITY
The incidence of renal scarring in children caused by VUR
is unknown. VUR is present in 29–50% of children with
UTIs.
MEGAPOLYCALICOSIS AND PRIMARY

MEGAURETER MEGAPOLYCALICOSIS
(OR CONGENITAL MEGACALYCES)
It is a non-obstructive enlargement of the calyces due
to underdevelopment of the medullary pyramids. This
entity is important because it can be confused with
obstructive or refluxing hydronephrosis. Distinguishing
FIGURE 4.29: MRU: Bilateral primary megaureter characteristics include preservation of the renal cortex
and the size and morphology of the calyces. There is
computed tomography increases sensitivity of the agents increase in the number of calyces. Moreover, these calyces
to changes of renal scarring and acute pyelonephritis. are polygonal in shape and faceted in appearance. The
Reduced accumulation of a cortical imaging isotope calyces have no definable papillary impressions on
within the renal margins is an indication of renal scarring. excretory urography. There is a prompt nephrographic
Radionuclide cystogram provides an alternative to VCUG phase but opacification of the pelvis and ureter may be
in identifying renal scarring. Cystograms have the primary delayed because the volume of contrast required to fill
disadvantage of poor anatomic resolution but the the calices is large.
advantage of a lower radiation dose. It is caused by underdevelopment of the papillae. The
Degree of confidence is high with renal cortical calyces are uniformly dilated with the medullary tip having
imaging performed by using technetium 99mTc dimercap- a semilunar shape which caps rather than projects as a
tosuccinic acid. cone into the calyx. Consequently, the calyces have a
Patient motion results in inaccurate imaging depiction polygonal shape and have a mosaic or faceted appea-
of isotope distribution and greater probability of rance. It is usually unilateral.
underestimating renal scarring (false-negative findings).
Hydronephrosis, anomalies such as ectopic and/or fused PATHOGENESIS
kidneys, and renal masses or cysts may result in the overcall Faulty ureteral bud division, primary hypoplasia of
of severity of renal scarring (false-positive findings). glomeruli in the juxtamedullary cortex, functional
infundibular achalasia due to maldevelopment of smooth
Angiography
muscle fibers of the pelvocalyceal junction, and transient
It does not play a role in the evaluation of reflux collection system obstruction in utero caused by involuting
nephropathy. folds in the proximal portion of the fetal ureter.
LIMITATIONS OF ALL MODALITIES IN GENERAL ROLE OF RADIOLOGY AND IMAGING
Renal scarring is detected and identified better by using Diagnosis should be considered only in patients without
nuclear medicine renal scanning with a cortical imaging prior or concurrent obstruction or vesicoureteral reflux.
agent (eg, 99mTc dimercaptosuccinic acid) than with Renal function is normal and this differentiates congenital
ultrasonography. megacalyces from postobstructive atrophy (Figure 4.30).
atrophy from vesicoureteral reflux by demostrating normal

renal function without permanent loss of nephrons.
Radionuclide tracers like I-131-iodohippurate sodium or
99m
Tc-DTPA that measure excretion show marked
prolongation of the excretory phase with a normal diuresis
phase excluding obstruction.
PRIMARY MEGAURETER
It is a congenital abnormality resulting from an aperistaltic
juxtavesical segment of ureter. Ureteral peristaltic
contractions are focally uninhibited secondary to faulty
development of the surrounding periureteral musculature.
It is akin to achalasia of the esophagus and it represents
the second most common cause of hydronephrosis in
the fetus and newborn. Most patients are asymptomatic.
The left ureter is affected three times more often than
the right and the finding is bilateral in 15–40% of cases.
Urine transit is delayed above the affected segment,
FIGURE 4.30: IVU: Congenital megacalyces on right side
typically at the distal third of the ureter, resulting in massive
dilatation of some or all of the more proximal ureter.
Clinically, stasis of urine in the enlarged calyces Reflux of urine does not occur. The calyces retain their
predisposes the patient to infection and stone formation. normal appearance which distinguishes this from ureteral
During excretory urography, maximum opacification obstruction. Other key radiographic findings include
of the pelvocalyceal system is delayed in the affected smooth, tapered narrowing of the ureter, no reflux and
kidney(s) because of the dilutional effect of the large no stenosis. Nonpropulsive to-and-fro motion in the
volume of urine in the enlarged, but otherwise normal, dilated segments may be seen (Figure 4.31).
infundibula and renal pelvis. Other features include
normal to increased number of calyces, normal to
increased kidney length, and decreased parenchymal
thickness due to expanded calyceal volume, thinning of
the medulla and shallow or absent papillary tips.
Congenital megacalyces is not progressive. Stones and
infection complicate the urographic appearance.
Ultrasound and CT findings of dilated calyces and
decreased renal parenchyma thickness do not help diffe-
rentiate congenital megacalyces from other nonobstruc-
tive causes of a dilated collecting system. Angiographic
nephrogram confirms that the reduction in parenchymal
thickness is due only to a medullary deformity and that
the cortex retains its normal thickness of 0.5 to 0.8 mm.
MR also demostrates the normal thickness of the cortex.
Quantitative renal radionuclide studies with 99mTc-
FIGURE 4.31: IVU: Bilateral congenital megaureters seen as dilated
DTPA or -DMSA differentiate nonobstructive congenital lower ureters without dilatation of pelvicalyceal system or proximal and
megacalyces from postobstructive atrophy or pressure mid parts ureters
Potential Complications CLINICAL FEATURES
Recurrent urinary tract infections Bladder Exstrophy is more likely to occur in males than
Urolithiasis females by approximately 2 to 1. The risk of having a
2nd child with bladder exstrophy is about 1 in 100 and
Differential Diagnosis 1 in 70 if one of the parents has bladder exstrophy.
Chronic ureteral obstruction. This misdiagnosis may occur
if megacalicosis co-exists with primary megaureter.
On plain films 60% of cases show separated symphysis
with extrophy-epispadic anomaly. 25% are associated
BLADDER EXSTROPHY
with epispadic anomalies alone. Radiologic evaluation
INTRODUCTION
is usually done postoperatively to exclude adencarcinoma
It is a congenital birth defect that is the malformation at the anastomosis site years after ureterosigmoidostomy.
of the bladder and urethra, in which the bladder is turned Ultrasound, CT, nuclear cystography and IVU are helpful
“inside out”. tools.
Bladder exstrophy results from a deficiency in develop-
ment of the lower abdominal wall musculature leaving ASSOCIATED ANOMALIES
the bladder open and its mucosa continuous with the Skeletal: Diastasis of the symphysis pubis correlates well
skin. This condition is associated with epispadias in which with the severity of the extrophy-epispadia complex. Over
the urethra is open dorsally and urethral mucosa covers 60% of diastases are associated with this complex and
the dorsum of a short penis. approximately 25% with epispadias alone.
The bladder does not form into its normal round shape
Gastrointestinal: Umbilical and inguinal hernias.
but instead is flattened and exposed outside the body.
The lower portion of the bladder, a funnel-shaped bladder Urinary: Ureteric obstruction; unilateral or bilateral
neck, made up of muscles that open and close the bladder, pelvicaliectasis due to ureterocele formation or fibrosis
fails to form correctly. The urethra and genitalia are not at the ureterovesical junction.
formed completely (epispadias) and the anus and vagina
appear anteriorly displaced. Additionally, the pelvic bones MANAGEMENT
are widely separated (diastasis). Rarely a vesicointestinal Ureteral diversion, bladder augmentation, and skin
fistual or prolapse (cloacal exstrophy) is seen in these grafting. Radiological assessment/monitory: mandatory
patients. The anus is ventral in position and may in the postoperative period, because adenocarcinoma
occasionally be imperforate. The genitalia are consistently may develop at the anastomosis years after uretero-
involved. Rectal prolapse can occur due to poor pelvic sigmoidostomy. This complication is not uncommon.
musculature.
CONGENITAL BLADDER DIVERTICULUM
PATHOPHYSIOLOGY
If a congenital diverticula (hutch diverticula), the bladder
It is unknown what causes Bladder Exstrophy. The outlet obstruction is usually secondary to a congenital
problem occurs somewhere between 4–10 weeks of urethral valve or urethral stricture. The congenital variety
pregnancy when various organs, tissues and muscles of diverticula are typically seen in boys. These diverticula
begin to form layers that separate, divide and fold. are characteristically located at the ureteral insertions.
Bladder Exstrophy is a Surgically Correctable Birth Defect. Often, vesicoureteral reflux is resultant. Bladder diverticula
most often occur as a result of outlet obstruction.
INCIDENCE Occasionally, a congenital weakness in the bladder wall
Bladder Exstrophy is noted in 1 of 30,000 to 50,000 live adjacent to the ureteral orifice results in a diverticulum.
births. This is termed a “Hutch” diverticulum.
In children, outlet obstruction causing a diverticulum umbilical ligament can lead to urachal abnormalities.
is rare and can be seen with urethral valves. In men, These may include, in addition to a patent urachus, a
diverticula are associated with outlet obstruction from urachal cyst resulting from failure of closure of the urachus
urethral stricture, prostatic hypertrophy, prostatic at the bladder attachment, or an umbilical cyst resulting
carcinoma etc. acquired diverticula are rare in women. from failure of closure at the umbilical attachment.
The urachus develops embryologically from the allantois
BLADDER DUPLICATION in the 5th gestational month. There is ventral
Is rare and may be complete or incomplete. communication from the umbilicus to the cloaca, from
Complete duplication demonstrates two bladders lying which arises the urinary bladder. There is usually complete
side by side, separated by a peritoneal fold. Each bladder regression of this communication by mid-fetal life; the
has normal musculature and mucosa and receives the structure persists in post-natal life as the median umbilical
ipsilateral ureter. Each also has separate urethral orifices ligament. There are four recognized types of urachal
that may drain into a common urethra and single penis, remant conditions which result from complete or partial
or there may also be complete duplication of the urethra failure of urachal obliteration. All but one type are typically
and penis. Lower GI anomalies are commonly associated. asymptomatic, except when exacerbated by infection or
Complete duplication may result from bifurcation of the tumor.
cloacal septum resulting in two bladders and urogenital INCIDENCE
sinuses.
Male:female ratio of patent urachus is 3:1.
Incomplete duplication is less common. A bladder
septum may divide the bladder into two equal or unequal CLINICAL FEATURES/COMPLICATIONS
chambers. Each chamber has a ureteric orifice but the
Clinical presentation of urachal cysts is usually during
septum may be eccentrically located leading to ureteric
adulthood because they are clinical silent until
obstruction, and aplastic or hypoplastic kidney. There is
infection supervenes. Calculi may form within urachal
a common urethral orifice and the external genitalia are
cysts and will be seen on plain films as small punctate
not duplicated. The bladder septum may be multiple,
calcifications above the bladder outline. An increased
dividing the bladder into several compartments.
incidence of adenocarcinoma arising within an urachus
Agenesis of the bladder is incompatible with life as
it leads to death from obstruction and renal failure. has been reported.
However, a few cases have been reported in children
VARIETIES OF ABNORMALITIES
(all female) who lived long enough to be diagnosed.
Patent Urachus
URACHAL ABNORMALITIES The urachus may rarely remain completely or partially
INTRODUCTION patent, allowing for a fistulous tract between the bladder
These run the spectrum from a patent urachus and and the umbilicus (patent urachus).
umbilical ligament in which urine can flow from the
bladder to the abdominal exterior via the umbilicus, to Urachal Sinus
urachal cyst and umbilical cyst. For a blind ending tubular structure extending from the
ANATOMY/EMBRYOLOGY umbilicus inferiorly (umbilical-urachal sinus).
As the bladder descends into the pelvis, its dome narrows
Urachal Cyst
to form the urachus. Normally, this umbilical attachment
of the bladder becomes obliterated to form the umbilical A non-communicating fluid collection along the urachus
ligament. Anomalous regression of normal closure of the (urachal cyst).
FIGURE 4.32: IVU: Urachal FIGURE 4.33: Cystogram: Delineation of endometrial FIGURE 4.34: IVU: Prune Belly
diverticulum cavity posterior to bladder–uterovesical fistula syndrome
Urachal Diverticulum the vast majorities of malignancies are adenocarcinoma,

typically at the level of the bladder, and represent greater
For an outpouching along the superior aspect of the
than 40% of all bladder adenocarcinoma. Urachal
bladder (vesicourachal diverticulum).
carcinoma is typically not differentiable from an infected
The most common (50%) urachal remant abnormality
remnant, and differentiation from a primary bladder
is patent urachus. This is a pure congenital abnormality
dome neoplasm typically relies on demonstration of
that results in persistent vesico-urachal fistula. The next
tumor extending towards the umbilicus, out of the
most common type is the urachal cyst (30%). This results
perivesical region. There is an approximately 30%
in a preperitoneal cyst just posterior to the midline rectus recurrence rate after surgical resection, which is the
muscles, with normal regression of the umbilical and treatment of choice.
vesical urachal tract. Next is the umbilical-urachal sinus
(15%), a persistence of the proximal urachus for few to ROLE OF RADIOLOGY AND IMAGING
several centimeters distal from the umbilicus. Last is the CT, ultrasound, and fluoroscopy are well-suited for
vesico-urachal diverticulum (5%), representing persistence demonstrated diseases of urachal remnants.
of the distal urachal-vesical communication (Figure 4.32). Cystography will be sufficient to make the diagnosis,
Complications of these anomalies include especially relying on oblique views.
development of infection, stones, and carcinomas, most
commonly adenocarcinomas. CONGENITAL UTEROVESICAL FISTULA
Urachal remnants tend to become infected, and have See Figure 4.33.
a propensity to undergo malignant degeneration,
representing about 0.5% of all bladder carcinomas PRUNE BELLY SYNDROME
(all histology). Although lined by transitional epithelium, See Figure 4.34 and refer to Chapter 18 for discussion.
Cystic Disease of Kidney 71
INTRODUCTION 2. Autosomal recessive

No single classification of cystic kidney disease is entirely • Autosomal recessive polycystic kidney disease
satisfactory but the following, based on genetic/nongenetic ARPKD)
disorders, is still the most widely acceptable. • Juvenile nephronophthisis.
Cystic disease of kidney can be broadly divided into: 3. Cysts associated with syndromes
1. Nongenetic cystic disease • Chromosomal disorders
2. Genetic cystic disease. • Autosomal recessive syndromes
• X-linked syndromes.
NONGENETIC CYSTIC DISEASES
PATHOPHYSIOLOGY
1. Cortical renal cysts
2. Cystic dysplasia or dysplasia. (Multicystic dysplastic Should divide the kidneys into unilateral or bilateral renal
kidney) disease; diffuse or localized cysts; size of the kidneys;
3. Multilocular cyst extrarenal manifestations, e.g. cysts in the liver or
4. Multilocular cystic Wilms’ tumor pancreas; evidence of portal hypertension, intracranial
5. Localized cystic disease of the kidney or cardiac abnormalities.
6. Parapelvic cyst The genetic disorders are often systemic and there
7. Calyceal cyst/diverticulum may be more than one abnormal chromosome causing
8. Medullary sponge kidney the disorder, e.g. autosomal dominant polycystic kidney
9. Acquired cystic kidney disease (in chronic renal failure). disease (ADPKD). Not all present in the newborn period.
The nongenetic conditions include both congenital and
GENETIC CYSTIC DISEASES acquired disorders.
1. Autosomal dominant The first imaging procedure should be a full US
• Autosomal dominant polycystic kidney disease examination, which remains the comer stone of imaging.
(ADPKD) However, in this difficult area there is an on-going need
• Tuberous sclerosis to integrate other renal imaging modalities such as IVU
• Medullary cystic disease and/or 99mTc-DMSA when certain differential diagnoses
• Glomerulocystic disease. are being considered. Other system imaging is usually
also required, either to look for associated abnormalities, Role of Radiology and Imaging
e.g. brain scan for intracranial tubers, or for complications Plain abdominal radiograph: Occasionally, one may
of the disease. see a cortical bulge projecting into the perinephric fat.
US will immediately identify if the abnormal kidney Calcification in a cyst wall is seen in only 1% of cases.
anatomy, that diseases is unilateral or bilateral,
Urography: A lucent mass may be seen within the renal
symmetrical or asymmetrical. The size of the kidneys and
parenchyma. A cyst will be well defined with a sharp
the presence or absence of dilatation of the collecting
interface with the surrounding normal renal cortex. It will
system can be easily ascertained. Heavy reliance is placed
not enhance, nor will it distort the adjacent parenchyma.
on the ultrasound examination in reaching a specific
However, a “beak” or “claw” sign may be seen if the
diagnosis and it also has a role in directing further
cyst extends beyond the surface of the kidney, and
appropriate imaging.
represents the adjacent stretched parenchyma. If the cyst
is completely intrarenal, the thickness of its wall cannot
NONGENETIC ‘CYSTIC KIDNEY’ DISEASE
be assessed. Radiographs taken 1–2 minutes after IV
The terminology used is often imprecise and inaccurate contrast injection optimally visualize a cyst.
and one should be sure that colleagues are discussing
the same condition with the different terms. USG: A simple cyst is a rounded homogenous mass
demonstrating a sharp interface with the surrounding
CORTICAL RENAL CYSTS parenchyma. It is echolucent and shows enhanced
Simple renal cysts occur in 50% of the population older through transmission.
than 50 years. They are considered to be acquired lesions Ultrasound represents the most cost efficient modality
that probably arise from obstructed ducts or tubules. A to confirm the presence of a simple cyst. When all the criteria
simple cortical cyst represents the most common renal for a benign simple cyst are present by any modality, further
mass lesion. evaluation is not indicated (however, cysts are usually not
visualized well enough by IVP to make this determination).
Incidence Since CT is the gold standard for evaluation of renal masses,
Uncommon in children or young adults, but is seen by it should be used to evaluate cysts when US has been
IVP in upto 20% of men with prostatic enlargement. A indeterminate or technically unsatisfactory due to overlying
cyst in a child must be carefully differentiated from a cystic bowel gas or the presence of obesity. Also, CT should be
Wilms’ tumor. the modality of choice if a urogram indicates that a mass
is complex or likely to be solid.
Pathology
CT: The density of cyst fluid can be measured by this
The cyst wall is composed of fibrous tissue and is lined
modality; it should be near than of water. A value greater
by flattened cuboidal epithelium. The cyst contains serous
than 15 Hounsfield units (HU) should raise suspicions
fluid and does not communicate with the collecting
for a complicated cyst or even a solid mass. A cyst should
system.
not enhance following contrast injection, although small
Clinical Features increases of 2–5 HU may normally be seen with contrast.
When evaluating wall thickness on CT, it is best to examine
Most cysts are asymptomatic and are detected as
a portion of the cyst that extends beyond the renal contour
incidental findings. Local pain may occur due to cyst wall
and does not lie adjacent to the “beak”.
distention or spontaneous intracystic bleeding.
Occasionally, hematuria may occur and rarely, a large MRI: A cyst will be low signal intensity on T1, and very
cyst may obstruct the collecting system or cause high signal intensity on T2 weighted images. As on CT,
hypertension. it will appear as a homogenous rounded mass with a
thin wall and sharp interface with the surrounding normal in a simple cyst. Bosniak has suggested the ~categoriza-
renal parenchyma. tion of cystic renal masses according to CT criteria in
an attempt to separate lesion~ that require surgery from
Angiography: It is not routinely used to evaluate a cyst.
those that do not. The application of Bosniak’s classifi-
On an angiogram, a cyst will be seen as an avascular
cation is most useful in evaluating and managing cystic
mass displacing adjacent blood vessels.
renal lesions.
Complications Category I Lesions.
Spontaneous cyst rupture into the collecting system or In Bosniak’s classification, category I lesions are classic
perinephric space may occur. This occurs due to buildup simple cysts.
of pressure within the cyst as a result of either intracystic Obviously they require no further evaluation or
hemorrhage or a change in composition of the cyst fluid management (Figure 5.2).
(Figure 5.1).
Category II Lesions.
Following rupture, the cyst may regress or disappear
Category II lesions are minimally complicated cysts that
completely. The patient may experience hematuria and
also usually, do not require surgery.
flank pain. Rupture is detected by IVP or retrograde
Minimally calcified benign cyst; category II lesion. A
pyelography if it leads to communication with the
contrast-enhanced CT scan reveals fine linear calcification
collecting system, in which case the cyst cavity may be in the cyst wall and septa. The lesion shows a thin wall,
opacified. Management of a ruptured cyst is conservative. and no focal nodules or areas of enhancement. It should
In most instances, the communication with the collecting be monitored with serial imaging, provided that the follow-
system closes spontaneously. ing CT criteria are fulfilled:
1. The lesion must be perfectly smooth, round, sharply
Bosniak Classification of Cortical Renal Cysts
marginated, and homogeneous;
Many renal cystic lesions do not fulfill the criteria for simple 2. The lesion should not enhance with intravenous
cysts listed previously. Such lesions vary from minimally contrast medium;
complicated simple cysts that usually do not require 3. At least one fourth of the lesion’s circumference should
surgery to cystic renal neoplasms that generally need extend outside the kidney so that the smoothness of
resection. Complicated cysts often result from bleeding some of the wall can be evaluated; and
FIGURE 5.1: CECT: Small exophytic renal cortical cyst with FIGURE 5.2: CECT: Category I simple exophytic renal cortical cyst
intracystic hemorrhage
4. The lesion should be less than 3 cm in diameter. that show internal echoes on ultrasonography. Hemor-
Although most lesions meeting these criteria are rhagic cysts appear on MRI as sharply marginated,
benign hemorrhagic cysts, cystic renal cell carcinoma smooth, round, homogeneous lesions. They have high
may rarely show similar characteristics on CT (Figures signal intensity on both Tl and T2 weighted sequences,
5.3 and 5.4). and they do not enhance after intravenous administration
MRI may also help in characterizing high-density renal of gadolinium DTPA. In addition, hemorrhagic cysts often
cysts that do not fulfill all the preceding CT criteria or show fluid-iron levels on MRI, probably because of
dependent settling of methemoglobin containing
sediment.
Category III Lesions
Lesions in category III are more complicated cystic lesions.
They exhibit some findings seen in malignant lesions,
including thick, irregular mural or septal calcification,
numerous or thick (> 1 mm), irregular septa, and uniform
or slightly nodular wall thickening. Most of the category
III lesions are benign (e.g. Multilocular Cystic Nephromas
and hemorrhagic renal cysts); others are cystic renal cell
carcinomas. All such lesions should undergo surgical
exploration, unless the procedure is contraindicated
because of the patient’s advanced age or poor general
medical condition (Figure 5.5).
Category IV Lesions.
Category IV lesions are clearly malignant lesions with large
cystic components; they should be resected. They may
FIGURE 5.3: CECT: Category II renal cortical cysts show marginal irregularity or solid vascular elements
(See Figure 5.2).
Summary
FIGURE 5.5: CECT: Multiseptate complex left renal cyst with

FIGURE 5.4: CECT: Category enhancing indistinct, irregular wall–category III Bosniak lesions
Briefly, the Bosniak classification of renal masses is as accepted as the correct term to describe these renal
follows: class I includes simple cysts; class II, minimally changes in children. The ultrasound appearances of renal
complicated but overwhelmingly benign masses with thin tubular ectasia without calculi are simply an increased
septa, hyperattenuation, or small amounts of mural or echogenicity of the medullae in normal kidneys of normal
septal calcification; class III, moderately complicated size. Periportal fibrosis is generally associated with ARPKD
masses with mural nodularity, thick septa, or irregular but some rare cases have been described in association
or thick calcifications that often require surgical with ADPKD.
exploration; and class IV, significantly complicated and
generally malignant masses with thick and irregular CALYCEAL DIVERTICULUM
enhancing regions and definite solid components. Introduction
DYSPLASIA/CYSTIC DYSPLASIA/ MULTICYSTIC Calyceal diverticulum, also known as pylogenic cysts,
DYSPLASTIC KIDNEY (MCDK) is typically a symptomatic and incidentally found on IVUs.
There is confusion over this condition because the terms They are found in both males and females of all ages.
used by many nephrologists, radiologists, pathologists and Calyceal diverticulums are composed of uroepithelium
urologists have different meanings. From a purist’s lined pouches that extend from the collecting system into
viewpoint, dysplasia is a histological diagnosis based on the renal parenchyma.
abnormal metanephric differentiation, with persistence Calyceal diverticula, the result of anomalous budding
of fetal kidney tissue in the form of nests of metaplastic of the calyceal system, is also associated with stone
cartilage associated with primitive ducts. However, the disease. In 10–40% of calyceal diverticula, stones are
pediatric nephrologist or radiologist may use the term present. These range from a few large calculi to many
as a generic description for a small, highly reflective, tiny seed calculi and to the microscopic “milk of calcium”.
poorly functioning kidney without establishing histology
by an invasive renal biopsy. From a management Types
perspective, all these kidneys function to some degree They can be of three types:
even though function may be very poor in childhood. 1. Type I originates from the minor calyx
Pathologically, renal cystic disease and renal dysplasia 2. Type II originates from the calyceal infindibulum and
are two distinct entities. This is true even though dysplastic 3. Type III from the renal pelvis.
kidneys often are cystic and many types of cystic kidneys These diverticuli may lead to urinary stasis, which
demonstrate some dysplasia. may predispose the patient to stone formation or urinary
tract infections. During an intravenous urogram, the
MEDULLARY SPONGE KIDNEY calyceal diverticulum will appear opaque later in the
Medullary sponge kidney is a term used to describe pyelographic phase. It typically remains radioopaque after
changes found on intravenous urography which show the remainder of the kidney has drained due to the slow
brush-like linear striations in the renal papillae, frequently exchange of urine caused by a narrow communicating
in association with renal calculi. It may be focal or involve neck. The diverticulum should have a round, smooth-
the whole of the kidney and usually is associated with walled appearance. It should not be confused with a
some enlargement of the kidney. It is not usually a disease hydrocalyx that develops from an infundibular stricture
of childhood although isolated cases are sometimes seen and has a squared off appearance.
with hematuria, nephrolithiasis and infections. A calyceal diverticulum is a lesion that results from
On IVU the blush in the medulla related to the calyces an outpouching of a portion of the collecting system that
has a very similar appearance to that seen in the ARPKD protrudes into the corticomedullary region. They can arise
and at times may be indistinguishable if the entire kidney in any part of the collecting system from a fornix to the
is involved. The term renal tubular ectasia is more widely renal pelvis. Size varies anywhere from a few millimeters
to several centimeters in diameter. They are uroepithelial- for malignant change, namely renal cell carcinoma. The
lined cavities that communicate via a narrow channel cysts may also be complicated by hemorrhage or
to a nearby calyx. They may be congenital, or acquired infection. Diagnosis is easily reached with ultrasound.
lesions.
It is not uncommon to see calcified stones charac- GENETIC CYSTIC DISEASE
teristically layering in the dependent portion of the AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE
diverticulum. The stones that form within the diverticulum
Autosomal dominant polycystic kidney disease (ADPKD)
may pass and cause symptomatic renal colic but they
is a renal cystic disorder which becomes manifest
are typically confined to the diverticulum due to its narrow
frequently after the third decade of life. However, there
neck connection to the distal collecting system.
is considerable phenotypic variation.
They are regions of urinary stasis and the dependent Incidence is 1:1000 with two and possibly three
sediment that eventually develops in a calyceal diver- genetic loci. Families have the gene located on the short
ticulum is referred to as “milk of calcium”. Larger stones arm of chromosome 16 (PKDI). The second gene is on
may form that are confined to the diverticulum and may chromosome 4 (PKD2). The location of PKD3 has not
be a source of chronic pain. yet been found. Although this is inherited as a dominant
On CT, the diagnosis is made with delayed imaging condition, nevertheless no family history is often encoun-
showing the diverticulum fill with contrast. Alternatively tered as spontaneous mutation seemingly accounts for
if stones are present, the patient can be rescanned in the many cases.
opposite position. If the stones settle dependently, and ADPKD is a systemic disease with both renal and
are confined to the lesion, the diagnosis can be made extrarenal manifestations. In PKDl families, 64% of
in the absence of delayed imaging. children affected under the age of 10 years will have cysts
and 90% between the ages of 10 and 19 years will have
Treatment cysts 31. Extrarenal manifestations, such as cysts in the
Management of symptomatic stone disease associated liver, pancreas or spleen, are rarely seen in pediatric
patients. Subarachnoid hemorrhage due to intracranial
with Calyceal diverticula has changed from an open
aneurysm is also rare in childhood. The occurrence of
surgical approach to include ESWL, percutaneous,
subarachnoid hemorrhage tends to cluster in families and
laparoscopic or ureteroscopic techniques. The choice of
routine screening in otherwise asymptomatic individuals
therapy depends largely on the anatomic location of the
is not recommended. Congenital hepatic fibrosis is
diverticulum.
generally associated with ARPKD but some rare cases
ACQUIRED CYSTIC RENAL DISEASE have been described in association with ADPKD.
The cystic abnormality differs from ADPKD in regard
This term usually refers to the cysts which develop in
to its localization into one part of the kidney and apparent
the native kidneys of patients in chronic renal failure. absence of cysts in the contralateral kidney. No family
There is no underlying cystic renal disorder and no other history or pattern of inheritance has been reported. Some
organ involvement. Acquired cysts can be found in up of the earlier reported patients with unilateral ADPKD
to 22% of patients in chronic renal failure but their almost certainly had this condition. It is suggested that
frequency increases with duration of dialysis up to 90% the family should be routinely screened for cystic kidneys
for those on dialysis for over 10 years. The incidence and the patient’s remaining kidney should be followed
is similar for those on hemo- and chronic peritoneal up for life. The lesions do not appear to be progressive
dialysis. After successful transplantation, the cysts tend in the few cases that have been reported and left in situ.
to regress in size. The importance of maintaining Hypertension has been documented and nephrectomy
surveillance of these kidneys is because of a potential is curative but the natural history is unclear.
ARPKD IVU: It helps to differentiate ARPKD from ADPKD,

It is the most common heritable cystic renal disease Glomerulocystic disease and renal dysplasia by demon-
occurring in infancy and childhood. It is distinct from strating bilateral and symmetrical enlargement of the
autosomal dominant polycystic kidney disease (ADPKD), kidneys and ‘streaky/chaotic’ nephrogram as the
which tends to occur in an older population. The clinical contrast clears in the ectatic collecting ducts.
spectrum shows a wide variability, ranging from perinatal
Ultrasonography: It is the primary modality for the
death to a milder progressive form, which may not be
evaluation of ARPKD, especially during the perinatal and
diagnosed until adolescence.
neonatal periods, which characteristically shows bilateral
Blyth and Ockenden initially classified ARPKD into renomegaly with uniformly increased reflectivity.
four groups:
1. Perinatal, CT and MRI: In older children, CT and MRI are often
2. Neonatal, used to evaluate liver disease.
3. Infantile, and For rest of the genetic cystic diseases of kidney, please
4. Juvenile. refer Chapter 4.
UROLITHIASIS or ectopic kidney, autosomal dominant polycystic

INTRODUCTION kidney disease, and vesicoureteral reflux.
2. Medullary sponge kidney
Passage of a urinary stone is the most common cause
3. Renal tubular acidosis (RTA)
of acute ureteral obstruction and affects as many as 12%
4. Immobilization
of the population. The pain may be some of the most
5. Hyperoxaluria
severe pain that humans experience, and complications
6. Other secondary causes:
of stone disease may result in severe infection; renal
• Milk-alkali syndrome
failure; or, in rare cases, death.
• Steroids
PATHOPHYSIOLOGY • Cushing syndrome
In patients with stone disease, more than 1 of 3 general • Hypervitaminosis D
mechanisms is likely to be active. These include the • Paraneoplastic Syndromes
following: • Multiple myeloma.
1. the possible presence or abundance of substances that
promote crystal and stone formation; TYPES OF RENAL CALCULI
2. a possible relative lack of substances to inhibit crystal 1. Calcium stones account for 75–85% of urinary
formation; and stones. Approximately one half of calcium stones are
3. a possible excessive excretion or concentration of composed of a mixture of calcium oxalate and calcium
salts in the urine, which leads to supersaturation of phosphate. They demonstrate intermediate fragility
the crystallizing salt. The greater the degree of super- to extracorporeal shock wave lithotripsy (ESWL).
saturation, the greater the rate of growth of the calculi. Approximately three eighths of calcium stones are
formed of only calcium oxalate dihydrate. These may
PREDISPOSING FACTORS be spiculated, dotted, mulberry, or jackstone in
1. Stasis or anatomic factors can also contribute to the appearance. Usually, these stones are fragile in
development of stone disease. These include response to ESWL. The remaining one eighth of
ureteropelvic junction (UPJ) obstruction, horseshoe stones are composed of calcium phosphate (apatite)
Calculus Diseases of Kidney 79
or calcium monohydrate. These stones are the densest therefore, metabolic evaluation is indicated.
and, consequently, the least responsive to ESWL. Combined obstruction and infection frequently cause
Calcium stones have numerous causes. Approxi- renal destruction and, potentially, renal failure if both
mately 85% of calcium stones are idiopathic, or kidneys are affected.
primary. Idiopathic hypercalciuria occurs in more than 3. Uric acid stones account for 5–10% of urinary
one half of patients with calcium oxalate stones. Most stones. These small smooth stones usually appear
causes of hypercalciuria are absorptive. Increased radiolucent on conventional radiographs but opaque
absorption in individuals after a normal diet causes on CT scans. Predisposing factors include acidic
an elevation of serum calcium levels and a suppression concentrated urine, excess urinary uric acid, small-
of parathyroid function as an abnormal response to bowel disease or resection, gout, and cell lysis (e.g.,
vitamin D. Approximately 10% of cases of primary resulting from treatment of leukemia or from
hypercalciuria are renal in origin. The inability of the starvation). Treatment and prevention for these stones
kidney to conserve calcium results in low serum is alkalinization and dilution of the urine.
calcium concentrations, which stimulate parathor- 4. Cystine stones account for only approximately 1%
of urinary stones. These ground-glass stones, which
mone secretion.
result from cystinuria (a rare autosomal recessive
The remaining 15% of calcium stones are secon-
metabolic disorder), are homogeneous; less opaque;
dary to some discernible etiology. Most commonly,
and less fragile than other stones, especially if they
they result from hyperparathyroidism, which is found
are smooth.
in 5-10% of patients with stones. In this situation,
5. Xanthine stones are relatively radiolucent stones.
hypercalcemia and increased absorption lead to
6. Several other less common forms of urolithiasis
hypercalciuria. Patients with the stones are treated with
may produce stones that appear relatively lucent, even
surgical removal of the parathyroid adenoma or
on CT scans. Inspissation of indinavir, an anti-
hyperplasia. Calcium stones can also occur in retroviral protease inhibitor used to treat HIV infection,
approximately 15% of patients with sarcoidosis in may cause stones that appear lucent on CT scans.
whom the production of activated vitamin D by 7. Matrix stones formed from inspissated mucoproteins
macrophages is abnormal. in patients with a chronic Proteus infection may demo-
2. Magnesium ammonium phosphate (struvite) nstrate soft tissue attenuation on CT scans. Stones
stones account for approximately 10–20% of urinary can also be caused by metabolic byproducts and drugs
stones. These stones are lucent but complex with (e.g. sulfa drugs, salicylates, triamterene ephedrine).
calcium phosphate. On occasion, they enlarge and 8. Orotic acid stones are rare to occur.
branch (staghorn*). Although they fragment easily,
patients with these stones usually are treated with INCIDENCE
percutaneous fragmentation and extraction because Renal calculi occur in 5–12% of the population, and they
of the large size of the stones and, usually, the presence are bilateral in 10–15% of patients. The prevalence of
of infection. Struvite stones are caused by urea-splitting urinary lithiasis is as high as 2–3% in the general
bacteria such as Proteus, Klebsiella, and Pseudomonas population. A slightly lower prevalence of urinary stones
species. However, as many as one half of patients is found in less developed countries, possibly because
have an underlying metabolic cause for stone disease; of diets lower in protein.
*“Staghorn calculi” resembles antlers of a stag. Composition: Struvite CLINICAL FEATURES

>> cystine or uric acid. Usually associated with recurrent urinary tract
infections from bacterial pathogens that produce alkaline urine (thus, • Acute ureteral obstruction by stone causes severe
F>M cases). Staghorn can be disrupted if infection complicates colicky (intermittent) flank pain that can radiate
obstruction related to the stone. Renal enlargement from pyonephrosis
or xanthogranulomatous pyelonephritis may produce a fragmented throughout the groin, testicles, back, and periumbilical
staghorn. region.
• Some patients with renal calculi may have no

symptoms at all
• Hematuria
• Occasionally, recurrent infection may result in
pyelonephritis or abscess. Stones can result in renal
scarring, damage, and renal failure.
• They are also more prevalent in highly developed
countries, possibly as a result of a higher protein diet.
• Sex: Males are at a greater risk than females, with
a male-to-female ratio of 3:1 (except for struvite stones
and in black populations).
• Age: Stones the peak age for development is in
persons aged 40–60 years.
1. Appendicitis (Figure 6.1)

2. Cholecystitis, acute
3. Cholelithiasis
FIGURE 6.1: KUB film: Appendicolith
4. Colon, diverticulitis
5. Crohn disease
29. Wilms tumor
6. Duodenum, ulcers
30. Xanthogranulomatous pyelonephritis.
7. Epididymitis
8. Gastric ulcer ROLE OF RADIOLOGY AND IMAGING
9. Gout The goals of imaging are to determine the presence of
10. Meckel diverticulum stones within the urinary tract, evaluate for complications,
11. Midgut volvulus estimate the likelihood of stone passage, confirm stone
12. Nephrocalcinosis passage, assess the stone burden, and evaluate disease
13. Obstructive uropathy, acute activity.
14. Ovarian torsion When acute flank pain suggests the passage of a
15. Ovarian vein thrombosis urinary stone, many methods of examination can be used.
16. Pancreatitis, acute Often, conventional radiography is initially used to screen
17. Pancreatitis, chronic for stones, bowel abnormalities, or free intra-abdominal
18. Papillary necrosis air. Radiographs can also be used to monitor the passage
19. Pelvic inflammatory disease/Tubo-ovarian abscess of visible stones.
20. Renal cell carcinoma IVU (excretory urography) provides important physio-
21. Renal vein thrombosis logic information regarding the degree of obstruction.
22. Retroperitoneal fibrosis Ultrasonography (USG) is useful in young or pregnant
23. Testicular torsion patients and in patients allergic to iodinated contrast
24. Transitional cell carcinoma material. USG is also helpful in problem solving.
25. Tuberculosis, Genitourinary tract All of these methods have become less useful with
26. Ureterocele the advent of more sensitive and specific nonenhanced
27. Ureteropelvic junction obstruction, congenital CT scanning. When CT is available, it is now considered
28. Vesicoureteral reflux the examination of choice for the detection and
localization of urinary stones. Almost all studies conducted in patients undergoing screening or follow-up observation
to date show that IVU provides no additional clinically for stones.
important information after nonenhanced CT is Typically, phleboliths are round or oval, and they may
performed. As a result of the higher radiation dose of demonstrate a central lucency. However, they are often
CT, conventional or digital radiography should be used difficult to distinguish from ureteral calculi. Phleboliths
to monitor the passage of stones if radiographic follow- in the pelvis are usually located lower than and lateral
up studies are indicated and if the stone is visible on to the ureter, but they overlap with the ureter. Because
conventional radiographs. gonadal veins parallel the upper ureters, contrast
enhancement may be needed to opacify the ureter and
Conventional Radiography demonstrate the extraurinary location of phleboliths in
Conventional radiography is often performed as a the gonadal veins.
preliminary examination in patients with abdominal pain Although 90% of urinary calculi are opaque on
possibly resulting from urinary calculi. These images abdominal radiographs, the sensitivity for the prospective
should be obtained before contrast material is adminis- identification of individual stones is only 50–60%, and
tered to prevent obscuring calcifications within the the specificity is only approximately 70%. Approximately
collecting system or calyceal diverticula. Conventional 10% of stones are radiolucent on conventional
radiographs should include the entire urinary tract, and, radiographs.
often, 2 images are required.
Limitations
Stones are often found at key points of narrowing
Because of the higher radiation dose with CT,
such as the UPJ, the ureterovesical junction (UVJ), and
conventional or digital radiography should be used to
the point at which the ureter crossing the iliac vessels.
monitor the passage of stones if radiographic follow-up
An addition site is on the right side where the ureter passes
is believed to be indicated and if the stone is visible on
through the root of the mesentery.
conventional radiographs. Pregnant or pediatric patients
Calcium stones as small as 1–2 mm can be seen.
Cystine stones as small as 3–4 mm may be depicted,
but uric acid stones are usually not seen unless they have
become calcified.
An erect or posterior oblique radiograph obtained on
the side of the calcification may help in distinguishing
urinary stones from extraurinary calcifications. This view
can also depict calcifications that are projected over the
sacrum or transverse processes on the frontal view.
(Figure 6.2).
Preinjection renal tomography may depict additional
stones, and it can be used to confirm the relationship
of stones to the kidneys.
Because stones are more visible with a lower peak
kilovoltage (kVp), maintaining a maximum of 60–80 kVp
is best, if possible. Larger patients may require a higher
peak kilovoltage for acceptable exposure and scatter. In
this situation, compression of the abdomen and
collimation is critical.
Mild bowel preparation may be helpful for increasing FIGURE 6.2: KUB film: Multiple small ‘gravel’ calculi in right
the sensitivity of conventional radiography for small stones kidney, right lower ureter and in the right ureterocele
may be imaged with US first to avoid radiation exposure.

The rare false-negative finding is usually due to reader
error or a protease-inhibitor CT-lucent stone. False-
positive results are usually due to phleboliths adjacent
to the ureter. In some cases, intravenous contrast material
may be needed to opacify the ureter.
Intravenous Urography
IVU is useful for confirming the exact location of a stone
within the urinary tract. IVU depicts anatomic abnorma-
lities such as dilated calyces, calyceal diverticula,
duplication, UPJ obstruction, retrocaval ureter, and others
that may predispose patients to stone formation or alter
therapy. Because contrast agents can obscure stones in
the collecting system, scouting the entire urinary tract prior
to their administration is critical.
When an acute urinary stone is the primary conside-
ration, compression may not be used to increase sensi-
tivity for detection of low-grade obstruction. A caveat is
FIGURE 6.3: IVU: Large right lower ureteric calculus with nonfunctioning
that the contralateral kidney may have an abnormality right kidney with focal caliectasis at upper pole of left kidney
that requires ureteric compression for adequate exami-
nation. In rare cases, the use of compression has been Limitations
associated with forniceal rupture. IVU is the traditional examination for the assessment of
When a stone causes acute obstruction, an obstructive urinary stone disease, and it does provide physiologic
nephrogram may be present. This may be prolonged and information related to the degree of obstruction. The
hyperopaque, with increasing opacity over time. The radiation dose is generally smaller than that of CT, but
nephrogram of acute obstruction is usually homogeneous, it is of the same order of magnitude. Intravenous contrast
but may also be striated or occasionally not visible on is required, with resultant risks of an allergic reaction or
radiographs. nephrotoxicity. IVU is less sensitive than CT, especially
Other signs include delayed excretion, dilatation to for small or nonobstructing stones (Figures 6.3 to 6.5).
the point of obstruction, or blunting of the calyceal
fornices. Immediately after the passage of a stone, residual Ultrasonography
mild obstruction or edema can be detected at the UVJ. On sonograms, stones are demonstrated as bright
Delayed images may be needed to opacify to the point echogenic foci with posterior acoustic shadowing. Stones
of the obstruction, but using gravity to position the more are visualized fairly well with USG in the kidneys and
opaque and more distal contrast material–laden-urine is the distal ureter at or near the UVJ, especially if dilatation
also possible by placing the patient in a prone or erect is present. USG is good for the visualization of
position. complications such as hydronephrosis (or other signs of
Extravasation of urine at the fornices may result in obstruction); however, some patients with acute
pyelosinus or pyelolymphatic extravasation, which is often obstruction have little or no dilation.
first indicated by blurring of the calyceal fornices. Greater In particular, USG is helpful in evaluating those with
extravasation may outline the collecting system, and the renal insufficiency or contraindications for the use of
contrast may dissect into the perinephric space; however, contrast media; however, USG is often skipped in favor
if the urine is not infected, this is usually clinically insignificant. of nonenhanced CT.
or after forniceal rupture. Absence of the ureteral jet, as

visualized with color Doppler on the symptomatic side,
is presumptive evidence for a high-grade obstruction in
a well-hydrated patient.
USG is very insensitive for stones, especially stones
smaller than 2 mm, stones at the UPJ, or stones in the
mid ureter. Fowler et al suggest that USG has a sensitivity
as low as 24%, compared with nonenhanced CT.
Furthermore, estimations of stone size may not be
accurate. Compared with nonenhanced CT, USG is more
dependent on the operator’s ability and more time
consuming.
Limitations: USG has limited sensitivity for smaller stones,
and does not depict the ureters well. It should be used
mainly in patients who are young, those who are
pregnant, or those undergoing multiple examinations (eg,
patients with spine injury).
FIGURE 6.4: Lateral view (overlapping of PCS on spine)
CT
With a sensitivity of 94–97% and a specificity of 96–
100%, helical CT is the most sensitive radiologic
examination for the detection, localization, and
characterization of urinary calcifications; therefore, helical
CT is considerably more effective than IVU.
Helical CT scans frequently depict non-obstructing
stones that are missed on IVU (Figure 6.6).
CT is faster and no contrast agent is needed in most
patients.
FIGURE 6.5: IVU: Filling defect of calculus seen in

left lower ureter with mild left hydronephrosis
In addition, USG is good for characterizing lucent

filling defects that are visualized as stones on IVU.
However, USG does not provide direct physiologic
information regarding the degree of obstruction.
Doppler imaging may demonstrate a high resistive index FIGURE 6.6: Non-contrast spiral CT scan section
in acute obstruction, but this may not occur immediately showing small nonobstruction bilateral renal calculi
To discern between phleboliths and urinary stones,

50 ml of low-osmolar contrast agent should be
administered. After 3–5 minutes, a 5 mm helical scan
is obtained through the area of concern. Fewer contrast-
enhanced studies are needed with increasing experience.
Soft tissue around the rim of a calculus can differentiate
it from a phlebolith. A phlebolith may have a comet tail
of soft tissue extending from it; this finding differentiates
it from a calculus. On CT scans, phleboliths do not have
radiolucent centers, as often seen on plain radiographs
(Figures 6.8 and 6.9).
FIGURE 6.7: Non-contrast CT: Left staghorn calculus
When contrast-enhanced scans are required to
evaluate pain not related to stones, routine abdominal
CT easily differentiates between non-opaque stones and/or pelvic CT should be performed. In this situation,
and blood clots or tumors (compared with IVU, which 100-150 ml of a low-osmolar oral and rectal contrast agent
may depict only a filling defect) (Figure 6.7). is used, and a 5 mm helical CT scan is obtained with
In addition, helical CT is better than USG or IVU in
detecting other causes of abdominal pain. In fact, in most
studies, IVU added little or no information.
Rarely, pure matrix stones may demonstrate soft-
tissue opacity on CT scans, and indinavir stones appear
lucent. However, all other stones appear opaque on CT
scans.
CT Protocol for Calculus Disease

Because stones in the collecting system may be obscured
by contrast material, nonenhanced CT is usually
performed. Helical CT is important to avoid missing
stones because of section misregistration. A 5 mm helical FIGURE 6.8: Non-contrast CT: Calculus in right lower ureter
technique with a pitch of 1.5:1 or less is preferred,

although some radiologists choose to use a pitch of as
much as 2:1. The kidneys and, if possible, the entire
abdomen should be scanned during a single breath hold
to prevent section misregistration.
Because patients with stones are often young and
because stone disease may recur, minimizing the radiation
dose is critical. A fairly high level of noise as a result of
the inherently high contrast levels is tolerable in most
patients.
Reported radiation doses for CT are 2.8–4.5 mSv
compared with 1.3–1.5 mSv for a 3-image IVU. However,
the uterine dose is approximately 0.006 Gy for 4 image
FIGURE 6.9: Non-contrast CT: Tiny right UVJ
IVU compared with 0.0046 Gy for nonenhanced CT. calculus pouting in bladder
a pitch of 1.5:1. Patient selection determines the number Limitations

of examinations needed. Nonenhanced CT results are usually diagnostic, but if
Stones at the UVJ may be difficult to distinguish from contrast material is needed, actions can be taken to
stones that have already passed into the bladder. If the decrease the risk of an adverse reaction in patients. The
distinction changes therapy, a repeat scan through the patient can be premedicated with steroids and histamine
UVJ in the prone position may be helpful. Stones that blockers. Use of low-osmolar contrast agent also helps.
have already passed into the bladder will drop into a Use of iodinated contrast agents should be avoided in
dependent location. patients who have had previous life-threatening reactions.
CT findings Nonenhanced CT is usually sufficient with the aid of USG
1. Stones in the ureter and MRI as problem-solving tools. Nuclear scintigraphy
2. Enlarged kidneys may also be helpful in confirming obstruction.
3. Hydronephrosis (83% sensitive, 94% specific) Usually, in patients with renal insufficiency,
4. Perinephric fat stranding ± fluid (82% sensitive, 93% nonenhanced CT is sufficient. Very poor renal function
specific) results in a failure to opacify the collecting system. As
5. Ureteral dilatation (90% sensitive, 93% specific) in pregnant patients, USG, MRI, and scintigraphy can
6. Soft-tissue rim sign (good positive predictive value be useful as problem-solving tools
with a positive odds ratio of 31:1)
7. White pyramid sign absent. MRI
The amount of perinephric fluid is correlated with the Stones are not directly visible on MRIs because they
degree of obstruction seen on IVU, and as with the produce no signal. However, they may be indirectly
obstruction, the amount of fluid is correlated with the visualized as a filling defect in the ureter or collecting
likelihood of stone passage. Normal hyperattenuating system on heavily T2-weighted images or on gadolinium-
renal pyramids sometimes are seen. These indicate that enhanced T1-weighted images. MRI can be useful as a
significant obstruction is not present. However, this finding
problem-solving tool if the use of iodinated contrast
has been seen with proven ureteral calculi and is often
material or radiation is contraindicated (e.g. during
absent in patients without stones. For this reason, the
pregnancy).
usefulness of IVU is limited. If contrast material is adminis-
tered, a delayed or hyperattenuating nephrogram may RNI
also be visible on CT scans if the ureter has an obstruction.
Nuclear medicine studies may demonstrate the retention
Conventional radiography may be helpful in visua-
lizing larger stones, once they are identified on CT scans, of activity in the cortex or collecting system when the
to provide a baseline to follow passage of the stone. If obstruction is ongoing. Nuclear medicine tests are useful
kidney, ureter, and bladder radiographs fail to depict the in determining differential renal function for treatment
stone, CT may be needed to follow its passage. planning and for assessing how much renal function might
Approximately 40–55% of stones are not visible on return after the obstruction is relieved. For example, a
abdominal radiographs. Almost no stones with attenua- kidney with very little function might be removed if very
tion values of less than 200 HU are visible, and repeat little function persists after a trial of drainage. Occasionally,
CT scans are usually required if passage of the stone is confirming the obstruction with nuclear medicine studies
to be followed. Cystine and urate stones have an is useful if the administration of iodinated contrast material
attenuation of 100-500 HU; calcium stones usually is contraindicated.
demonstrate attenuation higher than 700 HU. Consi-
derable overlap exists in the CT attenuation values of INTERVENTION
calcium stones. Retrograde or antegrade pyeloureterography may be
Individual CT signs are associated with varying indicated if the collecting system cannot be opacified
degrees of confidence, as noted in CT findings above. otherwise. This becomes much less useful as a diagnostic
examination when CT is available. Retrograde stent whereas stones larger than 8 mm pass in only
placement is indicated if obstruction is present with approximately 20% of patients.
proximal infection (pyonephrosis). Stent placement is Occasionally, recurrent infection may result in
performed to prevent sepsis and irreversible renal pyelonephritis or abscess. Stones can cause renal scarring,
damage. If retrograde stent placement is unsuccessful, damage, or even renal failure if they are bilateral.
nephrostomy or antegrade stent placement serves as a In 10% of patients, stones recur within 1 year. This
reliable backup. percentage increases to 50% within 10 years.
SPECIAL CONSIDERATIONS RADIOLUCENT UROLITHIASIS

In the diagnosis and treatment of kidney stones, special The prevalence of urolithiasis is approximately 2 to 3
concerns exist in patients who are pregnant, in those who percent in the general population, and the estimated
have contraindications to the use of contrast media, and lifetime risk of developing a kidney stone is about 12%.
in those with renal insufficiency. The classic presentation of renal colic is excruciating
Pregnancy does not predispose patients to stone unilateral flank or lower abdominal pain of sudden onset
formation; however, stone formation is a complication that is not related to any precipitating event and is not
in as many as 0.05% of pregnancies, and the diagnosis relieved by postural changes or nonnarcotic medications.
may be difficult to establish with imaging because of the With the exception of nausea and vomiting secondary
displacement and obscuration of organs by the enlarged to stimulation of the celiac plexus, gastrointestinal
uterus and fetus. Consider using USG first in a pregnant symptoms are usually absent. Approximately 75% of
patient, especially in the first trimester. IVU can be used, urinary calculi are composed of calcium oxalate, calcium
phosphate, or a mixture of the two. Phosphate-containing
but the views should be limited to scout and 10 to 30
stones account for about 15% of urinary calculi, with
minute images if possible. CT can also be useful, and
struvite (magnesium ammonium phosphate) being the
the radiation dose may be justified (especially if the clinical
most common. Uric acid stones account for approximately
picture is confusing), because any fetal damage is unlikely
5 to 10% of urinary calculi.
at the typical radiation doses. Minimize the dose by
The radiodensity of ureteral calculi also clearly affects
increasing the pitch and decreasing the milliamperage.
their visibility on plain radiographs. In general, calcium
MRI may be a useful tool for problem solving. phosphate stones have the greatest density, followed by
MORTALITY/MORBIDITY
calcium oxalate and magnesium ammonium phosphate.
Cystine calculi are only mildly radiodense. Uric acid stones
Passage of a renal stone is the most common cause and matrix stones are usually entirely radiolucent. It has
of acute ureteral obstruction. When this occurs, pressure been estimated that most (85–90%) of all renal stones
in the collecting system and renal blood flow acutely are radiopaque.
increase, followed by decreased blood flow after 1–2 There are several modalities used to make the
hours. Hematuria usually occurs. This can be intermittent diagnosis of urolithiasis. With its increasing availability
or persistent and microscopic or gross. However, as many and technical superiority, helical CT has been shown to
as 10% of patients with acute stones may not have be more accurate than KUB radiography, IVU, and US
hematuria. for the diagnosis of ureteral calculi. It has been proposed
Acute ureteral obstruction by stone causes severe, that because of its safety, speed, cost-effectiveness,
colicky (intermittent) flank pain that can radiate accuracy, and ability to assess other potential causes of
throughout the groin, testicles, back, or periumbilical flank pain, nonenhanced helical CT should replace IVU,
region. Some patients with renal calculi may have no in most instances, as the most effective study used for
symptoms at all. Stones smaller than 4 mm pass the diagnosis of ureteral calculus disease. Furthermore,
spontaneously in approximately 80% of patients. Stones essentially all stones, including uric acid stones, appear
that are 4–6 mm pass in approximately 50% of patients, dense on CT.
The primary use of CT in suspected renal stone disease in hyperparathyroidism. The term has now acquired more
is to differentiate stones from other causes of filling defects. of a radiologic concept and is used to describe diffuse,
Uric acid, xanthine, and cystine stones that are radiolucent fine, renal parenchymal calcification that is radiologically
on plain film are appropriately identified as high density demonstrable. This appearance is different from that of
on CT, with densities of 100 to 600 Hounsfield units (H). calcification within the lumen of the collecting system,
Uroepithelial tumors usually range from 8 to 30 H ureter, and bladder, which represents nephrolithiasis.
unenhanced, whereas blood clots are typically 50 to Many causes of nephrocalcinosis have been added
65 H with or without contrast. In other cases of since the original description. These include the several
obstruction, CT demonstrates the dilated collecting
causes of hypercalcemia and hypercalciuria. Nephrocal-
structures, delayed excretion of contrast agent, as well
cinosis can be subdivided into the cortical type, which
as calculi, tumor, or extrinsic masses.
is classically the result of acute tubular necrosis (ATN),
In addition to its sensitivity, a helical CT of the entire
and the medullary type, which may be an extension of
urinary tract can be performed in 30 seconds, allowing
a diagnosis to made much faster than urography, cortical nephrocalcinosis or seen in isolation with several
sonography, or radiography. CT for flank pain identifies metabolic disorders. In nephrology, the term nephrocal-
an alternative diagnosis up to 50% of the time, and cinosis is applied only to the medullary type. Nephrocal-
typically exposes the patient to less than half the radiation cinosis can be demonstrated on plain radiographs,
of an intravenous urogram with nephrotomography. sonograms, or CT scans. CT is the most accurate and
A circumferential rim of ureteral tissue (tissue rim sign) sensitive technique and therefore the modality of choice.
is seen around 90% of stones less than 4 mm in diameter, It can be unilateral or bilateral.
helping distinguish stones from phleboliths. Secondary
signs such as unilateral hydronephrosis, unilateral PATHOPHYSIOLOGY
hydroureter, unilateral perinephric stranding, and Disorders of Calcium Metabolism
unilateral nephromegaly may also be seen. In fact, Disorders of calcium metabolism, such as hypercalcemia
hydroureter with ipsilateral stranding around the kidney and hypercalciuria, may induce the formation of calcium
or ureter has a positive predictive value of 98% for the renal stones and deposition of calcium salts in the renal
presence of ureteral stone, and the absence of these two parenchyma (nephrocalcinosis). The extensive deposition
findings has a negative predictive value of 93%.
of calcium may lead to chronic tubulointerstitial disease
Stones >4 mm in the upper ureter are less likely to
and renal insufficiency. The first signs of damage induced
pass spontaneously, and are often associated with
by hypercalcemia are seen at the intracellular level, in
intractable nausea, vomiting, and fever.
the tubular epithelial cells. This results in mitochondrial
In the case of giant hydronephrosis, defined as the
distortion, and eventually, calcium deposits can be
presence of more that one liter in the collecting system,
demonstrated within the mitochondria, the cytoplasm,
nephrectomy is often performed due to severe renal
and the basement membrane.
failure. Giant hydronephrosis is seen more on the left
Calcified cellular debris results in occlusion of the
than the right, and more commonly in males than females.
Typical etiologies include PUJ obstruction, congenital tubules, leading to obstructive atrophy of the nephron,
abnormalities, or stones. The first diagnosis is often nonspecific inflammation, and interstitial fibrosis.
erroneous, with entities such as ovarian cysts or hepatic Impaired urine drainage through calcified tubules may
cysts considered initially. result in areas of cortical atrophy leading to scared
cortices. Functional abnormality of urine concentration
NEPHROCALCINOSIS is the earliest detectable renal change. This effect is related
to decreased chloride transport in the ascending thick
INTRODUCTION
segment of the nephron.
This was a termed coined by Albright in 1934 to describe Other defects of tubular function, such as tubular
the deposition of calcium salts in the renal parenchyma acidosis and salt-losing nephritis, may also occur.
The continuing and unchecked deposition of calcium 7. Milk-alkali syndrome

eventually leads to chronic renal insufficiency. Nephrocal- 8. Hypervitaminosis D
cinosis may be complicated by renal stone formation, 9. Sarcoidosis
which adds another element to the causation of renal 10. Nephrocalcinosis has been described in 16% of
insufficiency secondary to an obstructive uropathy. The preterm infants. On univariate analysis, gestation
histologic findings include calcium phosphate or calcium age, male sex, duration of ventilation, oxygen
oxalate crystal deposits that mainly appear in the renal dependency, duration and frequency of gentamicin
interstitium, but deposits may also be seen within the treatment, toxic gentamicin/vancomycin levels, low
renal tubules. Special stains, such as von Kossa and fluid intake, and postnatal dexamethasone were
Pizzolato stains, can be used to specifically depict these significantly associated with nephrocalcinosis.
deposits. 11. Nephrocalcinosis has been described in premature
infants treated with high doses of furosemide for
CAUSES OF CORTICAL NEPHROCALCINOSIS prolonged periods because of congestive heart
1. Acute cortical necrosis: This can follow placenta failure secondary to patent ductus arteriosus or
abruptio, placenta previa, septic abortion, transfu- pulmonary disease. Both nephrocalcinosis and
sion reactions, burns, snake bite, severe dehydra- nephrolithiasis may occur. These complications
tion, shock, severe heart failure, and abdominal occur 11–50 days after the commencement of
aortic surgery. In children, the condition may follow furosemide therapy. The addition of chlorothiazide
dehydration, fever, sepsis, and hemolytic uremic to furosemide prevents further calculi formation, and
syndrome. it may also lead to the dissolution of existing stones.
2. Chronic glomerulonephritis Therefore, preterm infants who are taking furo-
3. Alport syndrome semide should be regularly screened with renal
4. Prolonged hypercalcemia and/or hypercalciuria ultrasonography. Long-term furosemide abuse
5. Poisoning and toxicity (e.g. Ethylene glycol can also cause medullary nephrocalcinosis in
(antifreeze) poisoning) adults.
6. A severe form of primary hyperoxaluria 12. Hyperoxaluria
7. Methoxyflurane anesthesia toxicity 13. Miscellaneous causes
8. Rejected renal transplants can give rise to cortical 14. A variety of conditions can cause bone destruction
necrosis. associated with hypercalcemia and hypercalciuria.
9. Sickle cell disease is a rare cause of cortical nephro- These include bony metastases, multiple myeloma,
calcinosis. Sickle cell disease is related to infection. Paget disease, Cushing disease, and both
10. Vitamin B6 (pyridoxine) deficiency can be associated hyperthyroidism and hypothyroidism (though the
with xanthurenic aciduria related to deficiency of incidence associated with hypothyroidism is low).
phosphate dependent enzyme kynureninase. Chronic paraneoplastic hypercalcemia may also
Vitamin B6 (pyridoxine) deficiency is another cause cause nephrocalcinosis.
of secondary hyperoxaluria. It is a rare cause of 15. Sickle cell anemia is a rare cause of nephrocal-
cortical nephrocalcinosis. cinosis. A variety of radiographic renal abnormalities
have been associated with sickle cell disease,
CAUSES OF MEDULLARY NEPHROCALCINOSIS including renal enlargement, thickening of the renal
1. Hyperparathyroidism cortex, focal hypertrophy, papillary necrosis, and
2. Medullary sponge kidney changes associated with infection.
3. Tuberculosis of the kidneys 16. Ochronosis (alkaptonuria) is an autosomal recessive
4. Renal tubular acidosis disorder involving deficiency of the enzyme homo-
5. Renal papillary necrosis gentisic acid oxidase. It is a rare cause of nephro-
6. Immobilization calcinosis; renal stone formation is more common.
INCIDENCE Each normal kidney contains about 1,250,000

Regarding cases of nephrocalcinosis and nephrolithiasis nephrons. The gross anatomic renal functioning unit is
in children, 64% are associated with an underlying the lobule consisting of the medullary pyramid with its
structural renal lesion or urinary tract infection, 10% are base oriented toward the renal cortex, the apex oriented
associated with hypercalcemia or hypercalcuria, 6% are toward the calyx. Its sides are covered by renal cortical
associated with cystinuria, and 20% are idiopathic. columns. At the glomerulus, all the constituents of plasma
are filtered, except the cellular elements, and the plasma
Oxalosis and miscellaneous conditions may be involved.
proteins and are transported via of tubules to the renal
In adults, 40% of cases of medullary nephrocalcinosis
sinus. In healthy adults, 180 L of filtrate is formed per
are attributed to hyperparathyroidism, and 20% are
day. About 80% of the filtrate is reabsorbed in the
attributed to RTA. The remaining 40% are divided among
proximal convoluted tubule, and 20% is absorbed in the
the other multiple causes. Conversely, 5% of patients with
distal tubular structures, dependent on antidiuretic
hyperparathyroidism have nephrocalcinosis. The
hormone (ADH).
medullary type accounts for 95% of all nephrocalcinosis,
Disorders of calcium metabolism, such as
whereas 5% represent cortical nephrocalcinosis. In 70%
hypercalcemia and hypercalciuria, may cause extensive
of patients with RTA type I, both nephrocalcinosis and
deposition of calcium within the renal tubules, and chronic
nephrolithiasis eventually develop.
tubulointerstitial disease and renal insufficiency may
result. Calcified cellular debris results in occlusion of the
CLINICAL FEATURES
tubules, leading to obstructive atrophy of the nephron,
Sex: Both sexes are equally affected. nonspecific inflammation, and interstitial fibrosis.
Age: All age groups can be affected, but the disease is Impaired urine drainage through calcified tubules may
more common in childhood than at other times. result in areas of cortical atrophy, leading to scared
The clinical presentation is determined by the cortices. Functional abnormality of urine concentration
underlying etiology of nephrocalcinosis. Most cases are is the earliest detectable renal change (osmolality). This
asymptomatic, and nephrocalcinosis is identified as a is related to decreased chloride transport in the ascending
radiologic abnormality. Renal tubular disorders may be thick segment of the nephron. Other defects of tubular
first diagnosed on biochemical examination of the urine, function, such as tubular acidosis and salt-losing nephritis,
which often reveals glycosuria, aminoaciduria, and may also occur.
phosphaturia. Polyuria and polydipsia may be the
presenting features, with loss of the concentrating ability
of the renal tubules. Most cases of nephrocalcinosis are asymptomatic and
usually identified on plain abdominal radiographs.
Anatomy: The basic unit of renal function is the nephron Planar radiography provides a useful adjunct to plain
(Figure 6.1). The nephron consists of the glomerulus; the radiography. Findings: Plain radiographic detection is not
proximal convoluted tubule, the loop of Henle; and the possible until the attenuation of renal parenchyma
distal convoluted tubule, which finally drains into the exceeds 100 HU. The calcification resolution also depends
collecting ducts. Anatomically, the glomerulus and on the size of the stones (those <2 mm are rarely
proximal convoluted tubules are placed in the renal cortex, detected), the spatial resolution of the recoding technique,
while the descending loop of Henle enters the medullary and contrast factors. The characteristic cortical calci-
tissue. The ascending loop goes back to the cortex, where fication occurs within a few weeks after the onset of acute
it drains into the distal convoluted tubule. Finally, the cortical necrosis. When cortical nephrocalcinosis first
distal convoluted tubule drains into the collecting ducts, appears, the kidneys are still enlarged because of
which again enters the renal medulla and drains into the inflammatory edema of the kidneys. With time, the
calyx. kidneys atrophy.
Morphologically, three types of calcification have been before nephrocalcinosis can be identified on plain
described. The most prevalent is a single cortical, calcified, abdominal radiographs.
and thin peripheral band, often with calcified extensions
Findings: In medullary calcinosis, the margins of the
into the necrotic septa of Bertin (placed perpendicular
pyramids are echogenic, while the center of pyramids
to the cortical calcification). The medullary pyramids are
remains echolucent. The pyramids are well visualized as
usually spared, retaining the attenuation of the soft tissue.
rounded or echogenic structures. The pyramids may be
Initially, this pattern of nephrocalcinosis may be difficult
densely echogenic, and shadowing may be shown. These
to recognize because calcification is faint. However, the
findings may be evident on sonograms before they are
kidney margins appear well defined and appear penciled seen on plain radiographs.
in.
The second pattern seen with cortical nephrocalcinosis Computed Tomography
is the appearance of hyperattenuating tram lines. These Nonenhanced CT scans can also depict nephrocalcinosis,
lines may be continuous. More often, they are interrupted, and its apparent sensitivity and accuracy make it the
reflecting more patchy distribution of cortical necrosis. modality of choice.
The third pattern is a more diffuse distribution of punc-
Findings: CT is said to be the most sensitive modality
tate calcification. This punctate pattern of calcification
in the diagnosis of nephrocalcinosis. CT depicts
is thought to represent necrotic calcified glomeruli and
nephrocalcinosis at an early stage of the disease, it
tubules.
provides a better picture of the density and extent of
Medullary nephrocalcinosis typically produces clusters
nephrocalcinosis, and it may depict other changes such
of stippled calcifications, mainly within the regions of the
as renal cysts. In one small series of 13 patients,
renal pyramids.
intravenous urograms showed medullary sponge kidneys
Most conditions that cause medullary nephrocalcinosis
(24 kidneys), and nonenhanced CT scans showed
can also result in nephrolithiasis. Some nephroliths may
papillary calcifications in 11 kidneys. In 5 of these kidneys,
represent extruded deposits from the renal parenchyma.
the calcifications were not detectable on plain
The radiologic demonstration of both nephrocalcinosis
radiographs. Hyperattenuating papillae (55–70 HU)
and nephrolithiasis also helps in determining their etiology
without calcification were found in 4 other kidneys.
and thus significantly contributes to the management of
Hyperoxaluria is characterized by nephrocalcinosis on
the disease. Planar conventional tomography provides
radiologic examination, and it is associated with
a cheaper alternative to CT, and it may be a useful adjunct
nephrolithiasis and calcium oxalate crystal deposition in
to conventional radiography. multiple extrarenal organs. CT is a sensitive modality for
Findings the detection of systemic oxalosis (e.g., in the liver and
In medullary calcinosis, the margins of the pyramids are heart), in addition to nephrocalcinosis. Bilateral
echogenic, while the center of pyramids remains attenuating rings in the renal medulla have been depicted
echolucent. The pyramids are well visualized as rounded on nonenhanced CT scans in a patient with marked
or echogenic structures. The pyramids may be densely hypercalcemia and suspected hyperparathyroidism.
echogenic, and shadowing may be shown. These findings
may be evident on sonograms before they are seen on MRI
plain radiographs. MRI is rarely required to diagnose nephrocalcinosis since
it does not depict calcium, as such.
Ultrasonography
Renal cortical calcification causes increased cortical
This is noninvasive, ideal for use in young children, quick echogenicity with complete shadowing in severe cases.
to perform, and accurate. Early in its course, medullary Secondary pyramidal fibrosis may occur; these may cause
nephrocalcinosis can be seen as echogenic pyramids echogenic pyramids.
RNI stones (nephrocalcinosis). Parathyroidectomy may be

Findings: The role of nuclear medicine is confined to required to control hypercalcemia caused by hyperpara-
assessing renal function by means of isotope renography, thyroidism. This technique is good for stones smaller than
particularly when renal surgery is being considered. 5 mm, satisfactory for stones 5–10 mm, and rarely used
Intervention: Extracorporeal shock wave lithotripsy for stones larger than 10 mm because of the possibility
(ESWL) may be indicated in cases of intrapelvic renal of the debris load causing steinstrasse.
ACUTE URINARY OBSTRUCTION the most important in acute obstruction. Urine extravasa-
INTRODUCTION tes from a rupture of a calyceal fornix into the perinephric
spaces where it is resorbed, thus decompressing the
Acute obstructive uropathy is a commonly encountered
collecting system. Pyelovenous (a unique pathway),
condition in day to day clinical practice. Unilateral
pyelotubular, and pyelolymphatic backflow are other
obstruction to urinary outflow typically occurs, with little
compensatory mechanisms.
if any change in measured renal function in a healthy
The degree of obstruction is an important determining
individual. However, the less common bilateral form
factor in the development of nephropathy, with impairment
results in measurable changes in kidney function.
of function occurring in a more complete obstruction.
PATHOPHYSIOLOGY Complete recovery of function occurs when the obstruction
In a normally functioning kidney, urine formed within is relieved in 7 days. However, permanent loss of function
the tubular system empties into the calyces, where occurs if the obstruction continued for around 50 days.
pacemaker sites generate peristaltic activity to propel urine Acute obstruction also produces alterations in blood
into the pelvis. Urine is conveyed into the ureter, where flow, leading to ischemia. The renal capsule is a fixed
further peristalsis moves it in boluses into the bladder. container, and only with longstanding obstruction will the
The collecting system has a baseline pressure of 0–10 kidney significantly enlarge. Angiography may reveal
cm water, with peristaltic waves producing pressures in slight splaying apart and compression of arteries.
the range of 20–60 cm water. However, there is marked compression of the intrarenal
In acute obstruction, the pressures within the collecting veins, giving them a spidery appearance.
system and ureters above the point of obstruction can These effects are believed to be the result of interstitial
increase dramatically. Pressures in excess of 80 cm water edema secondary to increased hydrostatic pressure within
may be generated. Intratubular pressure also is elevated, the tubules. Other factors that are involved in the
leading to progressive renal impairment in the face of development of dysfunction include the health of the
continued obstruction. The kidney attempts to maintain kidney preceding the insult, as well as coexisting illnesses.
excretory function by mechanisms for urine resorption, Of special significance is a superimposed infection that
termed renal backflow. This includes pyelosinus backflow, can cause a rapid deterioration in renal function.
Obstructive Diseases of Kidney 93
INCIDENCE 3. Sloughed papillae secondary to papillary necrosis

Unilateral obstructive uropathy is predominantly the result occasionally may cause symptoms and signs of acute
of urolithiasis, which has an annual incidence of 1 in obstruction.
1000. Approximately 12% of the population will develop 4. Less commonly, in patients with diabetes and in
calculi before age 70 years. The less common bilateral debilitated patients, fungal balls from Candida or
form has an estimated incidence of 1 in 10,000 in the Aspergillus may produce obstruction.
general population. 5. Ureteral ligation is a well-recognized complication of
Ureteral injury occurs as a complication of gynecologic abdominal and pelvic surgery, especially gynecologic
surgery in approximately 2% of patients. With abdominal interventions. In addition, distal ureteral ischemia may
radical hysterectomy, a 10–15% incidence rate has been result during pelvic lymphadenectomy, as small blood
reported. vessels are stripped along with the lymphatics.
6. Pelvic trauma, both penetrating and blunt, may
produce obstruction to urine outflow from rupture of
ETIOLOGY
the ureter or compression from a retroperitoneal
1. The most common cause for acute obstructive hematoma.
uropathy is an impacted calculus. In most of the 7. Extrinsic compression from an abscess or
patients, the level of obstruction is at the ureterovesical inflammatory mass in patients with appendicitis,
junction; in the remainder of patients, the ureteropelvic pancreatitis, or Crohn’s disease also has been reported
junction or mid ureter are the points where calculi (Figure 7.1).
tend to lodge. Most calculi contain calcium, combined
with oxalate and/or phosphate. While metabolic
disorders such as hyperparathyroidism can contribute
to stone formation, idiopathic hypercalciuria occurs
in about half of patients with stones. Magnesium
ammonium phosphate (struvite) stones are the next
most common type, and are observed most frequently
laminated with calcium apatite. They form in the
alkaline environment created by urease-splitting
bacteria, especially Proteus species, and commonly
produce the ‘staghorn’ appearance of the
pelvocalyceal system. Uric acid stones are less
common. Hyperuricemic conditions such as gout only
account for a small minority of patients. An acidic
environment is necessary for uric acid stones to form.
These stones, along with the less common xanthine
and matrix calculi, appear radiolucent on plain
radiographs. Cystine stones are less opaque than
calcium stones and may be difficult to demonstrate
on plain radiographs.
FIGURE 7.1: Causes of obstruction to urinary tract
2. Intraluminal obstruction also may be the result of
blood clots and mucosal edema. These may occur CLINICAL FEATURES
with passage of calculi but also can occur following A patient with a calculus lodged in the urinary tract can
instrumentation of the ureter, trauma, or anticoagulant present with acute, colicky flank pain that radiates to the
therapy. groin. Initial management usually involves pain control
and aggressive hydration. Patients generally are treated UPJ, the area overlying the iliac bifurcation and where
as outpatients unless a complicated course necessitates the right ureter passes through the root of the mesentery.
hospitalization.
With other causes of acute obstruction, no symptoms ROLE OF RADIOLOGY AND IMAGING
may be present. If bilateral obstruction is present, anuria A. KUB and IVU

or oliguria is the earliest indication. Often, especially in In general, the sensitivity of plain radiography in detecting
inadvertent ligation of a single ureter, no detectable ureteral calculi to be approximately 45% and recommen-
change in urine output occurs. As a result, it remains ded that noncontrast helical CT replace the kidneys,
unrecognized for 10–30 days postsurgery, at which time ureters, bladder (KUB) film as the initial and possibly only
flank pain and fever alert the physician to this imaging study. While CT appears to have replaced plain
complication. radiography and IVU in the initial evaluation of acute
Sex: Urolithiasis occurs in a male-to-female ratio of 3:1. flank pain, these modalities remain involved in the follow-
Iatrogenic ureteral injury, mainly ligation, is more up evaluation of stone disease as well as surgical and
common in women, as it is most frequently observed interventional planning.
with gynecologic surgery. With the other etiologies, the Features on plain film and IVU: The plain abdominal
relative incidence likely is variable. radiograph or KUB film has long been the initial imaging
Age: Renal calculi usually appear in the third decade, study of choice in patients presenting with symptoms of
with a recurrence rate in untreated patients of 50% in acute flank pain. Calculi may have a variable appearance
10 years. on radiographs depending on their chemical composition,
especially the percentage and distribution of calcium
ANATOMICAL BASIS within them. They may appear homogeneously dense
The kidney is divided into a cortex and medulla. The and smooth to laminated and jagged.
renal pyramids are located within the medullary portion; While 90% of stones contain calcium, the sensitivity
they contain the loops of Henle and collecting ducts. At of plain films has been reported at only 50–60%, with
the apical portion of the pyramids, cone-shaped papillae a specificity of 70%. Therefore, in many centers, plain
convey urine from the distal collecting ducts into the minor radiographs are deferred in favor of CT in the acute
calyx. The peripheral projection of a minor calyx termed setting. In some instances, renal and ureteral calculi are
the fornix produces the characteristic cup-shaped detected on plain abdominal radiographs obtained for
appearance. another reason.
The minor calyces are arranged into 3 groups, the Most noncalcified stones, blood clots, and most other
upper, lower, and interpolar groups. Anterior and poste- intraluminal obstructive causes are radiolucent; therefore,
rior calyces are found within each group. Approximately they are not seen on abdominal radiographs. In trauma,
4–6 minor calyces converge into a major calyx or the presence of pelvic fractures and soft tissue mass in
infundibulum, which in turn forms the renal pelvis. The the abdomen or pelvis may alert the radiologist to the
fat-containing renal sinus envelops the entire collecting possibility of ureteral injury, a rare complication, or
system. obstruction from a large hematoma.
The pelvis tapers smoothly into the ureter at the IVU, also termed excretory urography, came into
ureteropelvic junction (UPJ). The ureter travels inferiorly clinical existence in the 1920s as a method of evaluating
along the psoas muscle, crossing over medially, anterior the structure and function of the kidney and urinary tract.
to the iliac bifurcation. It then descends posteriorly and It served as both a screening and diagnostic imaging study
laterally, inserting into the posteroinferior bladder wall in patients with acute renal or ureteral colic, demonstrating
at the level of the trigone. At the ureterovesical junction calculi and the presence of obstruction fairly accurately.
(UVJ), the ureter is most narrow; it also narrows at the Since 1995, noncontrast helical CT has superseded IVU
in this setting, at least as a screening study. Obstructive The pelvocalyceal system may not opacify for hours
uropathy demonstrated on unenhanced helical CT often or at all, depending on the severity of obstruction.
requires no further imaging studies. An estimated 15% Dilatation proximal to the obstructing process may be
of helical CT studies require radio-opaque contrast media seen as hydronephrosis and hydroureter, which may be
to confirm or exclude pelvic calcifications as stones or minimal in the acute situation. A standing column of
phleboliths. However, IVU may be performed if findings contrast may be observed proximal to the obstruction.
are equivocal or a noncalculus etiology is suggested. Pyelosinus extravasation of contrast and mild clubbing
IVU begins with a scout or preliminary radiograph. of the calyces also may be seen.
Additionally, oblique scout radiographs may be obtained. On an IVU, the findings of a persistent nephrogram
Pre-injection linear tomograms are helpful when the bowel and delayed passage of contrast through the urinary
pattern obscures the areas of interest. Following the system indicate with a high degree of confidence that
administration of the contrast agent, coned films and an obstruction is present. IVU is best performed following
tomograms are then obtained, with the intervals and total a screening study that produces either negative or
number of films being quite variable. In a normal kidney, equivocal results.
opacification of the renal parenchyma is observed within
1 minute. Within the next 2 minutes, the pelvocalyceal D/D of a Persistent Nephrogram
system may be seen, followed by the ureters approxi- 1. Pre-renal (15%)
mately 5–10 minutes post-injection. a. Renal artery stenosis
In an obstructed kidney, delayed accumulation of b. Hypotension
contrast is seen, resulting in a nephrogram that is initially 2. Renal (70%)
of lower density. Progressive concentration of contrast a. Glomerulonephritis
within the renal cortex and medulla occurs, producing b. Acute tubular necrosis
the classically described “obstructive nephrogram” that c. Papillary necrosis
may persist for some duration. Striations may occasionally d. Renal vein thrombosis
be seen in the parenchyma, representing contrast material e. Acute interstitial nephritis
within dilated tubules (Figure 7.2). 3. Post-renal (15%)
a. Obstruction.
Both false positives and false negatives are quite
common, as the sensitivity and specificity of plain
radiographs is poor. Small calcified stones, radiolucent
stones, clots, and mucosal edema are not visualized. In
addition, a large patient body habitus and overlying bowel
may obscure even densely calcified stones, resulting in
a false-negative interpretation.
Most false positives occur because of the presence
of other calcific densities within the perceived or expected
areas of the kidney and ureter. While gallstones usually
are multiple and have a faceted appearance, they may
be seen overlying the right kidney and, even on oblique
films, be indistinguishable from renal calculi. In the pelvis,
phleboliths often are misinterpreted as ureteral calculi.
The radiolucent center of a phlebolith may not always
FIGURE 7.2: IVU: Striated nephrogram on left side be present to help differentiate the two.
As reported in the literature, IVU sensitivity in the

evaluation of acute flank pain varies from 52–87%. The
detection of calculi as a cause for obstructive uropathy
depends on multiple factors, including stone size and
location as well as the degree of obstruction. As a result,
false-negative studies are not uncommon in the
urographic evaluation of calculi.
The presence of a standing column of contrast does
not always indicate the site of obstruction, as it may be
seen in normal individuals.
FIGURE 7.3: Right hydronephrosis due to small VUJ calculus
UROGRAPHIC FINDINGS IN ACUTE OBSTRUCTION
1. Spontaneous pyelosinus extravasation (up to 24% of B. Ultrasound
patients In the evaluation of acute flank pain, ultrasound (USG)
2. Increasingly dense ‘obstructive’ nephrogram’ is limited primarily to pregnant patients. While USG
2. Modest kidney enlargement (50% of patients) demonstrates renal calculi, it is poor at detecting ureteral
3. Delayed caliceal opacification stones.
5. Minimal to modest pelvicaliectasis. Features on ultrasound: The hallmark of obstruction
Limitations: Plain radiography and IVU have similar on USG is the presence of hydronephrosis (Figure 7.3).
drawbacks, including lower sensitivity because of Prominent anechoic structures within the renal sinus
superimposition of bone and other calcified structures represent a dilated pelvocalyceal system. Renal calculi
and obscuring bowel pattern. They are limited by stone also may be demonstrated as echogenic foci with or
size (<4 mm) and patient habitus. IVU, although without shadowing. This finding depends on the size of
providing both anatomic and physiologic information, the calculi, with smaller stones blending into the echogenic
has the added disadvantage of being time consuming renal sinus. Ureteral calculi and ureterectasis are detected
and dependent on administration of contrast material, less often. While UPJ and UVJ stones may be observed,
with its potential complications. It is also dependent on mid ureteral stones are extremely difficult to detect.
renal function, as the use of the test is greatly diminished Limitations: USG evaluates the ureters and detects
when the serum creatinine concentration is elevated when calculi poorly. The accuracy of USG in diagnosing
the patient has both intrinsic renal disease and an acute obstruction in the setting of acute flank pain was found
obstruction. to be approximately 66%. This is directly related to the
anatomic nature of the examination, which relies mainly
UROGRAPHIC FINDINGS IN CHRONIC OBSTRUCTION on the presence of hydronephrosis to make the diagnosis.
1. Renal size: Large in partial obstruction and small in In early obstruction, up to 36 hours, no dilatation of the
complete obstruction collecting system may be present, and a false-negative
2. Nephrographic density: Normal or decreased (may study may result.
increase with acute-on-chronic obstruction) The reported sensitivity of USG in the detection of
3. Parenchymal thickness: Reduced with or without a ureteral stone and associated obstruction is 60-70%.
‘Crescents’, ‘soap-bubbles’, ‘rims’, or ‘shells’ As a result, USG is not routinely used in the evaluation
Hydronephrosis of acute renal or ureteral colic. However, it is an important
4. Pyelogram: ‘Negative pyelogram’ on nephrogram screening examination in pregnant patients with acute
phase (Black pyelogram) and/or ‘Ball’ pyelogram flank pain and in acute renal failure. The gravid uterus
5. Ureter: Dilatation and tortuosity (low obstructions) and causes ureteric obstruction and a ureteric stone may be
mucosal striations may be seen. missed.
C. Color Doppler may be identified, along with the location and size of
It has been suggested as a method of indirectly deter- the stone. Secondary signs of obstructive uropathy,
mining obstruction by measuring the resistive index in including hydronephrosis, white pyramid sign, rim sign,
renal arteries and evaluating the direction and magnitude perinephric and periureteral stranding, as well as
of ureteral jets. Also twinkling artefact is a helpful Doppler ureterectasis, are well demonstrated on CT.
sign in cases of obstruction due to calculus disease. Several recent reports compare unenhanced helical
CT with intravenous urography (IVU), the established
Features on color Doppler imaging: Doppler
criterion standard in evaluating the genitourinary system.
sonography is an additional tool that may be employed
Advantages of NCSCT are many. CT has been shown
when radiation exposure is undesirable, such as in
to be more sensitive in detecting and characterizing
pregnant patients. The RI, which is a measure of
ureteral calculi and at least as sensitive in demonstrating
impedance to renal blood flow, may be raised when
the presence of obstructive uropathy. Additionally, CT
obstruction to urine outflow exists. In most centers,
may be performed rapidly, in approximately one third
obstruction is indicated by an RI greater than 0.70 or
the time of an IVU study, and does not require the use
a difference of greater than 0.10 between the kidneys.
of IV contrast material. The ability to diagnose noncalculus
However, any process causing decreased perfusion will
etiologies, as well as evaluate other intra-abdominal
result in an elevated RI. Ureteral jets also may be
pathologies that may mimic renal colic, affords CT an
evaluated; these may be absent or decreased in frequency
invaluable advantage.
in acute obstruction.
False positives/negatives: The presence of obstruction Features on CT:
is inferred by visualizing a dilated collecting system. This On unenhanced helical CT, calcified calculi appear
may be minimal or even absent in acute obstruction. In as in radiographs as opaque densities within the
addition, ureteral calculi are not well demonstrated. The genitourinary tract. Interestingly, calculi that are
false-negative rate is as high as 35% in patients with radiolucent on plain film also are of high attenuation on
uropathy secondary to a calculus lodged in the ureter. CT. Researchers have reported that Hounsfield values
Technical limitations to the study, such as patient obesity, may be used to differentiate the types of calculi, as this
bowel gas, and operator skill, also may result in a false- can provide the clinician information concerning an
negative study. underlying metabolic disorder as well as influence
The false-positive rate is approximately 10% in treatment decisions. However, the overlap in Hounsfield
patients with urolithiasis. The source of such errors values among the different stones makes this of limited
includes vascular calcifications that may simulate a use.
calculus. Peripelvic cysts, an extrarenal pelvis, or even These values become important in determining the
a normal collecting system may be misinterpreted as presence of an intraluminal blood clot, which appears
representing hydronephrosis. Doppler sonography can dense on CT but has a lower attenuation value than
help by readily distinguishing renal hilar vessels from a calculi. An exception to the normal high attenuation of
slightly dilated collecting system. calculi recently has been described in a small percentage
of HIV-positive patients being treated with Indinavir, a
D. Unenhanced Spiral Computed Tomography protease inhibitor. As the stones are of soft tissue
The advent of helical CT has dramatically altered the attenuation, they are not detected on non–contrast-
diagnostic imaging approach to patients presenting with enhanced CT.
acute flank pain. Unenhanced helical CT has both As mentioned previously, unenhanced helical CT is
a high sensitivity of 95-98% and a high specificity not a physiologic imaging study, and the sole finding of
of 96-100% in detecting ureteral calculi in the a stone is inadequate to make the diagnosis of obstruction.
acute setting. Both calcified and noncalcified calculi Therefore, secondary signs of obstruction as detected on
CT are necessary to confirm the diagnosis and obtain

an idea concerning the severity of obstruction. In the
presence of ureteral calculi, proximal ureterectasis is the
most commonly seen indirect sign. This also may be
observed with other causes of intraluminal obstruction.
Dilatation of the pelvicalyceal system is seen on CT
as anterior and medial bulging of the renal pelvis, which
is of low attenuation compared to the surrounding renal
parenchyma. Renal enlargement also may be observed
in some patients. Perinephric fat stranding, representing
engorged lymphatics and/or edema, is seen as linear
wispy densities in the normally low-attenuation fat.
Moderate-to-severe perinephric stranding generally
corresponds to the degree of obstruction present.
Periureteral stranding densities often are seen, again
representing edema in the surrounding fat. Other signs
of obstruction include the rim sign, which refers to the FIGURE 7.4: Coronal reconstruction: Severe right hydronephrosis
soft tissue attenuation halo around an intraureteral due to stricture in mid ureter
calculus, ureteral wall edema, and blurring of the renal

sinus fat. One or more of these secondary signs usually
is present, and the predictive values of the individual signs
have been reported in the literature. Ureterectasis, hydro-
nephrosis, and periureteral and perinephric stranding
each have a positive predictive value of approximately
90%.
Hydronephrosis and perinephric stranding may be
the only signs of obstruction in patients with inadvertent
ligation of the ureters, while the presence of an intra-
abdominal hematoma in a posttraumatic setting or an
inflammatory mass in a patient with abdominal pain
indicates a possible etiology (Figure 7.4). FIGURE 7.5: Sagittal section on CT: Pyelosinus extravasation
The administration of IV contrast material in the above
situations may help delineate the ureter and determine The largest source of false-positive errors in
if the obstruction is indeed secondary to extrinsic unenhanced helical CT results from phleboliths in the
compression. In many patients, IVU or direct visualization pelvis. On CT, the radiolucent center is not present to
help differentiate them from stones, and some stones have
of the urinary tract is needed to establish the diagnosis.
a lucent center. The false-positive rate in most studies
Contrast-enhanced CT in obstruction produces is approximately 4%. Administration of IV contrast may
findings similar to that first described with IVU, revealing help by outlining the ureter and separating it from
a prolonged parenchymal phase (Figure 7.4). The extraluminal densities.
persistent nephrogram, which appears either as a homo- The absence of secondary signs of obstruction, as may
geneous or mottled density kidney, results from delayed occur in early obstruction, can result in a false-negative
passage of contrast into the collecting system. Pyelosinus study, especially when no intraluminal or extraluminal
extravasation is well demonstrated on CT as areas of cause may be seen. The incidence of false negatives is
contrast material within the renal sinus fat (Figure 7.5). reported to be 0–6%.
Limitations: An inherent limitation of unenhanced Limitations: Doppler sonography is both operator

helical CT is in providing functional information. The dependent and time consuming. Determination of
severity of obstruction may be inferred by the presence ureteral jets and the resistive index has not been adopted
of perinephric stranding, which is believed to result from into widespread clinical use for acute obstruction because
forniceal rupture or inflammation in the perirenal fat from of technical limitations and questionable accuracy. Most
high calyceal pressures. The absence of this finding as pathologic processes decrease renal perfusion, thus
well as the other signs of obstruction does not exclude increase the resistive index (RI).
a significant obstruction. Features on RNI: Scintiscans and time-activity curves
Performing a contrast-enhanced CT may provide before and after administration of a diuretic (furosemide)
greater physiologic information by demonstrating an are obtained over a period of approximately 35 minutes.
obstructive nephrogram and pyelosinus extravasation of In a normal renogram, initial increased activity is seen
contrast material. An important consideration in choosing as tracer accumulates within the kidney. As tracer moves
this modality is the significant radiation dose to the patient, into the collecting system and ureter, the activity in the
compared to an intravenous pyelogram (IVP). This kidney starts to drop. In kidneys with dilated collecting
disadvantage restricts its use, especially in pregnant and systems, an up-slope in the time-activity curve occurs as
pediatric patients. tracer is retained within the kidney.
In patients with ureteral calculi, stone size alone Administration of furosemide in a nondilated kidney
determines initial treatment of patients, with no correlation results in a washout of tracer activity from the collecting
found between severity of obstruction as suggested by system, reflected as a down-slope on the time-activity
CT and the decision to treat conservatively or perform curve. T½ is defined as the time taken for reduction of
an intervention. The authors therefore suggest nonen- activity by 50%; a normal T½ is less than 10 minutes
hanced helical CT is adequate for the initial diagnosis with 99mTc MAG3.
and treatment of patients with stone disease. In a kidney with collecting system dilatation, a
decrease in activity with furosemide indicates a
E. Radionuclide Scan
nonobstructive cause of dilatation. In an obstructed
In nuclear medicine, the passage of radionuclide agents kidney, no response is observed following the injection
through the urinary tract is monitored with a gamma of furosemide. A prolonged T½, greater than 20 minutes,
camera. Outlines of the kidney and ureter are generated is highly indicative of obstruction. In addition, the
and a point of obstruction may be evident. More scintiscans, while providing the data for the time-activity
importantly, it provides physiologic information regarding curve, also may indicate the site of obstruction past which
the function and dynamics of the urinary system. The little or no activity is seen.
technique of choice in the evaluation of obstruction is To make this study definitive, renal function must be
diuresis renography, which is performed with technetium at least partially preserved. The glomerular filtration rate
99m
Tc labeled mercaptoacetyltriglycine (99mTc MAG3) in (GFR) must be greater than 15 mL/min for an obstructive
most centers. time-activity curve to be judged accurate. In patients
Diuresis renography: is not performed routinely in where the GFR is less than 15 mL/min, no response to
acute ureteral colic, as CT usually provides the necessary the administration of furosemide is observed. No washout
of activity occurs regardless of the presence or absence
information. However, it may be important in documen-
of obstruction. Thus, the study is deemed indeterminate
ting return of renal function in patients where urological
and further workup or intervention is required to make
intervention is required. Renography with Lasix is useful
the diagnosis.
in determining whether pelvocalyceal system dilatation,
often observed on a screening study such as USG, is Limitations: Diuresis renography, a physiologically
obstructive or nonobstructive in nature. driven study, depends on renal function. If marked
impairment of bilateral renal function is present, an

indeterminate study results. In patients who are
dehydrated, the expected response to the diuretic may
not be evident, leading to an inconclusive study.
F. MRU
Ultrafast T1 and T2 weighted techniques such as spoiled
gradient echo (T1) and single-shot fast spin echo (T2)
recently have been reported to be highly sensitive and
specific for determining the presence of both ureteral
calculi and acute obstructive uropathy. However, as with
US, in magnetic resonance urography (MRU) the degree
of dilatation must be sufficient to use the native urine
as a contrast agent (Figure 7.6).
Limitations: The use of MRU in the acute setting of
flank pain also is limited. Studies comparing MRU to CT
and IVU reveal that it misses small calculi and mild
dilatation. In addition, it is time consuming, expensive,
FIGURE 7.6: Left UVJ edema
and currently is limited to special situations, such as
pregnant patients.
in patients with staghorn calculi, which are commonly
INTERVENTION
infected and do not respond as well to other treatments.
Most calculi smaller than 6 mm in diameter pass In patients with obstructions with evidence of infection
spontaneously. Pain control and hydration usually are and/or sepsis, emergent percutaneous nephrostomy tube
all that is required in an uncomplicated situation. Larger placement serves to decompress the collecting system.
stones as well as stones lodged in the proximal ureter
are less likely to resolve on their own and require SPECIAL CONCERN DURING PREGNANCY
intervention. Treatment choice depends on the location Pregnant patient with acute flank pain from ureteral
and composition of the stone, presence of infection, obstruction presents a unique diagnostic and therapeutic
preceding treatment, and anatomy of the urinary tract. challenge. The incidence of an obstructing stone is
The placement of a ureteral stent by the urologist often approximately 1 in 1500 pregnancies. Compression from
is the primary intervention for ureteral calculi. If the the gravid uterus also may result in an obstructive
obstruction is not relieved, endourologic procedures such uropathy.
as retrograde ureteroscopy and lithotripsy may be The diagnostic imaging approach to these patients
attempted. Extracorporeal shock wave lithotripsy (ESWL) begins with an abdominal US to demonstrate the presence
commonly is performed on renal and ureteral calculi with of hydronephrosis. Remember that mild hydrouretero-
success but is contraindicated in obstruction, unless a nephrosis is observed in normal pregnant patients.
urinary diversion procedure allows for passage of the Transvaginal and transrectal US may be used to detect
stone fragments. distal ureteral calculi. Doppler sonography plays an
In ureteral obstruction, the interventional radiologist important role, using the RI and ureteral jets to diagnose
performs USG and/or fluoroscopic-guided percutaneous obstruction.
nephrostomy. As it provides access to the collecting Where USG does not provide a diagnosis, MRU or
system, nephroscopy, lithotripsy, and removal of large IVU may be the next step in imaging. MRU, if available,
renal calculi are possible. It is the treatment of choice is preferred, as no ionizing radiation exposure occurs.
One can make the diagnosis of an obstructing calculus recoverable renal function, even in the presence of severe
by the presence of a filling defect with associated dilatation of the collecting system.
perinephric or periureteral edema. Alternatively, an
extremely limited IVU may provide the necessary ADDITIONAL NOTE ON URETERAL OBSTRUCTION
information. The limited IVU consists of a preliminary Ureteral obstructions are commonly divided into intrinsic
film followed by a single postcontrast exposure to and extrinsic causes. Intrinsic causes include stones and
demonstrate the point and cause of obstruction. However, strictures, extrinsic causes include pregnancy, tumor,
the gravid uterus with the fetus can obscure a fibrosis, or iatrogenic. Patients often present with flank
nonpregnancy cause of hydronephrosis. pain. CT often will demonstrate the cause of the ureteral
As in other patients with ureteral calculi, management obstruction (i.e. calcification, tumor) and will often
is conservative, as most stones will pass with hydration. demonstrate secondary signs including hydronephrosis.
In patients with persistent symptoms, placement of a Patients will often have a delayed and persistent
ureteral stent or percutaneous nephrostomy is needed nephrogram depending on the grade of obstruction.
to relieve obstruction. This also may be necessary in Treatement involves re-establising the patency of the
patients with obstruction secondary to a gravid uterus, ureter often through stenting. Decompression can also
where conservative measures such as postural changes be obtained through percutaneous nephrostomy.
may be ineffective.
THE DIFFERENTIAL DIAGNOSIS OF
CHRONIC URINARY OBSTRUCTION (IVU SIGNS) HYDRONEPHROSIS
MORTALITY/MORBIDITY It includes both obstructive and non-obstructive causes.

Morbidity is related to the etiology, degree, and duration In the child, the most common cause of a dilated renal
of the obstruction. A stone that lodges in the urinary tract collecting system is ureteropelvic junction obstruction
is associated with excruciating pain and discomfort. usually from intrinsic dysfunction of the ureteral muscle.
Concurrent infection may produce both localized Other causes of the obstruction include ureterovesicle
sequelae, such as perinephric abscess, and systemic junction obstruction, ureterocele and posterior ureteral
manifestations including sepsis. Mortality can result valve.
from untreated superimposed infection. As the kidney
NON-OBSTRUCTIVE CAUSES
has no pain receptors, it is only with pressure distension
of the capsule, the site of the receptors, that pain is 1. Vesicoureteral reflux,
appreciated. 2. Congenital megaureter,
As opposed to the rim sign of hydronephrosis (in which 3. Eagle-Barret syndrome and
a rim of renal parenchyma enhances around a markedly 4. Polymegacalicosis (or congenital megacalyces)
dilated renal pelvis and collecting system, with 5. Pregnancy
enhancement of the cortical columns also frequently 6. Raped ureter.
seen), the crescent sign describes the appearance of The distinction between obstructive and non-
concentrated contrast material in the collecting tubules. obstructive causes can be made by diuresis renography
The collecting tubules are arranged parallel to the margin where the mechanically obstructed renal collecting system
of the dilated calyx, producing a thin line of contrast shows prolonged retention of radioactivity.
material that resembles a crescent. The change in tubule
DIFFERENTIAL DIAGNOSIS OF PRENATAL
orientation from vertical to near horizontal is thought to
(FETAL) HYDRONEPHROSIS
be the result of incomplete obstruction that produces a
gradual dilatation of the collecting system. UNILATERAL PATHOLOGY
It is important to distinguish the crescent sign from 1. Renal pelvic dilatation
the rim sign of hydronephrosis since the former indicates 2. VUR
3. Megaureter (with or without reflux) pelvis at 20 weeks gestation and a 10 mm pelvis in the
4. Multicystic dysplastic kidney third trimester or at full term. RPD is commonly unilateral
5. Complicated duplex kidney but may be bilateral; the importance of this distinction
6. Upper moiety dilatation - either ureterocele or ectopic is that if it is unilateral then there is no urgency in terms
drainage of investigation. The implication of this dilatation from
7. Lower moiety dilatation - usually VUR but rarely renal a management aspect is unclear since no natural history
pelvic dilatation only. studies have been undertaken.
All these infants are asymptomatic and their treatment
BILATERAL PATHOLOGY
has changed from operating early on most infants in the
1. Bilateral renal pelvic dilatation 1980s to a position where only about 25% now undergo
2. Bilateral VUR surgery in some teaching institutions. There are more
3. Bilateral megaureter (with or without reflux) children who are currently being followed expectantly so
4. Bladder pathology, e.g. neurogenic bladder
that a body of experience is building up suggesting both
5. Bladder outlet pathology (posterior urethral valves)
how to investigate and when to operate on these children.
6. Bilateral complicated duplex kidneys
The problem arises from the fact that the mere presence
7. Multicystic kidney on one side and cystic dysplastic
of isolated calyceal and renal pelvic dilatation does not
kidney on opposite side.
necessarily imply an obstruction that requires surgery.
Renal pelvic dilatation (RPD) can be defined
Obstruction is not easily defined physiologically but
postnatally as a kidney which on USG examination has
calyceal dilatation plus a dilated renal pelvis which the best definition may be a restriction to urine flow that,
measures greater than 10–15 mm in its AP diameter and left untreated, will cause progressive renal deterioration.
there is no USG evidence of a dilated ureter. This has The effects of obstruction are recognized as hydronep-
been termed PUJ obstruction or PUJ stenosis in the past. hrosis, parenchymal atrophy and impaired renal function.
The identification on antenatal USG is that of an AP renal These changes are the results of obstruction but do not
pelvis which is greater than 50% of the longitudinal length define or predict the potential for progressive renal
of the kidney. This equates to approximately a 5 mm deterioration.
Renal Infections 103
CLASSIFICATION The most common organisms are gram negatives. E. coli

ACUTE INFECTIONS is the most frequent organism, with Proteus, Klebsiella,
Staphylococcus saprophyticus, and Enterococcus being
1. Acute pyelonephritis
less common.
2. Pyonephrosis
3. Renal abscess Role of Radiology and Imaging
4. Emphysematous pyelonephritis.
The role of imaging is generally to define underlying
CHRONIC INFECTIONS pathologies like:
1. Xanthogranulomatous pyelonephritis • Obstruction
2. Renal tuberculosis • Reflux,
3. Schistosomiasis • Calculus
4. Malakoplakia. And to rule out complications like:
• Abscess
ACUTE INFECTIONS • Emphysematous pyelonephritis
• Changes consistent with chronic infection.
ACUTE PYELONEPHRITIS
IVP, CT, USG, can be normal in up to 75% of cases.
Introduction IVU
It is an infection of the upper urinary tract that usually • Enlarged kidney
occurs when colonic bacterial ascend from the lower • Striated nephrogram.
urinary tract to invade the renal parenchyma.
CT Findings
Causative Organism • Renal enlargement (edema); (Figure 8.1)
Acute pyelonephritis can be caused by hematogenous • Areas of decreased perfusion/enhancement; (Figure 8.2)
spread of bacteria or ascending urinary tract infections. • Stranding of the perirenal fat;
It may be a multifocal process and involve one or both • Striated nephrogram with contrast enhanced studies.
kidneys. It will usually respond to appropriate antibiotics. A striated nephrogram appears as alternating linear
FIGURE 8.1: Enlarged, inhomogenously enhancing right kidney with

patchy low density nonenhancing areas involving its parenchyma—
Acute pyelonephritis
FIGURE 8.4: Subcapsular perinephric infective collection
lucency/density parallel to tubules and collecting ducts

due to stasis of contrast material in dilated ducts
on a background of edematous renal parenchyma
(Figure 8.3).
• Perinephric collection can occur (Figure 8.4).
Differential diagnosis for a striated nephrogram:
(Pneumonic CHOIR C)
• Contusion;
• Hypotension;
• Obstruction (ureteral);
FIGURE 8.2: Focal nephromia in right kidney
• Intratubular obstruction;
• Renal vein thrombosis;
• Cystic diseases of kidney (infantile PCKD, medullary cystic
disease, medullary sponge kidney).
PYONEPHROSIS
Pyonephrosis refers to infection in an obstructed kidney
or accumulation of pus in an obstructed PC system.
Pyonephrosis can result in rapid destruction of the renal
parenchyma and must be treated promptly by relief of
obstruction by ureteral stent or nephrostomy tube
placement and antibiotics.
Ultrasound classically demonstrates a dilated
collecting system filled with layering echogenic ‘‘pus and
FIGURE 8.3: Left striated nephrogram–Acute pyelonephritis debris” (Figures 8.5 and 8.6).
enhancement (due to enhancement of the distinct

rounded and thick walls), a feature that distinguishes
abscess from fluid density renal cysts. Their contents
do not enhance.
• Alternatively abscesses may be ill-defined and
surrounded by zones of decreased parenchymal
enhancement, representing inflammation that has yet
not progressed to necrosis.
• Occasionally gas/air is found in it.
• Focal thickening of the adjacent fascia and stranding
in adjacent perinephric fat are common.
A perinephric abscess may ultimately result if the
infection extends out through the renal capsule.
FIGURE 8.5: PUJ obstruction with pyonephrosis—note
internal echoes
EMPHYSEMATOUS PYELONEPHRITIS (EPN)
Introduction
It is a life-threatening, fulminant, necrotizing upper urinary
tract infection associated with gas within the kidney and/
or perinephric space. Gas confined to the renal pelvis
should be called emphysematous pyelitis, and gas
confined to the perinephric space should be called
perinephric emphysema.
D/D of Air/Gas in PCS (Pelvi Calyceal System)

1. Retroperitoneal perforation of an abdominal viscus
2. Psoas abscess secondary to gas-forming organisms
FIGURE 8.6: Right hydronephrosis with pyonephrosis 3. Reflux of air from the bladder
and fungal balls 4. Fistulae (as may occur with xanthogranulomatous
pyelonephritis)
The preferred examination is CECT. On CT, patients
a. Bronchorenal
with pyonephrosis may have increased pelvic wall b. Enterorenal
thickness, inflammatory changes in the perinephric far, c. Cutaneorenal
and, in rare cases, layering of IV injected contrast agent 5. Air in a focal renal abscess (not life-threatening)
anterior to the pus in the dilated renal pelvis. PCS gas
in the absence of a history of urinary tract instrumentation, Incidence
is uncommon but strong diagnostic indicator of EPN is rare, but the frequency is higher in patients who
pyonephrosis. are immunocompromised, especially in patients with
diabetes, who account for 87–97% of patients.
RENAL ABSCESS
If inadequately treated, acute pyelonephritis may progress Predisposing Factors
to tissue necrosis, resulting in renal abscess. 1. EPN is usually a rapidly progressive and life-
threatening infection that is seen most commonly in
Features: persons with diabetes.
• On CECT, renal abscess usually have attenuation 2. EPN in a solitary, polycystic, transplanted kidney has
valves of about 30HU with or without peripheral rim been described recently, with a rare association with
xanthogranulomatous pyelonephritis. Often, multiple patients with creatinine levels greater than 1.4 mg/dL and
conditions are associated with EPN, such as poorly platelet counts of 60,000/mm3 or less are at high risk.
controlled diabetes, acidosis, dehydration, and The posttest probability of death increases from 69% to
electrolyte imbalance. 92% in type I EPN and 18% to 53% in type II EPN.
3. Females are affected twice as often as men, and Patients with creatinine levels of 1.4 mg/dL or less and
mortality rates can be as high as 80%. platelet counts greater than 60,000/mm3 are at much
4. Obstructive uropathy, urinary calculi, calyceal stenosis, lower risk. The posttest mortality risk in these patients
and neoplasms are significant predisposing factors. decreases from 69% to 27% and from 18% to 4% for
type I EPN and type II EPN, respectively.
Etiological Agents
The infecting organisms usually consist of mixed flora, Clinical Features
including: Patients usually present with chills, fever, flank pain,
1. Escherichia coli (68%), lethargy, and confusion. Septicemic shock may occur. A
2. Klebsiella pneumoniae (9%), crepitant mass may be present. Often, bacteriuria, positive
3. Proteus mirabilis and blood culture results, and leukocytosis are present. Patients
4. Other organisms include Pseudomonas; Enterobacter; are usually quite ill, but occasionally, the symptoms are
Candida; and, rarely, Clostridia species. mild, belying the severity of the disease. This is particularly
the case in persons with long-standing diabetes. The
Pathophysiology condition is bilateral in 5-7% patients.
EPN is an acute and chronic necrotizing pyelonephritis
Age: The average age of patients with EPN is 54 years.
with multiple renal abscesses. Mixed acid fermentation
of glucose by Enterobacteriaceae bacteria is the major Sex: The male-to-female ratio is 1:2.
pathway of gas formation.
Preferred Investigations
Types of EPN Plain abdominal radiography is the initial examination
Two subtypes of EPN based on CT appearances have of choice because it better depicts air in the renal collecting
been described. system and it is much more specific than ultrasonography.
However, in practice, sonography may be the initial
Type I EPN (33% of patients) is characterized by
examination performed.
parenchymal destruction with either absence of fluid
collection or presence of streaky or mottled gas radiating Features: Plain radiographs may show bubbles of gas
from the medulla to the cortex. A crescent of subcapsular within the region of the renal bed and in the upper renal
or perinephric gas may be present. The absence of fluid collecting system. These may be diagnostic in the
collection implies a poor immune response. The mortality appropriate clinical setting.
rate is high at 66%. Gas within the collecting system without evidence of
renal parenchymal gas may be seen in patients with
Type II EPN (66% of patients) typically has a confined
diabetes and does not have the same ominous prognosis.
bubbly intrarenal gas pattern, probably within abscesses
associated with renal and perinephric fluid collection, and Limitations: Plain radiographs are good for depicting air
gas within the renal pelvis. The mortality rate in type within the renal collecting system, but nonspecificity is
II is 18%. a problem because of the superimposition of gas from
Conversion from type I EPN to type II EPN has been the bowel. Moreover, gas in the retroperitoneum and gas
described. Wan et al have shown that serum creatinine within a renal or perinephric abscess may mimic EPN.
levels are the most reliable predictors of the outcome in IVU: Intravenous urography may be necessary if renal
patients with EPN. By calculating likelihood ratios, intervention is contemplated.
Features: Intravenous urography shows significant renal bronchorenal, and enterorenal, or cutaneorenal fistulae.
enlargement associated with delayed or absent excretion. These may occur with xanthogranulomatous pyelone-
Acute renal edema with obliteration of the renal pelvis phritis and focal renal abscesses.
can be seen. MRI
Limitations: MRI is not the modality of choice in the diagnosis of EPN.
When CT is available, it should be used instead.
USG: Features: MRI is not the modality of choice in the diagnosis
Ultrasonography is usually the first imaging modality for of EPN. MRI findings reported are a signal void on both
assessing renal pathology, but confirmation and better T1-weighted and T2-weighted images. Perinephric and
assessment entails the use of CT. intraparenchymal fluid collections are demonstrated well
on MRIs.
Findings: Intrarenal gas causes high-amplitude echoes
within the renal sinus/renal parenchyma associated with RNI: Because function is depressed or even absent on
dirty acoustic shadowing. the affected side, radionuclide studies are more
Ring-down artifacts may result from air bubbles appropriate for assessing renal function, particularly if
trapped in fluid. surgery is indicated.
Shadowing from gas bubbles in the perinephric space Findings: Radionuclide studies are nonspecific; therefore,
may be seen. These make visualization of the kidney they have a limited role in the evaluation of EPN.
difficult. However radionuclide study is an excellent modality for
Perinephric fluid, if any, tends to be obscured by gas. assessing differential function when nephrectomy is
contemplated. Scintigraphy has been used to evaluate
Limitations: USG is limited because gas within the kidney
responses to antimicrobial therapy.
and/or renal pelvis mimics renal calculi and produces
artifact due to reverberation echoes and shadowing. Limitations: The sole limitation of radionuclide imaging
is its lack of availability. Otherwise, it is an excellent
Computed tomographic (CT) findings are diagnostic modality, and it does not result in false-positive or false-
of the presence of air within the renal tract, and CT also negative diagnoses.
elegantly depicts the renal and perirenal anatomy and
the spread of infection to the perinephric tissues. Intervention
CT is the most reliable and sensitive modality Treatment involves aggressive antibiotic therapy, drainage
in diagnosing EPN. procedures to relieve obstruction, and prompt nephrec-
Features: tomy in life-threatening situations.
CT is the examination of choice for diagnosing With early diagnosis and the aggressive and prompt
administration of antibiotics, therapeutic success may be
EPN.
achieved in selected patients. Percutaneous nephrostomy
Intraparenchymal, intracalyceal, and intrapelvic gas
and percutaneous perinephric drainage may preclude
and extension into perinephric space are readily identified
nephrectomy in some patients. Occasionally, drainage
on nonenhanced CT scans.
procedures alone are successful; however most patients
Mottled areas of low attenuation extend radially along require immediate surgical intervention.
the pyramids.
Occasionally, pus may be seen extending into the renal Treatment
veins. It involves aggressive antibiotic therapy, drainage
Limitations: CT scans do not always depict other causes procedures to relieve obstruction, and prompt nephrec-
of intrarenal air, such as reflux of air from the bladder, tomy in life-threatening situations.
Mortality/Morbidity intervention are also key. Although nephrectomy offers

The mortality rate is 60–75% with antibiotic therapy and the best outcome, a trial of conservative treatment with
21-29% after antibiotic treatment and nephrectomy. drainage should be considered.
When the infection extends into the perinephric space,
the mortality rate increases sharply to 80%. CHRONIC INFECTIONS
XANTHOGRANULOMATOUS PYELONEPHRITIS (XGPN)
Summary
Emphysematous pyelonephritis (EPN) is a severe Introduction
infection of kidneys with accumulation of gas in the renal It represents an unusual suppurative granulomatous
parenchyma. The infection can be fatal if left untreated. reaction to chronic infection, often in the presence of
The mortality rate is 60-75% with antibiotic therapy and chronic obstruction from a calculus, stricture, or tumor.
21–29% after antibiotic treatment and nephrectomy. XGPN is characterized histologically by the presence of
When the infection extends into the perinephric space, foamy lipid-containing macrophages (xanthoma cells),
the mor tality rate increases sharply to 80%. diffuse infiltration with plasma cells, and histiocytes.
EPN is a rare condition, with a mean age of 55 years, XGPN is more common in women than in men;
and range of 19-81 years. Women are affected more often female patients with XGPN usually have a history of
than men. Ninety-five percent of patients have diabetes, recurrent or chronic urinary tract infections. Presenting
and of those, most are uncontrolled. Other factors include symptoms may include pyuria, flank pain, fever, dysuria,
immunocompromised state and ureteral obstruction/ pyuria, hematuria, proteinuria, or microscopic hematuria.
calculi. Other cases have been reported in patients with A palpable flank mass may be present, which may be
polycystic kidneys and end stage renal disease. tender or demonstrate costovertebral angle tenderness.
Interestingly, the left kidney is affected more commonly XGPN is rare in children.
than the right.
Forms
Escherichia coli is isolated in 66% of patients and
Klebsiella species are reported in 26%. Proteus, Two forms of XGPN are described:
Pseudomonas, and Streptococcus species have been also 1. Diffuse or global form (83–90%)
been reported. 2. Focal form (10–17%).
Patients typically present with fever, abdominal or XGPN has been termed the great imitator because
flank pain, thrombocytopenia, acute renal failure, shock, it may be misdiagnosed as a renal neoplasm, especially
altered sensorium, nausea and vomiting, or dyspnea. if the lesion is focal.
Laboratory data reveal leukocytosis with a left shift,
pyuria, infected urine, thrombocytopenia, an elevated Pathophysiology
creatinine level, and positive results from blood culture. XGPN almost always occurs unilaterally, and to the
Renal ultrasound images often reveal high echogenic author’s knowledge, only 1 patient with bilateral disease
areas (air) with dirty shadowing. Hydronephrosis and has been described. The kidney is involved either globally
perinephric fluid may also be seen. CT scan will show or focally. Changes of XGPN have been described in
kidneys with a streaky and mottled or bubbly appearance. kidneys destroyed as a result of pyonephrosis; in renal
Rimlike or crescent-shaped areas of gas may be found cell carcinoma; in transitional cell carcinoma; and, rarely,
in the perinephric area. Gas may also accumulate in the in a renal cyst. These focal pathologic changes are
adjacent structures, i.e. renal vein, inferior vena cava or detectable only by using histologic analysis, and they
along the psoas muscle. usually do not appear on images.
Fluid resuscitation and treatment with systemic On gross examination, the focal form of disease
antibiotics are cornerstone to initial patient management. usually is seen as a renal mass. On cut sections, the mass
Aggressive medical management and prompt surgical appears as a yellowish white, solid or semisolid structure
that is indistinguishable from renal cell carcinoma. In the kidneys require nephrectomy because of inflammatory
global disease, the kidney is enlarged, and indurated or conditions.
thickened perinephric fat is associated. The renal pelvis
Clinical Features
is dilated and often contains staghorn calculus. Soft,
yellow nodules replace the corticomedullary junction, and Sex: The male-to-female ratio is 1:3–4.
the calyces are filled with pus and debris. Age: Patients typically present at the age of 45–65 years,
Microscopically, both the focal and global forms but individuals of all ages may be affected, including
appear similar, with diffuse infiltration by plasma cells, infants.
histiocytes, and lipid-laden foam cells. The foam cells The typical patient is a middle-aged obese diabetic
contain neutral fat and cholesterol (ester granules) that female with a staghorn calculus, recurrent fever, dysuria,
test positive for periodic acid-Schiff (PAS) stain. PAS and flank pain unresponsive to antibiotics. Other
staining is useful in differentiating these cells from the symptoms include dysuria, pyuria, hematuria, proteinuria,
clear cells found in renal cell carcinoma. or microscopic hematuria. A palpable flank mass may be
Laboratory testing frequently shows an elevated present, which may be tender, or the patient may exhibit
erythrocyte sedimentation rate, leukocytosis, elevated costovertebral angle tenderness. XGPN is rare in children.
gamma-globulin levels, and anemia. Proteinuria and Frequently, urinary symptoms, fever, and flank pain
pyuria are frequent. Urine cultures may reveal Proteus are absent, and minimal, if any, leukocytosis is found;
mirabilis, Escherichia coli, Staphylococcal aureus, and therefore, excluding renal cell carcinoma is difficult on
Klebsiella, Pseudomonas, and Enterobacter species. Urine clinical grounds. Abnormal results in liver function tests
cultures may show negative results in 30–39% of patients are reversible in as many as 50% of patients and
despite positive results from the kidneys. Occasionally, occasionally are associated with hepatomegaly. Patients
different organisms may be isolated from urine and from (40%) are often symptomatic for 6 months before XGPN
the removed kidney, underlining problems with is diagnosed; however, the reported duration of symptoms
antimicrobial therapy. prior to presentation is extremely variable, extending from
Abnormal results in liver function tests are found in 3 days to 8 years.
as many as 50% of patients, and these are occasionally
associated with hepatomegaly. The cause of the liver Role of Radiology and Imaging
abnormalities is not known, but results of liver function Classic excretory urographic triad of XGPN:
tests return to normal after treatment. Inflammation 1. Obstructing stone
resulting from XGPN may extend into the perirenal space, 2. Renal enlargement
pararenal space, ipsilateral psoas muscle, colon, spleen, 3. Nonexcretion of contrast material from the involved
diaphragm, posterior abdominal wall, and skin. kidney.
Stages Plain KUB radiograph

Radiographic findings confirm the presence of renal
XGPN has been described in three stages, as follows:
calculi.
Stage 1: The lesion is confined to the kidney.
A plain scout radiograph obtained before intravenous
Stage 2: The pathologic process extends to the Gerota urography typically shows a staghorn calculus.
space. Further smaller calcifications may be seen scattered
Stage 3: The process spreads to the paranephric space throughout the renal area, and a soft-tissue mass may
and other retroperitoneal structures. be identified.
If present, perinephric extension may produce ill-
Incidence defined renal margins, and a large soft-tissue mass may
In the USG: XGPN is found in 1–18% of patients in whom be seen occupying the renal fossa.
A thickened Gerota fascia is occasionally demon- Renal cortical tissue is markedly thinned and may have
strated. scattered cystic collections representing abscesses.
The obstructing calculus usually does not create
IVU
shadowing.
After the administration of contrast material, findings
Although the ultrasonographic features are not specific
depend on the morphologic type of XGPN. An absent
to XGPN, the failure to depict a normal kidney associated
nephrogram or focally absent nephrogram may occur.
with a staghorn calculus suggests the diagnosis. Ultrasono-
On nephrotomography, some opacification of the
graphic results are not routinely helpful in identifying renal
kidney may occur, and a mass may be seen. A central
calculi, and sonography is less sensitive than other
opacified mass may correspond to a xanthoma.
modalities in demonstrating extrarenal spread.
When the inflammatory process extends into
perinephric tissues, the renal outline is obscured. CT Scans
Inflammation from XGPN may extend into the perirenal It demonstrates retroperitoneal involvement more clearly
space, pararenal space, ipsilateral psoas muscle, colon, than do other images.
spleen, diaphragm, posterior abdominal wall, and/or skin.
Findings: CT is the most useful investigation for the
This inflammation may obscure the renal outline.
preoperative assessment of XGPN. CT findings depend
Focal disease may appear as any other renal space-
on the morphologic type of XGPN.
occupying lesion, especially when it is not associated with
In diffuse disease, CT scans may show a staghorn
a calculus. The focal lesion usually shows no function
calculus within a nonfunctioning kidney.
or a patchy nephrogram; this may be associated with
Although the reniform shape is maintained, the renal
splaying and stretching of the calyces.
parenchyma is destroyed and is replaced by low-attenua-
Retrograde pyelography ting masses. The attenuation values in these masses vary
It may show a complete obstruction at the ureteropelvic from –10 to 30 HU, depending on the lipid content.
junction, infundibulum, or proximal ureter. The renal sinus may not be identifiable.
If a focal, solitary noncystic lesion is seen. The Fine calcifications are occasionally discerned within
differential diagnosis is that of a solid mass. the xanthomatous masses.
Small gas collections are sometimes seen in intrarenal
Ultrasonography
abscesses.
Is the initial examination in most patients of any age who
The Gerota fascia is invariably thickened and
have urinary symptoms.
identified.
Findings: Extrarenal extension of XGPN is well depicted on CT
Renal ultrasonographic findings demonstrate an enlarged scans and has been described both in the diffuse form
kidney that tends to maintain the reniform shape. and the focal form.
Loss of corticomedullary differentiation occurs. Enhanced CT images show ring enhancement in areas
Parenchymal calcification is uncommon, but when of granulation tissue; the xanthomatous tissue does not
present, it appears as central echogenic foci with enhance.
associated acoustic shadowing. Definition of cortical renal abscesses is improved on
The renal pelvis is small due to fibrosis, but hypoechoic enhanced images.
dilatation occurs in calyces, which have an echogenic In focal XGPN, a large mass is identified in an
rim. otherwise functioning kidney. The mass usually is low
The renal parenchyma is replaced by hypoechoic attenuating and fails to enhance on CT scans; however,
masses, which frequently have low-level internal echoes. rim enhancement may be seen.
Sound transmission is usually not enhanced because Calculus is often associated with the central portion
the hypoechoic areas are not simple fluid-filled spaces. of the mass.
None of the features described above are characteristic encased or occluded, but inferior vena cava thrombosis
of XGPN, but they are sufficiently typical of the diffuse has not been described.
form of XGPN to suggest a preoperative diagnosis in the Angiography no longer plays a diagnostic role in
appropriate clinical setting especially by low CT values. XGPN, although the authors occasionally use angio-
Focal disease cannot be diagnosed with confidence by graphy to characterize focal renal masses or to plan
using radiologic images, and they should be regarded surgery. The invasive catheter technique is being replaced
as malignant until proven otherwise. The importance of with CTA and MRA with reformatting images.
urine cultures in assessing an unexplained renal mass Isotopic renography
cannot be overemphasized. It is useful in demonstrating relative renal functions prior
MRI to surgery.
Documented findings include the presence of a large mass
Limitations of the Modalities in General
in the renal fossa that appears multiloculated. Abscesses
and calyces demonstrate intermediate signal intensity on No radiologic features are characteristic for XGPN, but
T1weighted images and high signal intensity on T2 in the diffuse form, some CT and ultrasonographic fea-
weighted images. Calcification and renal calculi appear tures may be sufficiently typical to suggest a preoperative
as signal voids. MRI appears to be extremely sensitive diagnosis in the appropriate clinical setting. Focal disease
in outlining perinephric spread. Residual parenchyma in cannot be diagnosed with confidence radiologically and
should be regarded as malignant until proven otherwise.
the unaffected kidney may appear normal.
MRI findings have been inconsistent, with varying
Differential Diagnosis
degrees of signal change described in areas involved by
the inflammatory process compared with normal 1. Renal fungal infections
2. Hydronephrosis
uninvolved renal tissue. In general, CT findings appear
3. Avascular tumor (on angiography)
to be more helpful than those obtained with other
4. Pyonephrosis
modalities.
5. Cystic renal carcinoma
Angiography 6. Lymphoma
It is occasionally performed to characterize a focal renal 7. Renal tuberculosis.
mass further and to plan surgery. CT angiography and
magnetic resonance angiography are replacing conven- Intervention
tional techniques. Needle biopsy may be helpful in differentiating XGPN
The features described in global disease include from other renal masses. Percutaneous nephrostomy may
stretching of segmental and/or interlobular arteries around be necessary to treat patients presenting with acute
large avascular masses, similar to those seen in hydro- septicemia due to an obstructive form of the disease
nephrosis. Attenuation occurs in major renal arteries, and associated with pyonephrosis.
peripheral vessels lack arborization. Inhomogeneity of the
nephrogram may mimic hydronephrosis. In the late Treatment
arterial phase, hypervascularity or arterial blush may be The treatment of patients with XGPN usually involves
noted around the periphery of the masses due to intensive antimicrobial therapy, but surgery is invariably
granulation tissue. required to completely eradicate the infection and the
The focal form of XGPN is usually hypovascular. accompanying calculus and/or obstruction. Kidney-
Intrarenal veins are stretched and compressed by sparing surgery may be undertaken in patients with focal
inflammatory renal masses. The renal vein may be disease.
RENAL TUBERCULOSIS irregular cavities. The cavities usually communicate with

Introduction the renal collecting system, generally a calyx, with
formation of fistulae and stricturing. Eventually, the kidney
The genitourinary tract is the second most common site
may become fibrotic and scarred.
for tuberculous infection after the lungs. The infection
The course of renal tuberculosis may be indolent, with
almost always affects the kidneys during the primary
the appearance of few, if any, symptoms. Presentation
exposure to infection but does not present clinically. The
is usually late and symptoms usually occur as a result
spread to the kidneys usually is hematogenous from the
as nonspecific urinary tract infection. Constitutional
lungs, bone, or a GI tract focus. The true incidence of
symptoms usually do not occur or are sparse. Renal
renal tuberculosis may be underestimated since radiologic
tuberculosis is bilateral although radiologic findings are
findings may be absent and diagnosis is made by urine
asymmetric and unilateral in 25% of patients. Ultimately,
culture. Genital tuberculosis is usually secondary to renal
the kidney becomes atrophic, scarred, densely calcified,
tuberculous infection.
and nonfunctioning (autonephrectomy) if not
Renal involvement may be indolent, with a latency
appropriately treated. Ureteric involvement occurs as a
period of more than 20 years after the primary infection
descending infection secondary to kidney infection.
to the appearance of urinary tract symptoms of hematuria
Tubercles may involve the transitional epithelium, causing
and stone disease. In patients with renal tuberculosis,
mucosal granulomas that project into the ureteric lumen.
treatment involves antituberculous drugs, with surgical
Eventually, fibrosis occurs in the ureter. These pathologic
excision as an adjunct to antituberculous therapy. The
processes can be demonstrated radiologically by the
urine can be free of bacteria in less than 72 hours but
appearance of a beaded, saw-toothed, corkscrew, or
anatomic changes can progress as part of the healing
pipestem ureter depending on the stage of disease.
process. Females with genital tuberculosis may present
Usually, the upper and/or lower third of the ureters are
with infertility, menstrual disorders, and pain. Pregnancy
is unusual in the presence of genital tuberculosis. When involved. The vesicoureteric junction may become fixed
pregnancy occurs, spontaneous abortion or ectopic and patulous, allowing vesicoureteric reflux. The kidneys
pregnancy usually results. As a result of the lack of clinical are always involved when ureteric tuberculosis is present.
features, diagnosis of genital tuberculosis may be difficult. Bladder tuberculous infection is almost always
secondary to renal involvement. Initially, interstitial cystitis
Pathophysiology occurs, eventually causing bladder mucosal ulceration and
Tuberculosis of the kidneys usually spreads by a thickening of the bladder wall. End-stage disease causes
hematogenous route from pulmonary disease, although scarring and bladder fibrosis, resulting in diminished
it occasionally may be secondary to tuberculosis of the capacity of the urinary bladder. Bladder wall calcification
GI tract or bone. By the time of diagnosis of renal is uncommon. Bladder tuberculosis may be complicated
tuberculosis, the primary source of pulmonary infection by fistulae or sinus tract formation, although these
may be inactive or calcified. True prevalence of renal complications are rare.
tuberculosis is underestimated since radiologic signs may Tuberculosis of the seminal vesicles usually occurs as
be absent. Moreover, tubercle bacilli are found in 7–29% a result of hematogenous spread. Descending infection
of urine samples in patients with extrarenal tuberculosis. is unusual. The same pathologic processes occur as within
The initial renal focus is usually a small tubercle in the the bladder (ie, mucosal tuberculomas, ulceration,
glandular and cortical arterioles. With the passage of time, fibrosis). Calcification is present in only 10% of patients.
these lesions progress to form necrotizing lesions. The Unlike seminal vesicle tuberculosis, tuberculosis of the
disease spreads to the renal tubules and renal medulla prostate is usually secondary to descending infection from
in which further tubercles develop, usually at the turn the kidney. However, the kidneys may occasionally
of the loop of Henle, coalescing into larger necrotic appear normal, suggesting subclinical infection or a
hematogenous prostatic infection. The tuberculous Renal tuberculosis may remain dormant for many
cavities or abscesses may discharge into the surrounding years after the kidneys become seeded during the primary
tissues forming sinuses or fistulae to the perineum or tuberculous infection. With reactivation, one or more renal
rectum, eventually resulting in a watering-can perineum. abscess is produced. Patients usually become
The scrotum and urethra may be involved, although symptomatic, with extension of the disease to the renal
rarely. Urethral involvement may be complicated by pelvis and ureters causing hydronephrosis. Specific
urethral strictures. symptoms may be lacking until the hydronephrotic kidney
Tuberculosis may cause chronic epididymitis and becomes secondarily infected.
epididymoorchitis. Tuberculous granulomas may develop Symptoms of frequency and urgency of urination and
within the testes and epididymis and rarely may be dysuria may ensue, with development of tuberculous
complicated by abscesses and discharging sinuses. cystitis. However, long before patients become
Thickening of the scrotal wall and tunica albuginea and symptomatic, sterile pyuria, albuminuria, and hematuria
moderate hydrocele also may be observed occasionally. are present but cultures for pyogens demonstrate negative
Female genital tuberculosis is invariably secondary results. Diagnosis usually is achieved using imaging,
to tuberculosis elsewhere, and spread may be hemato- cystoscopy, and culture of acid-fast bacilli from early
genous, via the lymphatic system, or by direct spread morning urine specimens. Needle aspiration biopsy is a
from adjacent organs. Patients usually present with last resort when urine cultures are negative.
infertility, menstrual irregularity, and pain. Pregnancy is
rare in the presence of genital tuberculosis and is often Preferred Examination
complicated by ectopic pregnancy or spontaneous
abortion. Plain KUB
Plain radiography may provide a clue to the diagnosis
Incidence and may guide further imaging.
The urogenital tract is the second most common site of Since the type and distribution of calcification may
tuberculosis after the lung. Renal tuberculosis is associated be suggestive of tuberculosis, CT scans (with the ability
with active pulmonary tuberculosis in 4–8% of patients. to depict calcification) may be helpful.
Genitourinary tuberculosis appears to be fairly common IUV
in developing countries. While intravenous urography remains the primary
modality used to image patients with renal, ureteric, and
Clinical Features
bladder tuberculosis, findings of urinary tuberculosis are
Race: Incidence of renal tuberculosis varies throughout also detectable on ultrasound, CT scan, or MRI.
the developing world where the infection is common. The While intravenous urography is the primary modality
disease is more common in higher socioeconomic groups, for imaging renal tuberculosis, CT scans clearly reveal
similar to the pattern found in Europe. Renal tuberculosis changes of renal tuberculosis, particularly in advanced
is uncommon in tropical Africa despite the fact that other disease. Changes such as calcification, calyceal dilatation
forms of tuberculosis are common. High prevalence is without a hydropelvis, parenchymal loss, and extrarenal
observed in Eastern Europe, Asia and, particularly, spread are well depicted.
Bangladesh, India, and Pakistan. On the Indian
subcontinent, renal tuberculosis is associated with Radiological Findings
diabetes.
Findings in the kidneys
Sex: Males are affected more often than females. IVP is abnormal in 85–90% cases and gold standard in
Age: Individuals of any age can be affected, but most diagnosis of Renal TB Distinctive features of Renal TB
patients present younger than 50 years. on IVU:
1. Moth-eaten Calyx “smudged” papillae (the earliest 18. Once communication with a tuberculous cavity is
sign) and diminished excretion of contrast, focally established, the involved calyx becomes an
or generally. ulcerocavernous lesion.
2. Hydrocalicosis with infundibular strictures (most 19. The finding of hydrocalyces with no pelvis dilatation
common sign) or an atrophic pelvis is highly suspicious for
3. Caliceal truncation tuberculosis. The cephalic retraction of the inferior
4. Phantom calyx/amputed calyx medial margin of the renal pelvis at the PUJ, or
5. Kerrkink sign (kinking of renal pelvis) “the hiked up renal pelvis,” is another suggestive
6. Parenchymal calcifications (amorphous/granular/ urographic/pyelographic change.
curvilinear/punctate/confluent tooth paste) involving 20. The infection spreads to the stem of the involved
entire kidney (putty kidney) calyx, which may develop a stricture, thus sealing
7. Cavities communicating with collecting system off the involved calyx (phantom calyx). This
8. Nonfunctioning kidney with dystrophic calcification change may be apparent as a mass lesion.
9. Lobar calcification (unique feature) 21. Dilated calyces often are associated with
10. Putty kidney (Tuberculous pyonephrosis) infundibular strictures and may be demonstrated
11. Autonephrectomy radiologically. The lesions may be depicted better
12. Nephrolithiasis on cross-sectional imaging.
22. If the ulcer or stricture extends to the renal pelvis
13. Signs of extrarenal active or inactive tuber-
or the pelvic ureteral junction, urine outflow ob-
culosis may be apparent, such as osseous or
struction may occur. In this instance, intravenous
paraspinal changes of tuberculosis and old healed
urogram may show delayed function, clubbed
calcified splenic, hepatic, lymph node, and adrenal
calyces, or absence of function. At this stage, a “putty”
granulomas. Chest radiographs may show evidence
kidney may be depicted. Ultrasound, CT scan, and
of active or healed tuberculosis in 50% of patients.
MRI can better depict an outflow obstruction.
The remainder have normal chest studies.
23. If tuberculous infection extends directly to the rest
14. Changes of renal tuberculosis are unilateral in
of the kidney, the entire kidney becomes a bag of
75% of patients.
caseous necrotic pus.
15. A tuberculoma usually starts as a localized
24. Plain radiographs may reveal dystrophic
caseating lesion, most commonly in the upper pole
calcification, but intravenous urography usually
of the kidney, although it may arise anywhere.
shows absence of function, although a faint
16. With time, the nidus of infection enlarges and nephrogram may be demonstrated if some function
ruptures into a neighboring calyx, discharging remains.
necrotic caseous material and distorting the calyx. 25. Associated renal calculi are found in as many
At this stage, a variety of radiologic abnormalities as 20% of patients with renal tuberculosis.
may be demonstrated, including smudged 26. The final outcome of renal tuberculosis is
papillae due to surface irregularity of the papillae, autonephrectomy, which represents a small,
a moth-eaten calyx (early sign), irregular tract shrunken, scarred, nonfunctioning kidney and
formation from the calyx to the papilla, and large is often associated with dystrophic calcification.
irregular cavities with extensive destruction 27. Renal calcification is present in as many as 50%
secondary to papillary necrosis. Changes may be of patients and may appear as an amorphous
detected on intravenous urography, retrograde granular opacification associated with active
pyelography, and on some CT and MRI scans. granulomatous infection and dense punctate
17. The kidney enlarges initially but subsequently calcification, which is associated with healed
may return to normal or become atrophic. tuberculomas.
28. Shell-like/egg-shell calcification within the 7. Symmetrical small spastic, and thickened bladder or
renal collecting system and a thinned-out renal small multilobular bladder (Thimble bladder). As
cortex are more a feature of autonephrectomy. compared to tuberculosis of kidney, that of bladder
29. Renal infection may extend into the perirenal or is rarely associated with calcification. Usually bladder
pararenal spaces and/or the psoas sheath. calcification in schistosomiasis is linear and continuous
and occurs chiefly at the bladder base going on to
Findings in the ureters
encircle the bladder lumen.
1. Although ureteral involvement is usually unilateral,
bilateral changes are asymmetric when they occur. Findings in the prostate
The most common site of involvement is the lower 1. Prostatic tuberculous cavities or abscesses may
third of the ureter. discharge into the surrounding tissues, forming sinuses
2. Mucosal granulomas may be demonstrated as or fistulae to the perineum or rectum and eventually
intraluminal filling defects on intravenous urogram or resulting in a watering-can perineum. These changes
retrograde pyelography. are demonstrated best on MRI scans.
3. Mucosal ulceration is difficult to depict radiologically 2. Plain radiographs may show dense calcification within
but rarely may show as irregularity in the contrast- the prostatic bed, which can also be demonstrated
filled ureter. on ultrasound and CT images.
4. The involved ureter may appear beaded or with a 3. Urethrography or a micturating cystourethrogram
corkscrew configuration as a result of alternating typically demonstrates filling of variable dilated
dilatations and strictures. prostatic ducts associated with destruction of the
5. Ultimately, the ureter may form a rigid, aperistaltic, prostatic parenchyma.
shortened tube. 4. Sloughing and irregular cavitation of the prostate
6. TB of ureter produces mucosal and wall ulceration, eventually may result in a smooth-walled cavity that
fibrosis, stricture and calcification. replaces the prostate.
Saw tooth ureter, beaded ureter, short, straight, rigid
Findings in the seminal vesicles
and narrow ureter especially terminal segment.
1. Calcification is depicted on plain abdominal
Calcification is however rare in ureteral tuberculosis
radiographs in as many as 10% of patients with
and when occurs involves lower segment to.
tuberculosis of the seminal vesicles.
Findings in the bladder 2. Calcification of the seminal vesicles is more common
1. When tuberculosis involves the bladder, progressive in patients with diabetes mellitus.
thickening of the bladder wall occurs with increasing
Findings in the epididymis and vas deferens
diminution of bladder volume.
1. Calcification of the epididymis and vas deferens may
2. Trabeculation may develop.
be visualized on plain radiographs of the male pelvis
3. The vesicoureteric junction orifice may become fixed
and must be differentiated from diabetes and schisto-
and patulous, resulting in vesicoureteric reflux.
somiasis.
4. The vesicoureteric junction may be affected by
progressive narrowing, causing stenosis and resulting Findings in female genital tuberculosis
in bilateral hydroureters and hydronephrosis. 1. Tuberculosis can affect any part of the female
5. Bladder wall calcification is rare. genital tract but more commonly involves the
6. Bladder tuberculosis may be complicated by fistulae fallopian tubes.
or sinus tract formation although these complications 2. Hydrosalpinx and pyosalpinx are usually large and
are rare and are demonstrated better on CT and MRI may appear as soft tissue masses on plain abdominal
scans. radiographs.
3. The pyosalpinx may be calcified. awareness of sonographic changes associated with

4. Tuberculous tuboovarian abscesses may calcify, are tuberculous infection may improve diagnostic accuracy
observed on either side of the pelvis, and sometimes and help avoid clinical mismanagement and surgical
appear as well-defined homogeneous masses, explorations in patients with genital infections associated
occasionally with areas of increased density with wet-type tuberculosis (peritonitis).
presumably due to the granuloma. Serpiginous or
Findings: Sonography is not as sensitive as intravenous
linear calcification can occur in the tubes.
urogram or CT scanning because of problems with
5. HSG findings may suggest a diagnosis. HSG
identifying calyceal, pelvic, or ureteric abnormalities.
appearances vary as widely as the pathologic
The kidney may appear entirely normal in both early
changes observed in this condition.
later stages of renal tuberculosis; later, hypoechoic/cystic
6. Tubal involvement is almost always bilateral but the
masses communicating with the collecting system may
degree of involvement varies between the two sides.
be observed, representing “excluded” calyces without
7. HSG may demonstrate a flask-shaped dilatation of
dilatation of the renal pelvis.
the fallopian tubes due to obstruction at the fimbria.
Large abscesses may distort the renal contour and
Occasionally, the obstruction is at the uterine end
may mimic tumors or cysts.
of the tube; therefore, the tubes are not depicted.
Usually, renal tuberculomas appear as a solid mass
8. HSG may demonstrate sacculation with infiltration
with diminished through transmission; however, a diffuse
of contrast material resembling salpingitis isthmica
infiltrative type of renal tuberculosis has been described
nodosa. Infiltration around the tube, which creates
in which the kidney may appear normal on ultrasound
a cloudlike appearance of the delicate sinus tracts,
images.
has also been described.
Fibrosis and scarring may appear identical to chronic
9. A characteristic HSG feature suggestive of
pyelonephritis or multiple renal infarcts.
tuberculosis has been described in which irregular
Calcification is common in the late stages and varies
contrast distribution occurs resembling a cotton-
from punctate foci to dense calcification of the entire
wool plug. Focal irregularity and areas of
kidney, which is associated with hydronephrosis or
calcification may occur within the lumen of the
atrophy.
fallopian tubes.
Bacille Calmette-Guérin (BCG) delivered intravesi-
10. Obstruction of the fallopian tubes is not
cally in the treatment of superficial bladder cancer may
pathognomonic for tuberculosis and may occur with
be complicated by a renal granuloma, probably as a result
other pathology.
of vesicoureteric reflux. The granulomas have been
11. In end-stage disease, the tubes become rigid, lack
reported as small intrarenal hypoechoic masses.
peristalsis, and resemble pipelike conduits.
Bladder tuberculosis causes fibrosis and mucosal
12. Within the endometrial cavity, tuberculous endo-
thickening, leading to a thick-walled small-volume bladder
metritis findings include adhesions, which may vary
with vesicoureteric reflux.
from very thin to very thick synechiae. In end-stage
Tuberculosis is an unusual cause of chronic epididy-
disease, the uterine cavity may be obliterated
mitis and epididymoorchitis. In tuberculous epididymitis,
completely.
the epididymis may appear heterogeneous and hypo-
13. Within the pelvis calcified lymph nodes and ovaries
echoic, associated with concomitant hypoechoic lesions
may be observed.
in the testes and a discharging sinus. The most notable
USG finding is an enlarged and heterogeneous epididymis,
Sonographic findings in the appropriate clinical setting predominantly in the body and tail.
may help avoid orchidectomy for benign testicular Testicular involvement is shown as a diffusely
disease. In patients with female genital tract tuberculosis, hypoechoic testis or focal intratesticular areas.
Thickening of the scrotal wall and tunica albuginea and 3. Brucellosis also may mimic tuberculosis.
moderate hydrocele may be observed occasionally. 4. The differential diagnosis of an adnexal mass is wide.
Follow-up scans may reveal intratesticular abscesses. The 5. A congenital megacalyx and focal papillary necrosis
testes may become as hard as stone, which is associated may mimic renal tuberculosis radiologically. Papillary
with extratesticular calcification. necrosis can result from tuberculosis.
Sonographic findings may suggest genitourinary 6. A tuberculous testicular granuloma may mimic a
tuberculosis in the appropriate clinical setting and findings testicular neoplasm on ultrasound images.
may help eliminate the treatment choices using renal 7. Small areas of calcification are difficult to detect on
surgery or orchidectomy for benign disease. In patients MRI scans although they are pivotal to the diagnosis
with female genital tract tuberculosis, awareness of of tuberculosis.
sonographic changes associated with the infection may 8. HSG findings are also nonspecific; blockage of the
improve diagnostic accuracy and avoid clinical mismana- fallopian tubes is not pathognomonic for tuberculous
gement and surgical explorations in genital infections salpingitis and may occur as a result of other forms
associated with wet-type (peritonitis) tuberculosis. of infective processes of the genital tract.
CECT RNI
CT scans may demonstrate dense prostatic calcification Isotope renography is the most sensitive imaging modality
in tuberculous prostatitis, sloughing, and irregular available for the assessment of renal function.
cavitation of the prostate, eventually resulting in a smooth-
Findings: The role of radionuclides in imaging patients
walled cavity that replaces the prostate.
with renal tuberculosis is confined to assessment of relative
Since the type and distribution of calcification features
renal function by renography when surgery or nephrec-
may be suggestive of tuberculosis, CT scans (with the
tomy is contemplated. The agents used are technetium
ability to depict calcification) may show relatively specific 99m
Tc diethylenetriamine pentaacetic acid (DTPA),
findings. While CT scans clearly demonstrate changes
technetium 99mTc mercaptotriglycylglycine (MAG-3), or
of advanced disease, sensitivity in early disease may be
iodine I 123 orthoiodohippurate (OIH).
low because scans do not demonstrate the detailed
calyceal anatomy. Mortality/Morbidity
MRI Untreated, the end result of renal tuberculosis is auto-
It is useful when fistulae or tuberculous tracts are formed. nephrectomy. The exact incidence of infertility in patients
Findings: MRI is good at depicting tuberculous cavities, with genital tuberculosis is unknown but in parts of the
sinuses tracts, fistulous communications, and extrarenal world where tuberculosis is frequent, genital tuberculosis
and extraprostatic spread. Multiplanar MRI allows is an important cause of infertility.
evaluation of the disease extent in the prostatic bed and
SCHISTOSOMIASIS (BILHARZIASIS)
the presence of sinuses and fistulae. MRI contrast agents
facilitate evaluation. MRI is also useful in the evaluation Introduction
of peritonitis and adnexal masses.
It is one of the most common parasitic infestations in
MRI is an excellent modality at depicting extraorgan
the world and is caused by the Schistosoma genus of
spread and discharging sinuses and fistulae but
fluke. The form of schistosomiasis affecting the urinary
calcification is not readily demonstrated.
tract involves Schistosoma haematobium. The other
Limitations of all techniques in general: forms, Schistosoma japonicum, Schistosoma mansoni,
1. All imaging findings may be normal in patients with and Schistosoma mekongi affect the gastrointestinal tract.
early genitourinary tuberculosis. The disease is endemic in the Middle East, India, Africa,
2. Genitourinary calcification may occur in patients with Central America, and South America, yet it is rare in the
diabetes mellitus and schistosomiasis. United States.
Pathophysiology symptoms due to central nervous system (CNS) compli-

Schistosomiasis typically affects the urinary system, cations.
especially the bladder, but ureteral involvement is found Urologic symptoms include either microscopic or gross
in as many as 30% of patients. The eggs of Schistosoma hematuria, dysuria, urinary frequency, and urinary
flukes are excreted into the urinary tract, causing an intense urgency.
granulomatous reaction and subsequent calcification.
The life cycle of the schistosome begins with the
passage of egg-containing urine into freshwater regions 1. Tuberculosis is the only real differential diagnosis. The
where the intermediate host is a snail. When the eggs propagation of both entities is different because
are hatched, miracidia are produced. These penetrate schistosomiasis starts in the bladder and ascends,
the snail and eventually form into cercariae. The cercariae whereas tuberculosis starts in the kidney and
penetrate the skin of human hosts and are eventually progresses distally. In addition, the bladder is usually
carried to the liver, lymphatic system, lungs, and venous distensible with schistosomiasis, but it is fibrotic and
system, where the fluke matures. The flukes live and limited in volume with tuberculosis.
2. Primary amyloidosis
copulate in the portal vein in most individuals, except
3. Cytostatic medications
for S haematobium, which migrates to the perivesical
4. Alkaline incrustation cystitis.
venous plexus through the hemorrhoidal plexus.
The fluke attaches to the walls of the venous plexus Role of Radiology and Imaging
by means of two suckers. The female fluke then deposits
eggs into the venules of the urinary bladder wall or distal Ureter: Involvement of the ureters occurs in as many
ureter. Some eggs penetrate the lumen of the bladder, as 65% of patients. The ureters commonly have persistent
but most become encapsulated in the vesical tissues, filling in the lower segment; dilated ureters are another
causing an inflammatory granulomatous reaction, fibrosis, finding. Ureteral strictures can be found, and, as the
foreign body reaction, and calcification of the dead ova. disease progresses, beading of the lower ureteral segment
The eggs, not the flukes, cause tissue damage to the host. may be observed. Subsequent ureteral fibrosis leads to
The degree of calcification is roughly correlated with the calcifications of the distal ureter, which have a
number of eggs deposited. The earliest calcification of characteristic pattern of linear or parallel calcifications
dead eggs occurs 50–120 days after deposition. The on plain radiographs. As many as 80% of the
female fluke can produce as many as 3500 eggs per day. strictures occur in the bladder wall near the
junction with the ureters. Dilatation of the ureter is
Frequency common. This is often caused by vesicoureteric reflux,
Schistosomiasis is the most common cause of bladder stenosis of the ureter, or an edematous ureteral wall that
calcification worldwide, causing as many as 56% of causes deficient peristalsis.
known calcifications in the bladder. It is endemic to the Bladder: With regard to the bladder, indistinctness or
Middle East and Africa. hazy changes are caused by submucosal edema and
pseudotubercles. The body forms an intense granuloma-
Clinical Features
tous reaction to the ova, and fibrosis ensues. This fibrosis
Sex: Schistosomiasis affects men more often than traps the ova in the tunica propria of the bladder wall
women, with a ratio of approximately 9:1. where the ova die and become calcified. The calcification
Age: Schistosomiasis usually occurs in individuals is not in the fibrous tissue, but it is caused by calcified
younger than 30 years. ova. The calcification spreads around the bladder wall
It typically causes a chronic low-grade infection with and can completely encircle the bladder, appearing as
flulike symptoms. Clinical symptoms may include fatigue, a curvilinear ring (curvilinear ring calcification of
headache, a stiff neck, a lack of energy, and neurologic bladder). A calcified area in the bladder has an estimated
500,000 to 1 million eggs per cubic centimeter. The linear, coarse, or floccular in schistosomiasis. Usually, the
extent of the calcification is roughly correlated calcifications are first seen in the base of the bladder on
with the number of eggs in the bladder lumen. plain radiographs, but they more commonly appear in
The bladder wall becomes fibrotic, but in contrast to the anterior wall of the bladder on CT scans. The thickness
tuberculosis, it is still distensible and maintains a of the ureteral wall is better evaluated with CT than with
normal capacity as schistosomiasis mainly involves the any other modality. Later fibrosis and calcification
submucosal layer, while in tuberculosis muscular layer develops which may be seen as ‘egg-shell or linear’
is primarily involved. calcification in the submucosa of the bladder and
Plain radiographs: Conventional radiographs are not the distal ureters.
useful until calcifications have developed in the bladder
Mortality/Morbidity
or ureters Calcification in the wall of the bladder or distal
ureters can be identified on plain radiographs. The most serious complication of urinary tract schisto-
Intravenous urography (IVU), retrograde uretero- somiasis is an increased incidence of squamous cell
graphy, or cystography carcinoma of the bladder. Additional complications
1. Mucosal irregularity, include urolithiasis, ascending urinary tract infection,
2. Inflammatory pseudopolyps urethral and ureteral stricture with subsequent hydronep-
3. Ureteritis cystica hrosis, and renal failure.
4. Ureteral dilatation and stricture, and Treatment
5. Reduced bladder capacity.
With regard to IVU, most of the findings are in the It involves mainly two drugs: praziquantel and metr-
bladder and distal ureter because the kidneys remain ifonate. Oxamniquine is another medication.
normal until late in the disease. MALACOPLAKIA
USG: Ultrasound may be normal in early or mild cases, It is a rare inflammatory disease usually associated with
but often shows wall thickened up to 1 cm or more and chronic E. coli infection.
single or multiple polypoidal lesions, which may be sessile The histologic hallmark of the disorder is the presence
or wave-like and may protrude in the lumen of ureter. of basophilic inclusions, called Michaelis-Gutmann
Changes are most marked in the bladder base and trigone. bodies, within large eosinophilic macrophages.
CT: Compared with other techniques, CT better It is usually unilateral. C findings are nonspecific, and
delineates the extent of the calcifications related to schisto- renal parenchymal malacoplakia may be indistinguishable
somiasis. Bladder calcifications are characteristically from a neoplasm.
RENAL TRAUMA and renal masses (e.g. angiomyolipoma) can manifest

INTRODUCTION with bleeding after a minor trauma.
Most renal injuries are associated with hematuria
Renal trauma is the most common urologic trauma and
(95%), which can be profused in more severe renal
occurs in 8–10% of patients with significant blunt or
trauma. However, in vascular pedicle injury or avulsion
penetrating abdominal trauma. In most cases, major renal
of the pelviureteric junction (PUJ), hematuria may not
injuries are associated with injuries to other major organs.
be present. Because the most contemporary trends in
The goal in trauma care is to resuscitate the patient,
trauma care, including renal trauma, call for less invasive
to diagnose injuries, and to implement appropriate procedures, trauma imaging by a skilled radiologist is
therapeutic measures as quickly as possible. Efficient increasingly important. By accurately distinguishing
organization of trauma centers with optimal resuscitation patients that can be optimally managed conservatively
techniques and early imaging leading to accurate staging from those who need surgery, radiologists can improve
are needed to determine appropriate clinical manage- the long-term outcome of patients.
ment. Radiologists serve an integral role in the multidis-
ciplinary approach to achieve that goal, playing a large Grading
part in the diagnosis and staging of injuries. Furthermore, Renal injuries are graded by the American Association
interventional radiologists help manage arterial injuries for the Surgery of Trauma (AAST) on the basis of the
using angiography with transcatheter embolization. depth of injury and the involvement of vessels or the
collecting system as follows (Table 9.1):
PATHOPHYSIOLOGY
Causes and Associated Features Grade 1
Hematuria with normal imaging studies
Renal trauma can result from a variety of mechanisms.
Contusions:
In the United States, motor vehicle accidents are the most
Nonexpanding subcapsular hematomas
common cause of blunt abdominal trauma leading to
renal trauma. Fall from a height and assault, including Grade 2
penetrating injuries, are less common causes. In rare Nonexpanding perinephric hematomas confined to the
cases, renal trauma occurs secondary to iatrogenic causes retroperitoneum
Urorenal Trauma 121
Superficial cortical lacerations less than 1 cm in depth or penetrating abdominal trauma. Blunt trauma is the
without collecting system injury. overwhelming cause of 80% of renal injuries.
Grade 3 CLINICAL FEATURES
Renal lacerations greater than 1 cm in depth that do not
Clinical findings that can exist in a patient with renal
involve the collecting system.
trauma include hematuria, flank hematoma, lower rib
Grade 4 fractures, and vital sign instability, such as hypotension.
Renal lacerations extending through the kidney into the About 95% of significant renal injuries are associated with
collecting system. hematuria; however, hematuria may be nonexistent,
Injuries involving the main renal artery or vein with especially with renal vascular injuries and UPJ avulsion
contained hemorrhage. or ureteral injuries. Of 1000 blunt abdominal trauma
Segmental infarctions without associated lacerations. patients with only microscopic hematuria and without
Expanding subcapsular hematomas compressing the hypotension, only approximately 1–5 have significant
kidney. injury of the urinary tract. Therefore, microhematuria
alone is not an absolute indication for imaging.
Grade 5
Shattered or devascularized kidney PREFERRED INVESTIGATIONS
Ureteropelvic avulsions
Indications in General
Complete laceration or thrombus of the main renal
artery or vein. Specific imaging of the genitourinary tract is indicated
for patients with gross hematuria, microscopic
INCIDENCE hematuria, and hypotension or in patients with
Renal trauma is the most frequent urologic trauma, injuries associated with renal trauma, such as the
occurring in 8-10% of patients with considerable blunt lumbar spine, lower rib, or transverse process
fractures. CT is the current preferred imaging technique
Table 9.1: Contrast-enhanced spiral CT grading of blunt renal injury used in these situations.
Injury Description or CT finding
grade Radiography
I. Superficial laceration(s) involving cortex
The use of radiography for blunt abdominal trauma is
Renal contusion(s)
<1 cm subcapsular hematoma nearly nonexistent, despite being an important tool in
Perinephric hematoma not filling Gerota’s space and no active the primary evaluation of chest and skeletal trauma. In
bleeding
II. Segmental renal infarction general, abdominal radiography has been replaced by
Deeper renal laceration extending to CT because of its widespread accessibility and, to some
Medulla, with intact collecting system
>1 cm subcapsular hematoma with intact renal function degree, ultrasonography. However, radiography still plays
Perinephric hematoma limited to and not a role in the assessment of penetrating trauma to the
Distending the perinephric space:
No active bleeding abdomen.
III. Laceration extending into collecting system with
Urine extravasation limited to retroperitoneum Intravenous Urography (IVU)
Perinephric hematoma distending perinephric space
Or extending into perineal spaces; no active bleeding Before the widespread use of CT, the traditional tools
IV. Fragmentation (3 or more segments) of the kidney used to search for genitourinary trauma were intravenous
(usually partially devitalized with large perinephric hematoma
Devascularization >50% of parenchyma urography (IVU), standard cystography, and retrograde
Main renal pedicle injury urethrography. Today, however, IVU has a more limited
Active bleeding by CT
Extravasation of urine into peritoneal
role, as CT has replaced many of its applications.
Cavity or extensive extravasation Occasions that may still warrant the use of intravenous
Subcapsular hematoma compromising renal perfusion
urethrography include the imaging of hemodynamically
unstable patients on their way to surgery or urologic should be confirmed in the event that significant unilateral
imaging of a patient already in the operating room. This renal injury warrants nephrectomy. However, the findings
approach is used to confirm that two kidneys are present on IVU may not always accurately specify the cause or
if nephrectomy might be needed. extent of renal involvement, while minor vascular injury
The traditional tools for assessing genitourinary injury or urinary extravasation may be missed. A non-visualized
have been IVU, standard cystography, and retrograde kidney (nephrogram/pyelogram) does not necessarily
urethrography. The role of IVU, however, has become represent significant renal trauma.
more limited as CT has become more available. IVU may
still be used if CT is not readily available, for unstable Retrograde Pyelography
patients going to surgery or for urologic imaging of a Retrograde pyelography is primarily useful when there
patient in the operating room. These studies are typically is a suggestion of ureteral, UPJ, or renal pelvic injury
performed as a one-shot IVU, which consists of the and delayed images were not made or were not sufficient
acquisition of a scout radiograph, a radiograph taken to exclude these injuries on CT or IVU. However, this
immediately after the injection of contrast material, and is not routinely needed; drawbacks to retrograde
a third radiograph obtained approximately 10 minutes pyelography include its impracticality in the emergent
after the injection. Additional delayed radiographs may evaluation of a severely injured patient and the fact that
be necessary to assess delayed excretion of contrast it does not characterize renal parenchymal injuries.
material if present and to detect the presence of urinary
contrast extravasation (Figure 9.1). VCUG
Findings that may be revealed by IVU include the If a trauma patient has blood at the urethral meatus, a
loss of the renal outline or psoas shadow if there is high-riding prostate, or an inability to void, urethral
perinephric hemorrhage, diminished or absent excretion trauma should be investigated by means of retrograde
or contrast extravasation. The ureters should also be urethrography. In almost all cases, this should be done
visualized to evaluate for ureteral injury or displacement, prior to the placement of a Foley catheter, unless the index
and the presence of a contralateral functioning kidney of suspicion is low. In this case, a pericatheter urethrogram
can be obtained later.
Ultrasonography
Has limited clinical usefulness in the evaluation of renal
trauma. The main application of this technique in the
trauma setting has been for the focused abdominal
sonography for trauma (FAST) scanning, with the
goal of detecting any free fluid in an unstable patient.
The primary advantage to this technique is that it can
be performed in a matter of minutes in the trauma bay
while a patient is being resuscitated. In many cases, the
presence of fluid is an indication for exploratory
laparotomy by surgeons.
Ultrasonography, however, is limited in the types of
injuries it can depict. First, although sonography can
depict-free fluid in the abdomen and pelvis, it lacks the
ability to distinguish the type of fluid or source of fluid.
FIGURE 9.1: Contrast extravasation with improper visualization of
Further, ultrasonography has not demonstrated significant
lower pole calyces on right due to trauma sensitivities and specificities to adequately search for solid
Urorenal Trauma 123
algorithm, there is some variability in the training of the

individual performing the examination. In some
institutions, radiologists or sonologists perform the study,
but in many centers, the trauma surgeon or emergency
physician do so. These specialists may have little training
in clinical sonography and virtually no training in its
technical aspects. Therefore, their ability to execute high-
quality examinations has been seriously questioned.
However, if the radiology staff is to perform trauma
sonography, the service must be readily available at all
times.
Although sonography can depict free fluid in the
abdomen and pelvis, it cannot be used to make the
clinically important distinction between extravasated
urine, blood, or other types of fluid. Moreover,
ultrasonography cannot depict the source of the bleeding.
Ultrasonography may demonstrate renal laceration, a
FIGURE 9.2: Subcapsular hematoma change in echogenicity of an injured kidney, or a decrease
in the usual perinephric echogenicity if perinephric fluid
or hemorrhage is present. However, if sonograms are
organ injuries. In most cases, even if a solid organ injury
negative and if noteworthy hematuria is present, or if
is found or if injury is clinically suggested but not found,
the sonogram is positive, CT is still indicated for evaluation
CT examination is still indicated if the patient’s condition
of the injury if the patient is stable. For this reason, the
is stable.
use of sonography is probably best reserved for the rapid
Findings: The use of abdominal ultrasonography for evaluation for intraperitoneal fluid in the unstable patient
trauma patients remains controversial, particularly for who may require urgent surgery.
detecting renal and urologic injuries. Despite this, in the
United States, ultrasonography has achieved moderate Computed Tomography
acceptance for evaluating a patient with blunt abdominal In general patients with blunt trauma and abdominal
trauma (Figure 9.2). symptoms, hypotension or an appreciably depressed level
In the trauma setting, sonography is usually performed of consciousness consistently undergo abdominal and
as a FAST scan for the primary purpose of identifying pelvic CT, which serves as the most comprehensive
free fluid in the unstable patient. In most circumstances, diagnostic tool for evaluating such a patient. Other
FAST scans can be executed in the few minutes during imaging modalities, such as ultrasonography, answer
patient resuscitation in a trauma bay. The examination questions of limited scope and do not afford the broad
includes probing six locations for the existence of free evaluation provided by CT. As such, CT is the primary
fluid: the right upper quadrant with the hepatorenal recess, tool for staging all injuries to the abdomen.
the left upper quadrant with the splenorenal recess, both If, during the CT examination, considerable
paracolic gutters, the pelvis and its various peritoneal perinephric fluid is noted (particularly on the medial side
cavity recesses, and the pericardial space. If the of the kidney), or if a deep laceration is noted, urinary
examination demonstrates the presence of fluid, surgeons extravasation should be investigated by using delayed
will generally perform an exploratory laparotomy. CT images.
In situations in which sonography has been used to While investigating the genitourinary tract, examiners
screen blunt abdominal trauma as part of a management should also thoroughly explore for active hemorrhaging,
as urgent surgery or embolization is frequently needed to appear. Furthermore, MDCT is advantageous because
in such situations to prevent exsanguination. of its improved ability to depict injuries such as active
If findings consistent with a bladder injury, such as arterial extravasation. Apart from faster scanning time
gross hematuria or pelvic ring fracture, are present, that MDCT provides, it also utilizes the tube-heat capacity
conventional cystography or CT cystography should be in a more efficient manner. This allows multiple,
performed after initial CT. Compared with a standard successive CT examinations to be carried out without
pelvic CT with intravenous contrast enhancement, the need to wait for the CT tube to cool.
cystography has a higher sensitivity for detecting bladder
Intravenous and oral contrast material: Intravenous
injuries. However, CT cystography is equal to or better
contrast enhancement is essential for abdominal CT.
than conventional cystography, if adequate bladder
distension can be achieved with contrast material. CT Without intravenous contrast enhancement, solid-organ
cystography also provides the ability to differentiate injuries such as renal lacerations can often be impercep-
between intraperitoneal, extraperitoneal, or combined tible. In addition, active arterial extravasation is only
bladder rupture. detectable with intravenous contrast material. Its usage
with helical CT and MDCT scanners has further increased
Limitations: CT is the overwhelming leader for
the frequency with which active arterial extravasation is
diagnosing and staging renal traumatic injuries. The main
seen. For adults, the typical contrast dose is 100–150
drawback to CT, however, is the time to complete a CT
mL, whereas for children, the dose is 1.5–2 mL/kg. The
examination, especially if CT equipment is not available
desirable injection rate is at least 2 mL/s; however, rates
near the trauma bay. For the most critically injured
of 3-4 mL/s allow for optimal enhancement of the
patients, this time is extremely limited; therefore,
vasculature and parenchyma. A low-osmolarity, non-ionic
ultrasonography has found some clinical appeal as a quick
contrast agent is standard.
method for searching for critical injuries.
Contrast material, when given orally, also has clinical
Findings: Across all imaging modalities, CT is the most utility in aiding in the detection of bowel injuries.
comprehensive diagnostic tool for assessing patients with Fortunately, oral contrast material is safe, even for
blunt abdominal trauma. CT can be used to evaluate children. As soon as an abdominal CT scanning is
a large breadth of intra-abdominal injuries with accuracy, requested, 400–600 mL of a dilute solution, for example,
and hence, it has a primary role in evaluating the trauma 4% diatrizoate meglumine in tap water, is given by mouth
patient. Further, the success of CT in staging abdominal or by nasogastric tube. Images of trauma patients are
injuries has contributed to the growing trend toward then obtained without delay. Thus, the stomach,
non-operative management of traumatic abdominal duodenum, and proximal jejunum are typically the only
injuries. As such, the CT scanner should be as close to
structures opacified; fortunately, these are the most
the trauma bay as possible to minimize patient transport
common sites of bowel injury. Finally, some authors
time.
suggest withdrawing the nasogastric tube into the distal
CT technique: To best evaluate blunt abdominal esophagus during the scan in an attempt to reduce upper
trauma, the technique with which a CT is obtained must abdominal streak artifact.
first be optimized. Conventional axial CT scanners can For CT, an image thickness of 5 mm or less prevents
provide sufficient scans; however, helical CT scanners major volume-averaging artifacts, and a scanner pitch
offer a considerable gain in speed and quality. of 1.5:1 for single-slice helical scanners optimizes speed
Multidetector-row CT (MDCT) scanners are even more while preventing excessive section-profile broadening. For
powerful than single-slice helical CT scanners because an MDCT scanner, a pitch greater than one but less than
their thin-section, high-quality images can be obtained two hastens image acquisition yet usually results in
more quickly. As a result of shorter scanning times, less excellent image quality. Further, by scanning at speeds
opportunity is available for motion or breathing artifact less than the maximum table speed or with a detector
Urorenal Trauma 125
configuration narrower than the image thickness usually

produced, thinner sections can be retrospectively
reconstructed. This is sometimes needed to evaluate
subtle injuries or associated spine or bony pelvic injuries.
Some institutions regularly scan through the kidneys
a second time during the urographic phase of
enhancement to aid in detection of subtle injuries of the
parenchyma and collecting system. At the author’s
institution, images of trauma patients are regularly
evaluated as they are obtained while the patient is still
in the CT scanner. If noteworthy perinephric or
periureteral fluid is found, urinary contrast extravasation
is investigated by taking images delayed at 5–15 minutes.
If clinical findings, such as gross hematuria or pelvic
ring fracture are present, and if bladder injury is a concern, FIGURE 9.3: Small right renal laceration involving cortex
cystography or CT cystography should be performed.
Standard CT with intravenous contrast enhancement has
a lower sensitivity for these injuries. CT cystography offers
a few advantages over conventional cystography. First,
the patient can be evaluated by CT cystography after
the initial scan without the need to move to another
location. CT cystography can also distinguish intraperi-
toneal, extraperitoneal, or combined bladder rupture.
When CT cystography is performed, the urinary
bladder is first drained by Foley catheter following the
abdominal CT scan. The CT cystogram is done with either
standard scans or scout imaging. The cystogram should
be performed before intravenous injection of contrast FIGURE 9.4: Deeper laceration extending to medulla
media. In the adult, a minimum of 300 mL of dilute but with intact PCS
contrast media is necessary. If this is normal, drainage
and wash out (bladder flushed with sterile fluid) may be
performed but is not routinely needed.
CT Interpretation (Figures 9.3 to 9.7): CT scans for
blunt abdominal trauma must be meticulously reviewed
for proper interpretation. On evaluation, urgent life-
threatening injuries, such as a large hemoperitoneum,
a large or tension pneumothorax, pneumoperitoneum,
signs of hypovolemic shock, or active arterial
extravasation, should be sought out first. This should be
followed by a thorough interrogation for injury of the
abdomen and pelvis: liver and right paracolic gutter;
spleen and left paracolic gutter; upper abdominal organs, FIGURE 9.5: Laceration extending to collecting system with
including the stomach, duodenum, pancreas, gallbladder perinephric hematoma
every organ”. With this method, renal injuries can be readily

identified and classified for proper treatment.
Grade 1 injuries
AAST grade 1 renal injuries include hematuria with
normal imaging, contusions, and nonexpanding
subcapsular hematomas; overall, this grade accounts for
80% of renal injuries. In CT images, contusions are
perceived as ill-defined or sometimes sharply marginated
areas of reduced enhancement and excretion. A
segmental infarction, which is an AAST grade 4 renal
injury, is differentiated from contusions by a lack of
enhancement altogether.
Subcapsular hematomas usually appear as a
hyperattenuating fluid collection between the renal
FIGURE 9.6: Fragmented left kidney with hematoma parenchyma and the renal capsule, at times deforming
the underlying kidney. These hematomas are less
common than perinephric hematomas in blunt abdominal
trauma. Small, subcapsular hematomas often take on a
crescent shape, whereas larger hematomas may appear
elliptical and compress the renal parenchyma. On rare
occasions, the hematoma may progressively enlarge and
compress the kidney enough to lower renal perfusion.
This may result in reactive hypertension (Page kidney).
Grade 2 and 3 injuries
Renal injuries that are classified as grade two include
nonexpanding perinephric hematomas contained by the
retroperitoneum and superficial cortical lacerations less
FIGURE 9.7: Devascularized left kidney
than 1 cm in depth without injury to the collecting system.
and biliary tree; retroperitoneum, including the adrenals, On CT, a perinephric hematoma often appears as an
kidneys, inferior vena cava, and aorta; small bowel, colon, ill-defined, hyperattenuating fluid collection located
and mesentery; pelvis, including the urinary bladder; between the Gerota fascia and the renal parenchyma.
muscles, including the abdominal wall, psoas, iliacus, More often than not, such a hematoma is associated with
and gluteals; bones, including the spine and pelvis; and underlying injury, though they can occur in isolation.
thighs. Thus, when a perinephric hematoma is discovered, a
To perform a complete evaluation, the entire scan must thorough investigation of the kidney should be
be scrutinized with three different window/level settings: undertaken to look for associated renal injury. Unlike a
soft tissue, lung, and bone. The entire systemic review has subcapsular hematoma, even a large perinephric
been called the “every-organ-on-every-slice” hematoma does not traditionally deform the kidney.
approach. The authors believe that, with the modern Renal lacerations are seen on CT as jagged or linear
Picture Archiving and Communication System (PACs) parenchymal disruptions that can contain fresh or clotted
workstation, image review is best accomplished by rapidly blood. The laceration may thus show attenuation higher
paging through the images multiple times, with special than that of water, but this would occur without the
attention to one organ at a time; hence, “every slice of contrast enhancement present in the renal parenchyma.
Urorenal Trauma 127
By definition, grade two renal lacerations are less than On CT, renal segmental infarctions appear as well-
1 cm in depth, while grade three lacerations are greater delineated, linear or wedge-shaped, often multifocal
than 1 cm in depth. Both grade two and grade three and nonenhancing areas that extend through the
renal lacerations, however, do not involve the collecting parenchyma in a radial or segmental orientation.
system. As such, there would be no evidence of urinary Thrombosis, dissection, and laceration of segmental renal
contrast extravasation on delayed CT. arteries are primary causes of segmental infarctions, and
The treatment of most grade one, two, or three renal such infarctions are frequently associated with other renal
injuries is usually conservative, except when a vigorous injuries. These injuries are treated conservatively, as they
active hemorrhage is present. In such cases, the active often resolve spontaneously or result in relatively minor
hemorrhage may be treated successfully with selective renal scaring. In 6–20% of patients, hypertension may
catheter embolization in an otherwise stable patient. develop as a delayed complication; however, this often
Occasionally, continued bleeding or extravasation can resolves or can be medically managed.
lead to complications and higher morbidity if not identified
Grade 5 injuries
and managed appropriately. Follow-up CT is useful for
Grade 5 renal injuries include a shattered or
restaging the renal trauma and helps in identifying the
devascularized kidney, UPJ avulsions, and complete
patients with progressive worsening on conservative
laceration or thrombosis of the main renal artery or vein.
management. Appropriate intervention in these patients
The first of these, a shattered kidney, essentially describes
can help prevent complications.
the extreme of multiple renal lacerations, often with
Grade 4 injuries devitalized areas due to infarction, and urinary
Grade 4 renal injuries include renal lacerations that extend extravasation resulting from injuries to the collecting
into the collecting system, injuries to the main renal artery system.
or vein with contained hemorrhage, and segmental A different type of grade five renal injury, the UPJ
infarctions without associated lacerations. The first of injury, characteristically involves a medial or circumrenal
these, renal lacerations with collecting system involve- urinoma on CT. Such injuries are caused by a shearing
ment, frequently produce extravasation of urine or force on the renal pelvis. Complete avulsion or partial
contrast agent. Extravasation such as this should be tear of the UPJ occurs when rapid deceleration of the
thoroughly sought any time a laceration extends through kidney pulls on the relatively fixed ureter and renal blood
the kidney or substantial perinephric fluid is seen on CT, supply. Imaging can distinguish a partial tear from a
especially if that fluid is around the renal hilum. Delayed complete avulsion by the presence of contrast agent in
images allow contrast material to filter into the collecting the distal ureter. In many cases, hematuria is absent or
system, providing adequate views of any urinary minimal. Treatment for complete UPJ tears is surgical
extravasation. repair, but some partial tears can be managed with
Under many circumstances, the healing of even large stenting and/or observation. When a UPJ injury is
urinary extravasations can occur with conservative undiagnosed and when the proximal collecting system
treatment; however, stenting is sometimes necessary to is not drained, an urinoma can develop. This may lead
facilitate the process. Surgical debridement or repair is eventually to a nephrectomy.
usually necessary only when the laceration is With CT imaging, a devascularized kidney appears
accompanied by significant devitalized renal tissue, nonenhancing. Often, little hematoma or other sign of
particularly when concomitant intraperitoneal injuries are injury is depicted. In some cases, CT angiography shows
also present. The main purpose of such a procedure is a blind-ending renal artery. Retrograde opacification of
to prevent the development of urinoma, infection, or the renal vein from IV contrast indicates an acute injury
abscess. In the absence of such repair, a nephrectomy indicating the need for immediate emergency surgery to
may be needed later to prevent sepsis. reestablish blood flow. In late evaluation, the renal vein
is thrombosed, and this reverse flow is not seen. The renal artery or lacerated kidney appears present,
cortical rim sign may be apparent, but not early. This immediate transcatheter embolization or surgery may be
usually indicates a dead kidney with rare recovery of renal needed to prevent exsanguination.
function (if it ever occurs). In the absence of other Delayed bleeding or rare cases of hypertension
associated injuries, hematuria is many times nonexistent. occasionally result from persistent pseudoaneurysms.
The most common cause of a devascularized kidney Renal lacerations from blunt or penetrating trauma can
is an incomplete renal artery tear with thrombosis; a also produce arteriovenous fistulas. Initially, these may
complete tear of the renal artery with an extensive be difficult to detect, but they can enlarge over time. The
hematoma or active bleeding is less common. When they results of this may be delayed bleeding, hypertension,
occur, these injuries are often present with other renal or high-output cardiac failure.
injuries. This association contributes to the poor renal
outcome after attempted repair; therefore, the care of MRI
stable patients is usually expectant. For patients with active Findings: For stable patients with a strong contraindication
bleeding or major parenchymal disruption, treatment is for iodinated contrast material, MRI with a gadolinium-
usually nephrectomy except in the case where there is based contrast agent can be helpful in assessing a renal
injury to or absence of the contralateral kidney. A potential injury; however, for the acutely injured patient, MRI is
complication of these injuries is hypertension; it can usually not practical because of motion artifacts and the
develop weeks to months after the initial injury in as many examination time.
as 40–50% of patients. This often resolves or can be
medically managed, but nephrectomy is sometimes RNI
necessary. It may be used to evaluate renal function after injury
Another less frequent form of vascular pedicle injury or to directly evaluate the patient for urinary injury,
is damage to the main renal vein. One type of such an especially those with an extravasation of urine.
injury is laceration of the renal vein. On CT, this usually Scintigraphy is generally not useful in the acute trauma
presents with medial or circumrenal subcapsular or setting because of its low specificity and inability to
perinephric hematoma. Thrombosis is a second type of evaluate for injuries outside the urinary tract.
renal vein injury that is depicted on CT as a filling defect
or as nonenhancement of the vein. A delayed or persistent Angiography
nephrogram may be present when thrombosis results in Angiography has played in the initial diagnosis of trauma
complete occlusion. This may be lethal in the adult. to the renal vasculature has diminished with the advent
Vascular extravasation of contrast material of faster CT scanners. Despite this development, use of
Contained or active hemorrhage is indicated by bright angiography in the management of vascular and
enhancement with attenuation similar to that of nearby exsanguinating solid-organ injuries has continued to
arteries within a laceration or around an injured kidney increase, with the trend toward nonoperative manage-
during the early phases of CT scanning. Active ment of trauma. Furthermore, angiography with
hemorrhage appears as an ill-defined, flame-shaped, or transcatheter embolization is becoming the standard of
waterfall-shaped area on CT, with an associated fresh care for treating stable patients with vascular injuries, such
as traumatic pseudoaneurysms and active arterial
hematoma. The hematoma often demonstrates
bleeding.
circumferential or dependent layering of older and fresher
hemorrhage. On the contrary, contained hemorrhage or Findings: Prior to the common availability of CT,
pseudoaneurysm is somewhat bound and contained angiography was often used to initially diagnose renal
within the renal parenchyma or laceration. If active arterial or parenchymal aberrations found on IVU. Today,
extravasation of arterial contrast material from the main however, faster CT scanners and the increased detection
Urorenal Trauma 129
of active arterial extravasation have limited the use of SUMMARY

angiography for the initial diagnosis of traumatic injuries. Blunt trauma, usually deceleration injury from motor
Moreover, CT can also depict many injuries not seen on vehicle accidents, may result in shearing injury to the
angiography.
renal arteries, resulting in hematoma formation, dissection
On the other hand, the role of angiography in the
or disruption. A hematoma may form between the renal
management of vascular and exsanguinating solid-organ
cortex and the kidney fibrous capsule. These subcapsular
injuries continues to rise in parallel with the increasing
hematomas accumulating in a closed space, may create
emphasis on nonoperative management of trauma.
increased tissue pressure and reduced tissue prefusion.
Angiography with transcatheter embolization is becoming
As renal perfusion decreases, the kidney reacts to
the standard of care for patients with many vascular
increase perfusion by secreting renin. In turn, this causes
injuries, such as pseudoaneurysms and active arterial
sytemic hypertention. This entity is referred to as Page
bleeding resulting from renal trauma.
kidney. This physiologic entity may also result from other
RADIOLOGIC INTERVENTION processes, including renal cystic disease or any renal mass.
Radiologic interventions in the setting of acute renal
trauma are mainly limited to those of a vascular nature. URINARY BLADDER TRAUMA
Examples include embolization of bleeding vessels and INTRODUCTION
arteriovenous fistulas.
Trauma continues to be a major cause of morbidity and
MANAGEMENT OF RENAL INJURY mortality in all areas of the world. Many immediately
life-threatening insults are to the vital organs or their blood
Treatment options must be weighed against related
supplies, and many diagnostic skills and studies are meant
mortalities and morbidities. In the evaluation for treatment
to rapidly reveal these complications. However, other life-
options, the AAST injury grade is correlated with the
apparent need for surgery to repair or remove the injured threatening conditions must be addressed in a timely
kidney. manner to avoid morbidity and occasionally mortality.
Non-operative management may be successful or Ideally, these conditions are diagnosed simultaneously,
even preferred in stable patients with high-grade injuries. rather than individually, and the search for a universally
The preservation of long-term renal function is often better applicable study is underway. Immediate surgery or other
when renal injuries are treated non-operatively. Thus, approach is commonly the diagnostic and therapeutic
unless there is extensive devitalized tissue, active procedure of choice if the patient is exsanguinating or
hemorrhage, a large injury to the collecting system with if his or her condition is deemed otherwise unstable by
progressive renal compression on follow-up or with the trauma physicians. One semi-emergent condition is
ureteral disruption, conservative management is often trauma to the bladder, especially trauma that results in
chosen for renal injuries. Overall, with modern manage- uroperitoneum. Sepsis can develop within 24 hours in
ment techniques, renal salvage rates approach 85–90%. these injuries if surgery with repair is not performed.
MORTALITY/MORBIDITY MECHANISM OF INJURY
Mortality and morbidity rates for renal injuries vary with Bladder trauma can occur in a number of settings. They
the severity of renal injury, the degree of injury to other are usually considered in the context of whether blunt
organs, and the treatment plan utilized. Thus, treatment or penetrating trauma were the inciting events. Blunt
options must be weighed against related mortalities and trauma is responsible for a rather large number of severe
morbidities. In the evaluation for treatment options, the associated injuries, such as pelvic, splenic, renal, aortic,
AAST injury grade is correlated with the apparent need bowel, and hepatic insults. Injury is not necessarily
for surgery to repair or remove the injured kidney. localized to areas obviously traumatized.
Penetrating injury is often secondary to missiles, the thigh or scrotum with disruption of the inferior fascia
impaling objects, and knives. These injuries are of varying of the urogenital diaphragm (perineal membrane). Urine
severity and often, but not always, result in damage to also may breach the Scarpa or Camper fascia, and it
more focal areas. Some missile injuries have a vaguely may be observed in the rectus sheath or even under the
estimable trajectory when one considers the entrance and skin.
exit wound, and many knife wounds still have the Type V, or combined rupture, consists of both intra-
impaling blade in place at the time of presentation. peritoneal and extraperitoneal urine. It occurs in only
5-12% of bladder ruptures and results from penetrating
CLASSIFICATION OF INJURY
and blunt trauma.
Any discussion of bladder trauma must include the
classification of injury and associated injury patterns. In CLINICAL FEATURES
bladder trauma, five grades of insults are recognized.
Sex: No significant difference.
Type I injuries are partial tears of the mucosa. This is
the most common injury pattern of multisystem trauma Age: Bladder trauma does not appear to be age specific.
patients and associated with blunt trauma for obvious Trauma to the bladder is associated with significant
reasons. These patients present with hematuria and trauma to the pelvis and intra-abdominal organs. Thus,
suprapubic pain, but no rupture is present, and findings patients present in a wide variety of ways with large
on cystography, conventional CT, and CT cystography differences in the stability of their condition. Commonly,
are normal, unless an intraluminal hematoma is present. they have few symptoms secondary to their bladder injury
This pattern is generally only mentioned to recognize that or even rupture. The literature suggests that they have
it is not a major bladder injury. no strong propensity to develop peritonitis initially, even
Type II, or intraperitoneal bladder ruptures, account for with a large amount of uroperitoneum.
approximately 10-20% of all major bladder injuries. Most Physical examination findings suggestive of bladder
commonly, this is the result of a direct blow to the trauma include vague peritoneal signs or abdominal
distended organ. The bladder is weakest at its dome where tenderness. However, more suggestive findings include
reinforcing tissue is absent, thus intraperitoneal rupture isolated suprapubic tenderness, pelvic instability, or lap-
usually occurs as a result of a rift in the bladder dome. belt ecchymosis. One study revealed that approximately
Intraperitoneal fluid is observed bathing loops of bowel
4% of children with lap-belt ecchymosis suffered bladder
and presents in the paracolic gutters and mesenteric folds.
rupture.
Type III, or interstitial pattern, is described as intramural
Unlike upper renal trauma, bladder injury is essentially
or partial-thickness laceration of the intact serosa. Only
ruled out when no RBCs are observed in the urine.
CT cystography is commonly diagnostic and
Urogenital injury is suggested when microhematuria is
demonstrates intramural contrast within the bladder wall.
present (defined as >25,000–35,000 RBCs per high-
This condition is most often secondary to blunt trauma.
However, laceration by pelvic fragments or a penetrating powered field). Gross hematuria is highly suggestive and
object, with minimal serosal damage, can present with certainly warrants full investigation. Gross hematuria is
a primarily interstitial pattern with minimal extracystic present in 95% of patients, and the remaining patients
urine demonstrated. have microscopic hematuria.
Type IV bladder injury is extraperitoneal. It is the most A problem can arise when bladder trauma is presumed
common bladder rupture. In blunt trauma, one theoretic to be the cause of hematuria because this finding is not
cause for laceration of the lower bladder is from secondary specific and can stem from more ominous sources such
injury from pelvis fragments. Simple extraperitoneal as renal fracture. Collect urine from the first few hundred
ruptures involve urine buildup in the perivesicular space. milliliters of the initial sample to prevent errors in
More complex patterns of urine tracking involve different interpretation. Furthermore, always rule out urethral
fascial planes. Urine or contrast material can be seen in trauma before placing a Foley catheter, especially when
Urorenal Trauma 131
gross hematuria is present. Suggestive findings include The pubovesicular, medial, and paired lateral umbilical
blood at the meatus, a high-riding prostate, the patient’s ligaments strengthen the association with the anterior
inability to void, perineal hematoma or scrotal swelling, body wall. The space of Retzius exists anterior to the
and pelvic fracture. bladder. This area is loose connective tissue that allows
Bladder injury is strongly associated with pelvic easy mobilization of the bladder from other surrounding
fracture. Of bladder ruptures associated with pelvic structures. There is little preventing motion of the bladder,
fracture, 80% are extraperitoneal. Pelvic fracture is in that the only rigidly fixed point is the bladder neck.
associated with bladder injury in 80% of patients, but This is the primary adaptation allowing distention during
the reverse is not true: Only 10% of pelvic fractures are filling.
associated with major bladder trauma. When a pubic rami
fracture is present or if pubic symphysis diastasis is present, PREFERRED INVESTIGATIONS
maintain a higher index of suspicion. A great deal of effort Radiologic examination is of paramount importance and
has been made to determine which pelvic fractures are should be performed to identify and classify the injury
associated with bladder injury. Patients with disruption and to plan surgical repair, but it should not hinder patient
of the pubic symphysis, pubic rami, and vertically unstable treatment and stabilization. Several radiologic evaluations
pelvic fractures have high degrees of concomitant bladder are appropriate, ranging from CT cystography to basic
trauma, whereas those with isolated acetabulum, femur, retrograde cystography. All have been moderately well
and ileac crest fractures have a low incidence of bladder studied and require different equipment, locations,
injury or rupture. protocols, and operator expertise. Always consider the
stability of the patient’s condition with regard to airway
ANATOMICAL BASIS patency and circulation during transfer and radiologic
The bladder is located within the bony pelvis and in adults evaluation of the patient.
is considered to be a mostly extraperitoneal organ. There, The amount and type of radiologic evaluation required
the bladder, prostate, and proximal urethra are protected depends on the patient’s condition and on the size of
by one of the most secure bony enclosures in the body. the area that may be affected. Many patients in stable
It has a tetrahedral form when empty and has four primary condition require extensive screening, thus diagnostic
surfaces. A superior, posterior, and two inferolateral procedures should ideally provide views of large area with
surfaces define the shape. The superior portion is quick and common preparation. For many patients in
triangular and lined completely by the visceral unstable condition or those with penetrating abdominal
peritoneum. This portion extends into the abdomen when injuries who are immediately treated in the operating
distended and has little support from other structures. theater (at the discretion of the surgeon) intraoperative
It is considered to be a dome when distended and contacts radiologic evaluation is needed.
the uterus in the female and the sigmoid colon in addition
to loops of bowel in the male. Retrograde Cystography
The posterior surface or fundus of the bladder is Retrograde cystography performed after urethrography,
anterior to the rectum but remains mostly retroperitoneal. was considered the criterion standard for evaluation of
A male bladder has the seminal vesicles coursing between bladder trauma. However, in recent years, support has
the posterior bladder wall and the rectum. The posterior grown for using CT cystography in proper diagnosis. Initial
base of the bladder is supported by the rectum and studies were not indicative of the reliability of CT when
secured by the rectovesicular ligaments. In females, the retrograde contrast enhancement was not used. However,
posterior and superior surface is loosely fixed to the upper contemporary studies have overwhelmingly
vaginal wall and uterus. demonstrated that the technique is both sensitive and
The pelvic floor musculature and overlying loose areolar accurate, provided that adequate bladder distention with
tissue support the inferolateral margins of the bladder. at least 300-400 mL of contrast material is achieved before
the study is performed. In diagnosing bladder rupture, procedure is less time consuming and, some would argue,
CT cystography, performed with 400 mL of contrast less costly. At 1 hospital cited in the literature, the cost
material administered in a retrograde fashion, is as of CT cystography was $500 or more, a marginal increase
accurate as plain radiography with retrograde over a plain radiographic examination. Costs should be
cystography. specifically evaluated at each institution.
A final step is the washout study. After the full-bladder
Ultrasonography
findings are recorded (on radiographs or CT scans), the
Ultrasonography is not sensitive or specific enough to bladder is drained. If no residual contrast enhancement
be useful for evaluation of bladder rupture. is present, the examination is completed, and the results
However, blood clots and at times site of tear in are negative. If residual contrast enhancement is present
bladder wall can be depicted (Figure 9.8). in the bladder area, fluid (e.g. sterile water) is used to
Retrograde cystograms have long been used for lavage the bladder. If no residual contrast enhancement
detecting bladder rupture. They are nearly 100% sensitive is noted after drainage, the examination is completed,
for detecting rupture, provided adequate distention is and the results are negative. If contrast enhancement
accomplished and postvoiding images are obtained. remains, a bladder wall injury is present.
However, they are time consuming, the examinations are
costly when one considers value relative to the benefit, CT Cystography
and they require extra radiography that does not occur CT cystography may be used somewhat less often in
in addition to necessary trauma evaluation. Furthermore, patients not undergoing CT for another reason. In a study
they are not useful in thoroughly evaluating other of 157 patients with hematuria, an absence of free fluid
structures present in the abdomen and pelvis. on abdominopelvic CT was a strong negative predictor
of bladder rupture. In these patients, not performing
CT Retrograde Cystograms
cystography may be reasonable.
CT retrograde cystograms are completed in the radiology Further study is warranted regarding this matter.
suite when routine spiral scans of the head, neck, chest, Perhaps one of the greatest advantages of retrograde CT
and abdomen and pelvis are performed. The same cystography with prior abdominopelvic CT is the ability
retrograde introduction of contrast agent is generally to detect renal parenchymal injury. In these patients,
performed as with retrograde cystography. However, intravenous urography is not necessary, as it commonly
multiple images, including postvoiding and oblique views, is with traditional retrograde cystography.
are not necessary as in plain radiography. Thus, this A few studies have focused on delayed evaluation
of the bladder. For example, the use of contrast material
for chest and abdominal CT (for which a large amount
is routinely required) has been studied. In these
examinations, the contrast agent was allowed to distend
the bladder in an anterograde fashion. However, this
distension occurs at the expense of valuable time, because
the Foley catheter should be clamped for at least
20–30 minutes to have any opportunity to achieve
accurate results. Furthermore, if preexisting renal
insufficiency or renal pedicle injury is present, this method
may be inadequate.
The author does not recommend this diagnostic
FIGURE 9.8: Large clot in bladder with tear in its posterior wall strategy for the reasons mentioned. Because results of
Urorenal Trauma 133
recent studies have also cast doubt on the consistent CT evaluation. This is true especially when microhema-
accuracy of this method in the evaluation of blunt trauma, turia is present and a possibility of renal trauma exists.
its use is discouraged.
MORTALITY/MORBIDITY
Limitations of Techniques Morbidity and mortality is most commonly infectious in
Cystography generally has served to greatly decrease nature. Therefore, complications are usually associated
trauma morbidity and mortality by helping in successfully with bladder rupture. Bladder disruption occurs in
screening for bladder rupture. Little doubt exists 5-10% of patients with pelvic fractures, and the type of
concerning the accuracy of plain-film cystography, as long perforation, with respect to classification, is important to
as a bladder hematoma does not occlude a rift in the prognosis. Broadly classified, approximately 50-85% of
bladder wall and prevent dye from flowing out into the ruptures are extraperitoneal (many of which have
surrounding spaces. The primary concern is that the associated pelvic fractures), 15-45% are intraperitoneal,
examination often does not occur in parallel with other and only 1-10% of disruptions are both.
radiologic examinations of patients with trauma who In the event of intraperitoneal rupture, morbidity and
require CT scanning. mortality are greatly influenced by preexisting urinary tract
A caveat should be noted: A normal cystogram finding infection. Thus, sepsis can ensue within 24 hours in
does not exclude bladder rupture. At surgery, certain circumstances. Treated properly with operative
intraperitoneal or extraperitoneal extravasation may be repair and urologic consultation, nearly all patients with
found. The consideration in this scenario is the spasm intraperitoneal bladder ruptures have few or no long-term
of the detrusor muscle, which is possibly secondary to complications.
the irritation effect of the contrast medium that causes
a leak to become sealed. With general anesthesia, the URETHRAL TRAUMA
detrusor relaxes; this is associated with the eventual INTRODUCTION
intraoperative leak.
Trauma to the urethra can be attributed to guns, knives,
CT cystography is faster than plain radiographic
surgical or urologic instruments, blunt trauma, straddle
studies; it has no labor-intensive requirements for
injuries, or penile fracture. Most male posterior urethral
completion; and it can be used to diagnose large
injuries, however, are the result of blunt pelvic trauma
hematomas of the bladder, which potentially could overlie
an occult breech in the bladder wall. Furthermore, most often associated with a vehicular accident or a fall
classification of bladder injury patterns requires CT from a height. Most cases of anterior urethral trauma result
scanning because cystography addresses perforations but from straddle injuries.
not more-subtle findings. The male posterior urethra is entirely encased within
Consider the cost in each prospective hospital, the rigid pelvis, a protective structure that must be
because the monetary costs, which favor classic disrupted before the posterior urethra can be damaged
cystography, may not reflect actual benefits. For instance, by blunt external trauma. The potential for urethral
because X-ray technologists currently are in short supply, trauma is thus influenced by the extent of the pelvic injury,
increasing their use adds to the expense. Furthermore, and this potential has been classified as no risk, low risk,
time is valuable in the trauma setting, especially because and high risk). Examples of no risk injuries include isolated
patients in seemingly stable conditions can deteriorate fractures of the acetabulum, ilium, or sacrum; low-risk
quickly, and a more-rapid evaluation can facilitate their injuries include single ischiopubic ramus or ipsilateral rami
transfer to the trauma intensive care unit or operating fractures; and high-risk injuries include straddle fractures
theater. In general, the author believes that evaluation or Malgaigne fractures. Overall, disruption of both the
with CT cystography is the study of choice when patients anterior and posterior sides of the pelvic ring introduces
already require transfer to the radiology suite for greater risk of urethral trauma.
Damage of the posterior urethra is thought to INCIDENCE

occur as its support mechanism becomes disrupted. As Most posterior urethral trauma cases in males result from
soft tissue becomes compressed, the puboprostatic pelvic injury. Among male pelvic traumas, the reported
ligament ruptures, disconnecting the prostate from the frequency of urethral injury varies widely from 1-25%
anterior pubic arch. This mobilizes the prostate and with an average of approximately 10%. Urethral
bladder. In many cases, a hematoma develops inferior injury in women with pelvic trauma is considered a less
to the prostate from sheared periprostatic vessels. The common event; however, some studies have reported
prostate is then driven cephalad by the growing incidences as high as 4-6%. Anterior urethral trauma
hematoma. The posterior urethra, however, is firmly is thought to occur less frequently due to its higher
attached to the pubic arch by the perineal membrane. mobility, but the frequency of occurrence has not been
The resultant shearing force stretches or ruptures the established.
urethra in varying locations as described by radiographic
ANATOMICAL BASIS
findings).
Anterior urethral injuries are seen in a small Historically, the male urethra has been divided into
minority of patients because of the mobility of the anterior anterior and posterior parts, which are demarcated at
the urogenital diaphragm. The proximal posterior urethra
urethra compared with the posterior urethra. Most cases
begins at the interface with the bladder, the internal
are the result of straddle incidents, in which the patient
urethral orifice, and the prostatic urethra. The prostatic
falls on the crossbar of a bike or the top of a fence. Force
urethra is entirely contained within the prostate and is
from the structure on the perineum compresses the
continuous with the membranous urethra at the prostatic
corpora spongiosum and bulbous urethra against the apex inferiorly. A principal support structure, the
pubic symphysis, disrupting the urethra. In some mild puboprostatic ligament, firmly attaches the prostate to
cases, the resulting injury will go untreated; however, a the anterior pubic arch. This anatomy is important for
stricture develops over time. The patient presents at locking the posterior urethra and prostate into their relative
this time with an inability to void. In some cases, the positions within the extraperitoneal pelvis.
patient is unaware of the relationship to the past straddle The membranous urethra is located within the anterior
injury. tip of the urogenital diaphragm and becomes the proximal
If the Buck fascia remains intact, the injury will be portion of the anterior urethra after passing through the
limited to the space between the fascia and the tunica perineal membrane. The principal mechanism of
albuginea. If, however, the Buck fascia is also disrupted, continence, the external urethral sphincter, is located
a hematoma may spread within the confines of the Colles within the urogenital diaphragm around the membranous
urethra. The Cowper glands are also located within the
fascia. Thus, blood or contrast material extravasation may
urogenital diaphragm adjacent to the urethra.
extend to the scrotum, perineum, or anterior abdominal
The bulbous urethra, a swelling in the proximal
wall. Contrast would not extend into the thigh because
anterior section, travels within the proximal corpus
of the insertion of the Colles fascia into the fascia lata
spongiosum and is continuous with the penile urethra.
of the thigh. The ducts to the Cowper glands drain into the bulbous
Penile fracture results only when the penis is erect, urethra. The penile or pendulous urethra extends the
and the injury results in disruption of the corpora and length of the penis where it ends as the fossa navicularis
tunica albuginea. and urethral meatus.
Among women, the most common types of urethral
injuries described are longitudinal tears and avulsion- CLINICAL DETAILS
distraction injuries, with the later attributed to more severe Sex: Urethral traumas are more frequent in the male
lateral compressive pelvic trauma. population than in the female population among women,
Urorenal Trauma 135
the most common types of urethral injuries described are urethral extravasation, it provides limited information
longitudinal tears and avulsion-distraction injuries, with about the details of surrounding soft tissue damage.
the later attributed to more severe lateral compressive Furthermore, imaging of the proximal urethra can
pelvic trauma. occasionally be inadequate. This is usually caused by
subpar contrast-agent filling of the proximal urethra or
Age: An age-linked risk of urethral injury associated
by gross extravasation of contrast blocking visualization
with pelvic fracture has been shown for children younger
of the proximal urethra.
than 15 years. The suggested cause for this pattern is
the difference in pelvic fracture severity seen between Technique: The standard imaging method used to
children and adults. For pelvic fractures in children, diagnose urethral trauma is RUG. While various
approximately 56% of cases are at high risk for urethral techniques have been described to implement RUG, the
injury. Among adults, only 24% are at high risk for urethral most common utilizes a Foley catheter. With this method,
injury. the patient is ideally positioned for imaging in an
A diagnosis of urethral trauma should be investigated approximate 45° oblique angle with the penis stretched
in the presence of pelvic fracture, straddle injury, so that the meatus points cephalad. This produces a C
penetrating trauma in the vicinity of the urethra, or penile configuration from the bladder level to meatus tip. If the
fracture. While there are no findings specific for urethral penile shaft points caudad, the femur may obscure the
trauma, there are many that suggest its presence. Findings opacified urethra. For some patients with multiple injuries,
can include blood at the urethral meatus, gross hematuria, this position is unobtainable. In this case, the patient
an inability to spontaneously void, and a high riding should be supine with the penis stretched perpendicular
prostate on rectal examination. to the leg. When the image is obtained in this antero-
For many patients with urethral injury, extravasation posterior projection, however, the urethra can appear
of blood contained within different fascial planes is also foreshortened, allowing for possible errors in inter-
present. pretation of extravasation.
The Foley catheter is then placed inside the urethra
ROLE OF RADIOLOGY AND IMAGING with the balloon inflated in the fossa navicularis.
Retrograde Urethrography Approximately 20-30 mL of 30% contrast material is
injected into the urethra, with the exposure being made
The possibility of urethral trauma should be properly
during the active injection of the last few mL of contrast.
investigated by retrograde urethrography. This should
Obtaining the image during the injection allows for
always be done prior to the insertion of a urethral catheter.
maximum filling of the deeper bulbar, membranous, and
If, however, a urethral catheter is properly in place prior
prostatic urethral sections. In the most ideal conditions,
to evaluation for urethral trauma, it should not be
the entire procedure should be performed under fluoro-
removed in order to perform urethrography. In such a scopic control; however, in the emergent environment
case, a pericatheter urethrogram may be obtained. this is often impossible.
After a diagnosis of urethral trauma has been made,
management and repair can be planned with the possible Classification of Retrograde Urethrography Findings
aid of other imaging modalities, such as MRI and
The most accepted and unified classification of RUG
ultrasonography. MRI has some utility in planning surgical
findings for urethral injuries is the Goldman classification,
approach for posterior urethral disruptions, and ultraso-
with its foundation in the earlier system developed by
nography has been used at times to aid in the repair of
Colapinto and McCallum (Table 9.2).
urethral trauma.
The Goldman classification of urethral trauma is
Limitations: While RUG provides clinically valuable defined entirely on the anatomical findings of the injury
information on the presence, location, and severity of and not on its mechanism.
Table 9.2: Classification of urethral injuries

Colapinto and McCallum Goldman and Sandler
Grade I Posterior urethra stretched, Posterior urethra stretched
But intact but intact
Grade II Posterior urethral tear above Partial or complete
Intact urogenital diaphragm Posterior urethral tear above
(UGD) intact urogenital diaphragm
Grade III Posterior urethral tear with Partial or complete
Extravasation through torn tear of combined
UGD anterior and posterior
Urethra with torn UGD
Grade IV — Bladder neck injury with
Extension to the urethra
Grade IVa __ Injury to bladder base with
Extravasation simulating type
IV (pseudo grade IV)
Grade V __ Isolated anterior urethral injury
This system defines five major types of urethral injuries depends upon the exact location of the injury and the
as seen in RUG. degree of disruption to the perineal membrane.
Type II or III urethral injury can be further classified
Type I urethral injury results when the puboprostatic
as a partial or complete urethral tear (Goldman, 1997).
ligament is ruptured, and the prostate is allowed to move
With RUG, partial tears are diagnosed when extravasation
superiorly. The urethra remains intact however, and is
of contrast material occurs with the presence of contrast
only severely stretched by the movement of the prostate. material in the bladder. Complete tears are diagnosed
No extravasation of contrast material is seen with radio- when extravasation is present and no contrast agent is
graphy, and continuity is maintained with the bladder. present in the bladder or in the proximal torn end of the
True cases of Type I urethral injury are uncommon. urethra. The relative frequency of partial tears versus
Type II urethral trauma is the classically described complete tears is highly variable in the literature, and
posterior urethral injury in which the urethra is torn no reason for this variance has been agreed upon.
superior to the urogenital diaphragm. In such an injury, Type IV urethral trauma is a tear to the bladder neck
contrast-agent extravasation is seen within the that extends into the proximal urethra. Contrast-agent
extraperitoneal pelvis, but contrast material is not present extravasation is seen in the extraperitoneal pelvis around
within the perineum. Here, the urogenital diaphragm is the proximal urethra. Such injuries can damage the
intact, preventing the spread of contrast material inferiorly. internal urethral sphincter, resulting in incontinence.
This type exists in approximately 15% of urethral trauma Proper diagnosis is therefore essential to ensure adequate
cases resulting from pelvic crush injuries. patient care.
A related injury, as described in the Goldman
Type III urethral injury: The most common type of classification, is type IVA. This is not a urethral injury;
urethral trauma has proven to be type III urethral injury. however, it can easily be mistaken for a proximal urethral
Type III urethral injury, like type II, shows disruption above tear. In this case, the base of the bladder is disrupted,
the urogenital diaphragm. Unlike type II, though, this with periurethral extravasation of contrast agent. The
injury extends through the urogenital diaphragm and resulting radiographs can easily mimic those of a true
includes the proximal bulbous urethra. In this injury, type IV urethral trauma. Distinguishing the two conditions
extravasation can be found within the extraperitoneal is important because type IV injury is typically treated
pelvis and within the perineum. The amount of contrast surgically and type IVA injury is not. Dynamic RUG under
material found above or below the urogenital diaphragm fluoroscopic control facilitates the differentiation.
Urorenal Trauma 137
Type V urethral trauma describes all cases that are historically described few applications of CT in diagnosing
isolated to the anterior urethra. Such an injury occurs urethral injuries.
distal to the urogenital diaphragm and is more associated A distance of 2 cm between the prostatic apex and
with perineal crush or straddle injuries. The resulting urogenital diaphragm was specific for type I urethral
urethral injury is usually a partial tear of the bulbous injuries. The CT findings specific for type II and type III
urethra, though complete tears can also occur. In this urethral trauma were contrast-agent extravasation above
case, contrast-agent extravasation occurs inferior to the the urogenital diaphragm and extravasation below the
urogenital diaphragm. If the Buck fascia remains intact, urogenital diaphragm, respectively. CT findings associated
the extravasation is limited to its confines, ie, the penile with but not specific for urethral trauma were distortion
shaft. If the Buck fascia is disrupted, the contrast material or obscuration of the urogenital fat plane, hematoma of
contained within the limits of the Colles fascia. In this the ischiocavernosus muscle, distortion or obscuration
case, contrast agent might be found in the lower abdomen of the prostatic contour, distortion or obscuration of the
and in the scrotum. bulbocavernosus muscle, and hematoma of the obturator
internus muscle.
Ultrasonography Because many patients with generalized trauma
Like MRI and CT, ultrasonography alone has not yet undergo CT before a specific evaluation for urethral
proven adequate and is not typically used for the primary trauma, CT might serve as an initial screening exami-
diagnosis of urethral trauma. However, a few reports nation for such injuries. Presently, no test supersedes RUG
suggest that ultrasonography can be used for defining for the confirmation of urethral trauma. CT might help
the extent of urethral damage in certain cases and for exclude unnecessary RUG.
preparing for surgical repair. Because few studies have been conducted to evaluate
High-frequency probes used in sonourethrography the accuracy of CT in identifying urethral trauma, further
provide a high spatial resolution; therefore, details of investigation is necessary for a well-defined degree of
urethral anatomy can be studied after the injection of confidence (Figures 9.9 and 9.10).
a saline solution. This saline solution technique uses a All specific findings are found only in patients with
Foley catheter in a similar manner as described for RUG urethral trauma. Among associated findings, distortion
to promote distension of the urethra. The presence of saline and obscuration of the urogenital fat plane was found
in the urethra produces high contrast relative to the urethral in 88% of those with urethral trauma and in 3% without
mucosa. Thus, this technique allows accurate visualization urethral trauma. Hematoma of the ischiocavernosus
of the urethral wall as well as the urethral lumen. muscle was found in 88% with urethral trauma and in
Only a limited number of reports exist in the literature 17% without urethral trauma. Distortion or obscuration
regarding the usage of sonourethrography. Such cases of the prostatic contour was present in 59% with urethral
include the use sonography for the diagnosis of urethral
trauma associated with penile fracture and in evaluating
anterior urethral trauma prior to delayed urethroplasty.
Sonourethrography has been shown to accurately depict
trauma to soft tissues surrounding the urethra, such as
the tunica albuginea. Other investigators have shown that
sonourethrography can more accurately measure stricture
length than RUG. This information could prove useful
for planning surgical repair for specific cases.
CT
Despite the prevalent use of CT as the initial screening
modality for general acute trauma, the literature has FIGURE 9.9: Posterior urethral rupture with contrast extravasation
MRI has proven clinical utility in its ability to define

damage to soft-tissue neighboring the urethral trauma.
Alone, however, MRI should not be used to investigate
urethral extravasation or to define urethral trauma as
partial or complete.
INTERVENTION
Interventional radiology has played only a small role in
the treatment of urethral injuries. In a few situations,
delayed realignment of the urethra following suprapubic
diversion has been accomplished under radiological
FIGURE 9.10: Extraperitoneal bladder rupture guidance.
Fluoroscopic guidance for percutaneous suprapubic
trauma and in 7% without urethral trauma. Distortion
cystotomy has also been suggested for clinical use when
or obscuration of the bulbocavernosus muscle was present
the bladder is distorted by a retroperitoneal hematoma
in 47% with urethral trauma and in 10% s without urethral or when the bladder has been displaced superiorly.
trauma. Hematoma of the obturator internus muscle was Treatment techniques for urethral tears include
found in 53% of patients with urethral trauma and in suprapubic cystotomy with delayed repair, immediate
13% without urethral trauma. realignment, and immediate suturing. For suprapubic
cystotomy with delayed repair, the incidence of stricture
MRI
is 97%, the incidence of incontinence is 4%, and the
Traditionally, MRI has not been used as an initial incidence of impotence is 19%. With immediate
diagnostic tool for urethral traumatic injuries; nevertheless, realignment, the incidence of stricture is 53%, the
some researchers have demonstrated the advantage of incidence of incontinence is 5%, and incidence of
using MRI as a preparatory tool when planning surgical impotence is 36%. For immediate suturing, the incidence
repair of urethral disruption. of stricture is 49%, the incidence of incontinence is 21%,
One of the most common methods of treatment for and the incidence of impotence is 56%.
urethral injury is delayed reconstruction after 3–4 months
MORTALITY/MORBIDITY
of suprapubic cystotomy. After the initial delay, the surgical
reconstruction is usually done with a transperineal or The three most common morbidities associated with
combined transperineal and transpubic approach with urethral trauma are stricture, incontinence, and
impotence.
pubectomy. The approach chosen is dependent on the
The incidence of these morbidities is dependent on
length of the urethral disruption, the degree of prostatic
the severity of the injury and the method of management
dislocation, and the amount of scar tissue present.
and repair.
MRI has shown positive results in evaluating the
anterior-posterior, superior-inferior, and lateral SUMMARY
displacement of the prostate; the degree of scar tissue Urethral trauma primarily affects males, because of the
around a urethral defect; and the precise length of a short length of the female urethra. The male urethra is
posterior urethral defect. In one study, the results of MRI divided into posterior (membranous and prostatic) and
preoperative evaluations changed the surgical repair anterior (bulbous and pendulous) portions, at the
approach in 26% of the patients studied. Because of its urogential diaphragm. Urethral injuries are associated with
superiority in defining local disruption to adjacent tissues, high rates of stricture, impotence and incontinence.
MRI can be an important tool in combination with RUG Anterior urethral injuries usually result from a low velocity,
in evaluating urethral trauma for management. compressive injury of the perineum –e.g. a straddle injury.
Urorenal Trauma 139
Again, blood at the urethral meatus, perineal hematoma, scarring, or spongiofibrosis. Scanning is performed during
hematuria and difficulty in voiding are often present. RUG retrograde injection of saline. Nodularity of the urethral
demonstrates contrast extravasation below the urogenital margins, acoustic shadowing, and narrowing of the urethra
diaphragm. all are indicative of spongiofibrosis.
Primary repair of urethral injuries is associated with Posterior urethral injuries are associated with high
severe stricture formation, incontinence, and impotence. velocity blunt force injury, pelvic fractures, and multi-
Initial treatment may consist of endoscopic realignment. organ trauma. The spleen, liver, bowel and bladder are
Catheter drainage for several weeks is then undertaken often also injured. A spectrum of injuries occurs, from
allow healing. Subsequent treatment of short segments stretching and elongation of the urethra, to laceration,
of scarring is frequently necessary. to transection.
In the evaluation of urethral strictures, the RUG is Exam findings include a high riding, boggy prostate,
again the initial examination. Voiding cytourethography blood at the urethral meatus, and perineal hematoma.
is complementary, as it allows better distension of proximal Hematuria and difficulty in voiding are also often present.
urethral segments. Both techniques can underestimate Retrograde urethrogram (RUG) is the initial study of
bulbar strictures because of the oblique angle of this choice, and demonstrates abnormal urethral contour.
portion of the urethra to the beam. Sonography of the Extravasation of contrast occurs in cases of laceration
urethra is helpful in these cases to provide a more or transection. If the urogenital diaphragm is intact,
accurate measurement of the length of the bulbar stricture, contrast will collect in the pelvic extraperitoneal space.
which has a major impact on operative planning. If the UGD has ruptured, contrast will be present in the
Ultrasound is also useful in the assessment of penile perineum.
RENAL NEOPLASMS (PEDIATRIC AND ADULT) Table 10.1: Most common age at presentation for solid renal
malignancies
CLASSIFICATION OF RENAL NEOPLASMS (TABLE 10.1) Renal neoplasm Age range Peak age
1. Wilms’ tumor Wilms’ tumor
2. Nephroblastomatosis Unilateral form 1–11 yr 3½ yr
Bilateral form 2 mo–2 yr 15 mo
3. Mesoblastic nephroma Nephroblastomatosis Any age 6–18 mo
4. Ossifying renal tumor of infancy Renal cell carcinoma 6 mo–60 yr 10–20 yr*
5. Multilocular cystic partially differentiated nephro- Mesoblastic nephroma 0–1 yr 1–3 mo
blastoma Multilocular cystic renal tumor
Cystic nephroma Adult female Adult female
6. Clear cell sarcoma Cystic partially differentiated
7. Rhabdoid tumor of kidney nephroblastoma 3 mo–4 yr 1–2 yr
Clear cell sarcoma 1–4 yr 2 yr
8. RCC (Gravitz tumor/Hypernephroma)
Rhabdoid tumor 6 mo–9 yr 6–12 mo
9. Multilocular cystic nephroma Angiomyolipoma 6–41 yr 10 yrt
10. Renal medullary carcinoma Renal medullary carcinoma 10–39 yr 20 yr
11. Oncocytoma Ossifying renal tumor of infancy 6 d–14 mo 1–3 mo
12. Angiomyolipoma Metanephric adenoma 15 mo–83 yr None
Lymphoma
13. Metanephric adenoma Hodgkin >10 yr Late teens
14. Lymphoma (Hodgkin and Non-Hodgkin lymp- Non-Hodgkin Any age child < 10 yr
homa) * von Hippel-Lindau syndrome
15. Metastasis.
t
Tuberous sclerosis, neurofibromatosis, von Hippel-Lindau syndrome
is the most common solid abdominal mass and by far

WILMS’ TUMOR/ NEPHROBLASTOMA
the most common renal pediatric malignancy.
INTRODUCTION
It is a malignant, embryonic neoplasm containing INCIDENCE
epithelial, blastemal, and stromal elements. It has almost Incidence is around three years of age; approximately
the same overall incidence as neuroblastoma, accounts 80% of all cases are detected between 1 and 5 years
for approximately 8% of all childhood malignant tumors, of age. An asymptomatic mass is the most common
Urorenal Neoplasms 141
clinical presentation. Uncommonly, it may present as tumors (multiple separate primary tumors separated in
abdominal pain, anorexia, hematuria and hypertension. time) have a survival rate of only 40%.
GENETICS STAGING OF WILMS’ TUMOR

Although most Wilms’ tumors are sporadic, 1% demon- Staging of Wilms’ depends on the involvement as
strate autosomal dominance with variable penetrance. determined by imaging, surgery, and pathology. Anatomic
A child with a sibling or parent with bilateral Wilms’ tumor staging of Wilms’ tumor is as follows:
has a 30% risk for development of the tumor. • Stage I, tumor limited to the kidney and completely
resected;
ASSOCIATIONS • Stage II, tumor extending beyond the kidney but
Wilms’ tumor is associated with (Acronym: WHAGR) completely resected;
• Hemihypertrophy (overgrowth disorders) • Stage III, residual tumor confined to the abdomen
• Aniridia (Sporadic) without distant metastasis;
• Genital and renal malformations. • Stage IV, hematogeneous metastases to lung, liver,
bone, brain;
In congenital hemihypertrophy and Beckwith-
• Stage V, bilateral renal involvement appearing initially
Wiedemann syndrome (macroglossia, hepatomegaly,
or during the course of treatment.
gigantism, omphalocele, Wilms’ tumor), the mean age
of diagnosis of Wilms’ tumor is similar to that of the ROLE OF RADIOLOGY AND IMAGING
general Wilms’ population. However, Wilms’ tumor
Imaging of Wilms’ tumor should define the size and
occurs at an earlier age when associated with aniridia
location of the primary tumor, any local spread, and any
and Drash syndrome (pseudohermaphroditism, renal
distant metastases. Imaging is also used for surveillance
failure). Weakly associated with Wilms’ tumor are Soto of individuals at risk for primary or recurrent Wilms’ tumor.
syndrome (cerebral gigantism), neurofibromatosis and
Klippel-Trenaunay-Weber syndrome. Plain Radiography
PATHOPHYSIOLOGY
Usually shows a soft-tissue mass that displaces bowel.
The dystrophic calcification which occurs in 5% of Wilm’s
Wilms’ tumor is usually bulky and replaces most of the tumors is curvilinear or amorphous is distinguished from
involved kidney. The renal capsule is usually intact; rarely, the stippled or flaky calcifications that occur in 55% of
the tumor breaks through this capsule and extends into neuroblastomas (Figure 10.1).
the extrarenal spaces. Wilms’ tumor may invade the renal
vein and inferior vena cava. Venous extension of Wilms’
tumor follows the “rule of 10’s”: 10% extend into the
renal vein; 10% of that group extends into the IVC; 10%
of the latter further extend into the right atrium. Distal
metastases most commonly involve the lungs; the liver
is next most common.
MORTALITY AND MORBIDITY

The most important prognostic factor in Wilms’ tumor
is histology. Approximately 10% of all Wilms’ tumors
have unfavorable histology with anaplasia. Wilms’
tumor is bilateral in approximately 5% of cases.
Synchronous tumors (bilateral tumors at initial FIGURE 10.1: Two well circumscribed nodules in right upper zone:
Metastases from Wilms’ tumor with, haziness of mass on right side
presentation) have a 87% survival while metachronous of abdomen without calcifications
IVU structures, identifies nodal involvement, and evaluates

See Figure 10.2. for liver metastases, and images the contralateral kidney.
The tumor mass is usually rounded, low attenuation and
heterogeneously enhancing. The vessels are displaced,
not encased as in neuroblastoma. Abdominal CT also
evaluates the renal vessels, IVC, lymph nodes, liver and
contralateral kidney. Chest CT is also performed,
as pulmonary metastases are present in up to 20% of
patients at the time of diagnosis. CT is also excellent for
surveillance after resection (Figures 10.4 to 10.6).
FIGURE 10.2: Displaced, stretched and distorted calyces due to

mass involving right kidney at mid and lower pole
Ultrasound
It is usually the first imaging modality for a child with
FIGURE 10.4: CECT-Axial section: well-defined, rounded low
a palpable abdominal mass. Wilms’ tumor is seen as a attenuation mass in the right side of abdomen showing heterogenous
large, sharply marginated, echogenic mass. Hypoechoic but bright enhancement
areas may represent hemorrhage, necrosis or dilated
calyces. Venous extension is diagnosed when intra-
vascular echogenic focus is identified (Figure 10.3).
FIGURE 10.3: Large, sharply marginated, isotohyperechoic mass

involving mid and lower pole of right kidney
Computed Tomography
CT confirms the presence of an intrarenal mass, deter- FIGURE 10.5: Coronal reconstruction image of same patient,
mines the extent of the Wilms’ tumor, visualized vascular showing lung metastases and the Wilms’ tumor, together
of the interaction of the advancing and bifurcating ureteric

bud which branch and form the collecting system of the
kidney and the metanephric blastema which give rise to
form epithelial and stromal elements of the kidney.
Metanephric blastema lies peripherally in the subcapsular
and interlobular spaces. Nephrons and associated
supporting structures arise from the metanephric
blastema. Nephrogenesis is complete in the normal fetus
at 34–36 weeks of gestation.
Nephroblastomatosis is defined as the persistence of
metanephric blastema, also known as nephrogenic rests,
into infancy and childhood.
Beckwith classified nephrogenic rests as:
1. Dormant (nascent),
2. Sclerosing (obsolescent) or
FIGURE 10.6: Coronal reconstruction of thorax showing 3. Hyperplastic/neoplastic.
lung metastasis
Thus, nephrogenic rests may regress, sclerose or
Magnetic Resonance Imaging become hyperplastic/mitotically active or give rise to larger
and frankly neoplastic rests such as Wilms’ tumor.
MRI is becoming the preferred imaging modality because
the multiplanar imaging capability and excellent contrast ROLE OF RADIOLOGY AND IMAGING
between vessels and adjacent soft tissues. In general,
Nephroblastomatosis can be differentiated from Wilms’
Wilms’ tumors are T1 dark and T2 bright.
tumor by its gross and microscopic appearance and
Differences between Wilms’ tumor and neuroblastoma
imaging characteristics. Nephroblastomatosis is usually
are important (Table 10.2)
diffuse, involves the entire subcapsular portion, has no
Table 10.2: Differentiating features of Wilms’ tumor and renal capsule, has a lobulated margin separating it from
Neuroblastoma
underlying uninvolved kidney and has a uniform pink-
Finding Wilms’ tumor Neuroblastoma
flesh appearance and firm consistency. On the other hand,
1. Location and epicenter Intrarenal Extrarenal
2. Shape Round or oval Irregular Wilms’ tumors are usually a bulky, spherical mass,
3. Hemorrhage and Common Rare occupies one portion of the kidney with areas of necrosis,
necrosis
4. Calcifications Rare Common hemorrhage and macroscopic cysts. On ultrasound,
5. Vasculature Invades Encases and nephroblastomatosis may show enlarged kidneys and
displaces
6. Crosses midline Uncommon Common hypoechoic foci in a diffuse or mutifocal pattern or normal
7. Distorts calyces Most Rare appearing enlarged kidneys. On CT, nephroblastomatosis
8. Retroperitoneal lymph Uncommon Common
nodes or contiguous may appear as a multifocal or diffuse subcapsular layer
extension
of abnormal hypodense tissue. Nephrogenic rests may
not demonstrate enhancement with IV contrast material.
NEPHROBLASTOMATOSIS Normal enhancing renal tissue is typically seen in the
INTRODUCTION central portion of the kidney (Figure 10.7).
It is a complex abnormality of nephrogenesis. Normal
developmental anatomy of the kidney occurs when the TREATMENT
ureteric bud contacts aggregates of primitive nephroblasts Treatment options for patients with nephroblastomatosis
(metanephric blastema) in the paraspinal region of the include follow-up with ultrasound or CT every 2–6 months.
developing embryo. Renal development occurs as a result Chemotherapy in nephroblastomatosis is controversial.
Imaging Findings
General features
• Best imaging clue
– CECT: Multilocular cystic mass herniating into
renal hilum
• Plain film
– Soft tissue mass (particularly if large and displaces
adjacent structures)
– Curvilinear/amorphous calcification CT Findings.
NECT
– Large/well-defined/encapsulated/multiloculated
cystic mass
– CT HU equal to water/higher than water (gela-
FIGURE 10.7: Right sided nephroblastomatosis tinous fluid)
– Solid component (CT depict very small cysts as
solid component)
Beckwith recommends use of chemotherapy in stage 1
– Curvilinear/amorphous calcification
WT and histologically proven nephroblastomatosis to
• CECT (Figures 10.8A and B)
decrease the size of the lesion. Although potential for
– Septa: Moderate enhancement (regular and thick)
malignant transformation is low, the mass effect of
– Capsule: Enhancement
growing nephrogenic rests may mechanically injure the
– Cystic component: No enhancement (Figures
kidney. These benefits may be outweighed against the
10.8A and B)
potential long-term side effects and unproven efficacy
(A) Cystic mass wit
of chemotherapy.
– Distortion of colli MR Findings
• T1 WI: Multiloculated I.
MULTILOCULAR CYSTIC NEPHROMA
Mass consisting of multiple noncommunicating cysts
herniates into the renal pelvis.
Key Facts
• Synonym(s): Multilocular cystic renal tumor/benign
cystic nephroma
• Definition: Multiple noncommunicating cysts within
a well-defined capsule. Other key facts
• Rare nonhereditary benign renal neoplasm
• Usually solitary but rarely multiple
• Location: Typically unilateral (usually lower pole)
• Usually symptomatic in adults/asymptomatic
palpable mass in children 0 tumor may grow slowly
over years or rapidly within months
• Males: Approximately 90% of tumors occur in first
2 years of life
• Females: Equally divided between < 5 years and FIGURE 10.8A: Schematic representation of multicolor cystic
between 40 and 60 years. nephroma (For color version see Plate 3)
Ultrasound Findings
• Large/well-defined multiloculated cystic mass
• Innumerable anechoic cysts+hyperechoic septa
• Thick hyperechoic fibrous capsule
• Hyperechoic areas within cystic tumor mimicking a
solid component (due to numerous tiny cysts causing
acoustic interfaces)
Angiography Findings
• Hypovascular mass (rarely avascular/hypervascular)
Imaging Recommendations
• NE+CECT; MR+CEMR; US.
• Multilocular cystic renal cell carcinoma (RCc)
FIGURE 10.8B: Axial CECT section of abdomen: Large, well defined,
• Usually has mural nodularity/solid component
encapsulated, multiloculated low-attenuation cystic mass in left side • May be indistinguishable from multilocular cystic
of abdomen with moderate enhancement of septae nephroma.
Cystic Wilms’ Tumor
• T2WI: Multiloculated / CET1WI: Enhanced excertory • Grows entirely by expansion of large cystic spaces
urography film. within stroma
• Depending on size and I. • Septa: Numerous and thick.
– Obstructed collect 0 : Tumor herniates/p Multicystic Dysplasia
tumor of collecting • Usually involves entire kidney
Differential Diagnosis • Segmental multicystic dysplasia: Associated with
ureteral duplication.
1. Multilocular Cystic Renal Cell
2. Cystic Wilms’ Tumor Pathology
3. Multicystic Dysplasia
4. Renal neoplasm General
(A) Cystic mass with thick septa and wall. • Embryology–Anatomy
– Tumor arises from metanephric blastema
(B) Mass herniates into renal hilum.
• Etiology–Pathogenesis
• Distortion of collecting system/herniation of mass into
– Unknown
renal hilum MR.
– Theories of pathogenesis: Dysplasia/hamartoma/
Findings neoplasia
• TlWI: Multiloculated hypointense mass (fluid intensity) • Epidemiology
• T2WI: Multiloculated hyperintense mass – Rare tumor
• CET1WI: Enhancement of thick septa. Gross Pathologic–Surgical Features
• “Honeycombed” cystic areas of varied sizes/thick
Excretory Urography Findings
fibrous capsule. Non-communicating locules
• Depending on size and location of mass
separated by thick fibrous septa
– Obstructed collecting system/pyelocaliectasis
• Mostly intraparenchymal tumors.
– Tumor herniates/protrudes into renal pelvis
(mimicking a primary tumor of collecting system) Microscopic Features
• Tomogram of nephrographic phase: May show • Locules: Lined by flattened/cuboidal epithelium
septations. • Septa contains fibrous tissue + tubular elements
• Cystic poorly differentiated nephroblastoma • Excessive weight

– Septa contain blastemal cells I other embryonal • Chronic dialysis use
elements • Several genetic syndromes (familial RCC, hereditary
• Cystic nephroma: Septa have no undifferentiated papillary RCC, von Hippel-Lindau syndrome, and
elements. tuberous sclerosis).
Clinical Issues PATHOPHYSIOLOGY

Presentation RCCs arise from the tubular epithelium and are usually
• Adults: Abdominal pain/I palpable mass/hematuria/ based in the renal cortex. Several pathologic subtypes
urinary tract (UT) infection have been described, including the clear cell, papillary,
• Children: Nonpainful palpable abdominal mass I granular cell, chromophobe cell, sarcomatoid, and
hematuria UT infection . Age: Biphasic age + sex collecting duct subtypes. These tumors vary from being
distribution nearly completely cystic to being completely solid. The
• Predominantly boys in childhood and women in imaging features reflect this heterogeneity. Bilateral RCCs
adulthood are common in von Hippel-Lindau syndrome, tuberous
• Size: Few cm to > 30 cm (average size 10 cm) sclerosis, and chronic dialysis; however, bilateral RCCs
• Lab: U/A may show RBC/WBC occur in only approximately 2% of sporadic cases of RCC.
• Complications: Local recurrence/obstructive uropathy/ RCCs are multicentric in as many as 25% of patients.
rarely malignancy. Spread by means of direct local invasion of adjacent
structures, such as the adrenal glands, liver, spleen, colon
Treatment
or pancreas, can occur. Local regional lymph node
• Surgical: Nephrectomy (complete/partial) metastases are also common. RCCs have a propensity
Prognosis to extend into the renal vein and, subsequently, into the
• Good: After nephrectomy inferior vena cava. The lungs are the most common sites
• Few cases: Local recurrence. of distant metastases. Liver, bone, adrenal gland, and
kidney metastases may also occur. Typically, skeletal
RENAL CELL CARCINOMA (RCC) metastases are purely lytic and expansile.
INTRODUCTION STAGING
It is the most common primary renal malignant neoplasm RCCs can be staged by using the American Joint
in the adult. It accounts for approximately 85% of renal Committee on Cancer TNM classification, as follows:
tumors and 2% of all adult malignancies. RCC is more • Stage 1: RCCs are 7 cm or smaller and confined to
common in men than in women (ratio, 2:1), and it most the kidney.
often occurs in patients aged 50–70 years. One-fourth • Stage 2: RCCs are larger than 7 cm but still organ
to one-third of patients have metastatic disease at the confined.
time of presentation. In only approximately 2% of • Stage 3: tumors extend into the renal vein or vena
sporadic cases are bilateral tumors seen at presentation, cava, involve the ipsilateral adrenal gland and/or
especially in von Hippel-Lindau syndrome. perinephric fat, or have spread to one local lymph
node.
RISK FACTORS • Stage 4: tumors extend beyond the Gerota fascia, to
• Increased age more than one local node or have distant metastases.
• Male sex, smoking Recent literature has questioned whether the cutoff
• Cadmium, benzene, trichloroethylene and asbestos in size for stage 1 and 2 tumors should be 5 cm instead
exposure of 7 cm.
Staging of RCC (Robson vcrsus TNM staging) (Figures ANATOMICAL BASIS

10.9 to 10.13B).
The kidney is a retroperitoneal structure surrounded by
Robson Disease extent a fibrous capsule and enclosed in the perirenal space
I Tumor confined to kidney with the adrenal gland and fat. In the general population,
Small < 2.5 cm 70–80% of individuals have single renal arteries to each
Large > 2.5 cm kidney, and the remainders have multiple arteries.
II Tumor spread to perinephric fat Multiple renal veins are rarer, occurring in approximately
IIIA Tumor spread to renal vein or inferior vena 10% of patients. The vascular anatomy becomes
cava important whenever minimally invasive surgery or
IIIB Tumor spread to local lymph nodes
IIIC Tumor spread to local vessels and nodes
IVA Tumor spread to adjacent organs, outside
Gerota’s fascia
IVB Distant metastasis.
INCIDENCE
Approximately 2% of adult malignancies.
FIGURE 10.9: Schematic: Stage 1 RCC-limited to capsule FIGURES 10.10A AND B: Stage 2 RCC-Perinephric spread been
(For color version see Plate 4) (For color version for figure 10.10A see Plate 4)
A
A
B B
FIGURES 10.11A AND B: RCC with thrombus in renal vein FIGURES 10.12A AND B: RCC with thrombus up to IVC
(For color version for figure 10,11A see Plate 5) (For color version for figure 10.12A see Plate 5)
nephron-sparing surgery is considered because control pain, or (less frequently than in the past) a flank mass.
of potentially bleeding vessels is paramount. Currently, nearly half of RCCs are discovered incidentally
during imaging for indications other than the assessment
CLINICAL DETAILS of RCC. In one series, 0.3% of all CT scans demonstrated
Sex: RCC is more common in men than in women, with incidental RCC. Incidental detection has also increased
a male-to-female ratio of approximately 2:1. on ultrasound. Occasionally, patients present with
Age: The incidence peaks in patients aged 50–70 years, systemic symptoms such as fever, nausea, anorexia, and
but the age distribution is broad. RCC rarely occurs in weight loss. Rarely, patients have symptoms related to
young children. humoral factors such as parathormone, prolactin,
Clinically, patients present with hematuria, flank erythropoietin, or renin.
FIGURE 10.13A: Left RCC with RV thrombosis
FIGURE 10.14: Plain radiography in case of RCC
stage the disease. If the lesion appears to be a cyst, USG

is the next best test in the patient’s work-up.
Findings of RCC are nonspecific and include mass
effect on the collecting system, distortion of the renal
contour, enlargement of a portion of the kidney, and
FIGURE 10.13B: RCC with right renal vein thrombosis calcifications. If good nephrotomograms are obtained at
peak renal enhancement, most RCCs are less attenuating
PREFERRED INVESTIGATIONS than surrounding renal parenchyma. Renal vein invasion
may be inferred if contrast material excretion by the
Plain Radiography
affected kidney is poor or absent. Alternatively, this finding
Plain radiographic findings are often unrevealing in may result from extensive involvement of the kidney or
patients with RCC unless the mass contains detectable ureteral obstruction caused by mass effect.
calcification or is large enough to distort the normal renal
contour. Plain radiography has no role in the primary USG
search for RCC or in the follow-up observation of patients The primary limitations of USG include problems related
with RCC because of its limited sensitivity and specificity to incomplete staging (bones, lungs, regional nodes)
(Figure 10.14). and to the detection of small non-contour deforming
masses. In addition, large patients are not good
Intravenous Urography
candidates for USG. For the work-up in RCC, USG is
IVU is also limited in depicting RCCs. Large lesions, which used primarily to differentiate solid masses from simple
can distort the renal contour or the collecting system, may cysts and to visualize the internal architecture of lesions
be detected. If RCC is suggested, further imaging with more effectively than can be accomplished by using CT
CT or MRI is necessary to confirm a solid mass and to or MRI.
Findings: On sonograms, RCC can be isoechoic,

hypoechoic, or hyperechoic relative to the remainder of
the renal parenchyma. Smaller lesions with less necrosis
are more likely to be hyperechoic and may be confused
with AMLs. Isoechoic tumors are detected only by
distortion of the renal contour, focal enlargement of a
portion of the kidney, or distortion of the central sinus
fat.
A prominent column of Bertin or fetal lobulation may
mimic a solid renal mass and can be resolved with
dedicated CT or MR.
CECT
FIGURE 10.15: Well circumscribed left RCC with central necrosis
Although a variety of modalities can be used in the
workup of patients with suspected RCC, the preferred
method of imaging these patients is dedicated renal CT.
In most cases, this single examination can be used to
detect and stage RCC and to provide information for
surgical planning without additional imaging.
Protocol: The dedicated renal CT examination consists
of thin-section (2.5–5 mm) helical imaging of the kidneys
before the intravenous administration of contrast agent,
followed by imaging after 60-70 seconds and after
3–5 minutes. The imaging parameters (kV, mA, field of
view, section thickness) should be kept constant for all
phases of imaging to enable comparison of the
attenuation measurements. The addition of an arterial
phase CT (either with bolus tracking or after a 20–25
second delay) with thin slices (1–2 mm) may be helpful FIGURE 10.16: IVC thrombus upto right atrium
to evaluate arterial anatomy, especially if partial resection
is contemplated or renal parenchymal or vascular
anomalies are suspected.
Findings: On nonenhanced CT scans, RCCs may appear
as isoattenuation, hypoattenuation, or hyperattenuation
relative to the remainder of the kidney. Calcifications may
be present and are usually amorphous and internal,
although rim-like calcifications can also be present.
On contrast-enhanced CT scans, RCC is usually solid,
and evidence of necrosis is often present. Sometimes RCC
is a predominantly cystic mass, with thick septa and wall
nodularity.
RCC may also appear as a completely solid and highly
enhancing mass (Figures 10.15 to 10.18). FIGURE 10.17: Right RCC with extension into IVC and RRV
than 8–10 mm in which pseudoenhancement may be

a problem. In these cases, USG may be of some use in
characterizing the lesions as cysts.
In addition, spread to regional lymph nodes in the
absence of lymph node enlargement can be missed.
If contrast material cannot be intravenously
administered, CT is a poor choice and MRI should be
performed instead.
MRI
Findings: MRI findings are similar to those of CT, with
masses ranging from predominantly cystic with septa or
nodularity to solid with enhancement. The numeric
criteria for enhancement are not defined for MRI as they
are for CT, but MRI signal intensity changes can be
FIGURE 10.18: Right RCC with multiple dilated vascular channels
measured.
supplying it–hypervascular mass On nonenhanced T1 eighted images, RCCs usually
appear isointense or hypointense relative to the remainder
of the kidney. With chemical shift imaging, some clear
cell carcinomas show focal or diffuse loss of signal
intensity. On T2 weighted images, RCCs are usually
hyperintense. Most often, they are heterogeneous.
The presence of necrosis or hemorrhage may alter
these signal intensity characteristics. MRI may be
especially helpful in imaging the superior or inferior poles
of the kidneys, in direct coronal or sagittal imaging, and
in determining invasion of venous structures (Figure
10.19).
MRI is limited by patient cooperation because MRI
is more sensitive to motion artifact than CT. In addition,
MRI is more expensive and less readily available than
FIGURE 10.19: Right RCC on T2 MRI
CT. Furthermore, patients with pacemakers, those with
certain types of medical implants, and those with severe
Staging of RCC, which can be performed by using
claustrophobia are excluded from undergoing MRI.
CT or MRI, includes the assessment of ipsilateral or
MRI has no advantage compared with contrast-
contralateral adrenal involvement, direct extension into enhanced CT for the diagnosis of RCC, but MRI is
adjacent organs, enlargement of retroperitoneal lymph superior to nonenhanced CT.
nodes, invasion of the ipsilateral renal vein (with or
without extension into the inferior vena cava), and distant RNI
metastatic disease (liver, bone, lungs). Retrocrural,
Findings: In a patient with a suspected renal mass, nuclear
subcarinal, or mediastinal lymph nodes can also be
medicine studies help in differentiating the mass from
enlarged.
a pseudomass (e.g. column of Bertin, dromedary hump,
Limitations: The primary limitation of CT is in the fetal lobulation). Scintigraphy with technetium dimethyl-
characterization of hypoattenuation in masses of smaller succinic acid demonstrates normal uptake in the region
of a pseudomass, whereas a real mass causes a focal Complication rates are low. Radiofrequency ablation can
photopenic defect. also be considered in patients with local recurrence. In
Bone scanning with technetium methylene diphos- patients considered for ablation, preprocedure biopsy
phonate is indicated to confirm bony metastatic disease may be important because, in one series, nearly 10
in a patient with RCC and symptoms referable to the (nearly 40%) of 27 patients referred for ablation had
skeleton. a benign diagnosis.
Angiography DIFFERENTIAL DIAGNOSIS
Noninvasive cross-sectional imaging (CT, MRI, US) has Oncocytomas cannot be reliably differentiated from
replaced angiography in the work-up of patients with RCC without pathologic analysis. Macroscopic areas
known or suspected RCC. Advances in CT angiography of fat in the tumor mass are reported in RCC, but they
and magnetic resonance angiography have diminished are extremely rare. Almost all renal tumors with
the need for preoperative conventional angiographic measurable areas of fat are angiomyolipomas (AMLs),
mapping of the renal vasculature prior to nephron-sparing but some AMLs do not contain visible fat and may be
mistaken for RCCs. In one series, homogeneous and
or minimally invasive surgery. Angiography is still
prolonged enhancement were valuable predictors for
occasionally used if the origin of a tumor (e.g., renal vs
differentiation of AML with minimal fat from RCC. High
adrenal) is not certain. In these patients, selective injection
attenuation on nonenhanced CT scans and the degree
of the renal and adrenal arteries, as well as additional
of enhancement were helpful but less valuable.
vessels, may be necessary.
MORTALITY/MORBIDITY
INTERVENTION
Because RCCs are usually resistant to chemotherapy and The prognosis of patients with RCC depends on its stage
radiation therapy, surgical resection offers the best at diagnosis.
likelihood of cure. Unresectable RCCs have a 5-year The prognosis is worst for patients with metastatic
survival rate of less than 20%. disease at presentation and best for patients with small
When a solitary mass is noted in a patient with masses confined to the kidney.
suspected RCC, image-guided biopsy is generally The size of the primary lesion also affects the prognosis
unnecessary. In one series, fine-needle aspiration of because larger lesions tend to be higher grade and also
solitary proven RCC had a yield of 40% definitely metastasize more frequently. Poorly marginated or
malignant, 36% questionably malignant, and 24% necrotic lesions also tend to be of higher grade.
negative tumors. In a patient with a prior malignancy, Unresectable RCCs are associated with a 5-year
lymphoma, or multiple masses, fine-needle aspiration or survival rate of less than 20%.
core biopsy guided with CT or US may prove helpful
in treatment planning. In one series, 31 of 54 biopsies LYMPHOMA OF THE KIDNEYS
performed for a new renal mass in a patient with a known
INTRODUCTION
malignancy proved to be RCC.
Image-guided radiofrequency or cryogenic ablation It is discovered at the time of autopsy in almost one half
has been used to treat patients with RCC, especially of cases. It rarely is discovered with conventional urologic
patients with a high surgical risk, aversion to surgery, studies, such as intravenous pyelography. However, in
or bilateral lesions. Recent studies have reported short- patients with known lymphoma who often undergo cross-
term success rates of up to 97% with 1 or 2 ablation sectional imaging, such as CT scanning for staging
sessions, with size (<3 cm) being a significant purposes, renal involvement with lymphoma is commonly
determinant of tumor eradication in a single session. diagnosed.
PATHOPHYSIOLOGY As a result of the proximity of the kidneys to the psoas

Familiarity with tumoral growth and the mechanism of muscles, the oblique course of these muscles causes slight
lymphoma spread is crucial to understanding the resultant lateral displacement of the lower pole of each kidney.
imaging patterns of organ involvement. Renal lymphoma Between the two kidneys are the aorta and inferior vena
is often a secondary process that is discovered in patients cava, as well as the celiac plexus and the ganglia of the
with known lymphoma. autonomic nervous system. The renal artery and vein
Hematogenous involvement of the kidneys usually anterior to the renal pelvis run medially and anteriorly
results in a bilateral distribution of the tumor foci within from the hilus of each kidney. Although the left renal vein
the renal cortex. Tumor proliferation begins in the inters- normally courses anterior to the aorta to drain into the
titium and the underlying nephrons; however, collecting vena cava, it can occasionally run behind (retroaortic
systems and blood vessels provide a framework for tumor renal vein) and anterior or posterior (circumaortic) to the
growth. Infiltrative growth results in preservation of the aorta.
parenchymal structures and renal contour. Consequently, The renal parenchyma is enclosed in a thin, fibrous,
detection is often difficult, and renal involvement can easily glistening membrane that represents a true capsule.
be missed. As lymphomatous tumors enlarge, the CLINICAL FEATURES
surrounding renal parenchyma is compressed and
destroyed, and continuous tumor infiltration results in the Race: Renal lymphoma demonstrates no racial
formation of expansile renal masses. Nonuniform growth predilection.
can result in single or conglomerate masses that extend Sex: Renal lymphoma demonstrates no sex predilection.
beyond the renal contour and displace the collecting system
Age: Renal lymphoma occurs in all age groups.
and thereby resemble primary renal neoplasms.
Patients with renal lymphoma may not be sympto-
Primary renal lymphoma is controversial and a rare
disease. matic; however, the renal function panel occasionally
reveals elevated blood urea nitrogen (BUN) and creatinine
INCIDENCE levels, which may suggest an obstruction. Therefore, the
patient may need to undergo radiologic evaluation.
Incidence of renal involvement in patients with lymphoma
Occasionally, symptoms of back pain are attributed
is 34–62% in several autopsy series. Renal involvement
to the patient’s known underlying lymphoma. Sometimes,
occurs more commonly in non–Hodgkin lymphoma than
patients present with nonspecific signs and symptoms,
in Hodgkin lymphoma.
including flank pain, weight loss, hematuria, or a palpable
ANATOMICAL BASIS
mass. The nature of lymphomatous infiltration around
the renal pedicle is such that it may not cause vascular
The kidneys are bean-shaped structures located in the
compromise; however, the tumor usually surrounds the
Gerota fascia in the retroperitoneal region. The lateral
renal vascular pedicle. The tumor mass can obstruct urine
edge of the kidneys is convex, whereas the medial border
flow, resulting in hydronephrosis.
is concave with a marked depression or notch termed the
hilus. In adults, each kidney is approximately 11 cm long, PREFERRED INVESTIGATION
2.5 cm thick and 5 cm wide; it weighs 120-170 g.
IVU
Usually, the left kidney is approximately 1.5 cm higher
than the right. When the patient is lying in the supine It provides information regarding involvement of the
position, the superior pole of the left kidney is at the level collecting system, the functional status of the kidneys,
of the 12th thoracic vertebra, while the inferior pole is and the severity of hydronephrosis.
at the level of the 3rd lumbar vertebra. In the erect position Intravenous urography may show distortion of the
or during deep inspiration, both kidneys may descend collecting system resulting from single or multiple renal
near the iliac crest or lower. masses. This finding cannot be differentiated from findings
in simple cysts or renal cystic disease. However, intra- CT scans demonstrate a wide variety of appearances
venous urograms of renal lymphoma can demonstrate in renal lymphoma. CT patterns depend on the technique
normal or near-normal findings. of CT scanning. Helical CT is universally accepted as
the technique of choice in patients with renal involvement,
Limitations: Intravenous urography can depict only the
and it has a high degree of sensitivity in the detection
renal collecting system and suggestive large masses that
of renal lymphoma.
have caused calyceal distortion. Small parenchymal
Breath-hold images can be quickly obtained during
masses and tumors around the kidney and in the the multiple phases of renal enhancement after the
retroperitoneum are often not demonstrated with this intravenous injection of contrast medium. These images
diagnostic examination. are essentially free of motion artifact and respiratory
Ultrasonography misregistration. This approach helps in accurately
detecting and characterizing most renal masses, including
It is a good screening modality, particularly for the smaller lesions that may involve the renal parenchyma
assessment and follow-up of patients with hydronephrosis. in lymphoma. In addition, the vascular phase of contrast
On sonograms, the masses are often hypoechoic, enhancement enables characterization of the renal
reflecting tissue homogeneity, and they can be vasculature, which may be involved in renal cell
misidentified as renal cysts. carcinoma but uncommonly so in renal lymphoma.
Although gallium-67 citrate is an isotope that often Without intravenously administered contrast material,
accumulates in lymphomatous tissue, it can be taken up infiltrative forms of renal lymphoma can be entirely missed
by inflammatory masses. on CT studies. Also, CT scanning should not be limited
Nonenhanced CT or MRI can cause masses to be to the cortical medullary phase of contrast enhancement
missed, particularly when they are small. because the lesions can be missed in this phase.
Findings: Sonography often demonstrates single or Lymphoma typically involves the kidney in one of
multiple renal masses that have low-level echoes. several recognizable patterns. These patterns include
Occasionally, the ultrasonographic findings may appear multiple renal masses, a solitary mass, renal invasion from
normal because of the small size of the renal nodules. contiguous retroperitoneal disease, perirenal disease, and
In addition, varying degrees of hydronephrosis may be diffuse renal infiltration.
seen because of compression of the renal hilum caused Multiple renal masses are most common in patients
by lymph nodes or obstruction of the ureters. with lymphomas –The masses are typically bilateral, but
On sonograms, a perirenal hypoechoic halo is they may also be unilateral. Multiple masses are seen
characteristic of perirenal lymphoma. in approximately 60% of patients, and they reflect
advanced disease in patients presenting for staging
CT evaluation. Depending on the size and expansile nature
Numerous diagnostic studies are available for the of individual neoplastic deposits, little mass effect on the
evaluation of renal involvement by lymphoma; but CT renal contour may be seen. Therefore, although renal
remains the most sensitive, efficient, and comprehensive involvement may be extensive, findings can be subtle,
examination. CT scanning is the diagnostic modality of and intravenous contrast enhancement is essential. The
choice in patients with suspected renal masses. CT depicts masses range in size from a few millimeters to several
renal involvement in most patients with lymphoma. CT centimeters and are seen as areas of low attenuation.
often helps in defining the extent of disease within and Though the masses are often homogeneous on CT scans,
outside of the kidneys, and it provides information for they can be heterogeneous or cystic as well; this is typically
staging of the patient’s underlying malignancy. In a sign of tumor necrosis in patients undergoing
addition, the result of treatment can be assessed on follow- chemotherapy. In approximately 50% of patients,
up CT scans. associated retroperitoneal adenopathy is noted.
Solitary masses are reported in approximately In patients who are immunocompromised, the
10–20% of patients. The mass can be as large as 15 cm, prevalence of lymphoma is increased. Lymphoma
and it can cause significant distortion of the renal contour. typically occurs in patients with human immunodeficiency
Solitary lesions resemble other renal neoplasms that grow virus (HIV) infection or in secondarily immunosuppressed
primarily by expansion, including renal cell carcinoma recipients of organ transplants. Lymphoma is particularly
or isolated metastasis. common in patients who have undergone cyclosporine
Continuous retroperitoneal extension into the kidney therapy. Typically, lymphoma in patients who are
is a common pattern of renal involvement in lymphoma immunocompromised is of small noncleaved cell or
and is seen in approximately 30% of patients. Continuous immunoblastic origin and has a B-cell phenotype. It often
extension typically results in a large, bulky retroperitoneal has an extranodal origin, and the most common sites
mass that envelopes the renal vasculature and invades include the central nervous system, bone marrow,
the renal hilum. In most patients, the renal vascular pedicle gastrointestinal tract, lungs, heart, and liver. Renal
is patent and engulfed by the retroperitoneal mass. involvement also occurs in these patients, but it is
Continuous extension of retroperitoneal involvement in uncommon.
the renal collecting system can often cause obstruction,
MRI
and patients commonly present with hydronephrosis.
Rarely, perirenal disease is seen in an isolated form It provides information for characterizing the lesion, and
without involvement of the renal parenchyma or it also is helpful in assessing the renal vascular pedicle.
retroperitoneal nodes. Occasionally, this feature is seen Findings: MRI findings in lymphoma are similar to those
without compression of the renal parenchyma or seen on CT scans, and they are diagnostic to the same
degree. Usually, lymphoma displays low signal intensity
functional impairment. This finding is rare and is virtually
on T1 weighted images, and it is either isointense or
pathognomonic. A variety of CT manifestations of
moderately hyperintense on T2 weighted images.
perirenal lymphoma, including renal sinus infiltration,
Lymphomatous tissue may be minimally enhancing, but
thickening of the Gerota fascia, and perirenal masses,
it does not enhance as much as normal renal parenchyma;
have been described. The normal renal parenchyma
therefore, it remains hypointense relative to the kidney
cannot be differentiated from the perirenal abnormality
on contrast-enhanced T1 weighted MRIs.
if intravenous contrast enhancement is not used in CT
scanning. RNI
Infiltrative disease is associated with lymphomatous
RNI particularly that using gallium-67 citrate, have a
proliferation within the interstitium of the kidney, which
higher degree of specificity in the detection of lympho-
is manifested as nephromegaly with preservation of renal
matous infiltration of the kidneys.
contour. Diffuse infiltration often is bilateral and is seen
Findings: Gallium-67 citrate has been used for diagnosing
in approximately 20% of patients. The diagnosis is often
and staging lymphoma. This radioisotope concentrates
subtle and depends on the finding of global renal
in lymphoma. Gallium is taken up by lymphomatous
enlargement. Usually, kidneys infiltrated by lymphoma tissue in the kidneys, and it is also concentrated in
have poor function, but the disease is often clinically silent. inflammatory masses of the kidney; therefore, obtaining
Atypical manifestations of renal lymphomatous mass, the clinical history of patients is important.
such as spontaneous hemorrhage, necrosis, The use of a newer agent, technetium- 99mTc–labeled
heterogeneous attenuation, cystic transformation, and antibody (LL2), has been advocated in imaging and
calcifications, are occasionally depicted on CT scans. staging lymphomas.
Often, these findings are the result of prior treatment.
More commonly, the findings of renal lymphoma regress Angiography
after chemotherapy, and the kidneys regain a normal CT Findings: Usually, lymphoma is a hypovascular neoplasm;
appearance. therefore, angiography cannot help in the diagnosis or
staging of these tumors. As indicated, with contrast- gray on cut sections because of their fat content. They
enhanced CT, the lesions remain hypovascular compared are composed of fat and vascular and smooth muscle
with the renal parenchyma when contrast material is elements. AML is not a hamartoma by definition, because
delivered to the kidney, via either intravenous injection fat and smooth muscles are not normal constituents of
for CT scanning or direct arterial injection for angiography. renal parenchyma. AML is a choristoma; that is, as the
Therefore, the degree of confidence with angiography name implies, it is composed of variable amounts of
is low, and the degree of false-negative findings is vascular, muscular, and fatty tissues.
high. The blood vessels in these tumors frequently have
an angiomatous arrangement. These vessels are tortuous
INTERVENTION
and thick walled. They do not have elastic tissue, but
Patients with renal lymphoma are treated with they do have a disorganized adventitial cuff of smooth
chemotherapy, and surgical intervention is not indicated. muscle. AMLs do not have a capsule, but they often are
The lesions are treated with systemic chemotherapy, and well marginated. The majority (88%) extend through the
the response is usually favorable. The tumors often renal capsule into the perinephric space. The tumor is
completely respond to such treatment without residual slow growing. It is truly space occupying; it displaces the
disease in the kidneys. renal parenchyma and distorts the collecting system, and
Occasionally, when a single mass is noted in a kidney, sometimes causes renal destruction. The characteristic
biopsy may be attempted to differentiate renal lymphoma absence of elastic tissue in the tumor vessels predisposes
from a primary renal cell carcinoma. Primarily, biopsy the patient to aneurysm formation and spontaneous
is performed because the treatments for the two hemorrhage. The amounts of fat, smooth muscle, and
neoplasms completely differ. In addition, in patients with blood vessels in the tumor vary. In 5% of the tumors,
another known primary malignancy, biopsy is needed fatty elements can be detected only at microscopy; this
to differentiate metastasis to the kidney from renal limitation makes accurate characterization on the basis
infiltration by lymphoma. of radiologic findings alone impossible.
MORTALITY/MORBIDITY
INCIDENCE
Because involvement of the kidneys usually indicates The frequency is 0.3–3%. Approximately 80% of cases
disseminated disease, the prognosis is poor if the patient occur in patients with tuberous sclerosis.
does not receive proper treatment.
With treatment, renal lesions may completely regress, CLINICAL FEATURES
often with minimal scarring of the renal parenchyma.
Sex: The male-to-female for isolated AML is 1:4 to 1:8.
The male-to-female ratio for AML associated with
RENAL ANGIOMYOLIPOMA (AML)
tuberculous sclerosis is 1:1 to 1:2.
INTRODUCTION
Age: With isolated AML, most patients are aged 27–72
It is a benign renal neoplasm composed of fat, vascular, years, with a mean age of 43 years. With AML associated
and smooth muscle elements. It has an incidence of about with tuberous sclerosis, the mean age of patients 1–17
0.3–3%. Two types are described: isolated AML and AML years.
associated with tuberous sclerosis. Isolated AML, which occurs sporadically, often is
solitary and accounts for 80% of the tumors. The mean
PATHOPHYSIOLOGY patient age at presentation is 43 years, and isolated AML
AML is a benign tumor. The majority of AMLs are small is about four times more common in women than in men.
and single, although they vary in size from a few Interestingly, 80% of the cases involve the right kidney.
millimeters to larger than 20 cm. At gross pathologic AML associated with tuberous sclerosis accounts for
examination, they are round or lobulated and yellow to 20% of the tumors; these lesions are typically larger than
isolated AMLs, and they are often bilateral and multiple. When AMLs are symptomatic, the main presenting
AML occurs in 80% patients with tuberous sclerosis. The symptoms are related to intratumoral or retroperitoneal
sex distribution of AML in patients with tuberous sclerosis hemorrhage. Symptoms related to hemorrhagic
is closer to being equal, but women still outnumber men complications occur in 87% of patients, and hematuria
in terms of prevalence. AML also occurs young women is reported in 40%. A palpable abdominal mass is present
with lymphangiomyomatosis without other stigmata of in 47% of symptomatic patients. Shock due to massive
tuberous sclerosis. AML and lymphangiomyomatosis are retroperitoneal hemorrhage most commonly occurs in
sometimes considered the forme fruste of tuberous white females with a sporadic unilateral tumor and aged
sclerosis. AML is considered benign, but rare cases of 40–50 years. In younger patients with AML and in those
extension into the renal vein and/or inferior vena cava with associated tuberous sclerosis, tumor size and
(IVC) and of deposits in the regional lymph nodes are symptoms are significantly correlated.
reported. This involvement possibly is related to Other symptoms reported in patients with associated
multicentric disease. tuberous sclerosis are flank or abdominal pain, weight
Most small lesions are asymptomatic and incidental loss, hypertension, fever, and nausea. In almost half of
findings on images. As many as 40% are symptomatic; these cases, the tumors are bilateral. This observation
these can cause a palpable abdominal mass, hematuria, illustrates the importance of follow-up in this group of
or flank pain. The solitary sporadic tumors may cause patients.
acute abdomen and shock as a result of spontaneous
hemorrhage in the tumor. The demonstration of fatty LIMITATIONS OF VARIOUS MODALITIES USED FOR
attenuation in renal tumor is virtually diagnostic of AML. DIAGNOSIS AND EVALUATION
The most characteristic sonographic feature of an AML

is its echogenicity. Also, AML may cause acoustic
• Plain abdominal radiography shadowing. The echogenic appearance is thought to be
• Ultrasonography related to its fat content and the presence of multiple tissue
• Computed tomography interfaces within it. However, this appearance in a mass
• Intravenous urography is not pathognomonic for AML, and it is described in
• Magnetic resonance imaging renal cell carcinoma. In one series with pathologically
• Angiography proven renal cell carcinomas, 32% of tumors with a
• Isotope renography and dimercaptosuccinic acid diameter smaller than 3 cm were echogenic. Not all AMLs
(DMSA) scanning are hyperechoic because the tumor constituents vary, and
• Percutaneous renal biopsy the fat content may be low. Hemorrhage, necrosis, and
• Renal arterial embolization. dilated calyces also may alter the echogenicity of the
tumor.
FINDINGS In a sonographically typical mass, fatty attenuation
The majority (60%) of AMLs are asymptomatic. In one on plain abdominal radiographs, planar tomograms, and
series, which included patients with tuberous sclerosis, CT scans is virtually diagnostic of AML. However, AML
82% of patients with a tumor 4 cm or larger were is not the only renal tumor that can contain fat. Rarely,
symptomatic, whereas only 23% of patients with tumors but most importantly, fat can be found in a renal cell
smaller than 4 cm were symptomatic. The development carcinoma because of the invasion of perinephric fat or
of abdominal ultrasonography, CT, and MRI in recent metaplasia in the tumor. Intratumoral fat also has been
years has increased the incidental detection of found in renal lipomas, liposarcomas, Wilms tumors,
asymptomatic AMLs. Recent imaging experience shows teratomas, xanthogranulomatous pyelonephritis, and
that AML is more common than previously reported. oncocytomas (engulfing of renal sinus fat).
If fat cannot be demonstrated within a renal mass in the symptomatic masses are often larger. Renal arterial
a patient with tuberous sclerosis, a diagnosis of renal cell embolization can be used to control hemorrhage.
carcinoma must be considered. Rapidly enlarging lesions
or dystrophic calcification within a mass may suggest the FOLLOW-UP
diagnosis. Although the natural history of renal cell No universal agreement exists regarding the follow up
carcinoma in tuberous sclerosis is not well known, the of AML. Because the risk of hemorrhage is greater in
risk of metastases likely increases with the size of the masses with a diameter of more than 4 cm in, the
tumor, and the risk probably is very low in tumors smaller restriction of follow-up to patients with multiple AMLs
than 3 cm in diameter. Therefore, the consideration of (i.e. tuberous sclerosis) or those with tumors larger than
nephron-sparing surgery may be reasonable in patients 4 cm may be reasonable. Small (<4 cm in diameter)
with tuberous sclerosis and lesions without demonstrable solitary AML masses are the most common. They have
fat that are enlarging and close to or larger than 3 cm little growth (<5% per year), are less likely to be
in diameter. complicated, and do not require follow-up unless the
Although routine follow-up seems reasonable in all diagnosis is in doubt.
patients with tuberous sclerosis, more intensive follow-
up is necessary to establish the need for diagnostic MORTALITY/MORBIDITY
resection in patients with tuberous sclerosis who have Most tumors have a benign course. If the diagnosis is
small masses (<3 cm in diameter) that are radiologically certain, patients can usually be treated conservatively and
atypical for AML. Apart from this group with tuberous followed-up with sonography. However, one subset of
sclerosis, follow-up may be reasonably restricted to patients who present with severe hemorrhage as the first
patients with sporadic tumors larger than 4 cm in diameter sign may have life-threatening condition. In cases with
who have a higher incidence of hemorrhagic recurrent episodes of hemorrhage or massive bleeding,
complications. the tumor can be resected. Surgery, whether planned or
performed emergently, has a certain risk of morbidity
TREATMENT
and mortality. Renal arterial embolization may also be
With advances in cross-sectional imaging, the diagnosis used to control hemorrhage.
of renal AML can usually be established without surgery.
Most tumors can be managed conservatively, particularly ONCOCYTOMA
if they are asymptomatic. In suitable patients in whom
INTRODUCTION
the diagnosis is not established with imaging findings,
partial nephrectomy enables pathologic diagnosis with It is the most common benign solid renal tumor.
a minimal loss of function. Nephron conservation is of First described by Zippel in 1942, this tumor represents
even greater importance in tuberous sclerosis, in which a distinct pathologic entity. Later, Klein and Vaensi
tumors are often bilateral. In cases with recurrent episodes published case series of patients with oncocytoma and
of hemorrhage or massive bleeding, the tumor can be highlighted the benign course of the disease and its
resected. In the acute severe presentation in which more discrete pathologic features.
conservative methods fail, radical nephrectomy may be
PATHOPHYSIOLOGY
the only option.
Partial nephrectomy is ideal for masses with a diameter Oncocytomas originate from the intercalated cells of the
smaller than 3 cm, and partial nephrectomy may be collecting duct. On gross pathologic examination, the
possible in masses with a diameter smaller than 5 cm tumors appear spherical and are large (average size,
that do not abut the hilum. Partial nephrectomy may be 7 cm), with a pseudocapsule. On cut sections, they appear
more suitable for the removal of indeterminate masses, homogeneous with a mahogany color in contrast to
rather than symptomatic masses, for diagnosis because yellow renal cell carcinomas. A fleshy central scar, a
characteristic finding of oncocytoma, may be observed Each kidney is supplied by main renal artery arising
in 33–54% of tumors. Necrosis, hemorrhage and directly from the aorta. Multiple renal arteries are common
calcification are rare findings. and important in nephron-sparing surgeries (partial
On histologic examination, oncocytomas are nephrectomy). A single renal vein drains each kidney.
composed of oncocytes, which are large cells with granular Multiple renal veins are uncommon. Lymphatics from the
eosinophilic cytoplasm that shows abundant mito- kidney drain into the lymph nodes in the renal hilum,
chondria on electron microscopy. which in turn drain into nodes in the para-aortic region.
FREQUENCY PREFERRED INVESTIGATION (TABLE 10.3)

This tumor accounts for approximately 3–7% of all renal
KUB Radiograph
neoplasms. About 2–12% are multifocal, and 4–14% are
bilateral. Plain radiographic findings are nonspecific, and images
may demonstrate a large, soft-tissue mass in the renal
CLINICAL FEATURES area with displacement of the fat planes. Calcification
Approximately 56–91% of the tumors are incidentally is rare. Excretory urography shows a large mass with a
detected on imaging studies performed for another renal-contour abnormality and compression of the
indication. However, 17–21% of patients present with collecting system.
symptoms such as hematuria, flank pain, and an
abdominal mass. In patients presenting with symptoms, USG
hematuria is more common than mass-like findings. Oncocytomas appear as well-defined, homogeneous,
hypoechoic to isoechoic masses. The central scar cannot
Sex: These tumors are more common in males than
be confidently identified on sonograms. However, when
females, with a male-to-female ratio of 2–3:1, which is
it is seen, especially in large lesions, the scar may appear
similar to the ratio for RCC.
echogenic. Color Doppler sonography may show central
Age: Can occur at any age. But, the mean patient age radiating vessels.
is 62–68 years at the time of resection.
CECT
ANATOMICAL BASIS
CT scanning of the abdomen with the intravenous
The kidneys are retroperitoneal organs enclosed in a administration of iodinated contrast medium is the
fibrous capsule. The kidneys are surrounded by perirenal examination of choice. CT is the best modality for
fat. The anterior pararenal fascia separates the kidneys the evaluation of a solid renal mass. It assists in
from the pancreas, and the posterior pararenal fascia the detection and localization of the tumor, and it may
demarcates the paraspinal muscles from the kidneys. The help in its characterization, especially if fat-containing
adrenal glands are located superomedially. lesions (e.g., angiomyolipomas) are present. Additionally,
Table 10.3: Differential diagnosis of transitional cell carcinoma

Uric acid calculi Diagnose with ultrasound or CT
Vascular Impression Linear extrinsic impression
Use compression
Oblique or prone films
Crossed calcies
Tuberculosis Narrowed infundibulum
Irregular calices
Pyeloureteritis cystica Bullous edema due to stones/infection
Papillary necrosis Irregular calices ± sloughed papillae
Renal cell carcinoma May invade pelvicaliceal system
Cholesteatoma Squamous metaplasia related to infected stone
Leukoplakia 10–20% associated with carcinoma
Fungus balls Diabetes
staging of the tumor can be performed to classify the T2weighted images. On the contrary, tumor necrosis, a
extent of the lesion, regional lymphadenopathy, vascular common feature of malignant masses, appears
involvement, and metastases. CT also helps in detecting hypointense on T1 weighted images and hyperintense
calcifications and in differentiating a complex cyst from on T2 weighted images. Rarely, the central scar may
a solid neoplasm. appear bright on T2 weighted images.
On nonenhanced CT scans, oncocytomas appear After the intravenous administration of gadolinium
isoattenuating or slightly hyperattenuating relative to dimeglumine contrast material, oncocytomas show
kidney parenchyma. On contrast-enhanced CT scans homogeneous enhancement, with a nonenhancing
obtained during nephrographic phase, the mass appears central scar. As discussed in the CT section above, features
less attenuating than the renal parenchyma. of an invasive tumor are absent.
Oncocytomas are well encapsulated and have
distinct margins, a smooth contour, and a homogenous Angiography
appearance. The tumors may range from 3 to 10 cm,
Typical “Spoke wheel” pattern of blood vessels supplying
and in symptomatic patients, they are most often larger
it are seen.
than 5 cm.
A central hypoattenuating scar may be observed RNI
in 33% of cases, but this scar cannot be differentiated
from the central necrosis commonly found in RCC. With Scintigraphy is not routinely performed in the evaluation
the advent of multisection CT, high-resolution, thin of renal tumors.
sections through the kidneys may improve detection of Findings: On DMSA scans, the lesion appears as
the central scar. photopenic area displacing the cortex and collecting system.
Calcification, necrosis, and hemorrhage are rare.
Typically, features of a malignant tumor, such as invasion PET Scan
or infiltration into the perinephric fat, collecting system, On fluorodeoxyglucose (FDG) positron emission
or vessels, are absent. Likewise, regional lymphadeno- tomography (PET), oncocytomas usually have less FDG
pathy and metastases are not encountered in patients uptake than RCCs. The amount of uptake is usually
with oncocytoma. Occasionally, multifocal or bilateral isointense to renal parenchyma. However, oncocytomas
tumors may be found. can occasionally have uptake in the range of RCC uptake.
With the advent of CT, routine angiography is not
performed to diagnose renal masses. However, the Limitations of Techniques
classical angiographic findings include a spoke-wheel The presence of a central scar on CT scans or MRIs and
arrangement of tumoral vessels, homogeneous tumoral a spoke-wheel pattern of vessels on angiograms are often
contrast during the capillary phase, sharp demarcation suggestive of oncocytoma but not entirely specific.
from the kidney and surrounding areas, and a peritumoral
halo (lucent-rim sign). The bizarre neoplastic vessels INTERVENTION
are conspicuously absent, in contrast to RCC. Percutaneous renal biopsy, fine-needle aspiration
cytology, and frozen-section biopsy have been used
MRI with variable success for the differentiation of
Findings: On nonenhanced T1weighted images, oncocytoma from RCC. Because of these limitations
oncocytomas are well-defined, homogeneous masses. in preoperative pathologic evaluation, the consensus
They may appear isointense to hypointense relative to is to treat a solid renal mass with partial or total
the renal cortex. On T2 weighted images, the tumors are nephrectomy.
typically isointense to slightly hypointense. However, slight
MORTALITY/MORBIDITY
T2 hyperintensity is also reported.
When present, the scar may be seen as a hypointense, Oncocytomas are benign tumors, and the prognosis after
stellate area in the center of the lesion on T1 and total or partial nephrectomy is excellent.
Medical Renal Disease 161
CAUSES OF UNILATERAL SMALL KIDNEY 4. Acute cortical necrosis

1. Reflux nephropathy 5. Arterial infarction
2. Ischemia 6. Proliferative necrotizing disorders
3. Post-obstructive atrophy 7. Multiple Myeloma
4. Radiation nephritis 8. GN (Glomerulonephritis)
5. Hypoplasia 9. Multisystem disease especially collagen vascular
6. Infarction and post-inflammatory atrophy diseases.
10. Bilateral hydronephrosis
CAUSES OF BILATERAL SMALL KIDNEYS (FIGURE 11.1) 11. Hormonal-Acromegaly, DM, hyperalimentation,
A. Vascular cirrhosis
Generalized arteriosclerosis 12. Developmental bilateral renal duplication,
Atheroembolic disease Horseshoe Kidney, PCKD
Benign and malignant nephrosclerosis 13. Glycogen storage disease
B. Renal 14. Hemophilia
1. Hereditary nephropathies 15. Sickle cell disease
a. Medullary cystic disease, i.e. Nephrophthisis 16. Fabry disease
b. Hereditary chronic nephritis (Alport syndrome) 17. Acute urate nephropathy
2. Chronic glomerulonephritis 18. Physiologic response to contrast material and
3. Amyloidosis (Late-Stage) diuretics.
a. Post-renal (papillary necrosis)
b. Cause of unilateral small kidney occurring INTRODUCTION
bilaterally. The kidneys are considered to have increased echoge-
nicity if the pyramids are unusually hypoechoic relative
CAUSES OF BILATERAL LARGE KIDNEYS
to the cortex. Echogenicity is considered increased if the
1. Amyloidosis echogenicity is greater than the liver or greater than or
2. ATN (Acute tubular necrosis) equal to the spleen. There is loose correlation with the
3. Acute RVT degree of echogenicity and the severity of disease.
Increased echogenicity is a nonspecific finding and biopsy Contd...
is required for a diagnosis. Small, echogenic kidneys B. Unilateral

usually indicates a chronic disease process with end-stage 1. Renal artery stenosis, Postirradiation
2. Hypoplastic kidney, Postobstructive atrophy
changes. 3. Papillary caliceal abnormality, Diffuse parenchymal loss
Obstructive nephropathy Generalized reflux nephropathy
No parenchymal loss papillary necrosis
1. TB
2. Medullary sponge kidney, Megacalices, Pelvicaliceal cyst
Focal parenchymal loss
1. Focal reflux nephropathy (chronic atrophic pyelonephritis)
2. TB
3. Calculus disease
USG
When imaging adult patients for evidence of the presence
parenchymal disease the essential features are:
1. Renal calcification or calculi
FIGURE 11.1: Bilateral small kidneys, left more than right, in a 2. Abnormality of kidney size or shape; generalized
patient with chronic renal disease thinning focal loss of renal substance; asymmetry
3. Abnormality of the collecting system, especially
DIFFERENTIAL DIAGNOSIS OF MEDICAL deformity the papillae/calices
RENAL DISEASE (TABLE 11.1) 4. Occasionally, abnormality of the nephrographic
1. Glomerulonephritis from: SLE, PAN, Wegener’s, pattern
Good-pastures, hemolytic uremic syndrome The most valuable observations for differential
2. Acute tubular necrosis (Deposition of cellular debris diagnosis, the presence or absence of:
in the collecting tubules) from: Hypotension, Drugs, 1. Papillary/caliceal abnormality
heavy metals, solvent exposure 2. Focal cortical loss
3. HIV nephropathy 3. Differentiation in size between the two kidneys.
4. Hypertensive glomerulosclerosis The term ‘papillary/caliceal abnormality’ is used here
5. Acute interstitial nephritis (Acute hypersensitivity to emphasize that deformity of the caliceal cups seen is
reaction) from Penicillin, Methicillin, Rifampin, Sulfa radiologically important deformity of the papillae. This
drugs, NSAIDs, Cimetidine, Furosemide and Thiazides. may arise primarily from the resubstance, as in papillary
6. Diabetes mellitus. necrosis and reflux nephropathy, from the collecting
7. Amyloidosis, primary and secondary (Secondary
system, as in obstructive nephropathy. 1 presence or
causes include multiple myeloma, rheumatoid
absence of papillary/caliceal abnormality and of cortical
arthritis, TB, renal cell carcinoma, Hodgkin disease).
loss can be used to divide the radiological appearances
Table 11.1: Differential diagnosis of chronic renal
renal parenchymal disease into four groups.
parenchymal disease
No papillary/caliceal abnormality, diffuse parenchymal loss
MRI
A. Bilateral
The differential diagnosis of low signal intensity of renal
1. Chronic glomerulonephritis, diffuse small-vessel disease,
Hereditary nephropathies parenchyma on magnetic resonance imaging can be
2. Focal parenchymal loss
3. Infarct previous trauma
divided into three categories: hemolysis, infection and
Contd... vascular disease.
First category includes paroxysmal nocturnal hemo- 10. Acidosis and respiratory distress
globinuria (PNH), hemosiderin deposition in the renal 11. Analgesics
cortex from mechanical hemolysis, and sickle cell disease. 12. Renal vein thrombosis
Second category includes hemorrhagic fever with renal 13. Diabetes mellitus with infection (no increase occurs
syndrome (HFRS). Third category includes acute renal with uncomplicated DM)
vein thrombosis, renal cortical necrosis, renal arterial 14. Dehydration (Severe)
infarction, rejection of a transplanted kidney, and acute 15. Severe neonatal jaundice.
nonmyoglobinuric renal failure with sever loin pain and
patchy renal vasoconstriction. PNH, hemosiderin CLINICAL FEATURES
deposition from mechanical hemolysis, and sickle cell Sex: Analgesic-induced renal papillary necrosis is often
disease involve the entire cortex including the columns observed in middle-aged and elderly women.
of Bertin. HFRS involves the medulla, especially the outer
Age: Renal papillary necrosis resulting from analgesic
medulla, whereas cortical necrosis involves the inner
use is observed more often in middle-aged and elderly
cortex including the columns of Bertin. Renal vein
women. Gastroenteritis and dehydration that are
thrombosis demonstrates low-signal intensity lesions associated with papillary necrosis are observed in infants
involving the outer medulla, resembling HFRS. Wedge and children.
shaped low signal intensity regions involving both cortex Clinical presentation may be related to symptoms of
and medulla are seen in arterial infarction. urinary tract infection, such as recurrent fever, malaise,
dysuria, flank pain, proteinuria, hematuria, and
RENAL PAPILLARY NECROSIS leukocytosis. Passage of sloughed papillae can cause renal
INTRODUCTION colic, ureteric obstruction and, rarely, urinoma. Rarely,
It refers to ischemic necrobiosis of the papilla in the renal papillary necrosis can present as acute oliguric renal
medulla of the kidneys. failure. In the advanced stage, renal function may be
It can be: impaired and anemia and uremia may be noted.
• Localized or diffuse
• Unilateral or bilateral. ANATOMICAL BASIS
Earlier in the disease, renal size and function are The renal cortex is subcapsular and arches around the
preserved. renal medulla. The renal medulla comprises triangular-
Function may deteriorate with eventual renal failure shaped, pale, striated, conical renal pyramids; their apices
in the later stages of the disease. converge to the renal sinus. The base of the renal pyramid
is capped by renal cortical tissue to form a renal lobe.
PATHOPHYSIOLOGY The conical renal pyramids project into calices as papillae.
Renal papillary necrosis occurs in following conditions: Each minor calyx receives 1-3 of these papillae.
1. Prolonged hypotension The minor calices unite with their neighbors to form
2. Pediatric gastroenteritis (infants) larger major calices, which in turn fuse with each other
3. Obstructive disease of kidney (Urinary tract to form the renal pelvis. The papilla is the site of drainage
obstruction) of the papillary ducts, which are a continuation of the
4. Sickle cell disease (Hemoglobinopathies) terminal uriniferous ducts. The numerous openings of
5. TB (Urinary tract infection) the papillary ducts at the papillary summit give rise to
6. Trauma area cribrosa. Beside the papillary ducts, the renal papilla
7. Congestive heart failure has a rich blood supply comprising the vasa recta, part
8. Cirrhosis of the loop of Henle, and meshes of capillary network.
9. Christmas disease, Hemophilia Medullary ischemia is the central finding in experimental
analgesic nephropathy. Necrobiosis of the loops of Henle

and the vasa recta is found as an early abnormality.
PREFERRED EXAMINATION
Plain radiographs demonstrate calcification in a
sloughed papilla, which is characteristically ring shaped
and may be the only abnormal radiologic finding in
necrosis in situ. Calcification is common in patients with
analgesic-induced papillary necrosis but has not been
reported in renal papillary necrosis associated with
hemoglobinopathy.
On plain radiographs, necrotic papillae can occa-
sionally demonstrate a ring of calcification.
Findings
Plain radiograph findings: Kidneys are normal in size and
contour except in the late stage in which they shrink and
demonstrate a wavy contour due to the prominence of
FIGURE 11.2: Configurations in papillary necrosis
the septal cortex around the atrophied centrilobular
cortex.
sponge kidney and in conditions associated with hyper-
Sloughed papillae may calcify and can be observed
calcemia. The presence of ringlike calcifications of up to
as curvilinear or ringlike calcification up to 5-6 mm in
5–6 mm in diameter is characteristic of sloughed papillae
diameter. The appearance of calcification infers a change
and calcification may be the only abnormal radiologic
in urine bacteriology to proteus organisms.
finding in papillary necrosis in situ.
Tiny calcifications may be observed in the region of
In the early stage when papillary swelling may be the
the liver, spleen, adrenal glands, and lymph nodes in
only abnormal radiologic finding, swelling is difficult to
patients with abdominal tuberculosis.
Calcification in a sloughed papilla is characteristically differentiate from normal findings. Necrosis in situ cannot
ring shaped and may be the only abnormal radiologic be diagnosed unless calcification has occurred.
finding in necrosis in situ. Calcification is common in Pyelosinus extravasation, which can occur with
patients with analgesic-induced papillary necrosis and has forceful injection of a large volume of contrast, can mimic
not been reported in patients who have papillary necrosis contrast tracking from the fornix in papillary necrosis.
associated with hemoglobinopathy. Inadvertent injection of air bubbles can produce filling
On excretory urography, persistent streaking of defects but the defects are seen as smooth and rounded
contrast from the fornix at the upper and lower poles and can be differentiated from the irregular filling defects
is almost diagnostic of renal papillary necrosis. Necrosis found in sloughed papillae.
in situ is difficult to diagnose because the necrotic tissue Urographic findings depend on the stage of the disease
does not slough. (Figure 11.2).
On excretory urography, the ulcerated papillae can Kidneys are normal in size and the contour is smooth
be observed. Sloughed papillae can cause filling defects until the late stage in which the kidneys shrink and
within the calyx, pelvocalyceal system, or ureter. demonstrate a wavy contour.
Findings of medullary calcification (nephrocalcinosis) In the early stage, papillary swelling may be the only
are nonspecific and can occur in patients with hyper- abnormal finding and papillary necrosis may be difficult
parathyroidism, renal tubular acidosis, and medullary to diagnose.
Later, necrosis of the papillae with disruption of the Occasionally, bright echoes produced by arcuate
urothelial lining causes tracking of contrast from the fornix arteries can be visualized at the periphery of the cystic
parallel to the long axis of the papillae. This can produce space. Sloughed papillae can appear echogenic and cast
the lobster claw sign. Then cavitation of renal papillae shadows when calcified.
occurs, which can be incompleted (medullary) or The collecting system may be dilated when obstructed
complete (papillary) and can be either central or eccentric. by sloughed papillae. Sonographic findings are
Shrinkage and sloughing of the necrotic papillae cause nonspecific. A hyperechoic medulla can be observed in
forniceal widening and calyceal clubbing. patients with hyperparathyroidism and medullary sponge
Sloughed papillae cause a filling defect in the kidney and in patients with conditions that cause
pelvocalyceal system and in the ureter. hypokalemia or hypercalcemia.
Persistent streaking of contrast from the polar fornix CT findings are not diagnostic but may be useful in
is almost diagnostic of renal papillary necrosis. Necrosis assessing urinary tract obstruction, hemoglobinopathies,
in situ is difficult to diagnose because necrotic tissue does and cirrhosis, which are recognized causes of papillary
not slough. Filling defects within the pelvocalyceal system necrosis.
and the ureter are nonspecific findings and opacification No major role exists for CT and MRI in the evaluation
of the collecting system is poor when renal function is of renal papillary necrosis. The usefulness of reformatted
impaired. multislice spiral CT has yet to be determined. CT findings
Retrograde pyeloureterography is sensitive, are not diagnostic but can be useful in patients with poor
especially in the presence of renal impairment or when renal function in whom intravenous urogram radiographs
urographic findings are inconclusive. Retrograde provide poor resolution. Medullary cavity and calcification
pyelography can be helpful when the renal collecting are nonspecific findings.
system opacifies poorly or when renal insufficiency is
Findings: CT does not offer much help in the diagnosis
present.
of renal papillary necrosis. Reformatted multidetector
Findings are similar to those of excretory urography.
images may change the role of CT.
Minor abnormal papillary findings can be demon-
CT is sensitive in detecting calcifications.
strated readily when urographic findings are indeter-
CT can demonstrate multiple bilateral ring shadows
minate. Retrograde pyelography is sensitive, especially
in the medulla, some of which are triangular.
in the presence of renal impairment or in patients in whom
Contrast can be detected filling the clefts in the renal
urographic findings are inconclusive. The procedure
parenchyma.
cannot help assess renal function or the renal
CT can be helpful in evaluating the nature of the
parenchyma.
material (sloughed papillae) that causes the filling defect
Ultrasound is a noninvasive technique that is
within the collecting system.
frequently used to assess the urinary tract. Findings are
CT findings are not diagnostic but the scans can be
nonspecific for papillary necrosis. On ultrasound,
useful in patients with poor renal function in whom
sloughed papillae can be revealed as echogenic material
intravenous pyelogram findings are not helpful. However,
within the collecting system, which is a nonspecific finding.
use iodinated contrast agents cautiously in patients with
Correlation with clinical and laboratory findings help to
compromised renal function.
distinguish renal papillary necrosis from other renal
MRI findings are nonspecific in papillary necrosis,
abnormalities with similar ultrasound features such as
although MRI may be useful in patients who are allergic
other causes of increased echogenicity (e.g., nephro-
to iodinated contrast medium because gadolinium may
calcinosis).
provide a useful alternative. Gadolinium-enhanced MRI
Findings: Sonographically, areas of cavitation in the is a useful alternative in patients with renal failure. MRI
papillae can be observed as multiple rounded or triangular is an expensive tool but may be useful in patients with
cystic spaces in the medulla arranged around a renal sinus poor renal function and in those with hypersensitivity to
echo-demonstrating a garland pattern. iodinated contrast media.
Findings: MRI has no specific role in the management of albumin being excreted in the urine increases, and
of papillary necrosis; however, it may provide a useful may be detected by ordinary urinalysis techniques. At
alternative to iodinated contrast in patients with depressed this stage, a kidney biopsy clearly shows diabetic
renal function and in those who are allergic to iodinated nephropathy.
contrast medium. Protein may appear in the urine for 5 to 10 years
Sufficient experience is not available with the use of before other symptoms develop. High blood pressure
MRI in the diagnosis of papillary necrosis. often accompanies diabetic nephropathy. Over time, the
Radioisotope scan findings provide a sensitive kidney’s ability to function starts to decline. Diabetic
index of renal function. Radioisotope studies play a nephropathy may eventually lead to chronic kidney
significant role in evaluating renal function but provide
failure. The disorder continues to progress toward end-
little anatomic information on the location of lesions. No
stage kidney disease, usually within 2 to 6 years after
role exists for isotope studies in the diagnosis of renal
the appearance of high protein in the urine (proteinuria).
papillary necrosis; however, they are useful in evaluating
Diabetic nephropathy is the most common cause of
renal function.
chronic kidney failure and end-stage kidney disease in
MORTALITY/MORBIDITY the United States. People with both type 1 and type 2
Progression of renal papillary necrosis can result in diabetes are at risk. The risk is higher if blood-glucose
eventual renal failure, anemia, and uremia. Transitional levels are poorly controlled. However, once nephropathy
cell carcinoma appears to be more common in patients develops, the greatest rate of progression is seen in
with analgesic nephropathy. patients with poor control of their blood pressure.
Diabetic nephropathy is generally accompanied by
GENITOURINARY MANIFESTATIONS other diabetes complications including hypertension,
OF DIABETES
retinopathy, and vascular (blood vessel) changes,
DIABETIC NEPHROPATHY (KIMMELSTIEL-WILSON although these may not be obvious during the early stages
DISEASE; DIABETIC GLOMERULOSCLEROSIS; of nephropathy. Nephropathy may be present for many
DIABETIC KIDNEY DISEASE) years before high protein in the urine or chronic kidney
Definition failure develop.
Diabetic nephropathy is a complication of diabetes. If
Role of Investigations
you have this condition, your kidney loses its ability to
function properly. The condition is characterized by high The first laboratory abnormality is a positive micro-
protein levels in the urine. albuminuria test. This means you are very likely to
develop diabetic nephropathy.
Causes, Incidence, and Risk Factors
Most often, the diagnosis is suspected when a routine
Each kidney is made of more than a million units called urinalysis of a person with diabetes shows too much
nephrons. Each nephron has a tuft of blood vessels called protein in the urine (proteinuria). The urinalysis may also
a glomerulus. The glomerulus filters blood and forms urine,
show glucose in the urine, especially if blood glucose is
which drains down into collecting ducts to the ureter.
poorly controlled.
The earliest detectable change in the course of diabetic
There may or may not be signs of other diabetic
nephropathy is a thickening in the glomerulus. At this
stage, the kidney may start allowing more albumin complications. High blood pressure may be present or
(protein) than normal in the urine, and this can be develop rapidly and may be difficult to control. Serum
detected by sensitive tests for albumin. This stage is called creatinine and BUN may increase as kidney damage
“microalbuminuria” (micro refers to the small amounts progresses.
of albumin). A kidney biopsy confirms the diagnosis. Most
As diabetic nephropathy progresses, increasing nephrologists do not need to perform the biopsy if the
numbers of glomeruli are destroyed. Now the amounts case is straightforward, with a documented progression
of proteinuria over time and presence of diabetic USG: Hypoechoic collection with air pockets and
retinopathy on examination of the retina of the eyes. moving internal echoes.
Should there be any doubt in the diagnosis, a biopsy
Emphysematous Pyelonephritis/cystitis.
may be performed to confirm the diagnosis and to study
KUB: Air lucency in renal region
the extent of the disease.
Air in PC system.
This disease may also alter the results of the following
Air in bladder.
tests:
USG: Hyperechoic (air) foci in renal parenchyma,
• protein electrophoresis—urine
P-C system and bladder
• creatinine—urine
CT: Hypodense foci CT density equal to air in KUB
• 24-hour urine protein.
region.
Imaging
Xathogranulomatous pyelonephritis (due to
Plain radiography: Proteus mirabilis)
Following things are to be seen: KUB: Enlarged kidneys
1. Calcification of aorta and great vessels Nephrolithiasis–Staghorn calculus usually
2. Pancreatic calcification associated.
3. Seminal vesicle calcification USG: Enlarged kidneys.
4. Emphysematous cholecystitis Diffuse/focal decreased echogenicity
5. Emphysematous pyelonephritis Calculi (+)
6. Emphysematous cystitis IVU: focal defect or complete nonfunctioning.
7. AVN of femoral capital epiphysis. CT: Enlarged kidney with fat density and calculus.
IVU:(Patient should be well hydrated) delayed
functioning with enlarged kidneys. Reproductive System
USG: Following findings may be noted in a patient of DM:
1. Enlarged kidneys • Vas deferens calcification.
2. Later progressive decrease in size due to nephro- • Genital infections; Fournier gangrene is the dreaded
sclerosis. one.
3. Diffuse cortical hyperechogenicity with Gradual loss • Postmenopausal tuboovarian abscess.
of CM • Increased risk of Ca endometrium.
4. Differentiation
Fournier gangrene is potentially lethal necrotizing
5. Resistive index in renal artery is > 0.7.
fascitis of the scrotum. Air pockets are noted in scrotal
Nephrocalcinosis wall and perineum with scrotal wall thickening in the
KUB: Triangular calcific lesion with central lucency. presence of normal testes.
USG: Hyperechoic foci s/o calcification noted at
papillac. Complications
Cystopathy (Neurogenic Bladder) Possible complications include:
MCU: Increased capacity due to decreased bladder • Hypoglycemia (from decreased excretion of insulin)
sensation. • Rapidly progressing chronic kidney failure
USG: Increase in post void residue. • End-stage kidney disease
Cystitis • Hyperkalemia
MCU: Irregularity and thickening of bladder wall with • Severe hypertension
trabeculations. • Complications of dialysis
Decrease capacity • Complications of kidney transplant
Renal and peri-renal abscesses • Coexistence of other diabetes complications
KUB: Increase in renal contour with effaced • Peritonitis (if peritoneal dialysis used)
perinephric fat planes. • Increased infections.
RENOVASCULAR HYPERTENSION (RVHT)/ Although MFP is a pathologic diagnosis, characteristic

RENAL ARTERY STENOSIS (RAS) change is seen at vascular imaging. The most common
INTRODUCTION finding is the ‘string-of-beads’ appearance caused by
It denotes nonessential hypertension in which a causal areas of relative stenoses or webs alternating with small
relationship exists between anatomically evident arterial fusiform or saccular aneurysms of the artery. Approxi-
occlusive disease and elevated blood pressure. RVHT is mately 10–30% of patients with RAS have MFP.
the clinical consequence of renin-angiotensin-aldosterone Diagnostic imaging plays an essential role in the
activation as a result of renal ischemia. diagnosis and treatment of RVHT. Although primary
Renal artery stenosis (RAS) is a major cause of RVHT. screening tool for RAS is radionuclide renography, CTA
Apart from the casual relationship of occlusive renal and MRA have much higher sensitivity and specificity.
artery disease and hypertension, RAS is also being
increasingly recognized as an important cause of chronic PATHOPHYSIOLOGY
renal failure. In older patients, atherosclerosis is
Development of RVHT
the most common cause of RAS. RAS due to
atherosclerosis is generally a progressive disease with A physiologically significant reduction in blood flow to
increasing luminal narrowing, which may eventually com- a region of the kidney causes decreased perfusion pressure
promise renal blood flow and renal function and structure. to the juxtaglomerular apparatus, leading to an increased
Arterial dysplasias (AD) are uncommon angiopathy release of renin. Renin converts angiontensinogen
associated with heterogeneous histologic changes that to angiotensin I. Angiotensin I is then converted to
may affect the carotid circulation and the visceral and angiotensin II by angiotensin converting enzyme
peripheral arteries. Medial fibroplasia (MFP), as a (ACE). Angiotensin II causes constriction of the efferent
cause of RAS usually affects young to middle-aged arteriole, thus maintaining the glomerular filtration rate.
adults, mostly women, but it can also affect However, angiotensin II also causes an increase in
children. It is an important cause of RVHT in children. systemic pressure. ACE inhibitors give rise to a decrease
The average age range of patients with MFP is 30–40 in glomerular filtration rate (in addition to a decrease in
years. systemic pressure).
Renal Vascular Diseases 169
RVHT is the clinical consequence of renin-angiotensin- efferent vasoconstriction effect by using angiotensin
aldosterone activation. Since Goldblatt’s work in 1934, blockade in the ischemic kidney may reduce the GFR.
RVHT has become increasingly recognized as a cause Angiotensin blockade can be achieved by the use of
of clinically difficult-to-control hypertension and chronic angiotensin-converting enzyme (ACE) inhibitors.
renal insufficiency. Goldblatt demonstrated that occlusion The use of ACE inhibitors causes a deterioration of
of the renal artery causes ischemia, which then causes renal function in some patients with renovascular disease;
an elevation of blood pressure by triggering the release this effect is particularly pronounced in patients with in
of renin. Increased renin levels help in the conversion bilateral RAS. Because angiotensin predominantly affects
of angiotensin I to angiotensin II, causing severe the efferent renal arteriole, the decrease in renal blood
vasoconstriction, aldosterone release. The ultimate flow caused by afferent vasoconstriction is less than the
cascade of events depends on the presence of a decrease in the GFR caused by efferent vasoconstriction.
functioning contralateral kidney. The net result is a decrease in the filtration fraction. By
The development of RVHT involves the activation blockading angiotensin, ACE inhibitors eliminate efferent
of both limbs of the renin-angiotensin-aldosterone system vasoconstriction and cause a decrease in the
and depends on the presence or absence of a contralateral intraglomerular pressure and the GFR.
kidney. Unilateral renal ischemia initiates an increased Normally, the perfusion of the kidney is increased by
secretion of renin, which accelerates the conversion of as much as five times, as compared with that of other
angiotensin I to angiotensin II and which enhances the organs, because it drives glomerular capillary filtration.
adrenal release of aldosterone. Aldosterone-mediated Both glomerular capillary hydrostatic pressure and renal
sodium and water retention is efficiently handled by the blood flow are essential components of the GFR.
noncompromised kidney, precluding the volume from In patients with RAS, chronic ischemia produces
contributing to the angiotensin II–mediated hypertension. adaptive changes in the kidney that are more pronounced
Atherosclerotic disease is usually diffuse and may in the tubular tissue. These changes include tubular cell
involve both kidneys, leaving a solitary ischemic kidney atrophy, atrophy of the glomerular tuft, patchy
that has little reserve capacity for sodium and water inflammation and fibrosis of tubular cells, tubular sclerosis,
excretion; hence, volume plays an additive role in the thickening and duplication of the Bowman capsule, and
hypertension. An ischemic solitary kidney is unable to intrarenal arterial medial thickening. In RAS, the GFR
perform the pressure diuresis required to handle the is dependent on angiotensin II and other modulators that
aldosterone-induced sodium and water retention. Thus, maintain the balance between the afferent and efferent
the resultant volume load further contributes to the arteries. However, when perfusion pressure decreases
hypertension and also suppresses the production of renin below 70–85 mm Hg, maintaining an adequate GFR may
by the stenotic kidney. no longer be possible. Significant functional impairment
Angiotensin II causes vasoconstriction of both afferent of autoregulation, leading to a decrease in the GFR, is
and efferent arterioles, with a preferential affect on the not likely to be observed until arterial luminal narrowing
efferent side. Under physiologic conditions, efferent tone exceeds 50%.
is essential to the maintenance of intraglomerular In adults, renovascular disease tends to appear at
pressure. Angiotensin blockade increases efferent renal different times, and it affects the sexes differently.
arterial blood flow, resulting in an increased intraglo- Atherosclerotic disease affects mainly the proximal third
merular pressure and optimized glomerular filtration rate of the main renal artery, and it is most common among
(GFR). older men. Fibromuscular dysplasia (FMD) involves the
In a kidney rendered ischemic by RAS with a reduced distal two thirds and branches of the renal arteries, and
afferent blood flow, the intraglomerular pressure and it is most common among younger women.
glomerular filtration are maintained by angiotensin II– Other conditions that may be associated with RVHT
mediated efferent vasoconstriction. Removal of the include cholesterol embolic disease, acute arterial
thrombosis or embolism, aortic dissection, neurofibro- Atherosclerosis

matosis, renal arterial trauma, arterial aneurysm, How the initial arterial epithelial injury occurs in patients
arteriovenous malformation of the renal artery, and with atherosclerosis is not clear. Nevertheless, lipid
polyarteritis nodosa and other vasculitides. abnormalities, hypertension, cigarette smoking, diabetes
mellitus, viral infection, immune injury, and increased
Evolution of RVHT
homocysteine levels have all been implicated in
The evolution of RVHT has been described as having
contributing to the endothelial injury. At the site of the
three stages.
endothelial atheromatous lesion, permeability to plasma
1. In the first stage, the immediate elevation of blood
macromolecules (e.g., low-density lipoproteins) is
pressure is a direct result of increased levels of renin.
increased, with a subsequent increased turnover of
Over days to weeks, the blood pressure remains
endothelial cells, smooth muscle cells, and intimal
elevated, but the course and presence of elevated renin
macrophages. When atherogenic lipoproteins exceed a
levels depends on the presence and function of the
certain critical mass, the mechanical forces may enhance
contralateral kidney. The mechanism by which
lipoprotein infiltration in these regions, leading to early
hypertension is produced in patients with renovascular
atheromatous lesions.
disease thus changes over time and varies with the
Atherosclerotic RAS may progress in as many as one-
sodium balance. With a normally functioning
third of patients, and ongoing ischemic renal parenchymal
contralateral kidney, volume expansion is avoided,
damage is of concern. Furthermore, despite adequate
and renin levels remain high. The t kidneys function
blood pressure control, this condition is associated with
out of synch: The ischemic stenotic kidney produces
reduced renal perfusion pressures, and renal function may
excessive renin and retains sodium, whereas the
deteriorate.
comparatively normal kidney continues to excrete
Studies about the natural history of atheromatous RAS
sodium and water to maintain normal volume levels.
obtained by means of sequential abdominal aortography
The end result is systemic hypertension that is renin
or duplex sonography in patients with documented and
and angiotensin mediated.
2. In the second phase, in the setting of an ischemic medially treated RAS have shown that progressive arterial
solitary kidney, sodium and water retention, together obstruction occurs in 42–53% of patients within the first
with the vasopressor effects of angiotensin II, act to two years of follow-up. In patients with a high-degree
maintain renal perfusion pressure. The stimulus to RAS, the rate of progression to complete renal artery
produce renin is impaired, and renin levels thus occlusion in these studies is 9–16%.
decrease. In these circumstances, angiotensin II no
Arterial Dysplasia
longer drives the hypertensive state, but the high blood
pressure instead results from volume expansion. Thus, In 1967, McCormack et al classified AD on the basis of
perfusion pressure tends to be maintained at the the primary site of involvement of arterial wall, as
expense of systemic hypertension and volume determined histologically.
retention. Renal perfusion may return to normal if Their classification of fibrosing lesions of renal arteries
blood flow is normalized during the first two phases, included the categories of:
and the blood pressure soon returns to a normal level. 1. Intimal fibroplasia,
3. In the third phase, hypertension persists, even after 2. Medial fibroplasia with microaneurysms,
patency of the renal artery is restored. Once phase 3. Subadventitial fibroplasia and
3 is reached, restoration of renal blood flow may not 4. Fibromuscular hyperplasia.
normalize the blood pressure, presumably because They first coined the term chain of beads to describe
of secondary irreversible vascular or renal paren- radiographic changes in MFP of the renal artery. The
chymal disease. term has subsequently been modified to string of beads.
MFP is the most common variety of AD and of patients with normal blood pressure and renal failure,
represents 85% of the cases. The string-of-beads sign 50% become hypertensive after renal transplantation.
is classically seen in MFP on angiography. With this complex background and the ever-present
A similar radiographic appearance can be caused by complication of graft rejection and acute tubular necrosis,
subadventitial fibroplasia, but in this variant, the size of one may loose sight of RAS as a treatable cause of
the aneurysms does not exceed the diameter of the renal hypertension.
artery. MFP may appear as a single stenosis of a visceral
artery, but it is more often seen as multiple stenoses with INCIDENCE
intervening outpouchings forming a chain. Radiogra- RVHT is the most common type of secondary hyper-
phically, this is depicted as a string-of-beads sign.
tension, accounting for less than 1% of cases in unselected
Neurofibromatosis populations and as many as 30% of cases in selected
populations.
Neurofibromatosis is a rare cause of RAS and usually
secondary to a direct effect of fibrous proliferation of the CLINICAL FEATURES
intima or media. Less commonly, neurofibromatous tissue
may affect the adventitia, producing periarterial fibrosis Age: The age of onset depends on the cause of the
indistinguishable from other causes of RAS. These lesions damage to the renal blood vessels. The average age range
are usually at the origin of the artery, and they may be is 30–40 years. RVHT tends to occur in patients younger
bilateral. than 30 years or older than 50 years. The youngest patient
with FMD of the renal artery was reportedly six months
Congenital Stenosis old.
Congenital stenosis (coarctation of renal artery) is RVHT often occurs in men older than 45 years with
extremely rare and assumed congenital because of its atherosclerosis and in women younger than 45 years with
discovery in early life. This type of stenosis is generally AD.
confined to the main renal artery, and it may be associated Sex: All types of AD, except intimal fibroplasia, more
with aortic coarctation. Some cases may eventually commonly affect women than men. For intimal fibro-
involve changes related to arteritis, FMD, or neurofibro-
plasias, the male-female distribution is equal. For FMD,
matosis.
the male-to-female ratio is 1:3-5.
Transplant RAS RVHT is most common in younger women and older
Transplant RAS is seen in about 10% of patients after men. In younger women, RVHT most commonly
renal transplantation, and it is the most important cause develops as a result of FMD affecting the distal two thirds
of treatable hypertension. In renal transplantation of the renal arteries and their branches. In older men,
patients, RAS may occur as a complication of surgery, RVHT most often develops as a result of atherosclerotic
as transplant rejection, or as intrinsic vascular disease; disease that affects the proximal third of the main renal
this RAS usually occurs in the first year after surgery and artery.
rarely after the third year. The presentation is usually with Although the incidence of atherosclerotic RVHT is
hypertension and, occasionally, an elevated serum independent of sex, Crowley et al showed that female
creatinine level. sex is an independent predictor of renovascular disease
The extent to which the RAS stenosis contributes to progression. Other such predictors are older age, elevated
the hypertension is difficult to determine because rejection serum creatinine level, coronary artery disease, peripheral
is frequent cause of vascular disease, and systemic vascular disease, hypertension, and cerebrovascular
hypertension often accompanies rejection. Of the 20% disease.
ROLE OF RADIOLOGY AND IMAGING 20-min-to-maximum ratio (using background-subtracted

Hypertensive Urography renogram curves) is less than 0.3. Abnormal renovascular
Hypertensive urography is of historical interest and no physiology is indicated by a significant decrease in the
longer used as a screening technique for RAS. CO2 glomerular filtration rate with the administration of
angiography is also obsolete with MRA and gadolinium captopril, and renal cortical retention of MAG3 (suggestive
imaging. CT angiography (CTA) with maximum intensity of physiologically significant renal artery stenosis).
projection (MIP) and the quantitative measurement of On giving captopril there is virtually no radionuclide
stenosis is an accurate noninvasive technique in the uptake on DTPA images in a patient with hypertension
diagnosis of visceral artery stenosis; this is fast becoming receiving captopril therapy. A repeat DTPA renogram
the diagnostic tool of choice with angiography reserved performed in the same patient after cessation of captopril
therapy revealed normal, bilateral DTPA uptake.
in cases in which vascular intervention is planned.
The administration of ACE such as captopril inhibitors
RNI prevents the conversion of angiotensin I to the active
vasoconstrictors angiotensin II and angiotensin III. By
Radionuclide renography technetium-mercaptoacetyltri-
glycine (MAG3)-captopril has a high sensitivity and inhibiting the compensatory increase in vascular tone at
specificity, and it adds a physiologic element to the the postglomerular arteriole, ACE inhibitors decrease
glomerular filtration in the setting of RAS. The
diagnosis of RAS.
The acceptance of radionuclide renography as a administration of ACE inhibitors in conjunction
primary screening tool for RAS has been hindered by with radionuclide renography provides a non-
invasive method for the detection of functionally
the lack of standardized protocols.
significant RAS.
Standard renography with iodine-131–labeled ortho-
In patients with RAS, effective renal plasma flow
iodohippurate (OIH) is of historical interest and no longer
(ERPF) is shown to increase after the administration of
performed in the investigation of RAS.
an ACE inhibitor because of the efferent arteriolar
The Captopril Scan vasodilatation, which increases blood flow through the
The renal captopril scan is used to assist in the diagnosis renal parenchyma and decreases blood flow through the
of renovascular hypertension. Although protocols vary, glomerulus. 131I-labeled OIH also closely estimates ERPF.
one protocol is as follows: Recently, technetium-labeled mercaptoacetyltri-
Captopril (an ACE inhibitor) is given orally, followed glycine (MAG3) has also been used to qualitatively
by IV MAG3 (a radiopharmaceutical) and IV Lasix assess ERPF.
approximately 60 to 90 minutes later. Dynamic, Since the introduction of captopril renography, various
sequential scintigraphic images are then acquired for 30 modifications have been made. Some centers only use
minutes. If the scan is normal, further imaging is not one agent, either DTPA or MAG3. A positive captopril
required. If the scan is abnormal, however, a “baseline” scan indicates that RVHT is present, and it also
scan is performed for comparison, again with IV MAG3 implies the existence of hemodynamically
and Lasix, but without pre-medication with captopril. significant RAS (>60–75% of the lumen).
Scan results: Perfusion of the kidneys should be relatively It is important to understand that the criteria for a
symmetric (generally considered 50% ± 5%), rapid positive study depends upon the tracer used. If DTPA
(seconds), and demonstrate normal renal shapes. Time is used, there should be a change in split function if RVHT
to peak absorption should be approximately 2 to 3 is present (since DTPA is dependent upon GFR for uptake
minutes, with 5 minutes at the upper limits of normal; there will be decreased initial uptake in the affected
followed by transit and washout of the MAG3 such that kidney). If MAG3 is used, split function will usually not
in a kidney with normal function and absence of change, but there will be increased cortical retention
significant pelvic or calyceal retention, the normal (usually measured as 20 minute to maximum ratio) in
the affected kidney. This is because initial MAG3 uptake Direct signs:
is primarily dependent upon tubular secretion rather than 1. In RAS, the peak systolic velocity increases by
GFR. However, later clearance of MAG3 is dependent more than 150 cm/s for angles less than 60° or 180
upon GFR (therefore decreased excretion is the primary cm/s for angles greater than 70°.
finding). Rarely, the split function will change when MAG3 2. Poststenotic spectral broadening may be present
is used in cases of severe RVHT. with or without flow reversal. Flow may be absent
Recent preliminary data suggests that aspirin during diastole in a stenosis of more than 50%.
renography may be as sensitive as captopril reno- 3. A ratio of the peak systolic renal artery velocity
graphy for detecting RAS. Considering that aspirin, to the aortic peak systolic velocity of 3.5 or
compared with captopril, reduces renal blood flow and more is said to be predictive of a stenosis of more
thus tubular tracer delivery in poststenotic kidneys, aspirin than 60%.
renography is expected to be more useful, particularly
Indirect signs:
if tubular tracers are used.
1. Presence of tardus-parvus pulse-demonstrated by
Color Doppler a gradual slope of Doppler waveform during systole
(pulse time increase of greater than 0.07–0.12 s) and
Doppler sonography can be used to measure the
attenuated Doppler waveform amplitude (peak
velocity of blood flow. It is a noninvasive technique, and
systolic velocity less than 20–30 cm/s).
it has high sensitivity in expert hands. Color flow Doppler
2. The acceleration index is determined by dividing
may demonstrate disorganized flow patterns and high
the slope of the systolic upstroke (in kilohertz per
velocity flow stream associated with hemodynamically
second) by the carrier Doppler frequency, and an
significant stenosis.
acceleration time is the time interval between the onset
Normal state: The peak systolic velocity in normal renal of systole and the initial peak. The acceleration index
arteries averages 120 cm/s ±12, with an average peak in RAS is greater than 3 m/s2.
systolic aortic velocity of 60 m/s ±15. Both velocities 3. The resistive index in RAS is usually less than 0.56.
decrease with age. The kidneys offer a low-resistance The early systolic peak may be absent in RAS.
vascular bed; thus the Doppler spectral waveform from 4. Disorganized flow patterns and a high velocity
the normal kidney is that of a constant forward diastolic flow stream associated with hemodynamically
flow. In renal parenchymal disease there is increased significant stenosis. A false-negative diagnosis may
vascular resistance, which in turn causes a decrease in occur with accessory renal artery, whereas a false-
the diastolic flow component and an increase in the positive diagnosis may be made with coarctation of
pulsatility of the Doppler spectral waveform. Parenchymal aorta.
diastolic flow velocities less than 20% of the peak systolic
velocities are consistent with renal parenchymal disease. RAS in a transplanted kidney
1. It is seen in about 10% of patients after renal
RAS transplantation, raising the questions of whether RAS
The diagnosis of RAS is based on systolic and diastolic it is the cause of post-transplant hypertension.
velocity changes throughout the length of the renal artery. 2. The site of stenosis is not at anastomotic site in
Renal artery flow patterns can be classified into most patients; this suggests the possibility of focal
four categories: rejection (with edema/fibrosis) as the cause of the
(1) Normal, hypertension. Duplex and color Doppler sonography
(2) Diameter-reducing stenosis less than 60%, has greatly impacted the diagnosis of RAS.
(3) Diameter-reducing stenosis more than 60%, or 3. The most reliable Doppler criteria for stenosis
(4) Renal artery occlusion. are a high velocity jet and distal turbulence.
Limitations: a branch renal artery in 4%, or a combination of the

Doppler sonography has several limitations in the 2 in 17%. MFP/FMD is bilateral approximately in 65%;
diagnosis of RAS. These include patient-related factors, the left-to-right ratio is 4:1.
anatomic factors, technical factors, and pathologic factors. Degree of confidence: Renal angiography remains
1. Patient factors include bowel gas, obesity, the criterion standard in the diagnosis of RAS.
respiratory renal movements, and poor patient Angiography is essential when renovascular
compliance. intervention is contemplated. The angiographic
2. Anatomic factors include multiple renal arteries measurement of the size of RAS, an important parameter
(16–28%), variation of renal veins (used as imaging in assessing the significance of RAS, is inaccurate.
landmarks), horseshoe kidneys, and crossed ectopia. Angiography provides only anatomic information, and
3. Technical factors include false-positive it does not enable the assessment of blood flow through
examinations due to suboptimal angles, variation in the stenosis.
operator experience, incomplete examination
(because complete renal evaluation is cumbersome), CT Angiography (CTA)
the need to visualize the entire length of artery, CTA with MIP and quantitative measurement of stenosis
transmitted cardiac and/or aortic pulsation (which may is an accurate noninvasive technique in the
obscure renal waveforms), and different emphasis on diagnosis of RAS. The advent of spiral and multislice
variable parameters. CT scanning has made CTA feasible. Continuous
4. Pathologic factors include false tracings (which may scanning through an area of interest during a single breath
be recorded from large collateral vessels and a hold provides sufficient data to reconstruct 3-dimensional
reconstituted main renal artery) and variable causes (3D) images.
of RAS that affects different sites (e.g., atheroma, Many scanning protocols are available. The general
fibromuscular hyperplasia, vasculitis, arteriovenous consensus is that both a timed bolus and rapid injection
fistula, retroperitoneal fibrosis, neurofibromatosis). rate improves image quality. No positive oral contrast
material should be used because it results in severe
Conventional Angiography degradation of the image quality. Immediately before the
Conventional angiography remains the criterion standard procedure, the patient ingests water, and glucagon is then
for the detection of RAS, although CTA and MRA are given intravenously to diminish bowel movement and
challenging it. The severity of the stenosis and the maximize bowel distention. The three most common
presence of collateral circulation to the kidney may be techniques used for 3D reconstruction are MIP, shaded-
assessed to determine the hemodynamic significance of surface display (SSD), and volume rendering. MIP is often
RAS. Lesions occluding more than 50% of the the single most useful technique for 3D reconstructions.
diameter of the artery are considered significant. Accessory renal arteries are reliably identified by
Both the angiographic and the nephrographic phases can means of CTA. In either the main stem artery or its
be studied because the latter may better depict ischemic intrarenal branches, RAS is detected with a high degree
changes. of accuracy.
Epinephrine may further restrict blood flow to the Limitations: CTA or MRA may cause the clinician to
kidneys and make the collateral circulation more obvious. overlook mild cases of FMD that are detectable with digital
Generally, flush aortography suffices for mainstem RAS, subtraction angiography (DSA). Most of the false-negative
but if branch stenosis is suspected, selective renal and false-positive findings of RAS arise from accessory
angiography may better define the lesion. DSA does not renal arteries. MRI is expensive, and its availability is
address the hemodynamic significance of RAS. limited.
RAS due to atherosclerosis generally affects the middle Measurements of the size of RAS on angiograms (an
and distal renal artery in 79% of the patients; it affects important clinical consideration) are imprecise, and do
not permit assessment of the cross-sectional area or, more MRA provides accurate information about the number
importantly, the flow through the stenotic segment. The of renal arteries, the size of the kidneys, and the presence
various histologic types of FMD are difficult to distinguish of anatomic variants.
on angiograms; this limitation has important clinical Gadolinium-enhanced MRA has been proven to have
bearing from a prognostic point of view. a high sensitivity for detecting stenosis in the main and
Standing waves in the renal arteries appear as multiple accessory renal arteries. At present, MRA provides
serrated indentations, symmetrically distributed at evenly anatomic information regarding a vascular stenosis but
spaced intervals. These waves are of no pathologic little information is provided about the functional
significance and may represent arterial spasm. They may significance of a stenosis. Recent reports indicate that 3D
also affect intrarenal branches.
MRA with gadolinium-based contrast agents (which have
A fibrous musculotendinous band may cause extrinsic
a low potential for nephrotoxicity) has a sensitivity of
compression of the renal artery.
96-100% and a specificity of 71-96% for the detection
Atheroma, FMD, thrombus, embolus, or arteritis may
of a main RAS of greater than 50%.
cause branch RAS.
When combined with cardiac synchronization, 3D
Klippel-Trenaunay syndrome is a congenital angio-
dysplasia consisting of a triad of angiomas, osteohyper- MRA can sharply delineate the entire length of the major
trophy and venous varicosities. Visceral involvement is renal arteries. However, MRA remains suboptimal for the
not uncommon and may cause life-threatening detection of hemodynamically significant lesions of distal,
complications. intrarenal, and accessory renal arteries, which may cause
The sensitivity and specificity of the spiral CT in physiologically significant RAS.
detecting RAS are approximately 98% and 94%, Limitations of MRA include its costs and lack of avai-
respectively. In patients with a plasma creatinine lability. Contraindications to MRA include claustrophobia
concentration higher than 1.7 mg/dL, the accuracy is and metallic implants, such as a pacemaker or surgical
lower (93% sensitivity, 81% specificity), possibly because clip.
of reduced renal blood flow.
INTERVENTION
MRA
Interventions appropriate for patients with RAS/RVHT
MRA provides accurate information about the number
may include medical therapy, percutaneous transluminal
of renal arteries, the size of the kidneys, and the presence
angioplasty (PTA), vascular stent placement, intravascular
of anatomic variants. The obvious advantages of con-
ultrasonography-guided atherectomy, and surgical
ventional angiography are its usefulness in determining
revascularization.
the clinical importance of suspicious lesions and the ability
to concurrently perform endovascular intervention.
Medical Therapy
Findings: MRA is fast becoming a clinical standard for
the safe and noninvasive detection of RAS, aneurysms, Treatment with antihypertensive drugs is indicated and
and occlusions. A comprehensive examination includes optimal blood pressure control is essential. ACE inhibitors
both 3D dynamic gadolinium-enhanced and 3D phase- should be avoided. Other risk factors that should be
contrast MRA techniques, which allow an evaluation of addressed include atherosclerosis, smoking, and hyper-
the renal arteries and other visceral arteries. The 3D lipidemia. Definitive therapy for the underlying cause
phase-contrast technique is flow based and subject to should always be considered to prevent the development
dephasing in the presence of significant arterial stenosis. of ischemic nephropathy. In patients with diffuse
The 3D gadolinium-enhanced MRA method produces atherosclerosis, the complication rate with both surgery
excellent contrast angiograms without the risk of iodinated and angioplasty is relatively high. Medical therapy may
compounds or radiation exposure. be preferred to other treatments.
Percutaneous Transluminal Angioplasty management of RAS. Early results suggest that stenting
PTA has become the procedure of choice for treatment may prove useful in patients with ostial disease, in those
of symptomatic stenoses. Patency rates after PTA are in whom restenosis occurs after PTA, or in those with
strongly dependent on the size of the vessel treated and complications (e.g. renal artery dissection) resulting from
the quality of inflow and outflow through the vessel. RAS PTA. Primary renal artery stenting in patients with
is an established cause of either RVHT or chronic renal atherosclerotic RAS has a high technical success rate and
insufficiency. Because of the excellent results obtained a low complication rate.
with renal angioplasty, it is the most commonly performed
Intravascular Ultrasonography-guided Atherectomy
procedure in symptomatic RAS.
Previously, angioplasty was considered a contra- In a single reported case, hypertension secondary to AD
indication in patients with a solitary or transplanted was successfully diagnosed with intravascular sonography,
kidney. This is no longer the case and, angioplasty is now and intravascular sonography-guided renal atherectomy
considered the procedure of choice for treatment of RAS was curative.
in these patients.
Surgical Revascularization
Technical success is achieved in more than 90% of
patients, and patency rates are 90–95% at two years for Currently, surgical revascularization is reserved for patients
MFP and 80–85% for atherosclerosis. Restenosis requiring in whom the main renal artery appears completely
repeat angioplasty has been reported in fewer than 10% occluded and in whom the surviving renal parenchyma
of patients with AD and in 8–30% with atherosclerotic is vascularized by collaterals. Surgical revascularization
stenosis. Improvement in blood pressure control with might also be used when an ostial stenosis is present with
fewer antihypertensive medications is achieved in a buttressing atheroma on either side of the ostium. Some
30–35% of fibromuscular lesions and in 50–60% of of these lesions may also be amenable to percutaneous
atherosclerotic lesions. A success rate of 83% has been vascular stent placement.
reported with PTA in RAS associated with renal Several surgical options are available. The stenotic
transplantation. segment may be excised and the artery resutured directly
onto either the aorta or surviving stump. A vein graft
Vascular Stent Placement may be transplanted or the kidney resected and
Vascular stenting is considered complementary to PTA. reimplanted in the iliac fossa with the renal artery
Many vascular stents are now available. Some stents are anastomosed to the iliac artery. Another novel method
metallic devices, which are either self-expanding or involves a splenectomy and anastomoses of the splenic
balloon expandable. The US Food and Drug Adminis- artery to the renal artery when RAS involves the left
tration (FDA) has approved a few of these for peripheral kidney. The underlying diagnosis determines the results
coronary work and transjugular intrahepatic of this surgery. With advanced diffuse atherosclerosis,
portosystemic shunt (TIPS) procedures. surgery may become less feasible because the certainty
The ultimate role of stents in the treatment of vascular that the RAS is the cause of the hypertension is less and
disease is not yet established, but these devices have the prognosis may be determined by comorbidities.
already had a dramatic impact on the practice of A potential complication is the release of cholesterol
interventional radiology. In studies from both the US and emboli during the surgery; however, 80–90% of patients
Europe, stenting of smaller vessels has resulted in an undergoing operation for atherosclerotic RAS benefit with
unacceptably high incidence of thrombosis. These cure or improvement. The perioperative mortality rate
problems are being addressed in the development of new is less than 5%. In patients with AD, the cure rate is as
stent materials and coatings. high as 80%, and morbidity rates are low. However,
Intravascular stents placed during angioplasty may similar results can be achieved with the minimally invasive
be helpful in the prevention of restenosis and the renal angioplasty technique, at a considerably less
morbidity, mortality, and expense. In patients with diffuse 2. Blunt or iatrogenic trauma, neoplasms such as renal
atherosclerosis, the complication rate with both surgery cell carcinoma, Wilms tumor, transitional and
and angioplasty is relatively high. metastatic disease, drugs (including birth control pills
and exogenous estrogens), hypercoagulable states
MORTALITY/MORBIDITY such as pregnancy and disseminated malignancy,
In patients with hypertension, atherosclerotic renal artery septic abortion, and extension of leg or pelvic venous
disease is a strong predictor of increased mortality relative thrombus all can predispose an individual to
to the general population. In the setting of renal dys- RVT.
function, RVHT is associated with the greatest mortality 3. In addition, extrinsic compression of the renal vascular
rate. pedicle observed in pregnancy, retroperitoneal fibrosis,
Major complications of RVHT include end-organ lymphomas, tumors, and abscesses may precipitate
damage due to chronically uncontrolled hypertension and thrombus formation in the renal vein or IVC. Finally,
progressive renal insufficiency, which is an important a number of systemic diseases, including polyarteritis
sequel of chronic renal ischemia. nodosa, diabetic glomerulosclerosis, sickle cell anemia,
The prognosis of patients with RVHT is difficult to and systemic lupus erythematosus, may also be
ascertain because it varies with the degree of RAS, the associated with RVT.
response of the patient to antihypertensive therapy, and
the effectiveness of revascularization procedures. INCIDENCE
Incidence of symptomatic or asymptomatic RVT is

RENAL VEIN THROMBOSIS (RVT)
16-42% in patients with nephrotic.
INTRODUCTION
In 1840, Rayer first described the entity of renal vein PATHOPHYSIOLOGY
thrombosis (RVT) and its association with nephrotic In general, patients with RVT have two primary modes
syndrome. Although trauma, infection, and tumor can of clinical presentation: acute and chronic.
cause RVT, nephrotic syndrome is now known to be the
most common cause of RVT. Acute RVT
Acute RVT is most commonly observed in infants and
ETIOLOGY
neonates secondary to dehydration. Acute RVT is
Primary Cause characterized by abrupt onset of flank pain, nausea,
Nephrotic syndromes: Among the various etiologies vomiting, and gross or microscopic hematuria. Physical
of nephrotic syndromes, the nephropathy most commonly findings may include a palpable kidney and hypertension.
associated with RVT is MGN, with MGN in 60% of On occasion, acute RVT may be bilateral, resulting in
patients, with membranoproliferative glomerulonephritis oliguric acute renal failure and flank pain. RVT results
(MPGN) in 40% of patients, and with focal sclerosis (FS) in an enlarged kidney that is tense, swollen, and cyanotic.
in 28% of patients). The renal pelvis is usually stretched, distorted, and
blurred. Bilateral acute obstruction in the adult may well
Secondary Causes be lethal irrespective of the cause.
1. Alterations of renal flow secondary to volume losses A characteristic, although rare, radiographic finding
(GI fluid loss, hemorrhage, dehydration, infection) or is notching of the ureter, which usually occurs when
functional states of decreased cardiac output and renal collateral veins in close relation to the ureters become
hypoperfusion (congestive heart failure, aortic tortuous and dilated. If swelling continues to increase,
insufficiency, constrictive pericarditis) can predispose arterial perfusion may be compromised and the kidney
individuals to RVT. may undergo hemorrhagic infarction and atrophy.
Chronic RVT sections of the renal vein or cessation of blood flow if

The chronic presentation of RVT is observed more the lumen is completely obstructed. Recently, power
frequently than the acute form. In general, it is Doppler USG has increased specificity in the diagnosis
asymptomatic, making the true prevalence of chronic RVT of RVT and in the assessment of caval tumor thrombus.
difficult to determine. Patients present with few or no
Computed Tomography
accompanying symptoms and normal renal function,
except for albuminuria, or gradual worsening in those CT findings include decreased nephrographic attenuation,
with nephrotic syndrome and resultant chronic edema. loss of corticomedullary differentiation, a low-attenuating
Many patients with RVT may develop DVT. They can thrombus in the renal vein (Figure 12.1), renal
initially present with pulmonary thromboembolism. DVT enlargement with persistent parenchymal opacification,
and pulmonary embolism are the most common and renal vein enlargement. In the acute stage, capsular
complications of RVT and nephrotic syndrome, with venous collaterals, thickening of the Gerota fascia, and
pulmonary embolism being the most common fatal pericapsular whiskering are also often observed.
thromboembolic complication.
In chronic RVT formation, regardless of the degree
of thrombosis, the collateral circulation always evolves.
Occasionally, evidence of venous collateralization in the
form of a varicocele or retrograde flow in dilated
superficial epigastric veins is observed. The presence of
a varicocele should prompt careful imaging of the kidney
to assess for bland or malignant RVT. In general, patients
with chronic RVT are older than those with acute RVT,
and they have a greater incidence of other thrombo-
embolic phenomena.

Because the classic presentation of hematuria, flank pain,
and a lumbar mass is rarely seen in RVT, a high index FIGURE 12.1: Low attenuation thrombus in IVC and left renal vein
of suspicion is essential for the diagnosis. Ultrasonography
(US), CT, and MRI have varying levels of sensitivity and Magnetic Resonance Angiography
specificity. Recent observations also suggest that magnetic resonance
angiography (MRA) appears to be a useful and accurate
Ultrasonography
alternative test for the diagnosis of RVT prior to
Currently, USG is the initial study of choice to exclude venography when US findings are equivocal). MRA
RVT. In an acute setting, USG may reveal an edematous produces high contrast between flowing blood, vascular
and enlarged kidney with decreased echogenicity caused walls, and surrounding tissues. Other advantages include
by diffuse edema, focal or diffuse disruption of the avoidance of contrast material, its noninvasiveness,
parenchymal architecture, and/or thrombus within the and the ability to image both the arterial and venous
renal veins. phases. Preliminary observations suggest that MRI and/
In addition, duplex Doppler USG demonstrates or MRA may be the diagnostic procedure of choice for
peaked, abruptly decreasing systolic-frequency shifts and RVT.
retrograde plateau-like shifts during diastole at the level
of the main renal artery and its proximal branches, with Renal Venography
an absent venous signal. Doppler US may reveal Presently, renal venography remains the diagnostic
increased blood velocity and turbulence in narrowed criterion standard. Assessing the venous phase of renal
arteriography also helps in making the diagnosis. heparin therapy. Because heparin works by enhancing
However, both are invasive procedures requiring the use the activity of AT-III, decreased levels AT-III, as observed
of potentially nephrotoxic contrast agents in patients with in nephrotic syndrome, may be a cause of failure from
existing renal compromise. US and MRA do not require heparin use. In general, prolonged therapy of several
the use of contrast agents. days’ duration may lyse more than 90% of those clots.
Radionuclide Renography Many investigators have demonstrated reversal of acute
renal failure after urokinase-mediated thrombolysis.
Radionuclide renography using technetium 99mTc DTPA
Currently, one thrombolytic agent does not appear to
also has been proven useful in the assessment of renal
have an obvious advantage over another.
perfusion. Arterial compromise from venous pressure
Although thrombolytic agents lead to faster and more
elevations in acute RVT is undoubtedly the primary factor
complete resolution, they have the potential to cause
determining ultimate organ damage (Keating, 1985). In
bleeding complications: Minor bleeding occurs in
such cases, scintigraphy reveals delayed or absent
perfusion to the kidney. When a scan indicates perfusion approximately 5% of patients, and serious bleeding
arrest, an aggressive therapeutic approach may be events, such as intracranial hemorrhage, occur in 1%.
warranted. Contraindications for the use of thrombolytic agents
include factors predisposing patients to bleeding,
TREATMENT intracerebral cancer, recent cerebral trauma, a prior
The treatment of RVT has evolved from nephrectomy history of cerebrovascular accident (CVA), recent surgery,
to thrombectomy to anticoagulation, which is presently or GI bleeding.
the standard treatment of choice in RVT. Anticoagulants The decision to choose systemic or local
act by preventing further propagation of the thrombus administration via venous or arterial administration
while allowing recanalization through the body’s own depends on risk and benefit factors. Systemic
fibrinolytic system and preventing thromboembolic administration is not invasive while local administration
phenomenon. Standard therapy has consisted of initiating allows for lower lytic dose and shorter infusion times.
systemic anticoagulation with intravenous heparin and Recent reports suggest that systemically administered
later switching the patient to oral anticoagulants. thrombolytic therapy is effective and safe if no obvious
Physicians recommend that, as long as patients have contraindications exist, and invasive procedures might
significant hypoalbuminemia (defined as a serum albumin be avoided.
level <2.5 g/L), anticoagulant therapy should be To date, no prospective studies have been performed
administered. For autoimmune disease, steroids and other to compare anticoagulation and thrombolytic treatment
immunosuppressive drugs are indicated. alone as the treatment of RVT. The general consensus
Thrombolytic Therapy is that heparin should be used initially. Thrombolytics
should be reserved for second-line treatment when
Thrombolytic therapy is warranted in patients with
heparin fails.
bilateral RVT and acute renal failure, extension of RVT
into the IVC, acute renal failure, a massive clot with high Surgical thrombectomy or nephrectomy is rare and
risk of acute embolic events, pulmonary emboli, or severe usually reserved for cases that are refractory to medical
flank pain . In a patient with unilateral RVT and no other therapy. Thrombectomy is indicated in situations of
signs of thromboembolic disease, an absence of flank unrelenting pulmonary emboli and oliguria refractory to
pain, and normal renal function, the risk-to-benefit ratio medical therapy from bilateral RVT or thrombosis of a
supports the use of anticoagulant therapy alone. solitary kidney. Consider nephrectomy in patients that
Thrombolytic therapy should also be considered present acutely with severe toxicity from hemorrhagic
in patients whose condition fails to improve with infarction of the kidney (Keating, 1985).
MORTALITY AND MORBIDITY

Pulmonary embolus is the most common and most
serious complication in patients with RVT.
RENAL ARTERIOVENOUS MALFORMATIONS

INTRODUCTION
These are somewhat rare clinical entities, may be
congenital, acquired or idiopathic.
CLINICAL FEATURES
The majority of patient experience hematuria. Other signs FIGURE 12.3: T1 W MRI
and symptoms include abdominal or flank bruits, diastolic
hypertension, cardiomegaly, and congestive heart failure.
ETIOLOGY
Renal arteriovenous fistulas are typically due to trauma;
itrogenic, congenital malformations are rare.
ROLE OF RADIOLOGY AND IMAGING (FIGURES 12.2 TO 12.4)

Characteristic features include dilated, tortuous vessels
within the renal collecting system, as well as early visua-
lization (Figure 12.5) of the renal draining vein and IVC.
Symptoms typically include gross hematuria.
Embolization is the non-surgical treatment.
TREATMENT
Small lesions may be successfully treated with
embolization, whereas larger lesions may require partial FIGURE 12.4: DSA image: Pre-embolization AVM
or total nephrectomy.
FIGURE 12.2: T2 W MRI FIGURE 12.5: Post embolization DSA image

FIGURE 12.6: MDCT: Renal artery aneurysm FIGURE 12.8: CT angiogram: Accessory right renal artery supply
upper pole of right kidney
RENAL ARTERY ANEURYSM

See Figure 12.6.
RENAL INFARCT
See Figure 12.7.
ACCESSORY RENAL ARTERY

See Figure 12.8.
FIGURE 12.7: CECT: Wedge-shaped left renal infarct

INTRODUCTION AND HISTORICAL ASPECT portable means to first detect most surgical emergencies
The first renal transplantation was performed in 1954. in such cases (Figure 13.1).
The kidney remains the most frequent organ transplanted.
PATHOPHYSIOLOGY
A major breakthrough occurred with the release of
cyclosporin in 1983; this drug helps to control transplant The process of organ transplantation begins with
rejection. With improved surgical techniques and medical the evaluation of patients with end-stage kidney
management of rejection, renal transplantation has
become the treatment of choice for end-stage renal
disease (ESRD).
The use of immunosuppressive agents such as
cyclosporin, OKT3, and FK506 has resulted in a 1-
year survival rate for mismatched cadaveric renal grafts
of 80%. A 90% 1-year graft survival rate has been
reported with nonidentical grafts from living related
donors and a 95% 1-year success rate for grafts with
identical human lymphocyte antigen. The half-life of grafts
from living related donors varies from 13–24 years.
Surgical techniques for transplantation have improved,
and the means by which kidneys are obtained from living
related donors was recently advanced with the use of
laparoscopic surgical techniques.
Perioperative complications occur in 15–20% of
renal transplants. Most initial complications can
be corrected if detected promptly. The radio-
logist should help to select the most effective
imaging methods for evaluating the many
problems encountered in renal transplantation.
Overall, ultrasonography (US) is a safe, rapid, and FIGURE 13.1: Transplanted kidney (For color version see Plate 6)
Radiology of Renal Transplant 183
disease. Chronic renal failure makes the potential ANATOMICAL BASIS

transplant recipient more susceptible to a wide range of The anatomic basis for renal transplantation reflects the
neoplasms, including carcinoma of the kidney. The need to create an arterial inflow and venous return for
significance of an atypical cyst or a small renal deformity the renal transplant. The transplanted kidney is generally
is much greater in chronic renal insufficiency. The renal
placed in the upper lateral portion of the pelvis. The ureter
transplant recipient is often chronically ill. Pulmonary
from the transplant is attached into the urinary bladder
hypertension greater than 35 mm Hg has been detected
via a submucosal tunnel.
in more than a third of patients receiving chronic
The vascular supply to the ureter of the transplanted
hemodialysis. Dialysis access-induced venous stenosis is
kidney is critical as well. The traditional surgical means
common. Stenosis of the superior vena cava may result
of harvesting the transplant kidney allows the selection
in severe upper extremity and cervical venous stasis.
of either the right or left kidney. In most cases, the left
The next stage in the renal transplantation
process involves the selection and examination kidney is favored because the left renal vein is longer
of a suitable donor. In the best circumstance, the renal than the right. The introduction of a laparoscopic
donor is alive and related to the transplant recipient. The approach for donor nephrectomy has further emphasized
success of transplants involving living related subjects is the selection of the left kidney in most cases. Single renal
much higher than those involving cadaveric transplants. arteries and veins are most common; however, the donor
Generally, kidneys are harvested from living kidney can have multiple renal arteries arising from the
related donors using a left-flank approach. Hand- aorta from T12 to L4. Multiple renal arteries are noted
assisted laparoscopic techniques have been reported to in approximately 25–30% of renal grafts. Allografts with
result in shorter operative and warm ischemia times. The multiple renal ar teries can be successful. The
need for excellent preoperative imaging is directly related pretransplantation donor evaluation must include an
to the challenge of the laparoscopic procedure as well accurate assessment of the number and the location of
as the additional time (27–30%) needed to transplant multiple renal arteries.
a kidney with two or more renal arteries. Concomitant Pretransplantation donor evaluations must include
surgery has been performed for live donors using a lapa- adequate evaluation for accessory renal arteries beginning
roscopic approach. Simultaneous surgical interventions from just below the diaphragm and proceeding distally
at the time of kidney donation benefit the donor and to include both external iliac arteries. Arterial assessment
do not compromise the overall transplantation process. should include the possibility of early renal artery
Renal transplantation surgery must address multiple
branching. Preoperative assessment is best performed
renal arteries, which may be treated with primary or
using a multidetector CT scanner with extensive post
secondary anastomosis. The left renal vein is longer
processing using multiplanar reformatted images and 3D
and easier to use; therefore, harvesting of the left
visualization.
kidney is favored. Recently described techniques allow
Renal veins can be multiple and present frequent
for longer arterial length on the harvested renal transplant.
anomalies. The most common venous anomalies include
Longer donor kidney vessels potentially make a right renal
donor kidney more acceptable. Dual pediatric donor the retroaortic position of the main left renal vein, multiple
kidneys are left connected to the donor aorta, which is veins, early branching of renal veins, and anomalies of
then anastomosed to the external iliac artery. The vessels the anastomosis between the renal veins and the lumbar
of the ureter must be preserved. In general, the ureter and gonadal veins. Anomalies in the potential renal donor
should be straight but not retracted. The distal ureter is may include urologic collecting system duplication, single
implanted into the bladder through a tunnel without kidneys, crossed-fused kidneys, and hydronephrosis.
opening the bladder. (Figure 13.2).
Contd...
3. Vascular
Arterial
• Anastomotic stricture (technical)
• Kinking or twisting of vessel
• Lobar vessel occlusion
• Renal artery stenosis
• Arteriovenous fistula
• Pseudoaneurysm
Venous
• Renal vein thrombosis
• Kinking
FIGURE 13.2: CECT: Transplanted kidney with bifid COMPLICATIONS OF RENAL TRANSPLANT
pelvicalyceal system (TABLE 13.1 AND FIGURE 13.3)
1. An incisional hernia was observed in 4% of transplants
The surgical placement of the donor kidney is subject in one series. The presence of large polycystic kidneys,
to variation depending on the size of the kidney compared bladder outlet obstruction, and chronic lung disease
with that of the recipient, the number of renal arteries,
was associated with the development of an incisional
and the presence of preexisting diseases such as polycystic
hernia. Both early and delayed incisional hernias were
renal disease and severe atherosclerotic disease of the
Renal
pelvic arteries. Repeat renal transplantation is influenced 1. Hyperacute rejection
by the location, condition, and size of the initial (failed) 2. Acute rejection
transplant. The primary arterial anastomosis may involve 3. Chronic
4. ATN
the external iliac artery or the hypogastric (internal iliac) 5. Cyclosporin toxicity
artery. Dual pediatric donor kidneys are left connected
to the donor aorta, which is then anastomosed to the
external iliac artery. Peritransplant
collections
Table 13.1: Complications of renal transplant • Pus
• Blood
1. Renal • Urine
Rejection • Lymph
• Chronic
• Acute
• Hyperacute
• Accelerated
Acute tubular necrosis
Cyclosporine A toxicity
2. Urological
Obstruction Urological Obstruction
• ‘Missed’ stone • ‘Missed’ stone
• Blood • Blood
• Pressure from adjacent collection (ureteric/bladder leak) • Pressure from adjacent collection
• Technical problem (ureteric/bladder leak)
• Technical problem
• Ischemic breakdown • Ischemic breakdown
• Anastomotic stricture • Anastomotic stricture
Urine leak
Peritransplant fluid collection: lymph, urine, pus or blood
FIGURE 13.3: Schematic representation of complications
Contd... of renal transplant
noted. Surgical repair was accomplished by primary 9. Occasionally, masses may arise from within the
facial approximation and polypropylene mesh transplanted kidney. Renal cell carcinoma, focal
reinforcement. Postoperative complications have been fungal disease, and B-cell lymphoma have been
reported secondary to the use self-retaining retractors. reportedly transmitted within renal transplants.
2. Direct trauma may occur due to compression of large 10. Infections (Figure 13.4).
bowel, femoral nerve compression, or other soft tissue
contusions related to the blade position.
3. Lymphocele collections may occur soon after renal
transplantation or they may develop on a delayed
basis. Whether its origin is from the recipient or donor
is rarely identified. A lymphocele may result in urinary
obstruction of the transplant or compromise blood
flow. Treatment generally involves fenestration of the
cavity into the abdominal space or, more recently,
laparoscopic fenestration.
4. Urinoma or urinary fistulas may occur in any renal
transplant patient but are more common in children
in whom urinary tract anomalies are present. FIGURE 13.4: CECT: Focal nephritis in a transplanted kidney
5. The application of bladder augmentation techniques
(ileum, ureter, sigmoid, stomach), incontinent urinary PREFERRED INVESTIGATIONS
conduits (ileum, colon), and continent urinary
CONVENTIONAL RADIOGRAPHY AND IVU
reservoirs is associated with surgical complications,
including stomal stenosis, stomal prolapse, renal Traditionally, the potential donor was examined with
artery stenosis, urine leaking, enterovesicular fistula, intravenous urography (IVU) and angiography. But,
and wound dehiscence and urinary tract stones. conventional radiographs provide a limited amount of
Similar complications may occur in the older useful information about the complex clinical presenta-
transplant patient who has undergone bladder or tions of renal transplantation. Often, chest radiographic
prostate surgery. findings are nonspecific because chronic interstitial
6. In the period immediately after renal transplantation, pulmonary disease is difficult to differentiate, whereas
hemorrhage may occur around the transplanted abdominal radiographs may fail to illustrate fluid collec-
kidney or in the peritoneal or retroperitoneal spaces. tions. Nowadays, preoperative evaluations are performed
Later, obstructive uropathy may occur because of with MRI and CT. CTU offers ease of performance and
stone formation, cross clamp stenosis, or ischemic a high degree of detection of renal stones, anomalies,
stenosis of the ureter or renal pelvis. and (less commonly) renal masses in the kidneys of
7. Renal arterial and venous stenosis may result in
potential donors.
hypertension or graft failure. The primary treatment
opinion is renal arterial or venous angioplasty often Limitations
with a stent placement. Surgical repair of transplant
renal artery stenosis has been described using both Traditionally, the potential donor was examined with IVU
direct vascular repairs as well as with preserved and angiography. That no longer occurs. Although a
cadaveric iliac artery grafts. number of pretransplantation urologic evaluations have
8. Vascular complications occur in up to 3% of renal been recommended in the literature, no one specific
transplants. Thrombotic, hemorrhagic, stenotic, and universally accepted protocol exists. The expense and
embolic complications related to the transplanted potential risk of using IV contrast material should limit
kidney have been reported. duplication of tests.
Donor anomalies related to the upper collecting system

and duplicated ureters may make the kidney from a living
related donor unacceptable. This is readily illustrated on
CT and MRI examinations, replacing IVU.
USG AND COLOR DOPPLER

The most commonly used imaging modality in the renal
transplant recipient is USG. To confirm two kidneys with
no gross abnormality, USG can be used as a screening
tool. Immediate complications related to surgery can be
demonstrated. USG enables suitable localization prior to
biopsy in most patients. Duplex USG also provide
important information concerning the vascular status of
the graft in cases of acute rejection. Delayed complications
related to the renal transplant, such as lymphocele, etc
FIGURE 13.5: Normal flow waveform in transplanted kidney
can also be very well documented. Fluid within the
peritoneal space is not a specific finding. Hypoechoic
peritransplant complications are associated with fluid
abdominal fluid may remain after peritoneal dialysis.
collections. The superficial nature of a typical renal
Urine, bowel content, sepsis, and hemorrhage may have
transplant allows good resolution with relatively few
variable sonographic appearances.
reverberation echoes. During the immediate post-
Features operative transplant period, most fluid collections in and
around the bed of the transplant are hematomas or
Diagnostic USG, including duplex vascular study, is the
urinomas. A hyperacute hematoma often presents as a
most commonly performed examination for routine
hypoechoic fluid-filled mass near the surgical site.
follow-up after renal transplantation. Typical renal
Hematomas may be stable in size but can enlarge rapidly.
transplant sonograms demonstrate the following: arcuate
arteries, echo-poor medullary pyramids, hyperechoic The enlarging hematoma may compress the kidney,
renal sinus, absence of hydronephrosis, stable renal resulting in a reduction of urine output. Later, a hematoma
volume (formula: 0.5 × length × transverse × antero- becomes complex with both hypoechoic and echoic areas.
posterior dimensions), normal position of renal transplant A second possible cause for fluid around the transplant
anterior to the external iliac vessels, and otherwise normal during the first 24 hours following transplantation is a
echogenicity in the parenchyma, collecting system, and urinoma. A urinoma results from a leak between the renal
surrounding tissues. transplant ureter and the recipient’s bladder. Such a leak
The measurement of the vascular resistive index (RI) may be the result of surgical failure or from necrosis of
is a part of the evaluation. The RI measures resistance the distal portion of the transplant ureter. A urinoma
to arterial flow within the renal vascular bed. RI is enlarges more slowly and remains hypoechoic. If the
calculated from the peak diastolic arterial waveform. The urinoma becomes infected the quality of the fluid may
normal RI is less than 0.7–0.8. An RI of greater than 0.9 become more complex as an abscess forms. After several
indicates transplant dysfunction but not the cause. In some weeks a spontaneous hematoma or a urinoma becomes
instances a nonfunctioning renal allograft can have less likely.
“normal” RI and pulsatility index (PI) (Figure 13.5). A lymphocele may form anywhere in the general area
of the renal transplant. While lymphoceles are more likely
Peritransplant Fluid Collections to occur soon after the transplant, they may develop long
Sonographic evaluation of renal transplant complications after the skin wound has healed. An uncomplicated
is often successful because many of the potential lymphocele is hypoechoic and remains hypoechoic unless
it becomes infected. While lymphoceles are slow to neously. Mild tissue rejection may cause transplant edema,
develop, a lymphocele can create significant pressure and a mechanical obstruction may cause hydronephrosis.
resulting in urinary or venous obstruction. Correlation with findings from functional examina-
An abscess may develop at any time during the life tions, including nuclear medicine study and dynamic
history of a renal transplant recipient. Abscesses may be MRA, should be performed whenever US cannot confirm
around the kidney, within the transplant, or in the the cause of the decreased renal function. Changes in
abdominal cavity remote from the transplant. Most native kidneys of renal transplant patients should include
abscesses have complex sonographic qualities. An abscess a survey to detect masses. Cysts frequently develop in
the kidneys of patients with ESRD. New patterns of
can be diagnosed best by comparing the sonographic
internal echoes or rapidly enlargement should be noted.
texture of a fluid collection to the white blood cell count
Correlation with CT, MRI, or aspiration biopsy results may
and the patient’s fever curve. In cases in which an abscess
be necessary to exclude carcinoma or other pathology.
is considered likely, sonographically guided aspiration is
recommended. Diagnostic ultrasound may both initially Limitations:
diagnose the fluid mass as well as guide the surgical or 1. Sonograms may fail to differentiate gas versus
radiologically based drainage. If percutaneous drainage calcifications, and bowel gas may obscure important
results in a recurrence of a lymphocele, laparoscopic findings.
drainage is indicated. 2. In double (pediatric) renal transplantation, one of the
kidneys may be much smaller than the other. The
Acute Kidney Transplant Rejection smaller kidney may be mistaken for a mass. Patients
may undergo more than one renal transplant. The
Sonographic findings include globular enlargement of the
contralateral, or prior, transplant is generally smaller
kidney, swelling and hypoechogenicity of the medullary
and can be mistaken for a mass.
pyramids, an indistinct cortical-medullary junction, and
3. Gas within the vascular or ductal systems may be
hypoechoic foci in the renal cortex. Among the early
interpreted as calcifications. Aneurysms and venous
findings of acute renal transplant rejection is renal
varices may be mistaken for lymphoceles unless
enlargement. The length of a renal transplant should not
duplex signals are routinely assessed. The degree of
increase from the stabilized posttransplantation hydronephrosis may be underestimated in a patient
measurements. with acute transplant rejection.
Although acute transplant rejection must be consi- 4. Tortuous renal arteries may suggest renal artery
dered in all cases of declining renal function after initially
stenosis in the absence of hemodynamically significant
successful renal transplantation, other acute events may
narrowing. Increases in the RI should be interpreted
occur in the immediate postoperative period or later
carefully. Variations in the location of the sampled
clinical follow-up. Perinephric hematoma and acute
arterial signal may suggest elevation of the RI (Figure
hydronephrosis are among the complications than can
13.6).
result in the acute loss of kidney function.
Perinephric hematoma appears as a peripheral,
mixed, hypoechoic rim or ring surrounding the kidney.
The bleeding may proceed away from the kidney. The
extent of the bleeding may be difficult to evaluate at US
because of overlying bowel gas. Acute or chronic
hydronephrosis has a presentation that is generally similar
to hydronephrosis in a native kidney. The central col-
lecting system may be dilated and filled with hypoechoic
fluid (urine), and a variable degree of dilatation is
generally preset in the renal collecting system. Several FIGURE 13.6: Figure on right shows bilateral small malfunctioning
diseases may affect the transplanted kidney simulta- kidneys and figure on left shows transplanted kidney in the same patient
CT Scanning and CT-guided Interventions medullary space and the renal pyramids. Nephrogram-
CT scanning and CT-guided interventions remain an phase images have been shown to be most sensitive in
important means for examining the preoperative renal the overall detection of renal masses. By reexamining
donor candidate and for evaluating the complications the kidneys, ureters, and bladder after a 2-3-minute delay,
that develop in patients who undergo renal transplants. accurate evaluation of the renal collecting system, ureters,
CT is not limited by bowel gas; however, the use of and urinary bladder is possible.
IV iodinated contrast material must be limited in many By carefully reviewing the axial CT images obtained
cases. CT and MRI studies of the abdomen and pelvis during the first 20–30 seconds after the IV bolus, most
renal artery duplications and many renal vein anomalies
offer similar information. In most cases, CT is preferred
can be detected. Image collimation of 2–3 mm with an
because CT-guided aspiration and drainage procedures
image index of 1–2 mm is necessary to separate small
may obviate surgical interventions.
vessels, which are closely positioned. The patient must
Findings: An increased interest in CT imaging has hold his or her breath during the arterial phase of CT
paralleled the improvement in the image quality and scanning. Special imaging reformatting and reconstruction
speed of the CT examination. With the introduction of methods improve the general diagnostic accuracy of renal
spiral CT imaging, both the resolution and speed of the CT angiography and, in selected cases, the demonstration
available examinations have improved. Concurrent with of small accessory arteries and venous anomalies that
improved imaging, the expanded role of interventional otherwise might not be detected. Such 3-dimensional
radiologic procedures has created an important role for (3D) imaging also provides the surgical team with an
CT imaging in transplant recipients (Figure 13.7). excellent overall review of the surgical anatomy.
CT scanning is useful in the evaluation of both
immediate and delayed complications of renal
transplantation. Multidetector CT urography has been
shown superior to conventional IVP in the detection of
urinary leaks and ureteral obstructions. In the immediate
posttransplantation period, perigraft hemorrhage and
urinary leaks can be detected with CT. In most cases,
either USG or functional nuclear medicine imaging should
be performed first. CT scanning is the best means for
surveying the most common intermediate and delayed
FIGURE 13.7: CECT: Narrowed supplying artery with segmental surgical complications. CT findings are related to density
infarct in a transplanted kidney differences between areas of hemorrhage, lymphoceles,
and abscesses.
CT of the renal donor may reveal small renal cell Regarding the donor, after a careful review of the axial
cancers, cysts, upper collecting system duplications, and CT images with a cine technique, the evaluation of
partial renal collecting system obstructions. Particularly maximum intensity projection images in multiple
important, the CT examination should be performed prior projections and variable slab thicknesses is useful.
to the IV administration of contrast agent to look for renal Coronal, curved, multiplanar, reformatted images best
stones, as well as immediately after administration of the outline the full length of each renal artery.
bolus. Immediate complications related to recipient renal
Early postinjection-phase images best demonstrate transplantation is best evaluated with combined or
the renal cortex, while images obtained approximately independent renal USG, functional renal nuclear
40 seconds after injection of the contrast agent bolus best medicine study, CT, and occasionally MRI. Acute perirenal
show the combined enhancement within the renal collections with a high CT attenuation of 28 Hounsfield
units (HU) or greater is most likely perinephric Findings: Gadolinium-enhanced MRA is rapidly
hemorrhage. Perirenal or pararenal fluid collections of becoming the technique of choice for imaging the renal
18–24 HU are most likely lymphoceles. Complex fluid arteries. MRA has several advantages compared with
collections anywhere in the abdomen or pelvis of more conventional digital subtraction angiography (DSA). MRI
than 28 HU on a delayed basis may be abscess cavities and MRA are noninvasive and use no ionizing radiation.
Gadolinium-based contrast media are safe to use in
or chronic (liquefying) hematoma. In general, the findings
patients with renal insufficiency because the agents have
of perirenal transplant complications have a pattern
minimal nephrotoxic effects. MRA provides true anatomic
similar to that of diseases of the abdomen in other
images and permits multiplanar reformatting. High-
immunocompromised patients. Multidetector CT performance MR gradient systems allow the acquisition
urography is more complete and precise compared to of 3D volumes in a single breath hold of shorter than
more conventional diagnostic techniques. The overall 30 seconds. Imaging of the renal arteries is important
diagnostic accuracy of CT urography has been reported in the examination of potential renal donors and of
to be 90%. transplant patients with suspected renal artery stenosis
(Figure 13.8).
Advantages of CT over MRI: The layering of excreted
gadolinium into the urinary bladder and ureters generates
artifacts that are less easily managed at MRI than at CT.
During MRI, changing the patient’s position requires
additional time in the scanner. In comparison, prone or
decubitus positioning at CT allows the mixing of contrast
layers, which enables a more complete evaluation of the
urinary bladder, including the anterior bladder surfaces.
In general, CT of the kidneys and urinary bladder is better
in the detection of renal stones and of renal, ureter, and
bladder anomalies. CT with CTA generally costs less than
MRI. In most applications, MRI is reserved for examination
of the transplant recipient, whereas CT and CTA are used
in the potential donors.
MRI
In more recent years, MRI of the abdomen has evolved
into an excellent alternative means for the diagnosis of
most renal transplantation complications and for the
examination of the living related donor. The contrast FIGURE 13.8: CEMRA: Transplanted kidney with
its vascular supply
agents used for MRI are nontoxic to the transplanted
kidney, and MRI often can be used to assess renal MRI offers several advantages in the pretransplan-
function, vascular supply, and postoperative complica- tation donor and recipient evaluation. MRI offers good
tions. However, MRI remains expensive and may be spatial resolution with excellent tissue differentiation. No
contraindicated in certain patients. Whenever 2 or more adverse effects related to radiation are known, and the
diagnostic techniques are used in the diagnosis of renal gadolinium-based IV contrast agents can be safely used
transplantation, comparison between the techniques is in patients with renal insufficiency. MRA with gadolinium
often very useful. Comparison of recent, current studies enhancement has been improved by the development
with remote, prior examinations is critical. New fluid of fast 3D gradient-echo sequences. The T1 shortening
collects are of greater significance than are slowly resolving effect of the IV injection of paramagnetic contrast material
fluid collections. significantly improves vessel imaging by eliminating signal
loss from saturation effects, minimizing dependence of agent, both images and activity counts are collected from
the inflow phenomenon, and possibly decreasing the area of the abdominal aorta and the renal transplant
intravoxel dephasing effects. site. Visualization of the renal artery and the renal
MRA demonstrates accessory renal arteries using transplant should occur within 20–30 seconds, with no
gadolinium agents with the same degree of accuracy as significant difference between the time of aortic and renal
CTA. MRA has been successfully applied to the diagnosis visualization.
of most of the complications related to acute renal The causes of diminished renal function include ATN,
transplant vascular thrombosis. Gadopentetate rejection, and drug nephrotoxicity. ATN is the most
dimeglumine (DTPA), when administered as an IV bolus common cause of delayed graft function. ATN is
during MRA, provides qualitative functional information associated with prolonged ischemia and reperfusion
concerning the renal transplant. It demonstrates the injury. ATN occurs after placement of most cadaveric
morphology in a manner similar to CT and is generally grafts. Recovery is usually spontaneous within 2 weeks.
superior to diagnostic USG. ATN is less common in patients whose transplants are
Limitations: The spatial resolution of MRI is generally from living related donors.
less than that of CT, while the functional information Functional activity of the renal transplant is assessed
available is generally qualitative compared with the using activity curves obtained from the renal transplant,
quantitative data provided by nuclear renal scans. Patients aorta, and background. Orthoiodohippurate 131 and
with pacemakers, cochlear implants, most intracranial
99m
Tc MAG3 studies demonstrate a delayed transit with
aneurysm clips, or severe claustrophobia cannot be safely a delayed time to maximum activity and a high ratio of
examined using MRI. The use of conscious sedation in findings at 20 minutes versus those at 3 minutes.
mildly claustrophobic patients and young children can Mechanical leaks from the renal pelvis, ureter, or urinary
be considered in MRI but this adds to the cost. bladder can be detected as extrarenal nuclide collections.
Limitations: Although complete thrombosis and renal graft
Nuclear Medicine Scanning infarction is easily determined with nuclide studies,
Nuclear medicine scanning and flow studies remain the moderately severe transplant renal artery stenosis may
primary means for evaluating vascular supply to the not have a clear pattern on a renal scan. In dual (pediatric)
transplant after surgery. The main advantage of nuclear renal transplants, the delay in the visualization of one
medicine scans is that they demonstrate the pathophysio- of the kidneys may indicate partial thrombosis or stenosis
logy involved. Recent developments in Doppler USG and of the arterial supply to that kidney. However, in most
MRI show promise in improving the quality of renal cases, only a single renal transplant is present, and
vascular imaging without the use of potentially nephro- comparing its activity with that of the other kidney is not
toxic intravenous (IV) contrast materials. There is certainly possible. The degree of renal dysfunction must be
complementary role of nuclear medicine studies and USG compared with the known surgical history and the current
laboratory value.
in the imaging evaluation of hydronephrosis, renal artery
stenosis, flank pain, renal masses, pyelonephritis, and
Angiographic Study
kidney transplant.
Angiographic study of the transplant may involve CT
Findings: Nuclear medicine studies have a role in all stages angiography, MR angiography, or intra-arterial catheter
of postrenal transplantation evaluation. Immediately after angiography if therapeutic procedures are to be
surgery, nuclear medicine studies can be used to evaluate performed. The donor evaluation is most likely done with
diminished renal function. The most immediate cause MRA and CTA.
of renal transplant failure may be renal artery occlusion. In general, CT of the abdomen and kidneys is
During the period immediately after the bolus IV injection performed, especially at the time of the evaluation of
of the technetium 99mTc mercaptotriglycylglycine (MAG3) the renal vasculature. If MRA is selected for the renal
vascular evaluation, an MRU can follow with sufficient INTERVENTION

spatial resolution to obviate the need for a standard IVU. Many renal transplant patients undergo renal dialysis
Conventional angiography was long considered the during the years prior to receiving the renal transplant.
criterion standard for the evaluation of the renal arteries Interventional techniques used to treat thrombosed
and veins of the living related donor candidate. Today, peripheral arteries have been applied to the removal of
as noted before, CTA (with spiral multidetector and
clots within arteriovenous (AV) shunts used for renal
multiplanar reconstruction) and MRA have become the
dialysis. At other times, double-lumen central venous
imaging techniques of choice. If vascular status, severe
access lines may be used to continue renal dialysis in
arteriosclerosis, or venous abnormalities of the pelvic
the event of AV access failure. Interventional radiology
arteries is suspected that might complicate the creation
plays an important role in the care and treatment of renal
of the renal artery anastomosis, MRA or CTA can be
transplant recipients.
performed. Angioplasty or stent placement may be
Historically, catheter angiography has been performed
required with a subsequent interventional procedure.
to evaluate the anatomy of the renal arterial and venous
Findings: Conventional angiography has been replaced systems of potential renal donors. To lower costs and
by CTA or MRA in many diagnostic applications both reduce risk, CTA or MRA has largely replaced
in the pretransplantation and posttransplantation periods. conventional DSA in the preoperative phase of renal
Cost, potential morbidity, and possible nephrotoxic effects transplantation. However, image-guided interventional
of iodinated contrast must be considered. In most cases, techniques play a vital role in the relief of mechanical
digital subtraction angiography (DSA) provides the most urinary obstruction, drainage of abscess and cystic fluid
fully diagnostic evaluation of the renal artery, the collections, and aspiration biopsy of neoplastic and
recipient’s vascular status, and the patency of the renal
infectious processes.
veins.
The development of lymphoceles in the surgical site
In select cases, renal transplant angiography may be
of a renal transplant is a diagnostic challenge.
preferred to CTA or MRA. In all cases requiring vascular
Occasionally, lymphoceles enlarge to cause compression
intervention, DSA angiography immediately precedes the
of the renal transplant pelvis and hydronephrosis.
interventional procedure. The potential diagnoses best
Drainage of lymphoceles enables diagnosis, and it can
made with angiography include renal artery stenosis, renal
relieve a mechanical obstruction; however, lymphoceles
vein thrombosis, aneurysms, and arteriovenous fistula.
Except for a pelvic location, the diagnosis of renal may recur after catheter drainage. Renal transplant
transplant circulation disease is similar to the diagnosis patients’ immunosuppression makes the development of
of renal artery disease in the native kidney. opportunistic infections more likely. Evaluation of
Direct visualization of the lumen of a blood vessel aspiration biopsy samples in spinal diskitis may reveal
is considered the most accurate means to determine the fungal or bacterial infections that are not often seen in
presence of arterial stenosis, venous thrombosis, and most other patients.
arteriovenous malformation (AVM) or arteriovenous Interventional techniques commonly used to treat
fistula (AVF). Because angiography is generally performed vascular stenosis, including renal artery angioplasty and
just before therapy, other testing typically is not performed renal artery stent placement, may be applied to the
to confirm a catheter-proven lesion. Vascular flow management of renal artery stenosis. Improvements in
gradients can be assessed at the time of the procedure the management of transplant rejection have made renal
to confirm a hemodynamically important lesion prior to transplant arterial stenosis less common. Percutaneous
treatment and to confirm the absence of a gradient after nephrostomy may be applied to temporarily relieve a
angioplasty with or without stent placement. mechanical renal outlet obstruction.
• Radiological anatomy of urethra Type 3: unrelated to the verumontanum; present in the

• Urethral pathologies membranous urethra; consist of a disc-like membrane
– Urethritis with central pinhole orifice; rare.
– Periurethral abscess
– Urethral stricture CLINICAL FEATURES
– Urethral calculus
Occur only in males.
– Urethral polyps and diverticuli
Posterior urethral valves are the most common cause
– Urethral caruncle
– Congenital urethral lesions of obstructive symptoms and of ascites in the newborn.
– Urethral tumors. Complete obstruction may lead to renal failure,
oligohydramnios, and intrauterine death, calyceal rupture,
POSTERIOR URETHRAL VALVE (PUV) fetal intracapsular, perirenal or intrarenal urinomas or fetal
PATHOPHYSIOLOGY urine ascites. Other symptoms and signs may include
Posterior urethral valves refer to thick valve cusps that palpable kidneys and bladder, abdominal distention,
result from abnormal fusion and insertion of the plicae straining to void, absence of urinary stream and dribbling.
colliculi. Plica colliculi are the inferior extensions of the BUN and creatinine are usually elevated.
midline urethral crest (2-4 in number) that take a spiral Renal function commonly returns to normal after
course and end inferiorly in the membranous urethra. obstruction is relieved. Occasionally, chronic renal failure
The urethral crest is the continuation of the inferior aspect intervenes despite surgical decompression.
of the verumontanum.
Young children may present with symptoms of urinary
CLASSIFICATION tract infection—fever, vomiting, hematuria, or failure to
Young, 1919: thrive-resulting from vesicorenal reflux and pyelonephritis.
Type 1: present in the bulbo-membranous urethra below Older children or young adults with mild obstruction may
the verumontanum; most common type; produce clinical not be diagnosed until infection supervenes. Therefore,
manifestations. posterior valves must be included in the differential of
Type 2: extend proximally from the verumontanum to UTI or outlet obstruction in male infants, children and
the bladder neck; rare. young adults.
Urethral Diseases 193

Neonates (Figures 14.1 to 14.3)
Best examined with ultrasound.
Renal isotope studies (99mTc-DTPA or 99mTc-GH) can
assess degree of residual renal function and renal damage,
differential renal function, degree of hydronephrosis, and
site of obstruction. Addition of furosemide will produce
diureris and help distinguish from nonobstructive
hydronephrosis. Excretory urography is less useful in this
age group and is contraindicated in severe renal
impairment.
Older Children and Young Adults

Valves are best demonstrated by voiding cystourethro-
graphy, although catheter insertion may be difficult with
type 3 valves. The valves are recognized by a spinnaker
FIGURE 14.2: MCV: Type 3 PUV with dilated prostatic urethra,
sail sign that consists of the dilated posterior urethra and bladder neck hypertrophy and thick trabeculated bladder wall
a linear radiolucent defect in the bulbo-membranous
urethra. The urinary stream is reduced distal to the valves.
The bladder is of large capacity, trabeculated, and shows
diverticula, reflux and occasionally a periureteric Hutch
diverticulum that shows up best during reflux.
Transrectal voiding sonourethrography may
demonstrate bladder abnormalities and a dilated prostatic
urethra, but the valves themselves will rarely be visualized.
FIGURE 14.3: MCV: Type 1 PUV with grossly dilated posterior

urethra, trabeculated bladder right renal and agenesis left VUR
In Utero Diagnosis
Fetal outlet obstruction is diagnosed by ultrasonographic
findings of dilated renal pelves, dilated tortuous ureters,
distended bladder, oligohydramnios, and possibly,
FIGURE 14.1: Diagrammatic representation of PU valves pulmonary hypoplasia and a small thorax. Pelvicalyceal
(For color version see Plate 6) dilatation may be absent if a calyceal fornix has ruptured
and decompressed into an intracapsular, perirenal, or

intrarenal urinoma. A dilated posterior urethra is almost
pathognomonic of outlet obstruction due to posterior
valves. Outlet obstruction is an indication for intrauterine
surgery or early fetal delivery to reduce the incidence
of chronic renal failure in infants.
URETHRAL STRICTURES
INTRODUCTION
During urination, the bladder empties through the urethra
and out of the body. Urine passes through an opening
called the bladder neck into a portion of the urethra FIGURE 14.4: Ascending urethrogram tight urethral
stricture at penobulbar region
surrounded by the prostate, called the prostatic urethra.
The next segment of the urethra is called the membranous
urethra and it contains a muscle called the external urinary
sphincter. This sphincter allows a patient to voluntarily
hold urine and to stop during urination. Together, the
prostatic urethra and the membranous urethra make up
the posterior urethra, and are approximately one to two
inches long. The urine then enters the bulbar urethra,
followed by the penile urethra. The penile urethra is the
segment that runs along the bottom surface of the penis.
The exit at the tip of the penis is called the meatus. The
bulbar urethra, penile urethra and meatus make up the
anterior urethra, which is 9 to 10 inches long.
A urethral stricture is a scar in or around the urethra,
which can block the flow of urine, and is a result of FIGURE 14.5: Ascending urethrogram: Urethral stricture with
outline irregularity and spicking urethral carcinoma
inflammation, injury or infection.
penis up into the bladder to allow urine to drain until
ETIOLOGY a repair can be performed. Trauma such as straddle
Stricture disease may occur anywhere from the bladder injuries, direct trauma to the penis and catheterization
to the tip of the penis. can result in strictures of the anterior urethra.
The common causes of stricture are trauma to the In adults, urethral strictures may occur after prostate
urethra and gonorrheal infection. However, in many surgery, removal of kidney stones, urinary catheterization
cases, no cause can be identified. or other instrumentation.
Stricture of the posterior urethra is often caused by In children, urethral strictures most often follow
a urethral injury associated with a pelvic bone fracture reconstructive surgery for congenital abnormalities of the
(e.g. motor vehicle or industrial accident). Patients who penis and urethra, cystoscopy and urethral catheter
sustain posterior urethral injuries from pelvic fracture drainage.
generally suffer a disruption of the urethra, where the
urethra is cut and separated. These patients are ROLE OF RADIOLOGY AND IMAGING
completely unable to urinate and must have a catheter Evaluation of patients with urethral stricture disease
to realign the urethra. The catheter is placed through the includes:
1. Urethral imaging (X-rays or ultrasound) and progress, leading to a narrowing of the urethra and
2. Urethroscopy. difficulty passing urine. Other symptoms include soreness
The retrograde urethrogram is an invaluable test and itching in the area and sometimes ulceration.
to evaluate and document the stricture.
URETHRAL POLYPS
Combined with antegrade urethrogram, length of
the stricture can be determined. The retrograde A urethral polyp is an irregularity that is usually present
urethrogram is performed as an outpatient X-ray at birth. It is usually composed of fibrous tissue but may
procedure and can indicate the number, position, length include some smooth muscle, small cysts or nerve tissue
and severity of the stricture(s). This study involves all covered by a thin protective layer of tissue. Symptoms
insertion of contrast dye (fluid that can be seen on an include a lump in the vulva, blood in the urine or a
X-ray) into the urethra at the tip of the penis. No needles blockage. Urethral polyps are diagnosed with cystoscopy,
or catheters are used. The retrograde urethrogram study a fiber-optic technique that allows a urologist to readily
allows doctors to see the entire urethra and outlines the view the polyp, and a voiding cystourethrogram (VCUG).
area of narrowing at the stricture (Figures 14.4 and 14.5). By combining an X-ray of the urethra with dye in the
Ultrasound is performed by placing a small, high area, the doctor can easily view the structures.
frequency ultrasound probe on the skin over the stricture
to view it and surrounding tissue. PARAURETHRAL CYST
Urethroscopy is a procedure where the doctor gently
Also known as Skene’s glands, paraurethral glands are
places a small, flexible, lubricated telescope into the
located in the urethrovaginal wall at the opening into
urethra and advances it to the stricture. This study permits
the urethra in females. A paraurethral cyst will be evident
the doctor to see the urethra between the tip of the penis
to a doctor by its appearance — a glistening, tense and
and the stricture.
bulging yellowish-white mass reducing the size of the
Recently MRI is also being used for evaluation of
urethral opening. Other symptoms include a misdirected
urethral lesions.
urinary stream and possibly painful urination.
OTHER BENIGN URETHRAL LESIONS URETHRAL CARUNCLE
Causes can include abscesses, pelvic fractures, straddle Urethral caruncles are polypoid (or stalk-like) masses,
injuries, infections or injury caused by surgical instruments hanging from one area of the external urethral opening.
(e.g., catheters, cystoscopes, resectoscopes, etc.). The primary sign of this problem is a thin, reddish
BENIGN NEOPLASMS
membrane protruding from one portion of the urethral
opening. Other symptoms include bleeding and urination
Linked to the presence of genital warts on the penile shaft, problems such as frequency, urgency and pain. A urethral
these lesions are often the product of human papilloma caruncle is usually spotted during an examination for
virus (HPV). Urethral wart-like growths are suspected another condition. They are relatively common in the
when there is bleeding from the urethra, a visible lesion urethral epithelium of women who do not use hormone
on the opening of the urethra or changes in the urinary replacement therapy (HRT) after menopause. Marked by
stream, accompanied by a history of genital warts. a purplish mucosal mass, this condition can cause a
BALANITIS XEROTICA OBLITERANS variety of symptoms, including difficult or painful
urination, blood in the urine and tenderness.
Balanitis xerotica obliterans is a chronic condition with
unknown causes that affects the end of the penis and URETHRAL DIVERTICULUM
is marked by pale, shiny, whitish skin around the opening Urethral diverticulum are outpouchings of urethral
of the urethra. The scarring and thinning of the membrane mucosa through their weak walls (Figures 14.6 and
begin (in most cases) in young adulthood and can 14.7).
FIGURE 14.6: MCU: Urethral diverticulum FIGURE 14.9: Urethrogram: Beaded urethrogram-multiple short
segment urethral strictures typical of TB
FIGURE 14.7: MCU: Well defined, smooth, oval filling

defect in mid urethra urethral polyp
FIGURE 14.10: Hypoplastic penis with underdeveloped urethra
FIGURE 14.8: Ascending urethrogram: Normal anterior urethra FIGURE 14.11: MCU: Meatal stenosis
FIGURE 14.12: Plain X-ray: Calculus in prostatic urethra FIGURE 14.13: Ascending urethrogram: Filling defect due to
calculus in penile urethra
FIGURE 14.14: USG: Calculus in prostatic urethra with dense post-acoustic shadow
FIGURE 14.15: MCU: Urethral trauma with narrowing of bulbo- FIGURE 14.16: MCU: Large periurethral collection of
membranous urethra and extravasation of contrast periurethrally contrast abscess cavity
DUPLICATION OF URETHRA URETHRAL CALCULUS

See Figures 14.8 and 14.9. See Figures 14.12 to 14.14.
AGENESIS OF URETHRA PERIURETHRAL ABSCESS

See Figure 14.10. See Figures 14.15 and 14.16.
MEATAL STENOSIS
See Figure 14.11.
Urinary Bladder Diseases 199
CLASSIFICATION congenital urethral valve or urethral stricture. The

• Bladder diverticulum congenital variety of diverticula are typically seen in
• Neurogenic bladder boys. These diverticula are characteristically located
• Vesical calculus at the ureteral insertions. Often, vesicoureteral reflux
• Emphysematous cystitis is resultant. Bladder diverticula most often occur as
• Bladder carcinoma a result of outlet obstruction. Occasionally, a congenital
• Rhabdomyosarcoma of bladder weakness in the bladder wall adjacent to the ureteral
• Primary lymphoma of bladder orifice results in a diverticulum. This is termed a
• Urachal carcinoma “Hutch” diverticulum.
• Bladder outlet obstruction (BOO). In children, outlet obstruction causing a diverti-
culum is rare and can be seen with urethral valves.
In men, diverticula are associated with outlet obs-
BLADDER DIVERTICULUM
truction from urethral stricture, prostatic hypertrophy,
The diverticula are full thickness outpouchings of mucosa, prostatic carcinoma, etc. acquired diverticula are rare
adventitia, and detrusor muscle. There is usually a in women.
communicating channel with the main bladder lumen. • If it is an acquired diverticula, the bladder outlet
Diverticula can have wide or narrow necks. The wide obstruction is usually secondary to BPH, carcinoma,
necked variety empty urine readily. The narrow neck types or prostatitis. Acquired diverticula can occur anywhere
are slow to empty and therefore, are more likely to have there is a bladder wall weakness, although, as in the
urinary stasis. congenital variety, the most vulnerable area for
Bladder diverticula are categorized as: presentation is at the ureteral insertions.
• Congenital or Diverticula usually occur on the lateral bladder walls,
• Acquired. rarely the dome. They are often multiple. Large diverticula
Whether congenital or acquired the etiology is often displace the bladder and or ureters.
secondary to bladder outlet obstruction. Infection, tumor and stone formation can occur as
• If a congenital diverticula (hutch diverticula), the a result of urine stasis within a diverticulum. Tumor
bladder outlet obstruction is usually secondary to a formation in a diverticulum is more likely to spread
beyond the bladder because the diverticulum wall consists CYSTITIS (Figure 15.2)
only of urothelium without muscle. • Ascending urinary tract infection is extremely common
Bladder diverticula can be evaluated with excretory in sexually active females.
urography, ultrasound, CT and cystoscopy. • Urethritis and cystitis can occur.
CONGENITAL URETHRAL DIVERTICULUM • Burning of micturation is the clinical hallmark
• Wall thickness upto 5 mm may be normal, however
INCIDENCE
thickend, hypoechoic and irregular bladder with
Occurs mainly in male infants and children. echoses in urine may favor cystitism USG.
Female ureteral diverticulum is acquired.
ANATOMICAL BASIS EMPHYSEMATOUS CYSTITIS
Arises from the ventral surface of the anterior urethra
Embryology: result of either failure of closure of urethral
folds or an abortive attempt at urethral duplication (in
which case there are anterior urethral valves in the
penoscrotal junction region).
Diverticulum fills slowly during voiding and has a narrow
neck. As it fills it may obstruct drainage from the true
urethra, leading to incomplete bladder emptying and
infection. At the termination of micturition, the
diverticulum causes dribbling (Figure 15.1).
TREATMENT
FIGURE 15.2: Ultrasonographic section: Cystitis
Urethroplasty to repair the defect in the urethral floor.
INTRODUCTION
It is a rare entity. Typical patients are immunocompro-
mised or elderly and debilitated, and 50 percent of cases
are seen in diabetics.
CLINICAL FEATURES
Patients may present with anything from no symptoms

to an apparent acute abdomen. Pneumaturia is rare.
Prevalence in females is twice that in males. Often
associated with urinary stasis, and growth of gas forming
organisms (most commonly E. coli, but others include
Enterobacter aerogenes, Proteus mirabilis, Staphylo-
coccus aureus, streptococci. Clostridium perfringens, and
Candida albicans).
Plain abdominal radiographs are generally sufficient

FIGURE 15.1: MCU: Multiple pseudodiverticuli c ‘pine-tree’
appearance of urinary bladder in a case of neurogenic bladder to confirm the diagnosis. This disease is often not
suspected until discovered on the abdominal radiograph. is endemic are expected to have a higher incidence of
Findings include curvilinear gas lucencies throughout the TCC.
bladder, often described as a “cobblestone” or
“beaded necklace” appearance. Gas may ascend the PATHOPHYSIOLOGY
ureters and reveal an air pyelogram. In rare cases, where Causative Agents
plain images are negative despite high clinical suspicion,
1. Chemical carcinogens act locally on the epithelium,
CT may show intramural bladder gas.
causing a field change; their action is enhanced by
This appearance differs from that of bladder gas
the contact time (exacerbated by urinary stasis or
introduced by trauma, instrumentation, or vesicocolic
diverticulae).
fistula, where gas is confined to the bladder lumen, and
Chemical carcinogens:
does not exhibit the curvilinear appearance.
Tobacco
Treatment of emphysematous cystitis involves early
Aniline dyes
broad spectrum antibiotics, drainage of the bladder, and
Benzidine
management of hyperglycemia if present.
Aromatic amines
Prognosis in patients diagnosed and treated early in
Rubber; and
the disease process is usually good. The development
Azo dyes used in textiles, printing, and plastic
of emphysematous ureteritis, nephritis, or adrenalitis manufacturing.
portends a poor prognosis. 2. Partial obstruction may account for increased exposure
to the upper tracts (e.g. increased incidence of TCC
TRANSITIONAL CELL CARCINOMA (TCC) OF in horseshoe kidney).
URINARY BLADDER 3. Abuse of analgesics (e.g, phenacetin, which increases
INTRODUCTION the risk 8 times); cyclophosphamide therapy (6-y lag
time), particularly after drug-induced hemorrhagic
Embryologic derivation of the collecting system of the
cystitis.
urinary tract occurs from the fetal mesonephros. As a
4. Balkan nephritis, with progressive renal failure; and
result of this development, tumors of the renal collecting multiple bilateral tumors (etiology unknown); and
system arise from cell origins different from the renal 5. Recurrent or chronic infection and urinary calculi
parenchyma. Their classification is based on their (squamous cell carcinoma [SCC] is the most common
respective mesodermal or epithelial tissue origin. Primary entity in this case).
neoplasms of the renal collecting system represent
10% of the renal tumors, of which approximately 80% Polychronotropism
are malignant. Most are transitional cell carcinomas Of great importance is the multiplicity of TCCs of both
(TCCs). synchronous and metachronous natures. This issue
Bladder TCC is 50 times more common than renal includes a frequent association of papillomas in patients
pelvic tumors. Often, TCCs are multiple, involving any with TCCs. Almost 25% of patients with renal pelvic
part or all of the collecting system. The tumors are papillomas develop a carcinoma. Of those with multiple
traditionally classified into papillary and nonpapillary papillomas, 50% develop carcinomas. Ureteric TCC may
types. Nonpapillary tumors are considered malignant. be solitary, but TCC has marked propensity for unilateral
multicentricity. The reason is unknown, but this
INCIDENCE distribution may result from antegrade and/or retrograde
The true incidence of TCC internationally is not known; seeding or the fact that the entire ureteral mucosa is
however, regions of the world where schistosomiasis exposed to the carcinogen.
Pathology/Histology
TCCs usually have the following broad classification:
1. Exophytic papillary lesion (85%) with a frond-like
structure and central fibrovascular core lined by an
epithelial layer (broad based and pedunculated);
2. Nonpapillary, noninfiltrating;
3. Infiltrating, usually of higher grade and less common;
and
4. Carcinoma in situ.
STAGING OF TCC
Although no staging method is universally recommended,
the following tumor, nodes, metastases (TNM)–American
Joint Committee on Cancer (AJCC) technique is useful
in upper tract and ureteral lesions:
• Tis 0 indicates an in situ lesion; Ta, noninvasive
papillary carcinoma;
• T1 I, invasion of the subepithelial connective tissue;
• T2 II, tumor confined to the muscularis layer;
FIGURE 15.3: Staging of urinary bladder carcinoma
• T3 III, invasion of the renal parenchyma and/or
peripelvic soft tissues; and
• T4 IV, extension beyond the renal capsule. commonly occurs as a result of edema after endoscopy
and/or endoscopic resection and as a result of fibrosis
A staging classification that incorporates the TMN
from radiation therapy.
and Jewett-Strong-Marshall (JSM) systems is
useful. The stages are as follows:
METASTASES
• T1 A indicates lesions involving the mucosa and
submucosa; Hematogenous spread is less frequent with renal pelvic
• T2 B1, invasion of the superficial muscle layer; tumors than with hypernephromas.
• T3a B2, invasion of the deep muscular wall; Lymphatic supply is extensive, and lymphogenous
• T3b C, invasion of perivesical fat; involvement occurs early in the disease process.
• T4a D1, extension to perivesical organs; Metastases occur to the lung, lymph nodes, and liver,
• T4b, invasion of the pelvic and/or abdominal wall; with direct extension into the retroperitoneum being
and D2, distant metastases (Figure 15.3). common.
Metastases from ureteral TCC are far more common
GRADING OF TCC
than those from bladder cancer partly because the ureteral
The grade is usually correlated with the stage, as follows: wall is thin and acts as a poor barrier. Metastases occur
1. Indicates the cells are slightly anaplastic; in approximately 11% of cases, in the following sites:
2. Intermediate features are observed; and • Retroperitoneal nodes (34%);
3. Marked cellular pleomorphism is present. • Distant lymph nodes (17%);
The stage of tumors in the bladder as assessed by • Liver (17%);
the depth of infiltration is the single most important • Lumbar vertebrae (13%);
prognostic parameter when treatment is initiated. • Lungs (9%);
Clinical staging has an accuracy of 50% when compared • Kidneys (8%);
with that with CT (32–80%) or MRI (73%). Overstaging • Adrenals (4%);
• Spleen (2%); PREFERRED EXAMINATION

• Sacral vertebrae (2%); Elevated urinary lactate dehydrogenase (LDH) levels
• Brain (2%); have been reported, but this finding is nonspecific.
• Pancreas (2%); However, cytology can play a significant role, with a
• Skin (2%); and 60% accuracy rate in the diagnosis of renal pelvic and
• Isolated metastases to the colon, broad ligament, ureteral TCC. Cytology is particularly useful in more
diaphragm, humerus, omentum, pericardium, pleura, advanced stage tumors, with an accuracy rate improved
prostate, and vagina. to 80%. Selective lavage, ureteral urine collection, brush
biopsy, or ureteroscopy may be performed to obtain
ANATOMICAL BASIS specimens.
Embryologic derivation of the urinary tract collecting
SUGGESTED SYSTEMATIC APPROACH TO EVALUATE
system occurs from the fetal mesonephros. As a result
ANY UB MASS AND ITS EXTENSION
of this development, tumors of the collecting system of
the renal tract arise from cell origins different from the • Identify the tumor.
renal parenchyma. Their classification is based on their • Identify the capsule and possible invasion.
respective mesodermal or epithelial tissue origin. • Evaluate for periprostatic fat infiltration.
The uroepithelium is the mucosal lining of the calyces, • Evaluate the neurovascular bundles and other
infundibula, ureters, urinary bladder, and portions of the periprostatic vascular structures.
urethra. The uroepithelium has a characteristic micro- • Evaluate abnormalities within the seminal vesicles.
scopic appearance that is midway between glandular and Determine whether the base of the prostate is involved
squamous tissue and hence, its name transitional epi- and whether extraprostatic extension at the base is
thelium. Following the distribution of the uroepithelium, present.
TCC can develop anywhere from the calyces down to • Identify the apex of the prostate and evaluate for
parts of urethra with uroepithelial lining. possible tumor involvement and apical extension.
• Identify any regional or extraregional lymph nodes.
CLINICAL FEATURES Identify any bone involvement.
Sex: The male-to-female ratio is 3–4:1. ROLE OF RADIOLOGY AND IMAGING
Age: The mean age of onset is 50–70 years. The work-up of patients in whom TCC is suspected
Hematuria is a common symptom that occurs in as includes ultrasonography and IVU. However, CT scanning
many as 80% of patients; it may be frank or microscopic. is the best modality for the detection and staging of TCC,
Hematuria may occur early or late in the development usually without the need for additional imaging.
of the disease. For upper tract TCC pain, abdominal mass
Plain Radiograph
and pyuria occur with approximately the same frequency
as that of other renal based tumors. Dysuria and frequency Plain radiographic findings, such as calcifications, are not
are more commonly reported with ureteral tumors. Pain specific in the diagnosis of urothelial tumors, and they
is usually dull and colicky and results from obstruction, usually contribute little to the diagnosis.
Plain radiographic findings are nonspecific, parti-
although a second type of pain results from direct tumoral
cularly the presence of calcification, which has a wide
extension. This is frequently severe, constant, and
differential. Filling defects in the renal collecting system
penetrating.
and the bladder, as seen with IVU, CT, and retrograde
Rare instances of spontaneous urinary extravasation
pyelography, are present in a number of other patho-
due to rupture have been reported; this often results in
logies. These findings are not specific for TCC.
an acute abdomen. Other rare findings include
hypercalcemia, secondary amyloidosis, and elevated Findings: Plain radiography may be first study to alert the
chorionic gonadotropin levels. radiologist to presence of a renal mass or bladder mass.
Occasionally, a large renal outline may be seen in a intravenous contrast material is delivered. Occasionally,
completely obstructed kidney. Uncommonly, radio- contrast material may be trapped within a blood clot
graphically discernible areas of punctate calcification may formed in the ureter and then extruded into the bladder
be seen in TCC. The calcification is on the surface of the as a stringy mass that may trap contrast material in an
tumor and not within the mass duct. Intrinsic calcifications irregular fashion. However, this entrapment is coarser
suggest an adenocarcinoma or the unusual cell type. than that seen with neoplastic stippling.
TCC is associated with analgesic nephropathy. Plain Fungus balls or mycetoma may also occasionally
radiographic findings of displacement of renal calcifi- entrap contrast material, but the pattern of entrapment
cations, which occur in analgesic nephropathy, may be is lamellar and frequently associated with gas formation
the first sign of TCC. Surface calcification of a bladder (frequently, patients have poorly controlled diabetes with
tumor may be seen on plain radiographs in 1% urinary tract infections). Rarely, stippling may be observed
of cases. Osseous expansive destruction or lung in tubular ectasia when papillae containing dilated ducts
metastases may be seen in the presence of renal of Bellini are seen en face. Inspection of the other papillae
malignancy. may make the diagnosis obvious.
Phantom calyx represents the failure of a calyx to
Intravenous Urography opacify due to obstruction. Oncocalyx represents calyceal
IVU is a common diagnostic test in patients with distension due to the tumor.
hematuria, although the early detection of small urothelial In contrast to renal TCC, ureteric tumors show a single
tumors may be difficult. A meticulous IVU technique is or multiple filling defects in only 19% of cases. This
required. observation may be associated with hydronephrosis and
hydroureter in 34% of cases or to a nonfunctioning kidney
Findings: The most common findings in TCC of the kidney
in advanced cases.
are a single or multiple filling defects in renal pelvis, as
Bladder TCC also shows an irregular filling defect
seen in 35% of cases. About 26% of patients with renal
with broad base and fronds. Increased thickness of the
TCC may show a dilated calyx due to partial or complete
bladder wall in the region of the tumor should indicate
obstruction of infundibulum. Amputation of the calyx may
infiltration.
be seen in 19% of cases. No visualization of the affected
kidney may occur in 13–31% of cases. This is usually Retrograde Pyelography
secondary to hydronephrosis but can be the result of
RGP is useful when the kidney cannot be visualized by
tumor infiltrating and replacing functioning kidney or the
means of IVU or when IVU cannot be performed because
occlusion of the renal vein. Hydronephrosis with renal
of renal disease or an adverse response to the contrast
enlargement due to tumoral obstruction of ureteropelvic
agent. Retrograde pyelography also has the advantage
junction may be seen in 6% of cases.
that it can be combined with various biopsy techniques.
Various signs have been described in renal TCC. The
stipple sign is a rare sign that occurs when contrast Findings: Retrograde pyelography in ureteric TCC may
material is trapped within the interstices of a papillary show focal expansion of ureters around and distal to the
tumor. When this is seen en face, the tumor appears to mass called the champagne, or goblet, sign. This finding
contain multiple stipples. This sign is highly suggestive is helpful in differentiating TCC from other causes of focal
of TCC. When this sign is seen, blood clots and lesions such as calculi or blood clots. Typically, calculi
radiolucent calculi are excluded, and further investigations and blood clots do not demonstrate a goblet sign as they
are superfluous. Punctate calcification on a TCC may do not progressively stretch the ureteral wall from slow
mimic the stipple sign. growth. Occasionally, the catheter may coil below the
Punctate calcification is unusual in TCC, and such mass during retrograde catheterization; this is called the
calcification is apparent on the scout image before the Bergman sign.
Retrograde pyelography is useful when the kidney Limitations: On sonograms, calculi may be confused with
cannot be visualized with IVU or when an intravenous high-grade TCCs, which can be densely echogenic. No
approach cannot be performed because of renal disease sonographic features are specific for TCC, and many filling
or an adverse reaction to contrast agents. Retrograde defects within the renal collecting system and bladder
pyelography may be combined with brush biopsy for a may give rise to a nonspecific appearance. Ultrasono-
highly reliable method in the definitive diagnosis of TCC. graphy also has problems in depicting nondilated ureters.
The results are considered diagnostic in 85 percent of cases.
If spot filming is the imaging technique, this should CT
be performed in an appropriate fluoroscopic digital unit Multidetector-row CT (MDCT) with postprocessing image
where serial images can be obtained. This would at both manipulation (contour imaging) is rapidly challenging
diuresis urography (infusion IVU with 300 mL of IVU.
30% contrast media) or at antegrade/retrograde CT is rapidly increasing its sensitivity in staging early
pyelography. TCC, as noted above. However, developments such as
virtual cystoscopy and postmicturition scanning should
Angiography
improve the overall accuracy of staging by using CT.
It has no role in the diagnosis of TCC, but it may have On CT scans, confusion may occur with a blood clot
a role in preoperative planning, particularly when and a hyperattenuating TCC, particularly in early stages
neoplasm sparring surgery is being considered. However,
of the disease. Also, as alluded to above, any cause of
here also, CT angiography (CTA) and magnetic resonance
inhomogeneous renal fatty attenuation in a patient with
angiography (MRA) are challenging the catheter studies.
undiagnosed early-stage TCC may hinder accurate
Ultrasonography diagnosis.
It is inaccurate for diagnosing early TCC, and it is useful Multidetector-row CT is replacing IVU in the
in the diagnosis of obstructive uropathy. Ureteric lesions evaluation of hematuria, although IVU is still
are particularly difficult to visualize unless they cause being used. The early detection of small urothelial
hydronephrosis and hydroureter. The other limitation of tumors demands a meticulous IVU technique. Accurate
ultrasonography is that it is inaccurate in the staging of demonstration of the pelvocalyceal system requires
bladder TCC, particularly Ta and T1 tumors, and also abdominal compression to distend the collecting systems,
in detecting pelvic lymph-node involvement. and oblique imaging or tomography may also be
necessary. Diuresis multidetector-row CT with contour
Findings: The most common sonographic appearance reformatting of the axial records may be useful. This
of TCC is a hypoechoic renal collecting system mass technique does not require abdominal compression. Early
splitting the central echocomplex with varying degree of experience with this method has been most positive
infundibular dilatation. Focal hypoechogenicity of
because the CT technique demonstrates not only the
adjacent renal cortex reflects local invasion. Occasionally,
opacified lumen but also the surrounding soft tissue
the central echocomplex may be only segmentally
anatomy.
amputated.
Ultrasonography is inaccurate for diagnosing early Findings: Filling defects with dilated calyces are easily
TCC, and it is useful in the diagnosis of obstructive seen and readily diagnosed. Hydronephrosis with renal
uropathy. Ureteric lesions are particularly difficult to enlargement is easily confused with uncomplicated
visualize unless they cause hydronephrosis and primary pelvic hydronephrosis, and antegrade/retrograde
hydroureter. The other limitation of ultrasonography is pyelography is usually required for diagnosis.
that it is inaccurate in the staging of bladder TCC, Adequate depiction of the ureters is difficult, and
particularly Ta and T1 tumors, and also in detecting pelvic prone, oblique, or even spot images obtained during
lymphnode involvement. fluoroscopy may be required at urography. If an area
of constant narrowing of the ureters is seen on more the upper urinary tract, both at the time of the initial
than 1 image and if it cannot be explained by a crossing diagnosis and during the follow-up of urothelial cancer.
vessels or peristaltic wave, a tumor must be suspected. The statistics for multidetector-row CT are now in
A meticulous urographic examination (radiographic or development.
multidetector-row CT) can prevent unnecessary Approximately 5% of ureteric TCCs grow intramurally,
pyelography/ureterography in the majority of cases. producing a stricture rather than a filling defect.
Importantly, if the demonstration of the pelvicalyceal Radiologically, distinguishing this type of tumor from
system at excretion urography or CT is poor, an upper- stricture is impossible. However at CT, a thickened ureteric
tract tumor may be missed, particularly when a decoy wall with stranding may be seen.
of a bladder tumor is present. The coexistence of upper-
and lower-tract tumors is not rare (Figures 15.4 and 15.5). MRI
The sensitivity and specificity with regard to the upper It has the advantage of high intrinsic soft-tissue contrast,
urinary tract pathology are 67% and 91%, respectively, its direct multiplanar capability, and the availability of
for IVU and 56% and 94%, respectively, for nontoxic renally excreted contrast agents. MRI appears
ultrasonography. For both techniques combined, the to be at least as useful as CT in the evaluation of
sensitivity and specificity are 79% and 88%, perivesical fat involvement, and it may be superior to
respectively. IVU has a 70% accuracy rate in the diagnosis CT in the detection of invasion of the adjacent organs.
of bladder TCC. The role of radiology is in demonstrating However, MRI cannot depict superficial invasion of the
upper urinary tract TCC well.
Both CT and MRI have been shown to perform better
than cystography in the diagnosis of tumors in the bladder
diverticula that are not depicted on cystograms because
of obstruction at the diverticular orifice.
MRI is expensive and has limited availability. False-
positive diagnosis has been reported. In staging bladder
TCC, tumor extension is overstaged according to the TNM
classification in 7.5% of patients and understaged in
32.5% of patients.
FIGURE 15.4: Focal bladder wall thickening in a case of RNI

Carcinoma bladder
Isotope renography is an extremely sensitive means of
assessing relative renal function and obstructive uropathy
prior to surgery. Isotope bone scanning is a useful
technique in the investigation of skeletal metastases.
Findings: Isotope renography is an extremely sensitive
means of assessing relative renal function and obstructive
uropathy prior to surgery. Isotope bone scanning is a
useful technique in the investigation of skeletal metastases.
D/D OF FILLING DEFECTS
1. Radiolucent or uric acid stones,

2. Blood clots,
FIGURE 15.5: Focal bladder wall thickening carcinoma at bladder
bases with coincidental right UVJ calculus 3. Sloughed papilla due to papillary necrosis or
4. Fungal balls in patients with diabetes may mimic Because of these serious limitations, the percutaneous
a TCC. method is reserved for high-risk patients with a solitary
5. Vessel crossing may cause a linear extrinsic kidney low-grade lesion, a negative history of bladder
impression; in these cases, oblique or compression TCC, and negative preoperative cytologic results in the
images may help in differentiating them from upper urinary tract.
urothelial tumors. Unlike renal cell adenocarcinomas, TCC is prone to
6. Tuberculosis can cause narrowed infundibulum and spread after percutaneous interventional procedures (e.g.,
irregular calices and is the major imaging differential nephrostomy).
diagnosis.
7. Other mimics include pyeloureteritis cystica. MORTALITY/MORBIDITY
8. Renal cell carcinoma invading the pelvicalyceal Approximately 90% of TCCs are curable in patients with
system, or superficial, confined tumors. Those with deeply invasive
9. Choleastoma types, which are still confined to renal pelvis and ureter,
10. Papilloma or polyp have a 10–15% likelihood of cure. Usually, distant
11. SCC is radiographically indistinguishable from TCC; metastases that penetrate the urothelial wall cannot be
however, SCC is less likely to be a polypoid tumor. cured with current forms of treatment.
Although a diagnosis may be made on the basis
SUMMARY
of urograms or cystograms, a small bladder tumor,
especially one of the infiltrative types, can remain Bladder cancer is most common in ages 50-69, with a
undetected. Furthermore, a dense concentration of male: female ratio of 3:1. 95% of malignant bladder
contrast material may obscure the intraluminal part cancer is carcinoma, originating from urothelial
of the urothelial tumor. (transitional cell), squamous, or glandular cells. 90 percent
are transitional cell, 5% are squamous, and
INTERVENTION 2% are adenocarcinoma. Less common, non-epithelial
Nephrostomy may be indicated in renal or ureteric bladder cancers include leiomyosarcoma and lymphoma,
inoperable neoplastic obstruction or as a stop-gap though invasion from carcinoma of the prostate, cervix,
procedure prior to surgery to improve renal function. The uterus, and sigmoid colon can also occur.
conventional and standard technique in the treatment Risk factors for TCC include industrial chemical
of TCC is a nephroureterectomy with either a standard exposure (azo or aniline dyes), cigarette smoking,
surgical technique or a laparoscopic approach. Percuta- analgesic abuse, and prior radiation. Most TCC’s originate
neous renal endoscopy has been used in the diagnosis in the bladder trigone and posterolateral bladder wall,
and treatment of TCC affecting the renal collecting system; and 75 percent are superficial papillary lesions. Upper
however, this approach remains controversial. tract metachronous tumors occur in 2–3% conversely,
In high-risk patients and in those with a solitary kidney, up to 40% of patients with upper tract TCC will develop
a less-invasive approach may be considered. A percuta- bladder TCC.
neous nephrostomy tract is created to access the tumor, Risk factors for SCC of the bladder include schisto-
which is then removed by using a resectoscope with somiasis, neurogenic bladder with resultant chronic
electrocautery or an Nd:YAG laser. irritation and infection, indwelling catheter, and recurrent
cystitis. Increased risk for adenocarcinoma includes the
SPECIAL CONCERNS presence of urachal remnants and bladder exstrophy.
Although percutaneous nephrostomy is successful for the For TCC, depth of invasion is a good predictor of
removal of most renal collecting system tumors, the recurrence, metastatic disease, and survival. Muscle-
recurrence rate is high, and nearly one third of patients invasive tumors have a poor prognosis due to resultant
have recurrent tumors within two years. lymphatic and distant spread, with 5 years survival
dropping to 10–20% in this segment. UVJ obstruction

and hydroureter usually indicate muscular invasion. IV
urography is a useful adjunct to detect synchronous upper-
tract urothelial cancers. CT and MRI are used for staging,
but accurate assessment of the depth of invasion is not
possible. MRI is superior for the detection of invasion
of adjacent organs, and is thought to be better for
evaluating tumors of the bladder dome or base.
RHABDOMYOSARCOMA
INTRODUCTION
It accounts for 5–10% of all malignant tumors in patients
under 15 years and is most common malignant tumor
of the vagina, prostate, and bladder.
FIGURE 15.6: Precontrast T1 WMR
PATHOPHYSIOLOGY
Other sites of involvement include perineal region, head
and neck, skeletal muscle/soft-tissue, protahepatis,
peritoneal.
Most neoplasms of the bladder in children are
malignant, with Rhabdomyosarcoma most common. TCC
and Leiomyosarcomsa are rarely seen. Clinically bladder
tumors present with hematuria and retention and can
cause flank pain secondary to hydronephrosis and
constipation.
Rhabdomyosarcoma appears either as pedunculated
soft-tissue mass with bunch of grapes appearance
(Botryoid) or as focal or diffuse wall thickening.

On CT asymmetry of fat planes or direct soft tissue into
adjacent organs is useful for assessing stage. Pelvic lymph
nodes may be enlarged indicative of metastatic disease. FIGURE 15.7: Postcontrast T1 WMR-brightly enhancing
Metastatic disease to the nodes, liver, bone, lung, brain. rhabdomyosarcoma of bladder in a child
Distinguishing between localized benign and malignant

MORTALITY/MORBIDITY
tumors of the bladder may not be possible with CT
(Figures 15.6 and 15.7). Three year survival rates are 70–80%.
TREATMENT PRIMARY LYMPHOMA OF BLADDER

Genitourinary rhabdomyosarcoma is initially managed INTRODUCTION
by chemo. Patients with operable tumors undergo It is a rare malignancy of the bladder, and accounts for
surgery and radiation rendered for residual inoperable less than 0.2% of bladder neoplasms. Involvement of the
tumor. bladder by manifestations of systemic lymphoma is more
common, but still rare. It is generally thought that no

lymphoid tissue is present in the bladder. However, it
is felt that lymphoid tissue can develop in bladders affected
by chronic cystitis and this can give rise to the develop-
ment of lymphoma.
CLINICAL FEATURES
Primary lymphoma of the bladder is more commonly

seen in elderly woman who present with a history of
chronic cystitis. Obstruction of the ureter orifice with
resulting hydronephrosis is uncommon since the tumor
usually does not involve the ureteric orifice.
Patients most commonly present with symptoms of
periodic hematuria of variable intensity. This can be
FIGURE 15.9: CT: Bladder lymphoma adjacent prostate
associated with urinary frequency and dysuria. Associated
urinary tract infection occurs in about 50% of patients.
MORTALITY/MORBIDITY
ROLE OF RADIOLOGY AND IMAGING Prognosis and treatment of prostate cancer requires
staging. Prostate carcinoma confined to the gland has
Transrectal ultrasound is not able to acceptably stage local
a 5-year survival rate of 94%. With extracapsular
prostate cancer. Ultrasound is unable to consistently identify
extension, the 5-year survival rate is less than 70%.
the prostate capsule and subtle infiltration of tumor beyond
the prostate. With the use of endorectal surface coil, MRI
is able to routinely identify the tumor, the prostate capsule, URACHAL CARCINOMA
the neurovascular bundles and surrounding periprostatic INTRODUCTION
structures (Figures 15.8 and 15.9). It is a rare cancer accounting for less than 0.7% of bladder
cancers. Commonly, urachal carcinoma arises in the
TREATMENT
dome of the urinary bladder at the vesicourachal junction.
Treatment of bladder lymphoma is generally nonsurgical The tumor commonly invades the anterior abdominal
with patients undergoing chemotherapy. wall, and most patients have a poor prognosis.
Approximately 85% of urachal carcinomas are
adenocarcinomas, and these are 40% of bladder
adenocarcinomas.
PATHOPHYSIOLOGY
The pathogenesis of urachal carcinoma is poorly

understood. Adenocarcinoma is believed to arise from
malignant transformation of columnar metaplasia in as
many as 84% of patients. However, in 3%, it arises within
the transitional cell epithelium, and the transitional
epithelium undergoes metaplasia to become glandular
epithelium. Approximately 70% of urachal carcinomas are
mucin-producing adenocarcinomas, and 15% are
FIGURE 15.8: CT: Bladder lymphoma invading adjacent prostate non–mucin-producing adenocarcinomas.
INCIDENCE Findings: A mass, often with relatively low attenuation,

Urachal carcinomas are rare. may be depicted at the anterior dome of the bladder in
the midline of the body or just off the midline. A mass
ANATOMICAL BASIS in bladder dome with stippled calcifications is pathogno-
The urachus is a musculofibrous band that is an extension monic for urachal adenocarcinoma. Extension into the
of the urogenital sinus. This band extends from the perivesical fat, anterior abdominal wall, and through the
bladder dome to the umbilicus; in the fetus, it is contiguous space of Retzius is common. Calcification is easier to
with the allantois. The urachus lies in the space of Retzius, diagnose by using CT than with plain radiography.
between the transversalis fascia anteriorly and the Calcifications often are dotted, stippled, or curvilinear.
peritoneum posteriorly. In 70% of adults, a small lumen
lined by transitional epithelium remains in the urachus. MRI
The persistence of a urachal lumen can lead to a urachal The MRI appearance of urachal carcinoma depends on
fistula, urachal cyst, or urachal sinus. In some patients, the composition of the tumor. Many tumors have
this epithelium can undergo metaplasia to become extremely high signal intensity on T2 weighted images
glandular epithelium. The result may be malignancy, because of their mucoid composition.
usually mucinous adenocarcinoma. Most (90%) urachal
carcinomas are juxtavesical, specifically, supravesical, INTERVENTION
anterior to the bladder, or in its midline. Treatment for localized bladder dome urachal cancer
includes surgery to remove the cancer and the bladder,
CLINICAL FEATURES in whole or in part. This removal may be accompanied
Sex: Approximately 75% of urachal carcinoma cases by en bloc resection of the umbilicus and bilateral pelvic
occur in men. lymphadenectomy. Many advocate the use of radiation
therapy because the most common area of recurrence
Age: Patients usually are aged 40–70 years.
is at or near the bladder dome. The use of chemotherapy
Patients can have mucous micturition, although this
in treating urachal cancer remains experimental.
occurs in only 25% of patients. More commonly,
hematuria is present in as many as 75% of patients. MORTALITY/MORBIDITY
Uncommonly, blood, pus, or mucus can drain from the Mortality is based on the stage at presentation. The
umbilicus. Nonspecific abdominal pain is often a prognosis with urachal carcinoma is poor compared with
symptom. that of other types of bladder carcinomas. Early detection
is important in preventing death because urachal cancers
that are found early have a good prognosis. However,
Plain Radiography in 50% of patients, the disease is fatal. The 5 years survival
rate of less than 16%. The poor prognosis is a result of
Plain radiographic findings usually are normal, although
the late presentation of symptoms, a tendency for early
a few urachal carcinomas can be calcified enough to be
local invasion, and pulmonary and osseous metastasis.
depicted on plain radiographs.
BLADDER OUTLET OBSTRUCTION
CT
It is the best modality to depict urachal carcinoma, Congenital Causes
although MRI can be helpful in select patients. MRI is 1. PUV
helpful for the evaluation of the fluid or mucous content 2. Bladder neck hypertrophy (Marion’s disease)
of the urachal segment and for multiplanar depiction of 3. Caecourterocele
the carcinoma. 4. Meatal stenosis
FIGURES 15.10A AND B: Cystocele with back pressure changes
Traumatic Causes 2. Ca Prostate

Urethral trauma/rupture 3. Urethral malignancy
Infective causes
Miscellaneous
1. Severe UTI
2. Urethral stricture Cystocele
3. Severe urethritis Urethral calculus
4. Urethral abscess For example, Cystocele (Figures 15.10A and B).
5. Chronic balanoposthitis
NEUROGENIC BLADDER
6. Pimosis
Can be autonomic, automatic bladder due to parasym-
Neoplastic Causes pathetic and sympathetic dysfunction (Figure 15.1).
1. BHP
CLASSIFICATION 12. Leiomyoma vaginal tumors

• Benign prostatic hyperplasia 13. Miscellaneous.
• Carcinoma of prostate
• Prostatic utricle cyst BENIGN PROSTATIC HYPERPLASIA (BPH)
• Prostatic abscess Benign prostatic hypertrophy (BPH) is the commonest
• Prostatic calcification cause of bladder outlet obstruction in older men. As the
prostate enlarges, it gradually compresses the urethra.
CAUSES OF BLADDER OUTFLOW OBSTRUCTION Since much of the prostate lies below the bladder base,
1. Benign prostatic enlargement Prostatic cancer there may be considerable hypertrophy and urethral
2. Other prostatic lesions obstruction without much of an impression upon the
3. BPH inferior aspect of the bladder shadow. Conversely, the
4. Wegener’s granulomatosis, lymphomatoid granulo- presence of a classic bladder-base defect from median
matosis, malignant lymphoma, hemangioperi- lobe hypertrophy does not guarantee outlet obstruction.
cytoma, rhabdomyosarcoma For so common a problem as BPH, it is remarkable
5. Malignant schwannoma, fibrous mesothelioma how much confusion and controversy surround the
6. Anatomical versus functional postsurgical, traumatic indications for imaging examinations and the significance
7. Large pelvic tumors of findings.
8. Bladder neck obstruction: Acquired bladder neck
PATHOPHYSIOLOGY
stricture, Bladder-sphincter dyssynergia, hydro-
colpos The cause of BPH is unknown but is believed to be caused
9. Hydrometrocolpos Hematocolpometra by changes in hormonal status (declining levels of
10. Cervix, low uterine segment androgens).
11. Foe example Leiomyoma, fibroma, rhabdomyo-
CLINICAL FEATURES
sarcoma, carcinoma Calculus, Brunn’s cyst,
epidermolysis bullosa, gelatinous plug, trigonal It is present in 50% of men between 40 and 60 years
polyp, prolapsing ectopic ureterocele of age and 95% of men over 70 years of age.
Diseases of Prostate 213
Clinical significant symptoms occur in 5–10% of men over

60 years of age.
Overall the gland is enlarged, often reaching massive
size. Small nodules are present throughout the gland.
Some larger nodules show cystic changes. Infarction of
a nodule is common and may be associated with acute
swelling that may precipitate acute pain and urinary
retention. When infarction of a periurethral nodule occurs,
the patient may develop hematuria.

Bladder size is readily evaluated with cross-sectional FIGURE 16.1: USG: Benign prostatic
imaging on the urogram. Massively distended bladders enlargement with median lobe hypertrophy
containing 1500 ml or more are not rare in men with
overflow micturition and decompensated bladders. tract events are reversible. As an example, consider the
Abdominal radiographs may show an oval soft-tissue patient who develops acute urinary retention. US at this
shadow in the pelvis displacing gas-filled intestinal loops
stage always shows upper tract dilatation. Catheter
upwards. US can immediately confirm the presence of
drainage of the bladder reverses the hydrouretero-
massive bladder distension.
If the patient empties his bladder as documented by nephrosis within minutes in most patients. Those few
imaging, we learn only that the bladder has not yet whose dilatation persists despite bladder decompression
decompensated. Indeed, there may be serious but have partial obstruction of the intramural ureters
compensated outlet obstruction at the expense of very secondary to fibrosis or severe muscle hypertrophy.
high micturating pressure. Lower tract urodynamic With time, even these unpromising-looking ureters
evaluation is important to provide objective data about return towards normal, although some permanent
the presence or absence of obstruction. The symptoms functional deficit persists. The same principles apply to
of an unstable bladder may overlap those of BPH and chronic bladder outlet obstruction from other causes and
the two conditions often coexist. in other age groups. Men with tight urethral strictures and
The radiographic counterpart of elevated micturating boys with posterior urethral valves are examples.
pressure is bladder wall thickening. Unfortunately there BPH is one of the common cause of BOO in old age
are no reliable criteria for judging when a bladder wall
(Figure 16.1).
is too thick. The apparent thickness (distance between
Complications include development of bladder
intravesical contrast and extravesical fat) varies with
bladder filling. Even experienced uroradiologists and diverticula, urinary infection, hydronephrosis and chronic
urologists have trouble here. renal failure. Treatment of prostatic hyperplasia is surgical.
With detrusor hypertrophy, small outpouchings called
DIFFERENTIATING POINTS FROM
cellules develop between adjacent muscular trabeculae. CARCINOMA OF THE PROSTATE
Later these may progress to full-fledged diverticula.
Cellules and diverticula are reliable indicators of bladder Prostate cancer can cause urinary obstruction in several
wall hypertrophy and certainly suggest bladder outflow ways. Most commonly urethral narrowing comes from
obstruction of some type, either anatomicalor growth of intraglandular tumor. Symptoms resemble those
neuropathic. The size of the intravesical prostate on US of BPH. Tumor shrinkage after orchidectomy, hormonal
or CT may help to decide on transurethral versus open therapy, or chemotherapy can reverse urethral
resection of the prostate. obstruction. In the absence of a beneficial response to
Large residual bladder volumes may lead to therapy, transurethral resection of malignant tissue is
hydroureteronephrosis, although fortunately these upper required to establish at least temporary urethral patency.
Prostatic cancer, spreading locally around the bladder squamous cell carcinoma, basal cell carcinoma, adenoid
base, can block one or both ureters, and metastases cystic carcinoma (basaloid), signet-ring cell carcinoma,
to iliac or periaortic lymph nodes may also displace and neuroendocrine cancer.
and/or obstruct the ureters. The latter is particularly Prostate cancer is frequently multifocal within the
common at the common iliac artery crossing, but ureteric prostate. In the prostate, 70% of cancers occur in the
obstruction may develop above, even at the renal pelvis. peripheral zone (Peripheral zone), and approximately
Like urethral obstruction, ureteric obstruction may resolve 20% are found in the transition zone (Transitional zone).
after successful hormonal therapy or chemotherapy. Some have claimed that Transitional zone prostate cancers
Temporary percutaneous nephrostomy drainage may are relatively nonaggressive, whereas Peripheral zone
preserve renal function until the tumor regresses and cancers are more aggressive and tend to invade the
ureteric patency is re-established. periprostatic tissues.
Contemporary biopsy strategies concentrate on the
Peripheral zone and largely ignored cancer in the
PROSTATE CANCER
Transitional zone. A recent study of 148 patients with
INTRODUCTION Transitional zone cancers based on radical prostatectomy
It is an important, growing health problem, presenting specimens revealed the details of Transitional zone tumors.
a challenge to urologists, radiologists, and oncologists. of the Transitional zone tumors, 80% were organ confined.
Prostate cancer is the most common nondermatologic A secondary tumor was found in the Peripheral zone in
cancer, yet despite this frequent occurrence, the clinical 52% of cases. About 15% of the cases had capsular
course is often unpredictable. Many men are found to penetration, 2.7% had seminal vesicle invasion, and 3.4%
had lymph node metastases.
have had incidental microscopic foci of prostate cancer
at postmortem examination, and most prostate cancers
HISTOPATHOLOGIC DIAGNOSIS OF PROSTATE CANCER
are slow growing and do not manifest during the man’s
lifetime. Thus, many men die with prostate cancer rather Disturbances of architecture, invasion, and anaplasia are
than die from prostate cancer; however, some cancers the important histopathologic criteria for the diagnosis
are aggressive, with a rapidly worsening course. of prostate cancer. Dysplastic lesions of the prostate occur
Currently, many men are identified as having early and are characterized by loss of cellular polarity, increase
prostate cancer through the use of prostate-specific in nuclear size with hyperchromicity, and pleomorphism.
antigen (PSA) screening, but few indicators currently Such dysplastic lesions are termed prostatic intraepithelial
distinguish progressive prostate tumors from the indolent neoplasia (PIN). PIN is a histologic appearance that many
cancers. pathologists consider to be a preinvasive stage of some
The treatment of prostate cancer is controversial, and prostate cancers. In its most severe form, PIN is regarded
different options range from early aggressive treatments, as carcinoma in situ. PIN may be classified as low grade
such as radical prostatectomy and radical radiotherapy, or high grade.
to deferred treatments, i.e. treating men when and if the
disease progresses and becomes symptomatic. GRADING
Various grading systems have been proposed; the
PATHOPHYSIOLOGY Gleason system is one of the most widely used
Approximately 95% of prostate cancers are adenocar- internationally. It recognizes a primary and a secondary
cinomas developing in the acini of prostatic ducts. Other pattern and in each, 5 subpatterns. The sum of the
rare histopathologic types of prostate carcinoma occur 2 patterns contributes the Gleason score. The scores are
in approximately 5% of patients; these include small cell of prognostic significance. Patients with a Gleason score
carcinoma, mucinous carcinoma, endometrioid cancer of 4 or below do well, and patients with a score of
(prostatic ductal carcinoma), transitional cell cancer, 8–9 do poorly.
STAGING T3b - Tumor invading the seminal vesicles

The prostate does not have a true capsule, but it does T4 - Tumor fixed or invading adjacent structures other
have an outer fibromuscular band called the capsule. than seminal vesicles, bladder neck, external
Tumor spread outside the prostate may occur by means sphincter, rectum, levator muscles, and/or pelvic
of capsular penetration, invasion of the seminal vesicles, wall
or local extension along the neurovascular bundles. The
usual sites for metastases from prostate cancer are the Regional Lymph Nodes (N)
lymph nodes, bones, and lungs. Skeletal metastases are NX - Regional lymph nodes not assessable
common in advanced prostate cancer, but hepatic N0 - No regional lymph node metastasis
metastases are uncommon. N1 - Regional lymph node metastasis
The spread of prostate cancer to the lymph nodes
involves the obturator and from there to the common Distant Metastasis (M)
iliac and para-aortic lymph nodes. Pelvic lymph nodes MX - Distant metastases not assessable
are involved initially; the inguinal nodes are not involved. M0 - No distant metastasis
Metastatic spread to bone is common in patients with M1 - Distant metastasis
advanced prostate cancer; this typically occurs as M1a - Nonregional lymph node metastasis
osteoblastic sclerotic metastases. Lytic metastases are seen M1b - Bone metastasis
occasionally. M1c - Metastasis at other sites.
The TNM (tumor, node, metastasis) staging system
INCIDENCE
revised in 1997 is considered the international standard
for prostate cancer staging and categorizes the prostate Prostate cancer is the most common nondermatologic
as follows: cancer and the second most common cause of cancer-
related deaths in the men.
Primary Tumor (T)
CLINICAL PRESENTATION
TX - Primary tumor not assessable
T0 - No evidence of primary tumor Patients with prostate cancer may be asymptomatic.
T1 - Clinically inapparent tumor, not palpable or Cancer is detected when abnormal digital rectal
visible by imaging examination (DRE) results or elevated PSA levels are
T1a - Incidental histologic finding in 5% or less of tumor further investigated. Alternatively, cancer may be detected
resected (tissue obtained during transurethral in tissue obtained during transurethral resection to treat
resection for symptoms of outflow tract a urinary outflow tract obstruction. Patients may present
obstruction) with symptoms of advanced disease with weight loss,
T1b - Incidental histologic finding of in more than 5% lethargy, bladder outflow obstruction, or bone pain. (Men
of tissue resected (tissue obtained during with newly diagnosed prostate cancer and PSA levels of
transurethral resection for symptoms of outflow less than 20 ng/mL are unlikely to have skeletal metastases
tract obstruction) resulting from prostate cancer.)
T1c - Tumor identified by needle biopsy (performed Age: The incidence of prostate cancer increases markedly
because of elevated PSA levels) with age. In fact, the incidence is exponentially related
T2 - Tumor confined within the prostate to age.
T2a - Tumor involving 1 lobe Prostate cancer is rare in men younger than 50 years.
T2b - Tumor involving both lobes One half of all cases occur in men older than 75 years.
T3 - Tumor extending through the prostatic capsule The maximum mortality rate occurs in those aged 85
T3a - Extracapsular extension (unilateral or bilateral) years.
ANATOMICAL BASIS prostate biopsy, transurethral resection of the prostate,

McNeal first proposed the histologic division of the and urethral catheterization.
prostate into an outer Peripheral zone, a central zone Serum PSA levels are lowered with finasteride
treatment for benign prostatic hyperplasia, and this
(Central zone), and an inner Transitional zone. In the
treatment must be considered when PSA levels are
young adult prostate, approximately 5% of prostatic
assessed and before the decision to perform prostatic
glandular tissue is in the Transitional zone located on both biopsy is made.
sides of the prostatic urethra. This is the area where benign
hyperplasia develops in older patients. RISK FACTORS FOR PROSTATE CANCER
The Transitional zone is separated from the Peripheral 1. High fat consumption is a possible risk factor. Diets
zone and Central zone by the surgical capsule, in which low in animal fat and protein decrease the risk.
calcified corpora amylacea may be found. The Central 2. A family history of prostate or breast cancer is a risk
zone is relatively resistant to disease processes and factor.
constitutes approximately 25% of the glandular tissue of 3. Farming or exposure to radiation and cadmium are
the prostate in the young adult. The Central zone is risk factors.
situated at the base of the prostate, and the ejaculatory 4. Race: Blacks are at increased risk for prostate cancer.
ducts reach the verumontanum by passing through 5. Neither alcohol consumption nor cigarette smoking
Central zone tissue. The Peripheral zone constitutes 70% is associated with a risk of prostate cancer.
6. Substances that may offer some protection include
of the prostate and lies on the posterior and lateral aspects
vitamin E, selenium, and lycopene from tomato-based
of the gland surrounding the Transitional zone. Its ducts
foods. High soya consumption may be protective
drain into the urethra distal to the verumontanum. because of the ingestion of plant phytoestrogens.
Of all prostate cancers, 70% occur in the Peripheral zone,
and approximately 20% occur in the Transitional zone. PREFERRED INVESTIGATION
Rhabdomyosarcomas of the prostate and pelvis TNM Staging Algorithm
represent a childhood malignant tumor. These appear 1. For the staging of prostate cancer, MRI is preferred
as soft-tissue masses infiltrating the bladder and prostate. to CT because it permits more accurate tumor staging.
As such, they have a presentation and radiologic features 2. Both techniques can be used in Nodal staging, and
completely different from those of prostatic adeno- they have equivalent accuracy.
carcinomas. 3. Bone scintigraphy is used in staging of Metastases.
PSA SCREENING CXR and Skeletal Survey

PSA screening is currently the single best test for prostate A chest radiograph may be considered in the evaluation
cancer, but it does not help in determining whether the of a patient with known prostate cancer to assess chest
detected cancer will cause clinically significant disease. symptoms, weight loss, localized bone pain, or
constitutional symptoms.
Some men with prostate cancer may have normal PSA
Skeletal radiographs may show sclerotic metastases
levels. PSA is an excellent marker for the follow-up of
or lytic lesions with bone destruction.
patients with established prostate cancer.
PSA is produced by both abnormal and normal Transabdominal Sonography
prostate tissue. A moderate elevation of the PSA level See Figures 16.2 to 16.4.
(4 – 10 ng/mL) has a low specificity for prostate cancer.
Some men with prostate cancer have PSA levels in the TRUS
reference range. An elevated PSA level is not specific for TRUS plays a central role in the contemporary diagnosis
prostate cancer, and elevated serum PSA levels may also of prostate cancer because it enables accurate image-
be associated with prostatitis, prostate infarction, PIN, guided biopsy of the gland. Patients are usually referred
for TRUS because of an abnormality is found during DRE

or because the serum PSA level is elevated.
Features: Using TRUS, the prostate is shown to be
divided into an outer gland (Peripheral zone and Central
zone) and an inner gland (Transitional zone). Calcification
in the corpora amylacea in the surgical capsule between
the outer and inner parts of the prostate is common.
Particular attention should be paid to the
Peripheral zone in prostate cancer diagnosis
because about 70-80% of prostatic malignancies
are located peripherally.
The most frequently noted abnormality due to prostate
cancer is a hypoechoic area in the Peripheral zone. Rarely,
FIGURE 16.2: # demonstrates large lobulated
prostate with heterogeneous echotexture cancer may appear as a hyperechoic area.
Both prostate cancer and prostatitis may have
increased vascularity, as shown on color and power
Doppler sonograms. This focal alteration in the prostatic
vasculature is most commonly found in hypoechoic areas
in the Peripheral zone, as depicted on gray-scale images.
No cancer-specific flow pattern has been identified, and
some cancers that are demonstrated clearly on gray-scale
imaging show no focal hypervascularity.
Lymphoma of the prostate tends to present in younger
men, and large hypoechoic masses in both the Transitional
zone and Peripheral zone have been reported.
Prostate cancers frequently demonstrate isoechoic
FIGURE 16.3: Bladder base invasion by the prostatic carcinoma findings. This observation is the basis for the systematic
biopsy approach in which multiple cores are taken from
both lobes in a standardized manner. Color and power
Doppler results have been disappointing, and they have
not been significantly helpful in detecting cancers that
are isoechoic on gray-scale examination (Figure 16.5).
Advantages:
1. Detailed sonographic appearances of the rarer
histologic variants of prostate cancer can sometimes
be described by TRUS. For example, in comedocarci-
noma, the most malignant form of prostate cancer,
stippled multiple small hyperechoic foci within the
hypoechoic area of the cancer can be found. In
FIGURE 16.4: Invasion of bladder base lead to involvement of patients with adenoid cystic carcinoma of the prostate,
both UVJ and backpressure changes more on left side multiple small cysts in the prostate may be seen.
Color or power Doppler may also be used to

identify areas for biopsy under TRUS guidance. In the
future, interventional MRI equipment may be used to
guide needle biopsy of the prostate.
CECT
Features: Arterial-phase multisection CT scanning can
help in differentiating the prostate Peripheral zone and
Transitional zone, but it cannot demonstrate intraprostatic
pathology in the prostate. CT has a limited role in
assessing prostatic cancer, but it may be helpful in
detecting nodal involvement.
FIGURE 16.5: TRUS: Arrows point to the prostatic mass
For the evaluation of lymph node metastases, CT and
MRI depict lymph node enlargement, and both have
similar accuracy. CT can be used to search for lymph
2. TRUS may be used for local staging of prostate cancer
node metastases and to stage the primary tumor by
because it can demonstrate bulges of the prostate
depicting extracapsular spread in patients in whom
capsular outline or overt extracapsular extension.
advanced disease is suspected, particularly when
However, TRUS findings have been found to be
radiation therapy is being planned.
inaccurate in the staging of localized prostate cancer.
CT cannot depict T1 or T2 tumors accurately. T3
However, Peripheral zone tumors with contact with
tumors may show invasion of periprostatic fat or seminal
the fibromuscular rim surrounding the prostate of
vesicles. Evidence-based guidelines for the use of CT in
longer than 2.3 cm on TRUS images may be prostate cancer staging have been produced. CT also may
associated with extracapsular invasion. be used to depict soft-tissue metastases elsewhere in the
3. TRUS is widely available, it has a relatively low cost, body (Figures 16.6A and B).
and it provides the opportunity for accurate biopsy
of the gland. Currently, TRUS offers the best Limitations: Current CT techniques cannot demonstrate
opportunity to demonstrate prostate cancer. intraprostatic pathology, and signal intensity changes in
4. TRUS provides the opportunity for accurate and
comprehensive biopsy of the prostate gland while
providing an imaging examination.
Limitations
1. Although, currently TRUS offers the best opportunity
to demonstrate prostate cancer, it does have major
limitations in compeletely demonstrating prostate
cancers because many prostatic tumors are both
isoechoic and multifocal.
2. Furthermore, TRUS has low specificity because many
pathologic conditions may demonstrate similar appea-
rances as hypoechoic areas in the Peripheral zone of
the prostate. For this reason, diagnostic assessment
of cancer in the prostate must be made by means
of the histologic interpretation of biopsy samples. FIGURE 16.6A: Axial CT: Localized prostatic carcinoma
be complicated by postbiopsy hemorrhage, and MRI

should not be performed until at least 3 weeks after biopsy.
The current role of MRI is the assessment of
local extracapsular extension and invasion of the
seminal vesicle. The optimal MRI technique for the
staging of prostate cancer has not been established. Signs
of extracapsular spread include the following: irregular
bulging of the prostatic outline, breech of the capsule
with extracapsular spread, asymmetry of the neuro-
vascular bundles, and loss of the rectoprostatic angle.
Contiguous areas of low signal intensity extending into
the seminal vesicles from the base of the prostate are
evidence of invasion of the seminal vesicle. On T2-
FIGURE 16.6B: Axial CT: Prostatic carcinoma weighted images, reduced signal intensity in the seminal
with bladder base invasion
vesicles may be seen after radiation therapy or prostatic
biopsy.
the Peripheral zone on MRIs have a low specificity, as
Endorectal MRI is more accurate than body-
with TRUS.
coil MRI in the local staging of the primary tumor.
Nodal staging relies on assessment of lymph node size,
Dynamic endorectal MRI with gadolinium enhancement
and neither CT nor MRI can demonstrate cancer within
may provide optimal visualization of cancer in the
lymph nodes that are not enlarged. For the evaluation
prostate. Magnetic resonance spectroscopy performed by
of lymph node metastases, CT and MRI have similar
accuracy. CT cannot depict T1 or T2 tumors accurately. using citrate and choline can provide specific information
regarding prostatic metabolism. These data may be
MRI useful in assessing the biological potential of the primary
tumor and the extracapsular extension of the tumor
The most accurate imaging technique for staging prostate
cancer appears to be endorectal MRI, but even this may (Figures 16.7 to 16.9).
cause significant understaging in approximately 30% of
prostate cancers.
Features: MRI can demonstrate the internal anatomy
of the prostate, and it can identify areas of altered signal
intensity, which representing focal pathology in the gland.
MRI provides the most complete evaluation of
patients with prostate cancer because it can be
used to assess primary disease in the prostate and
involvement of the local lymph nodes.
On T1-weighted images, the prostate appears
homogeneous with medium signal intensity. Neither the
zonal anatomy nor intraprostatic pathology is displayed.
These are depicted on T2 weighted images, on which
the cancer appears as an area of low signal intensity in
the hyperintense Peripheral zone. The specificity of this
appearance is low.
As with TRUS, MRI cannot accurately depict cancer
in the Transitional zone. Cancer assessment with MRI may FIGURE 16.7: Normal prostate on MRI with ER coil
Interventional MRI: Although, MRI is used primarily

for staging, but the availability of interventional MRI units
means that MRI is likely to have a future role in the precise
diagnosis of prostate cancer.
RNI
Radionuclide bone scanning after the injection of a
99m
Tc tracer is the standard method for assessing
potential bone metastases from prostate cancer. With
diffuse bone metastases, a so-called superscan may be
seen. This superscan demonstrates high uptake
throughout the skeleton, with poor or absent renal
excretion of the tracer.
FIGURE 16.8: Normal prostate T1 MRI
Immunoscintigraphy
The use of immunoscintigraphy to assess prostate
cancer is under investigation. With this method,
radiotracer-labeled antibodies to acid phosphatase
and PSA are used. Initial studies used iodine-131–
labeled antiprostatic acid phosphatase antibody, and
subsequent studies have used indium-111–labeled
antibody. The use of labeled anti–carcinoembryonic
antigen antibodies is being investigated. The most
commonly used monoclonal antibody (mAb) is
ProstaScint. This is 111In-labeled mAb 7E11-C5.3
(CYT-356, which recognizes an intracellular epitope of
PSMA. This technique is approved for the imaging of
soft-tissue metastases from prostate cancer but not bone
metastases. The sensitivity and specificity of this method
for lymph node metastases is 62 and 72%, respectively.
The sensitivity and specificity for prostatic fossa recurrence
FIGURE 16.9: Normal prostate T2 MRI is 49 and 71%, respectively.
MR lymphography: A new technique to detect Positron Emission Tomography

clinically occult lymph node metastases using “MR Positron emission tomography (PET with 18-fluoro-2-
lymphography” with a highly lymphotropic MR contrast deoxyglucose (FDG) may have a role in the detection
agent is being used since 2003, in which intravenous of lymph node metastases from prostate cancer,
lymphotropic paramagnetic nanoparticles of iron oxide particularly in patients with relapsed disease after primary
are administered, and patients are examined by MR 24 treatment. Localized disease within the prostate and pelvic
hours after contrast administration. Small lymph node lymph nodes can be difficult to image because of the
metastases are identified with higher sensitivity than with proximity of activity of the bladder. The sensitivity of
conventional MR scanning; this potentially valuable test FDG PET for detection of recurrence after radical
needs further evaluation. prostatectomy currently is less than 50%. C11-acetate
and C11-choline have shown promise as alternatives to transitional zone. Little attention has been paid to assessing
FDG in prostate cancer, but are less readily available than hypoechoic areas in the Transitional zone because of the
FDG. FDG PET has a repor ted sensitivity of lower frequency of cancer in the Transitional zone and
approximately 50% for the detection of skeletal prostatic the perceived lower potential for metastatic spread of
metastases. In general, FDG PET has an excellent primarily Transitional zone cancer. No specific studies in
detection rate for lytic skeletal metastases, but it has a the literature report the biopsy results in focal Transitional
poor detection rate for sclerotic metastases. Disease zone hypoechoic areas or in areas of specific focal
localizing within the prostate and pelvic lymph nodes can alterations of Transitional zone vascularity, as identified
be difficult to image because of the proximity of activity by using color or power Doppler imaging.
of the bladder. The sensitivity of FDG PET for detecting
disease recurrence after radical prostatectomy is currently FUTURE PERSPECTIVES OF
IMAGING IN PROSTATIC CANCER
less than 50%. Carbon-11 acetate and carbon-11 choline
imaging have shown promise as alternatives to FDG 1. Currently, research studies are underway to investigate
imaging in prostate cancer, but these are less readily whether sonographic contrast agents have a role in
available than FDG techniques. the identification of cancer in the prostate and whether
by demonstrating tumor vascularity they have a role
INTERVENTIONS in establishing prognosis of a patient with biopsy-
TRUS-guided Biopsy detected prostate cancer. The use of ultrasonographic
contrast agents increases the time and cost of
The original systematic approach for biopsy included the
sonography-guided prostate biopsy procedures. No
acquisition of 6 cores, 3 cores from each of the lobes
marked improvement has been found in the accuracy
of the prostate at the base, mid gland, and apex in a
of prostate cancer diagnosis with contrast agents.
parasagittal plane. Current practice is to obtain an
These agents remain experimental, and they have not
increased number of cores, i.e. lateral Peripheral zone
been adopted into standard uroradiologic practice.
cores, mid gland cores, or Transitional Zone cores, in
The impact of sonographic contrast agents on
addition to the standard 6 cores. A 10-core biopsy
radiologic practice could be considerable if future
incorporating the traditional 6 parasagittal samples and research proves that they enable the quantitative
2 lateral samples from the right and left prostatic lobes, preoperative assessment of microvascular density or
is now a standard technique for systematic biopsy. that they provide prognostic information in an
Systematic biopsy may be supplemented with cores individual patient.
obtained through hypoechoic Peripheral zone lesions.
Focal areas of hypervascularity in the Peripheral zone
of the isoechoic prostate, as shown on color Doppler
examination, may also be targeted.
Opinion differs regarding whether Transitional zone
cores should be routinely obtained during an initial biopsy
procedure or whether the samples may be obtained
during repeat biopsy in a patient with an elevated PSA
level after the initial systematic biopsy results are negative
for malignancy.
Most Transitional zone cancers are found by analyzing
systematic biopsy cores specifically obtained from the FIGURE 16.10: Prostatic utricle cyst
FIGURE 16.11: Prostatic abscess
2. Research studies are also being conducted to assess FIGURE 16.12: Prostatic calcification
the value of elastography in the diagnosis of prostate
cancer; however, the role of this technique is still PROSTATIC ABSCESS
unclear. See Figure 16.11.
PROSTATIC UTRICLE CYST PROSTATIC CALCIFICATION

See Figures 16.10. See Figure 16.12.
Urorenal Interventions 223
VARIOUS INTERVENTIONAL URORADIOLOGY 3. Obstruction causing renal failure

TECHNIQUES Benign
• FNAC and biopsy of renal masses a. Stones
• Percutaneous nephrostomy b. Sloughed papillae
• Percutaneous nephrolithotomy c. Iatrogenic—operative damage to the ureter pro-
• Percutaneous endopyelotomy ducing edema/stricture
• Percutaneous resection of urothelial tumors of renal d. Retroperitoneal fibrosis
pelvis Malignant
• Renal cyst puncture a. Retroperitoneal tumor
• Ureteric stone manipulation b. Pelvic tumor.
• Percutaneous balloon dilatation of ureteric strictures
Elective Percutaneous Nephrostomy
• Therapeutic ureteric occlusion
• Prostatic dilation and stenting 1. Obstruction with minor symptoms before treatment
• TRUS guided biopsy of prostate 2. Diversion of urine to heal urinary fistula
• ESWL. 3. Pressure flow studies for equivocal upper tract
obstruction
4. Percutaneous access for manipulation within kidney
PERCUTANEOUS NEPHROSTOMY
or ureter. Careful clinical supervision is required to
INDICATIONS
ensure that there is adequate fluid replacement.
• Emergency PCN Thus, broadly PCN is commonly indicated for one
• Elective PCN. of following reasons:
• Relief of urinary obstruction
Emergency Percutaneous Nephrostomy • Urinary diversion
1. Obstruction and infected urine • Access for therapeutic interventions
2. Obstruction and severe pain • Access for diagnostic interventions
The first indication is the most common and occurs aid to the ultrasound-guided puncture.
when bilateral obstruction has occurred or in the If intravenous contrast medium opacifies the
individual with only one functioning kidney. It is especially pelvicaliceal system, then it is simple and quick to use
important when the patient demonstrates hydronephrosis a 19 gauge sheathed needle for the caliceal puncture,
in the setting of obstruction and fevers, leukocytosis or so that a 0.35 guide wire can be advanced into the renal
evidence of frank sepsis. The most common indication pelvis. With a poorly functioning kidney an initial puncture
for percutaneous nephrostomy is relief of urinary with a fine needle allows opacification of the system so
obstruction. This may be secondary to either acute or that a calyx may be selected for the fluoroscopically
chronic obstruction. Common causes include uretero- guided, sheathed-needle puncture. This double-puncture
lithiasis, extrinsic compression from abdominal or pelvic technique is usually used if the first two or three passes
neoplasms, or direct invasion from malignancies. with the sheathed needle fail.
The second indication, diversion of urine, may be
necessary when there has been trauma to the urinary Ultrasound Guided
system. An ultrasonically guided puncture requires a certain
The third indication, access for diagnostic inventions, degree of expertise, but is the best method of guidance
includes biopsy, antegrade pyelograms and the Whitaker for puncturing a posterior calyx. When obstruction is
test. severe, ultrasound is required to localize the dilated
Access for therapeutic interventions includes stricture collecting system. It can be used before cleaning and
dilatation, stent placement, foreign body removal and draping the patient to enable surface markings to be made
stone therapy. and to give some idea of the angle and depth of the
Relief of urinary obstruction is necessary to preserve collecting system. With a small system and a difficult
renal function. Most often, only one kidney is affected, puncture, ultrasound can be used to direct the needle
but bilateral disease does occur. In an effort to maximally during the procedure, using a sterile sheath over the
preserve renal function, treating the least affected side ultrasound probe. Some operators like to use a guide
first is recommended. attachment on the ultrasound probe to direct the needle,
while others prefer to puncture ‘freehand’ with the needle
TECHNIQUE
in one hand and the ultrasound probe in the other.
Percutaneous nephrostomy can usually be performed
under local anesthesia with the patient in the prone
position. Anxious patients requiring bilateral SUMMARY
nephrostomy may require intravenous sedation, and During the procedure, the patient is placed in the prone
young children always require general anesthesia. The position. Conscious sedation is recommended for this
puncture may be guided by fluoroscopy alone, having procedure. Ultrasound is used to confirm dilation of the
used ultrasound beforehand to establish the site and depth
collecting system and to guide placement of the needle
of the collecting system before puncture, or preferably
into a posterior calyx. Posterior calyces are desired
by real-time ultrasound.
since accessing them is associated with less
Two techniques:
chance of vascular injury than does accessing an
• Single-puncture technique
• Double-puncture technique. anterior calyx or infundibulum. Once the percutaneous
needle is in place, a small amount of contrast is injected
Fluoroscopy Guided to confirm appropriate access to the kidney. Oblique
The general rule that the simpler a technique is, the more images are then obtained to demonstrate that the needle
effective it will be, applies to percutaneous nephrostomy. does not pass through the colon. Guidewires and dilators
If renal function is adequate, intravenous contrast medium precede placement of percutaneous nephrostomy drains.
given before the puncture will give additional fluoroscopic The tube is then secured by coiling its tip within the renal
pelvis and suturing the drain to the patient’s flank.
Miscellaneous Genitourinary Conditions 225
CONDITIONS steroids in RPF remains controversial; however, some

• Retroperitoneal fibrosis authors believe that steroids can be used as an adjuvant
• Prune belly syndrome to surgical ureterolysis. More recently, immunosuppressive
• Tuberous sclerosis drugs, such as azathioprine, cyclophosphamide, and
• VHL. tamoxifen, have been used to treat RPF.
PATHOPHYSIOLOGY
RETROPERITONEAL FIBROSIS (RPF)/ Grossly, RPF appears as an exuberant mass of white,
ORMOND’S DISEASE woody, fibrous tissue covering the retroperitoneal
INTRODUCTION structures such as the aorta, vena cava, ureters, and psoas
The French urologist Albarran first described retroperi- muscle. It may extend from the renal pedicle to below
the pelvic brim. The center of the plaque is usually located
toneal fibrosis (RPF) in 1905, but with Ormond’s publi-
at the level of the fourth or fifth lumbar vertebra, overlying
cation in 1948, the disease became an established clinical
the aortic bifurcation. Not uncommonly, the fibrous tissue
entity.
bifurcates and follows the common iliac arteries. Rarely,
In most patients (approximately 68%), no etiologic the fibrous process extends into the root of the mesentery
factor is found. Therefore, the term idiopathic RFP is used. or passes through the crura of the diaphragm to continue
Recent evidence suggests that RPF is an autoimmune as fibrous mediastinitis.
response to an insoluble lipid called ceroid that has leaked Histologically, the predominant finding is a fibrous
through a thinned arterial wall from atheromatous tissue consisting of collagen fibrils and fibroblasts. A
plaques. Other implicated causes include drugs, subacute nonspecific inflammatory reaction is often
abdominal aortic aneurysm, ureteric renal injury, present, or completely hyalinized fibrosis may be the only
infection, retroperitoneal malignancy, postradiation finding. The cellular infiltrate includes polymorphonuclear
therapy, and chemotherapy. No genetic predominance cells, lymphocytes, eosinophils, or plasma cells. In the
is seen in malignant retroperitoneal fibrosis. chronic phase, the only finding may be an acellular fibrosis.
RPF can be diagnosed on the basis of the history and The coexistence of RPF with primary biliary cirrhosis,
the radiologic observations. At times, the diagnosis is not fibrosing mediastinitis, panhypopituitarism, glome-
established firmly until surgical exploration. The use of rulonephritis, rheumatoid arthritis, systemic lupus
erythematosus (SLE), polyarteritis nodosa, ankylosing PREFERRED INVESTIGATIONS

spondylitis, and Riedel or Hashimoto thyroiditis support Plain Radiography and IVU and RGP
the hypothesis of an immune-mediated mechanism. The On plain abdominal radiographs, the psoas shadows may
association of RPF with the prolonged use of certain drugs not be visible. The renal outline may be enlarged if
and with the improvement of clinical symptoms (in most hydronephrosis has occurred. Features related to the
instances after cessation of the drug therapy) indicates complications of RPF may be visualized; these include
that the fibrotic process is the end stage of vasculitis bowel dilatation due to obstruction and splenomegaly
resulting from a hypersensitivity reaction to various secondary to portal hypertension. Findings related to
antigens such as drugs and diseases. Drugs implicated associated bone conditions may be seen. Such findings
in causing RPF include methysergide, beta-adrenergic include metastasis, ankylosing spondylitis, and
blockers, lysergic acid diethylamide, methyldopa, tuberculosis of spine.
amphetamines, phenacetin, pergolide, and cocaine. On chest radiographs, signs of noncardiogenic
INCIDENCE
pulmonary edema may be seen secondary to fluid
overload. Pulmonary fibrosis can occur if associated with
RPF is relatively uncommon, with an incidence of 1 case
SLE and ankylosing spondylitis. Mediastinal widening
per 200,000 population.
may occur due to a soft-tissue mass associated with
CLINICAL FEATURES mediastinal fibrosis.
The findings in RPF are nonspecific, and most findings
Race: No racial preponderance has been recorded.
are related to the late complications of fibrosis, such as
Sex: The disease is three times more common in males bowel obstruction and pulmonary edema secondary to
than in females; however, methysergide-related RPF has renal failure. Contrast-enhanced studies, such as barium
a female-to-male ratio of 2:1. No sex predominance is follow-through and barium enema examinations, can
noted in malignant RPF. show the level of bowel obstruction. The diagnosis of
Age: The age range of patients is 7–85 years, with RPF has often been suggested on the basis of the excretory
predominance in patients aged 40–60 years. urographic findings due to extensive changes in the
Most patients present with nonspecific symptoms of urinary tract. Retrograde pyelography is used in patients
less than 12 months’ duration. Children may present with with severely impaired renal function.
hip or gluteal pain. Limitations
In the early stage, signs and symptoms originate from Findings on plain radiography and contrast-enhanced
the disease process; in the advanced stage, clinical features studies are nonspecific, demonstrating the late effects of
represent the effects of obstructive uropathy and renal fibrosis. Excretory urography helps in establishing a
failure. The most common presentation is pain (92%) diagnosis in the early stage of the disease.
occurring in the flank (42%), the back (32%), the scrotum Characteristically, medial deviation of the ureter is
(8%), or the lower abdomen (28%). Other presentations present, usually at the middle third, beginning at the level
include fever, weight loss (38%), nausea and vomiting of the third or fourth lumbar vertebra. The medial
(32%), malaise (18%), polyuria (18%), polydipsia (18%), deviation of the ureter is not a constant finding in patients
anorexia (15%), nocturia (13%), oliguria (10%), urinary with RPF, and approximately 20% of patients with normal
frequency (8%), and hematuria (2%). urographic findings have medial deviation of ureters
Laboratory findings include anemia, azotemia, and without demonstrable evidence of pathologic change in
an increased erythrocyte sedimentation rate (ESR). the urinary tract. Most retroperitoneal neoplasms displace
Elevated white cell counts and pyuria can occur. The the ureters in a lateral direction, but medial deviation
diagnosis of RPF often is delayed because of its can occur. Other causes for medial displacement of the
nonspecific presentation. ureters include aneurysm, metastatic tumors, and bladder
diverticulum. Medial displacement may be seen after Ultrasonography

abdominoperineal resection and removal of pelvic Ultrasonography can be used as a noninvasive technique.
malignancy. Sonograms may or may not help in identifying the
Retrograde pyelography is an invasive technique with retroperitoneal mass, but they can readily demonstrate
no additional value compared with excretory urography. the degree of obstruction to the ureters and kidneys.
Attempts have been made to differentiate benign RPF
Excretory Urography
from malignant RPF by using color Doppler imaging.
Characteristically, medial deviation of the ureters occurs, On sonograms, RPF may appear as a relatively echo-
usually at the middle third, beginning at the level of third free mass centered on the sacral promontory. Imaging
and fourth lumbar vertebrae. Varying degrees of ureteric the retroperitoneum may not be possible, particularly in
obstruction with resultant hydronephrosis, hydroureter, obese patients or in those with excessive bowel gas.
and tapering of the ureter at the level of L4-L5 vertebrae
may be evident. Findings: On sonograms, RPF may appear as a well-
defined, smooth-marginated, hypoechoic, retroperitoneal
Retrograde Pyeloureterography soft-tissue mass encasing the aorta and inferior vena cava.
Distal extension beyond the sacral promontory and the
Small ureteral catheters can be passed easily beyond the
absence of lobulation suggest a benign etiology.
obstruction, despite the appearance of complete occlusion
Ultrasonography may demonstrate dilatation of the
on excretory urograms, because of restricted peristalsis
rather than intraluminal constriction. pelvocalyceal system and the ureters. Sonograms may
show features of coexisting primary biliary cirrhosis, bile
Aortography, Venography, and Lymphangiography duct dilatation due to common bile duct stricture, portal
hypertension due to portal vein compression, focal or
Aortography, venography, and lymphangiography help
diffuse pancreatic mass due to sclerosing pancreatitis, and
in assessing the level and extent of occlusion; however,
dilated bowel loops due to obstruction.
findings from these examinations can be normal in
Some have attempted to use Doppler ultrasonography
advanced disease.
to differentiate benign RPF from malignant RPF. However,
Findings: Angiography and venography help in recent findings suggest that color Doppler ultrasonography
assessing the level and degree of obstruction. Abdominal has no role in differentiating benign masses from
aortic aneurysm can be demonstrated on angiograms. malignant retroperitoneal masses.
Lymphangiographic findings in RPF include a delay Ultrasonography is not a sensitive examination
in the passage of contrast material through the aortic and compared with CT or MRI. Color Doppler imaging is not
para-aortic lymphatics. Lymphatic flow may be obstructed helpful in differentiating benign masses from malignant
at the L3-L4 vertebral level, resulting in nonvisualization masses because they have no distinguishing features.
of the lymphatics above the fourth lumbar vertebra, filling
of collateral lymphatic channels, and small irregular filling CT
defects in the mesenteric and para-aortic lymph nodes.
CT is the imaging modality used to diagnose RPF and
The absence of lymph node metastasis helps in excluding
to perform follow-up studies. CT scans help in assessing
malignancy.
the extent of disease and in studying the effects on other
Lymphangiography can be a complementary study
organs. Differentiating benign RPF from malignant RPF
to excretory urography. The retroperitoneal lymphatics
may not be possible.
are delicate structures; therefore, obstruction of the
lymphatics occurs before compression of the ureters or Findings: On CT scans, RPF may appear as a rind of
major vessels. Angiography often causes underestimation soft tissue around the aorta and inferior vena cava
of the size of the aorta; therefore, aneurysms cannot be extending between the renal hilum and sacral promon-
excluded. tory.
Laterally, RPF spreads to involve the ureters, causing RNI

varying degrees of obstruction. Findings: In the early inflammatory stage of the disease,
The fat plane between the mass and the psoas muscle gallium-67 scintigraphy may show increased uptake. Little
may be obliterated. or no uptake may occur in the later stages of the disease.
The mass tends not to displace the aorta anteriorly. Therefore, gallium imaging may be helpful in predicting
The attenuation value of the mass is similar to that the therapeutic response to steroids and to monitor the
of muscle and shows variable degrees of enhancement response to treatment. The absence of gallium uptake
depending on the stage of the disease. in the mature stage of the disease makes the lesion less
Certain CT features can help in differentiating benign likely to be of malignant origin. Both benign inflammation
masses from malignant masses. The mass in RPF may and malignancy can show increased uptake.
be bulky but not as massive as neoplastic lesions; however, On FDG-PET scanning, benign RPF masses exhibit
some exceptions exist. The presence of enlarged low uptake, whereas malignant masses exhibit increased
mesenteric nodes and displacement of the aorta from uptake.
the spine by the periaortic mass favors malignancy, The role of nuclear medicine studies is yet to be
although some displacement can occur in RPF. Unlike defined. Such studies may be useful in assessing the
RPF, most retroperitoneal neoplasms displace the ureters activity of the disease and in monitoring the response
laterally. to treatment. The use of FDG-PET scanning is still in
RPF does not produce local bone destruction. the preliminary stages.
The CT value and contrast enhancement have no role
in distinguishing benign masses from malignant masses. DIFFERENTIAL DIAGNOSIS
RPF must be differentiated from other conditions that
MRI can cause ureteric obstruction and renal failure, such as
Findings: RPF appears as a mass with low signal intensity retroperitoneal abscess, infections, inflammation, pelvic
on T1 weighted images and variable signal on T2 surgery, and radiation therapy. Unusual causes that can
weighted images, depending on the activity of the disease. cause ureteric obstruction include barium granuloma after
On T2-weighted images, RPF may appear hyperintense colon perforation, use of sclerosing agents for treatment
when active inflammation is present and hypointense in of inguinal hernia and hemorrhoids, and methyl
end-stage fibrosis. In homogeneous signal intensity on methacrylate cement used for joint replacement.
T2-weighted MRIs suggests malignancy. Imaging appearances similar to those of RPF can be
demonstrated by abdominal aortic aneurysm; tumors
Isotope Renography inducing desmoplastic response, such as lymphomas,
Isotope renography is useful in the serial assessment of sarcomas, and pancreatic carcinomas; and metastatic
renal function. Gallium scintigraphy may demonstrate malignancies from the breast, lung, stomach, colon,
increased uptake, depending on the activity of inflam- kidney, bladder, prostate, and cervix. Other conditions
mation. The use of 2-[fluorine 18]-fluoro-2-deoxy-D- that may simulate RPF include periaortic hematoma and
glucose positron emission tomography (FDG-PET) in amyloidosis.
differentiating benign from malignant RPF is promising.
With CT and MRI, the fibrosis can be shown in more INTERVENTION
detail. The symmetric distribution and geometric shape Biopsy can be performed under CT guidance to differen-
are highly suggestive of RPF. CT scans may not be helpful tiate benign masses from malignant retroperitoneal
in differentiating benign RPF from malignant RPF. MRI masses. Drainage of the upper urinary tract can be
may help in differentiating the two, but the diagnosis performed as a temporary measure. Percutaneous
cannot be certain by using MRI. nephrostomy and the insertion of a double-J stent helps
restore renal function and allows time to improve fluid, abdominal wall, leaving the upper abdominal musculature
electrolyte, and acid-base balance prior to surgery. with a normal appearance. The muscular defect may be
The use of steroids in RPF remains controversial; partial or asymmetric.
however, some authors believe that steroids can be used The overlying skin has a wrinkled appearance like
as an adjuvant to surgical ureterolysis. More recently, a prune. Older children may instead demonstrate a
immunosuppressive drugs, such as azathioprine, cyclo- “potbelly” appearance.
phosphamide, and tamoxifen, have been used to treat
RPF. ROLE OF RADIOLOGY AND IMAGING
In drug-related RPF, cessation of the drug therapy can The kidneys are asymmetrically affected: one tends to
result in restitution of the urinary tract and disappearance be normal while the other is dysplastic. The ureters are
of the symptoms. tortuous and dilated resulting from a deficiency in smooth
muscle. Vesicoureteral reflux is common and
MORTALITY/MORBIDITY hydronephrosis is often present. The bladder is typically
Although fatalities do occur, a satisfactory outcome can very large and may be associated with a patent urachus.
be expected if renal impairment is not too severe. The prostatic urethra is dilated secondary to prostatic
Unlike idiopathic (nonmalignant) RPF, a distinctly poor hypoplasia, and it tapers rapidly at the membranous
prognosis is associated with malignant RPF, which occurs urethra, giving rise to an appearance resembling that of
in response to metastatic tumor cells in the retro- posterior urethral valves. The anterior urethra is normal.
peritoneum. An association with megalourethra has been described.
Most patients live only 3–6 months after receiving a The cryptorchid testes usually lie in an abdominal
diagnosis of malignant RPF, although prolonged survival location. Extraurinary abnormalities include intestinal
has been reported. Close follow-up care is critically malrotation, microcephaly, imperforate anus, congenital
important to ensure the absence of progressive or heart defects, and musculoskeletal deformities such as
recurrent disease. polydactyly, hip dislocation, and scoliosis.
An incomplete manifestation of the syndrome exists,
PRUNE BELLY SYNDROME (EAGLE BARDETT consisting of urinary tract abnormalities, but normal
SYNDROME) abdominal musculature.
INTRODUCTION
TUBEROUS SCLEROSIS
A classical triad consisting of absent abdominal
INTRODUCTION
musculature, undescended testicles, and urinary tract
abnormalities. The full syndrome only develops in males. It is a neuroectodermal syndrome characterized by
formation of hamartomatous tumors in the skin, brain
PATHOPHYSIOLOGY
and viscera, including most commonly lungs and kidneys.
Precise cause is disputed: Theories:
INCIDENCE
1. The muscular defect is secondary to a distended
urinary system that prevents abdominal muscle Incidence of tuberous sclerosis has been reported be as
development rare as 1 in 150,000 and as common as 1 in 10,000.
2. prostatic dysgenesis and transient neonatal ascites It has significant morbidity and mortality with 75 percent
3. mesodermal deficiency leads to urinary tract abnor- of patients dying by the age of 20 - either from CNS,
malities and abdominal wall defect. cardia, or renal complications.
CLINICAL FEATURES CLINICAL FEATURES

Syndrome is detected at birth due to abdominal mus- The mean age of presentation of renal lesions is about
culature defect, which typically affects the lower 9 years.
The most common renal lesion is the angiomyolipoma hemorrhage. Therapy for very large AMLs can include
(AML) which is present in about 80 percent of cases. The prophylactic arterial embolization or tumors.
second most common renal lesion is renal cysts.
VHL
Ultrasound, CT and MRI can all be used for evaluation INTRODUCTION
of renal tuberous sclerosis. CT is the most sensitive and It is a neurocutaneous syndrome characterized by
specific imaging modality for evaluating renal tuberous cerebellar hemangioblastoma, retinal phakomas/
sclerosis. The finding of multiple, less than 3 cm, often angiomas, renal and pancreatic cysts, and bilateral RCC
bilateral cysts with fat-containing tumors is highly in few cases.
suggestive of tuberous sclerosis. AMLs of tuberous
sclerosis are not prone to malignant degeneration and ROLE OF IMAGING
so no yearly follow-up is needed. However, AMLs of CT is the most sensitive and specific technique for
tuberous sclerosis can grow very large and are prone to evaluating renal component of VHL.
Recent Advances in Uroradiology 231
RECENT ADVANCES INDICATIONS
• CT urography (CTU) CT urography is most vital for the evaluation of patients

• MR urography (MRU) with hematuria. The main causes of hematuria are:
• TRUS and guided biopsies • Urinary tract calculi
• Renal tumors, urothelial tumors and
CT UROGRAPHY (CTU) • Urinary tract infections.
CT urography is the best single diagnostic
INTRODUCTION
examination for diagnosing all of these pathologies, with
IVU is associated with a substantial radiation dose. the exception of infection, which is effectively diagnosed
Ultrasound is good for imaging the kidney parenchyma in most cases by microbiological analysis of the urine.
and for detecting hydronephrosis, does not require the CT urography offers ease of performance and a high
administration of iodinated contrast, and avoids radiation degree of detection of renal stones, anomalies, and (less
exposure. However, ultrasound is not good for detecting commonly) renal masses in the kidneys of potential
urinary tract calculi, and does not adequately image the donors before consideration of renal transplant surgery.
renal collecting system or ureters. For these reasons,
multidetector CT imaging has become the gold TECHNIQUE OF CTU
standard for the diagnosis of urinary tract calculi, CT urography requires the use of contrast agent to opacify
the investigation of hematuria, and the charac- the collecting system, the ureters, and the bladder. In
terization of renal masses and has largely replaced addition to optimal opacification, distension appears to
both plain film excretory urography and ultrasound be an important requirement for thorough evaluation of
examinations for these purposes. the renal collecting system and ureter. For this reason,
Dedicated CT protocols have been developed for intravenous saline is given at the same time as the contrast
these new high speed machines for different clinical material to aid in the detection of subtle filling defects
indications including “stone protocol” for the evaluation and the discrimination between urothelial neoplasms
of urinary tract calculi, CT urography for the evaluation and other filling defects. Image reconstruction tech-
of patients with hematuria and “renal mass protocol” for niques are used to create images of the entire length of
the characterization of known renal masses. the urinary system from the kidneys to the bladder.
Multiplanar 3 D reconstruction can provide the anatomic MAGNETIC RESONANCE UROGRAPHY (MRU)
detail required to correlate the finding with retrograde INTRODUCTION
ureterography or to perform an endoscopic evaluation.
Interest is rapidly growing in the use of magnetic reso-
nance (MR) imaging to assess genitourinary diseases,
ADVANTAGES
particularly with the advent of faster imaging times
The advantages of multidetector CT urography over allowing breathhold image acquisitions and contrast
conventional plain film excretory urography (also known material-enhanced studies with dynamic measurements.
as IV pyelography and IV urography) and ultrasound for Intravenous excretory urography (IVU) has been
the evaluation of the urinary tract are numerous. regarded as the imaging modality of choice for the
1. CT has been shown to detect parenchymal masses detection of urinary tract disorders in patients with acute
in the kidney with a sensitivity of 94 percent, compared flank pain. Functional as well as anatomical details are
to 67 percent for plain film excretory urography and provided by this imaging technique. The use of ionizing
79 percent for ultrasound. radiations and contrast material are considered to be the
2. Another potential advantage of CT is that recons- major drawbacks of IVU.
tructed images can show tumors in a filled bladder Recently, the use of magnetic resonance urography
opacified with contrast agent (“virtual cytoscopy”). using half-Fourier acquisition single shot turbo spin-echo
However, conventional cytoscopy remains the gold (HASTE) and rapid acquisition with relaxation
standard for the detection of tumors of the bladder, enhancement (RARE) sequences has been described in
as it will detect early color-changing mucosal lesions patients with urinary tract disease. High resolution images
that do not deform the contour of the bladder wall. call be achieved with breath-hold and rapid acquisition
In addition, cytoscopy has the added capability of sequences. The HASTE and RARE can demonstrate acute
biopsy of suspicious lesions. urinary obstruction and show perirenal high intensity
3. Multidetector CT scanning is fast, taking around 15 signal, however, information about renal function is not
seconds for image acquisition from the kidneys to the achieved. Small calculi are difficult to detect and non-
bladder during a single breath-hold. dilated urinary tract is not fully visualized. In this aspect
4. The images have good spatial resolution, little Gadolinium enhanced three-dimensional fast low angle
misregistration due to respiratory movement, and the shot (3D FLASH) sequence provides an alternative rapid
imaging technique for urinary tract evaluation.
acquisition of multiple thin slices allows excellent two-
Gadolinium enhanced 3D FLASH MRU is a highly
and three-dimensional reconstructions of the abdo-
sensitive and specific imaging modality in investigating
minal anatomy, making it possible to detect pathologies
patients with acute flank pain and could replace
outside the urinary tract as well those within.
Conventional IVU when the later is contraindicated or
DISADVANTAGES undesirable. MRI also offers the possibility to screen the
1. Although, Iodinated contrast agents are not usually abdominal cavity and retroperitoneal space to rule out
required for the detection of renal stones, but are other pathological conditions. The combined use of both
routinely used in CT urography. HASTE and 3D FL4SH sequences will ensure better
2. In comparison to CT, Conventional IVU offers confidence in the evaluation of acute suspected renal colic.
excellent delineation of calyceal and papillary
anatomy, the ureters and bladder, but it is inferior TECHNIQUE
to multi-detector CT for imaging of the kidney MR urography has been perfor med with both
parenchyma. T1 weighted and heavily T2 weighted pulse sequences
3. Both CT and plain film excretory urography are (e.g. rapid acquisition with relaxation enhancement, fast
associated with a substantial radiation dose. spin-echo [SE], and half-Fourier acquisition turbo SE).
Recent Advances in Uroradiology 233
Gadolinium-enhanced excretory MR urography simultaneously or closely in time, could provide a rapid

supplemented with low-dose diuretics such as furosemide determination of split glomerular filtration rate (GFR),
has been proposed as an accurate alternative to a fundamental index of renal health and viability.
conventional excretory urography for imaging the Coronal breath-hold images of the kidney (three or
morphology of the urinary tract. four sections, 8 mm thick) are to be acquired before and
Gadolinium-enhanced MR imaging to differentiate after bolus injection of contrast material to simulate
obstructive from nonobstructive hydronephrosis, nephrotomogram.
employing a renogram-like dynamic format to depict
signal intensity (SI) changes in the cortex and medulla DISADVANTAGES
in normal and hydronephrotic kidneys. 1. Only a limited spectrum and intensity of hydro-
A strategy for estimating single-kidney hemodynamic nephrosis. Obstructive hydronephrosis was diag-
functions by using MR imaging with gadopentetate nosed in only two patients, and none had acute
dimeglumine enhancement to determine the single- obstruction.
kidney filtration fraction has also been described. 2. Greater clinical experience will be required for
assessing the strengths and weaknesses of dynamic
ADVANTAGES and functional MR urography. The compatibility of
MRU provides detailed anatomic, dynamic, and the dynamic MR renograms with known renal
functional information on normal and abnormal kidneys. physiology, radionuclide renograms, contrast medium
No technique other than MR imaging has the potential renal pharmacokinetics, and laboratory reports
for accomplishing all of these goals. provides support for these preliminary findings.
Currently used MR contrast agents, such as gadopen-
tetate dimeglumine and gadoversetamide, are small COMPARISON WITH IVU, CTU, USG, AND RNI
molecules, similar to inulin, that after intravascular Nuclear medicine renography provides functional
injection are distributed initially in the vascular space and information but relatively poor spatial resolution.
then rapidly into the extracellular space. These agents US has better spatial resolution than nuclear medicine
can serve as extracellular fluid markers and are freely but provides no functional information, although new
filtered in the glomeruli. Since they are neither secreted US contrast agents could be promising for the study of
nor absorbed, they are an excellent indicator of renal renal dynamics.
function, serving as an “MR-visible inulin”. CT offers excellent spatial resolution and the potential
MR contrast agents also produce a T1 effect on to assess renal dynamics, but multiple exposures would
images, rendering the calices, ureters, and bladder highly be needed, resulting in inordinately high radiation doses.
visible on T1-weighted MR images. A previously CT also requires iodinated contrast agents, with an
unexploited value of these molecules is their effect on increased risk of systemic and renal toxicity.
the T1 of moving blood, which is linearly related to their With use of predominantly breath-hold pulse
concentration in blood . Thus, the T1 of the incoming sequences, the potential for MR to provide excellent pre-
blood in the renal artery will be different from that of and postcontrast anatomic images, tomographic coronal
the outgoing blood in the renal vein, in a relationship dynamic images with renograms, and unilateral renal FFs
that reflects the percentage of plasma filtered, the FF. Pulse as a measure of kidney function not available with any
sequences used to determine renal blood flow, performed other clinical technique.
CONTRAST MEDIA It occurs iatrogenically after the administration of

INTRODUCTION intravascular contrast. The underlying pathophysiology
Iodinated contrast media are widely used today in many is usually acute tubular necrosis. A common definition
radiological procedures. of CMN is impairment, either transient or permanent,
Nonionic low-osmolar iodinated (iopamidol, iohexol, in renal function (rise of at least 1.0 mg/dL) within two
etc.) contrast media are preferred over ionic (sodium and to five days after exposure to contrast.
meglumin salts of diatrozoate). Risk factors for development of CMN include
preexisting renal insufficiency (especially diabetic
PRECAUTIONS FOR A CONTRAST MEDIUM INJECTION nephropathy), decreased renal perfusion (congestive
• Explain to the patient about the examination and risk. heart failure, diuretics, etc.), hypertension, age over 60,
• Anxiety-free examination atmosphere. concurrent nephrotoxic medications, and repeated
• Secure that first line drugs and instruments are exposure to contrast within a 72 hour period. Intraarterial
available. contrast administration places patients at higher risk for
• The patient should be well hydrated. CMN than does intravenous administration.
• Determine whether the patient is at risk of any contrast The risk of CMN in patients with normal renal function
medium reaction. is less than 0.5%. Prospective studies have found the risk
• Patients at risk should be carefully monitored during of CMN in patients with renal impairment to be 12–27%.
the examination. CMN occurs after the use of both low-osmolality contrast
• When absorption or leakage into the circulation is media (LOCM) and high-osmolality contrast media
possible (for example after intracavitar use) take the (HOCM); however, studies show that there is generally
same precautions as for intravascular administration. a decreased risk of CMN with LOCM, especially diabetic
patients with renal insufficiency.
CIN (CONTRAST MEDIA-INDUCED NEPHROTOXICITY) N-acetyl cysteine has been found to be very helpful
The findings of contrast media-induced nephrotoxicity in treating CIN.
(CIN) include persistent bilateral, diffuse nephrograms Methods for prevention of CMN include vigorous
and occasionally vicarious excretion of contrast by the hydration, careful patient selection, and increased interval
biliary system. between contrast examinations.
Radiological Contrast Media 235
Main risk factors and prophylactic measures on use of contrast media in imaging
Adverse reaction Risk factors/conditions Measures to reduce the risk
Previous generalized • Previous adverse reaction, either moderate Use non-ionic contrast media.
contrast medium reaction (e.g.urticaria, bronchospasm, moderate hypotension) Corticosteroids may be given :
or severe (e.g. convulsions, servere bronchospam, Prednisolone 30 mg orally 2 hours or
pulmonary edema, cardiovascular collapse) – Methylprednisolone 32 mg orally 12 and 2 hours
anaphylactoid reactions. before injecting contrast medium.
• Asthma Effects of antihistamines [H1 and H2] are not
• Allergy requiring medical treatment. definitely proven.
Contrast medium • Raised s-creatinine levels, particularly secondary • Make sure that the patients is well hydrated
induced nephrotoxicity to diabetic nephropathy. [give at least 100 ml. (oral (e.g. soft drinks) or
• Dehydration intravenous (normal saline) depending on the
• Congestive heart failure clinical situation) per hour starting 4 hours before
• Concurrent administration of nephrotoxic drugs, to 24 hours after contrast administration – in warm
e.g. non-steroid anti-inflammatory drugs areas increase the fluid volume]
• Age over 70 years old. • Use non-ionic contrast media
• Stop administration of nephrotoxic drugs for
at least 24 hours
• Consider alternative imaging techniques,
which do not require the administration of iodinated
contrast media.
Lactic acidosis Patients treated with biguanide-type anti diabetics • Serum creatinine level measurement < 1 week
• Serum creatinine normal
• Stop biguanide intake, give contrast medium
• Serum creatinine abnormal
• Consider alternative imaging techniques, which do
not require the administration of iodinated contrast
media.
Postpone contrast medium examination for 48 hours.
Anxiety Nervousness, sweating, etc. Consider benzodiazepines
Iodine induced • Patient with Graves’ disease In very selected high-risk patients prophylactic therapy
hyperthyroidism • Multi-nodular goiter with thyroid autonomy, especially may be given by endocrinologists in advance.
elderly patients and patients living in areas of iodine
deficiency
Extravasation • Pressure injector with insufficient fixation of Appropriate injection site, careful injection
needle placement Prefer non-ionic contrast medium in combination with
• Fragile or damaged veins pressure injector
Risk for late reaction • Previous contrast medium reaction Patient should be informed and observed
(1 hour to 7 days) • Interleukin-2 treatment
Hypotension Dehydration Hydrate adequately
First line treatment of contrast medium adverse reactions

Subsequent steps if needed should be taken by care of resuscitation team.
Acute adverse reactions ( < 1 hour ).
Nausea/Vomiting Moderate, transient : Supportive treatment
Severe, protracted : Appropriate antiemetic drugs (e.g. odansetron, dimenhydriant )
Urticaria Scattered, transient : Supportive treatment including observation
Scattered, protracted : Appropriate H1 antihistamine (e.g.clemastine) intramuscularly or intravenously should be
considered. Drowsiness and/or hypotension may occur.
Profound : Consider Adrenaline 1:1000, 0.1-0.3 ml. (0.1–0.3 mg.) intramuscularly in adults, 0.01 mg/kg
intramuscularly up to 0.3 max. in children. Repeat as needed.
Bronchospasm 1. Oxygen by mask (6-10 l/min)
2. B-2-agonist metered dose inhaler, 2–3 deep inhalations
3. (e.g. salbutamol)
4. Adrenaline intramuscularly depending on blood pressure
Normal blood pressure
1:1,000, 0.1–0.3 ml (o.1–0.3 mg) [use smaller dose in a patient with coronary artery disease or elderly patient]
In children : 0.01 mg/kg; mad dosage : 0.3 mg
Decreased blood pressure
1:1000, 0.5 ml (0.5 mg), in children : 0.01 mg/kg max. dosage : 0.3 mg
5. For further treatment : resuscitation team.
Laryngeal edema 1. Oxygen by mask (6–10 l/min)
2. Intramuscular adrenaline (1:1000), 0.5 ml (0.5 mg) for adults, repeat as needed
3. Intubation via resuscitation team.
Hypotension Isolated hypotension
1. Elevate patient’s legs
2. Oxygen by mask (6–10 l/min)
3. Intravenous fluid : rapidly, normal saline of lactated Ringer’s solution
4. If unresponsive : adrenaline : 1:1000, 0.5 ml (0.5 mg) intramuscularly, repeat as needed
Vagal reaction (hypotension and bradycardia)
3. Atropine 0.5–1.0 mg intravenously, repeat if necessary after 3-5 min, to mg total (0.04 mg/kg) in adults
In pediatric patients give 0.02 mg/kg
Intravenously (max. 0.6 mg/dose) repeat if necessary to 2 mg total
4. Intravenous fluids : rapidly, normal saline or lactated Ringer’s solution
Hypotension and tachycardia
2. Intravenous fluids : rapidly, normal saline or lactated Ringer’s solution
Anaphylactoid 1. Call for resuscitation team
generalized reaction. 2. Suction airway as needed
3. Elevate patient’s if hypotensive
5. Intramuscular adrenaline (1:1000), 0.5 ml (0.5 mg) in adults. Repeat as needed.
In pediatric patients 0.01 mg/kg to 0.3 mg max. dose.
6. Intravenous fluids rapidly (e.g. normal saline, lactated Ringer’s)
7. H1-blocker, e.g. diphendydramine 25–50 mg intravenously
Seizures, Convulsions Diazepam 5–10 mg rectally (or intravenously)
Radiological Contrast Media 237
Late adverse reactions ( 1 hour – 7 days)

Symptomatic and similar to the management of other drug-induced skin reactions
Extravasation • Conservative management is adequate in most case
Limb elevation
Apply ice packs
Careful monitoring
• If a serious injury is suspected, seek the advice of a surgeon
Iodine induced Very late reaction (several days/weeks)
hyperthyroidism Seek the advice of an endocrinologist
Contrast medium induced Symptomatic
nephrotoxicity Close monitoring as in any other patient with reduced renal function (due to other causes)-Consult a nephrologists
Dialysis seems to have no effect on the incidence on contrast media induced nephropathy
Lactic acidosis • Monitor renal function ( serum creatinine), serum lactic acid and pH of blood
• Look for symptoms of lactic acidosis (vomiting, somnolence, nausea, epigastric pain, anorexia, hyperpnea,
lethargy, diarrhea and thirst). Blood test results indicative or lactic acidosis: pH < 7.25 and lactic acid
> 5 mmol
References 239
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Index
A Adult polycystic kidney disease 46 B

clinical features 47
Abdominal ectopy 35 incidence 47 Beckwith-Wiedemann syndrome 51
Accessory renal artery 181 pathophysiology 46 Beer-drinker’s hydronephrosis 31
Acute infections 103 preferred investigation 47 Benign urethral lesions 195
acute pyelonephritis 103 angiography 48 balanitis xerotica obliterans 195
causative organism 103 CT scanning 48 benign neoplasms 195
role of radiology and imaging 103 MRI 48 paraurethral cyst 195
emphysematous pyelonephritis 105 plain radiographs 47 urethral caruncle 195
clinical features 106 RNI 48 urethral diverticulum 195
etiological agents 106 ultrasonography 47 urethral polyps 195
incidence 105 mortality/morbidity 48 Bilateral large kidneys 161
intervention 107 Agenesis of urethra 198 causes 161
pathophysiology 106 Alport syndrome 88, 161 Bilateral small kidneys 161
predisposing factors 105 Anomalies of urorenal tract 27 causes 161
preferred investigations 106 kidney 27 Bladder 1, 33
treatment 107 anomalies of form 27 congenital bladder diverticula 33
types 106 anomalies of fusion 27 exstrophy 33
pyonephrosis 104 anomalies of number 27 Bladder diverticulum 63, 199
renal abscess 105 anomalies of position 27 Bladder duplication 69
features 105 Antegrade urography 9 Bladder exstrophy 68
Acute urinary obstruction 92 advantages 11 associated anomalies 68
anatomical basis 94 disadvantages 11 clinical features 68
clinical features 93 indications 10 incidence 68
etiology 93 Aphorism 59 management 68
incidence 93 Ascent of the kidneys 27 pathophysiology 68
intervention 100 Aspirin renography 173 role of radiology and imaging 68
pathophysiology 92 Autosomal recessive polycystic kidney Bladder outlet obstruction 210
role of radiology and imaging 94 disease 44 congenital causes 210
KUB and IVU 94 anatomical basis 45 neoplastic causes 211
urographic findings in acute obstruction classification 44 traumatic causes 211
96 clinical features 45 Bochdalek’s hernia 37
urographic findings in chronic incidence 45 Bosniak classification of renal masses 75
obstruction 96 mortality/morbidity 46
nephrographic density 96 pathophysiology 44
C
parenchymal thickness 96 preferred investigations 46
pyelogram 96 CT 46 Calyceal diverticulum 52
renal size 96 MRI 46 treatment 53
ureter 96 ultrasonography 46 types 53
Captopril renography 173 differential diagnosis 31 pathophysiology 214

Captopril scan 172 incidence 30 preferred investigation 216
Caroli disease 51 pathophysiology 30 PSA screening 216
Chronic infections 108 role of radiology and imaging 31 risk factors 216
malacoplakia 119 treatment 31 staging 215
renal tuberculosis 112 Congenital urethral diverticulum 200 prostatic abscess 222
clinical features 113 anatomical basis 200 prostatic calcification 222
incidence 113 incidence 200 prostatic utricle cyst 222
mortality/morbidity 117 role of radiology and imaging 200 Drooping lily sign 56, 61
pathophysiology 112 treatment 200 Duplicated collecting systems 54
preferred examination 113 Congenital uterovesical fistula 70 anatomical basis 56
radiological findings 113 Crossed fused renal ectopia 28, 37 clinical features 55
schistosomiasis (bilharziasis) 117 clinical features 38 incidence 55
clinical features 118 age 38 mortality/morbidity 58
differential diagnosis 118 anatomical basis 34 pathophysiology 55
frequency 118 etiology 38 preferred investigations 56
mortality/morbidity 119 incidence 38 antegrade pyelography 57
pathophysiology 118 pathophysiology 38 arteriography 58
role of radiology and imaging 118 preferred investigations 38 CT with contrast 57
treatment 119 MRI 39 IVU/excretory urography 56
xanthogranulomatous pyelonephritis ultrasonography 39 magnetic resonance urography 57
108 Cystic disease of kidney 71 MCU/VCUG 57
clinical features 109 classification 71 plain radiography 56
differential diagnosis 111 genetic cystic diseases 71 scintigraphy 58
incidence 109 nongenetic cystic diseases 71 ultrasound 57
intervention 111 pathophysiology 71 terms relevant to duplex collecting
pathophysiology 108 Cystitis 200 systems 56
role of radiology and imaging 109 Cystography and cystourethrography 22 bifid system 56
stages 109 ascending urethrogram 22 double ureters 56
treatment 111 combined ascending urethrogram and duplex kidney 56
Chronic urinary obstruction 101 MCU 22 duplex system 56
additional note on ureteral obstruction MCU 22 upper and lower pole ureters 56
101 upper or lower pole 56
hydronephrosis 101 D Duplication anomalies 29
differential diagnosis 101 Duplication of urethra 198
non-obstructive causes 101 Diseases of prostate 212
mortality/morbidity 101 benign prostatic hyperplasia 212
E
Cobra-head deformity 61 clinical features 212
Column of Bertin 6 differentiating points from carcinoma Ectopic orifices 31
Complete urinary collecting system of the prostate 213 Ectopic ureteral 58
duplication 53 pathophysiology 212 associated anomalies 58
clinical features 54 role of radiology and imaging 213 clinical features 58
differential diagnosis 54 prostate cancer 214 embryology 58
pathophysiology 53 anatomical basis 216 imaging 58
role of radiology and imaging 54 clinical presentation 215 incidence 58
treatment 54 grading 214 Ehlers-Danlos syndrome 51
varieties 54 histopathologic diagnosis of prostate Embryology 27
Congenital bladder diverticulum 68 cancer 214 Emphysematous cystitis 200
Congenital PUJ obstruction 30 incidence 215 clinical features 200
clinical features 31 interventions 221 role of radiology and imaging 200
Index 243
Excretory urography 11 H M
bowel preparation 14
Horseshoe kidney 28, 39 Malrotation 29
contraindications 14
anatomical basis 41 Meckel diverticulum 80
contrast administration 17 clinical features 40
bolus versus infusion 17 Medical renal disease 161
horseshoe kidney with associated
dosage 17 anomalies 40 differential diagnosis 162
demerits 21 isolated horseshoe kidney 40 role of radiology and imaging 162
exposure factors 15 complications 41 MRI 162
film sequencing 17 incidence 40 USG 162
intervention 44 Medullary sponge kidney 50
general considerations 17 pathophysiology 40
indications 13 associated syndromes 51
preferred investigations 41
informed consent 14 clinical features 50
CECT 42
limitations 21 IVU 41 incidence 50
limited urogram 20 MRI 43 intervention 52
modifications 21 plain radiographs 41 mortality/morbidity 52
RNI 43 pathophysiology 50
patient preparation 14
ultrasonography 42 preferred examination 51
dehydration 14
physiology 12 CT 52
plain film of abdomen 15 I IVU 51
postvoid film 20 MRI 52
Interventional uroradiology
psychological preparation 14 plain radiograph 51
techniques 223
tailoring 20 RNI 52
Intrathoracic kidney 35, 37
tomography (adult) 19 USG 51
Intravenous urography 11, 16
compression 19 Megapolycalicosis and primary megaureter
introduction 11
Extracorporeal shock wave lithotripsy 78 megapolycalicosis 66
Ischial spine 4
pathogenesis 66
primary megaureter 67
F K differential diagnosis 68
Fibromuscular band 5 Kidney 1 potential complications 68
Foley balloon 63 mesonephros 1 role of radiology and imaging 66
Fused pelvic kidney 28 metanephros 1 Mesodermal tumors 63
pronephros 1 Metal stenosis 198
G Milk-alkali syndrome 88
MR anatomy of prostate 5
Genetic cystic disease 76 L
MRI 26
ARPKD 77 Leukopenia 30 MR urography 26
infantile 77 Levator ani muscle 5 Müllerian ducts 2
juvenile 77 Lymphoma of kidneys 152 Multicystic dysplastic kidney 29, 49
neonatal 77
anatomical basis 153 differential diagnosis 50
perinatal 77
incidence 153 imaging findings 49
autosomal dominant polycystic kidney
intervention 156 CT/MR findings 49
disease 76
Genitourinary manifestations of diabetes mortality/morbidity 156 nuclear scintigraphy 49
166 pathophysiology 153 ultrasound 50
diabetic nephropathy 166 preferred investigation 153 incidence 49
causes 166 angiography 155 intravenous urography 50
complications 167 CT 154 prognosis 50
imaging 167
IVU 153 retrograde ureterogram 50
incidence 166
reproductive system 167 MRI 155 Multilocular cystic nephroma 144
risk factors 166 RNI 155 cystic Wilms’ tumor 145
role of investigations 166 ultrasonography 154 clinical issues 146
gross pathologic-surgical features O Prenatal (fetal) hydronephrosis 101

145 bilateral pathology 102
Oncocytoma 158 differential diagnosis 101
microscopic features 145
anatomical basis 159 unilateral pathology 101
pathology 145
clinical features 159 Primary lymphoma of bladder 208
prognosis 146
frequency 159 clinical features 209
treatment 146
intervention 160 mortality and morbidity 209
Muscular hypertrophy 63
mortality/morbidity 160 role of radiology and imaging 209
pathophysiology 158 treatment 209
N preferred investigation 159 Prune Belly syndrome 70
Nephroblastomatosis 143 angiography 160 Prune Belly syndrome (Eagle Bardett
role of radiology and imaging 143 CECT 159 syndrome) 229
treatment 143 KUB radiograph 159 clinical features 229
Nephrocalcinosis 87 limitations of techniques 160 pathophysiology 229
causes of medullary nephrocalcinosis 88 MRI 160 role of radiology and imaging 229
clinical features 89 PET scan 160
incidence 89 RNI 160
R
pathophysiology 87 USG 159
causes of cortical nephrocalcinosis Radiological contrast media 234
88 P Radiology of renal transplant 182
anatomical basis 183
disorders of calcium metabolism 87 Page kidney 126 complications 184
preferred investigations 89 Pelvic kidney 35 historical aspect 182
computed tomography 90 Penis and urethra 33 intervention 191
MRI 90 duplication of urethra 34 pathophysiology 182
RNI 91 epispadias 34 preferred investigations 185
ultrasonography 90 hypospadias 34 limitations 185
Neurogenic bladder 211 phimosis and paraphimosis 34 USG and color Doppler 186
Nongenetic cystic kidney disease 72 posterior urethral valves 34 acute kidney transplant rejection 187
acquired cystic renal disease 76 urethral meatal stenosis 34 angiographic study 190
calyceal diverticulum 75 urethral stricture 34 CT scanning and CT-guided
treatment 76 Percutaneous nephrostomy 223 interventions 188
types 75 indications 223 features 186
cortical renal cysts 72 elective percutaneous nephrostomy MRI 189
Bosniak classification 73 223 nuclear medicine scanning 190
clinical features 72 emergency percutaneous peritransplant fluid collections 186
complications 73 nephrostomy 223 Radiolucent urolithiasis 86
incidence 72 technique 224 Ranal arteriovenous malformation 180
pathology 72 fluoroscopy guided 224 clinical features 180
role of radiology and imaging 72 ultrasound guided 224 etiology 180
dysplasia/cystic dysplasia/multicystic Peripheral zone 5 role of radiology and imaging 180
dysplastic kidney 75 Periurethral abscess 198 treatment 180
medullary sponge kidney 75 Plain KUBU radiograph 7 Recent advances in uroradiology 231
Normal anatomy 3 mimics of renal colic 7 CT urography (CTU) 231
anatomy of 3 Posterior urethral valve 192 advantages 232
kidney, ureter and bladder 3 classification 192 disadvantages 232
prostate 3 clinical features 192 indications 231
retroperitoneum 3 pathophysiology 192 technique 231
Normal variants (pseudotumors) 6 role of radiology and imaging 193 magnetic resonance urography 232
Nubbin sign 56 Potter’s syndrome 27 advantages 233
Index 245
disadvantages 233 pathophysiology 163 intravascular ultrasonography-

technique 232 preferred examination 164 guided atherectomy 176
Renal agenesis 34 CT 165 medical therapy 175
associated anomalies 35, 36 MRI 165 percutaneous transluminal
bilateral renal agenesis 35 plain radiographs 164 angioplasty 176
clinical features 36 retrograde pyeloureterography 165 surgical revascularization 176
incidence 35 ultrasound 165 vascular stent placement 176
pathophysiology 35 Renal scintigraphy 26 mortality/morbidity 177
role of radiology and imaging 35, 36 arteriography 26 pathophysiology 168
angiography 37 dynamic scan 26 arterial dysplasia 170
CT 37 renal cortical scintigraphy 26 atherosclerosis 170
ultrasound 36 static scan 26 congenital stenosis 171
urography 36 Renal trauma 120 development of RVHT 168
Renal angiomyolipoma 156 clinical features 121 evolution of RVHT 170
clinical features 156 incidence 121 incidence 171
findings 157 management of renal injury 129 neurofibromatosis 171
follow-up 158 mortality/morbidity 129 transplant RAS 171
incidence 156 pathophysiology 120 role of radiology and imaging 172
mortality/morbidity 158 causes 120 color Doppler 173
pathophysiology 156 features 120 conventional angiography 174
preferred investigations 157 grading 120 CT angiography (CTA) 174
treatment 158 preferred investigations 121 hypertensive urography 172
Renal artery aneurysm 181 angiography 128 MRA 175
Renal cell carcinoma 146 intravenous urography 121 RAS 173
anatomical basis 147 MRI 128 RNI 172
clinical details 148 radiography 121 Retrocaval utreter 33
differential diagnosis 152 retrograde pyelography 122 Retrograde urography 8
incidence 147 RNI 128 indications 9
intervention 152 ultrasonography 122 Retroperitoneal fibrosis /Ormond’s disease
mortality/morbidity 152 VCUG 122 225
pathophysiology 146 radiologic intervention 129 clinical features 226
preferred investigations 149 Renal vein thrombosis 177 differential diagnosis 228
angiography 152 etiology 177 incidence 226
CECT 150 primary cause 177 intervention 228
intravenous urography 149 secondary causes 177 mortality/morbidity 229
MRI 151 incidence 177 pathophysiology 225
plain radiography 149 mortality and morbidity 180 preferred investigations 226
RNI 151 pathophysiology 177 aortography 227
USG 149 acute 177 CT 27
risk factor 146 chronic 178 excretory urography 227
staging 146 role of radiology and imaging 178 isotope renography 228
Renal dysplasias 29 computed tomography 178 lymphangiography 227
Renal ectopia 28 radionuclide renography 179 MRI 228
Renal hypoplasia 29 ultrasonography 178 plain radiography and IVU and RGP
Renal infarct 181 treatment 179 226
Renal papillary necrosis 163 thrombolytic therapy 179 retrograde pyeloureterography 227
anatomical basis 163 Renovascular hypertension 168 RNI 228
clinical features 163 clinical features 171 ultrasonography 227
mortality/morbidity 166 intervention 175 venography 227
Rhabdomyosarcoma 208 clinical features 229 RNI 61

mortality and morbidity 208 incidence 229 ultrasound 60
pathophysiology 208 role of radiology and imaging 230 voiding cystourethrography 60
role of radiology and imaging 208 Urethra 1
treatment 208 U Urethral calculus 198
Role of MDCT in urologic diseases including Urethral strictures 194
the imaging protocols 23 Ultrasonography 22 etiology 194
hematuria protocol 24 advantages 22 role of radiology and imaging 194
indications 23 disadvantages 22 Urethral trauma 133
renal mass protocol 24 Unilateral small kidney 161 anatomical basis 134
stone protocol 24 causes 161 clinical details 134
nephrolithiasis 24 Urachal abnormalities 69 incidence 134
urolithiasis 24 anatomy/embryology 69 intervention 138
clinical features/complications 69 mortality/morbidity 138
complications 70 role of radiology and imaging 135
S
incidence 69 CT 137
Sacral kidney 35 role of radiology and imaging 70 MRI 137
Sickle cell disease 88 varieties of abnormalities 69 retrograde urethrography 135
Space of Retzius 210 patent urachus 69 ultrasonography 137
Stenosis 31 urachal cyst 69 Urinary bladder trauma 129
Supernumerary kidney 27 urachal diverticulum 70 anatomical basis 131
urachal sinus 69 classification 130
T Urachal carcinoma 209 clinical features 130
anatomical basis 210 mechanism 129
Transitional cell carcinoma of urinary
clinical features 210 mortality/morbidity 133
bladder 201
incidence 210 preferred investigations 131
anatomical basis 203
intervention 210 CT cystography 132
clinical features 203
mortality/morbidity 210 CT retrograde cystograms 132
incidence 201
pathophysiology 209 limitations of techniques 133
intervention 207
role of radiology and imaging 210 retrograde cystography 131
mortality/morbidity 207
pathophysiology 201 CT 210 ultrasonography 132
causative agents 201 MRI 210 Urogenital septum 1
pathology/histology 202 plain radiography 210 Urolithiasis 78
polychronotropism 201 Ureter 30 clinical features 79
preferred examination 203 Ureterocele 32, 58 differential diagnosis 80
role of radiology and imaging 203 classifications 58 incidence 79
angiography 205 clinical features 59 intervention 85
CT 205 differential diagnosis 62 mortality/morbidity 86
intravenous urography 204 incidence 60 pathophysiology 78
MRI 206 intervention 62 predisposing factors 78
plain radiograph 203 mortality/morbidity 62 role of radiology and imaging 80
retrograde pyelography 204 pathophysiology 59 conventional radiography 81
RNI 206 role of radiology and imaging 60 CT 83
ultrasonography 205 antenatal ultrasonography 60 CT protocol for calculus disease 84
staging of TCC 202 CT 61 intravenous urography 82
grading of TCC 202 intravenous urogram 61 MRI 85
metastases 202 MRI 61 RNI 85
Tuberous sclerosis 229 renal/bladder ultrasonography 61 ultrasonography 82
Index 247
special considerations 86 mortality/morbidity 66 genetics 141

types of renal calculi 78 pathophysiology 63 incidence 140
calcium stones 78 role of radiology and imaging 64 mortality and morbidity 141
cystine stones 79 angiography 66 pathophysiology 141
magnesium ammonium phosphate intravenous urography 64 role of radiology and imaging 141
(struvite) stones 79 RNI 65 computed tomography 142
matrix stones 79 ultrasonography 65 magnetic resonance imaging 143
orotic acid stones 79 VCUG 64 plain radiography 141
several other less common forms of Vesicourethral canal 1 ultrasound 142
urolithiasis 79 staging 141
Vestibule 2
uric acid stones 79 Wolffian duct 2, 55
VHL 230
xanthine stones 79
role of imaging 230
X
V
W Xanthogranulomatous pyelonephritis 80
Vesicoureteral reflux 32, 63
anatomical basis 63 Weigert-Meyer rule 54, 59
Z
clinical features 64 Wilms’ tumor 80, 51
grades 64 Wilms’ tumor/nephroblastoma 140 Zonal anatomy 5
incidence 63 associations 141 Zuckerkandl’s fascia 3

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Uroradiology

Text and Atlas

Suresh M Bakle DMRD MD (Radiology)

JAYPEE BROTHERS MEDICAL PUBLISHERS (P) LTD

Uroradiology: Text and Atlas

Bibliography ................................................................................................................................... 239

ADPKD Autosomal dominant polycystic kidney disease

1. The earliest structure, the pronephros, forms at about

FIGURE 1.2: Development of collecting system of kidneys

On either side, the retroperitoneum is divided into 1. Cortex

zonal anatomy is well delineated, with the prostatic urethra

Modality Column of Bertin Renal neoplasm

VARIOUS IMAGING MODALITIES AND Satisfactory bowel preparation is important prerequi-

PLAIN KUBU RADIOGRAPH MIMICS OF RENAL COLIC (PLAIN KUBU FILM)

INDICATIONS ANTEGRADE UROGRAPHY (AGP)

FIGURE 3.15: Colon studded with fecoliths FIGURE 3.16: Enteroliths

• Decompression of PUJ obstruction • Longer duration required

Table 3.1: Intravenous urography

Nephrogram Thick slice tomogram To improve definition of the renal outlines

Frusemide IVU Administration of 20 mg of frusemide If suspected pelviureteric junction obstruction is being

FIGURE 3.20: Normal IVU

FIGURE 3.19: Schematic diagram showing important renal structures

Table 3.2: Recommended film sequence

the possibilities of ureteral tortuosity or kinking. COMPRESSION

junction and vice versa. Coned oblique views of the

are pregnant. The effective radiation dose from this

Modern non-ionic contrast agents do not provoke an

retrogradely will fail to show valves. When catheterization 1. Widely used

ASCENDING URETHROGRAM DISADVANTAGES

ROLE OF MDCT IN UROLOGIC DISEASES

Calculus itself as a cause may be seen.

Table 3.4: Urinary tract imaging protocols

Renal Mass Protocol

STONE PROTOCOL detection of stones on follow-up plain film radiographic

MRI administration. The typical dose is 5 mCi for adults.

INTRODUCTION Agenesis: Bilateral renal agenesis (absence of both

Duplex kidney (double kidney) consists of a single renal

CLINICAL FEATURES Also, percutaneous nephrostomy followed by balloon

FIGURE 4.5: Ultrasound scan: Bifid pelvicalyceal system

FIGURE 4.6: CT scan: Coronal reconstruction—Bifid pelvicalyceal

prophylaxis, or significant and progressive renal scarring

surgery consists of division of the ureter with uretero-

6. Duplication of urethra Urethral Stricture

It is between 1:500 and 1:1500 births.

Failure of formation of the ureteral bud or because of

BILATERAL RENAL AGENESIS

This is extremely rare and is incompatible with life. Males

Ultrasonography (USG) Accuracy is high with MRI, primarily as a result of

Horseshoe kidney is probably the most common fusion

COMPLICATIONS OF HORSESHOE KIDNEY

duplex system, hydronephrosis). It can also be used to RNI

Angiography 6 (PKHD1) as the genetic locus. The frequency of the

ducts are dilated with minimal liver involvement. INCIDENCE

Generally, there is a reciprocal relationship between PATHOPHYSIOLOGY

With small cysts, ADPKD can be confused with CLINICAL FEATURES