The biliary system consists of the organs and ducts (liver, gallbladder,

bile ducts, and associated structures) that are involved in the production
and transportation of bile.
The gallbladder is a gastrointestinal organ located within the right
hypochondrial region of the abdomen. This pear-shaped sac lies within a
fossa formed between the inferior aspects of the right and quadrate lobes
of the liver.

The primary function of the gallbladder is to concentrate and

store bile which is produced by the liver.
The transportation of bile follows this sequence:

When the liver cells secrete bile, it is collected by a system of

ducts that flow from the liver through the right and left
hepatic ducts.

These ducts ultimately drain into the common hepatic duct.

The common hepatic duct then joins with the cystic duct from
the gallbladder to form the common bile duct.

This runs from the liver to the duodenum (the first section of
the small intestine). However, not all bile runs directly into
the duodenum. About 50% of the bile produced by the liver is
first stored in the gallbladder.
Then, when food is eaten, the gallbladder contracts and
releases stored bile into the duodenum to help break down
the fats.
In humans, the 2 primary bile acids cholic acid and chenodeoxycholic
acid, are synthesized in the liver. Before excretion, they are conjugated
with glycine and taurine.
In response to a meal, contraction of the gallbladder delivers bile acids to the intestine
to assist in fat digestion and absorption. After mediating fat digestion, the bile acids
themselves are reabsorbed from the terminal ileum. They return to the liver via portal
blood, are taken up by liver cells, and are reexcreted in bile.
In an adult, this enterohepatic circulation involves 90-95% of the circulating bile acid
pool. Bile acids that escape ileal reabsorption reach the colon, where the bacterial
flora, through dehydroxylation and deconjugation, produce the secondary bile acids,
deoxycholate and lithocholate.
❑ The gallbladder is congenitally absent in approximately 0.1% of the
population.
❑ Hypoplasia or absence of the gallbladder can be associated with
extrahepatic biliary atresia or cystic fibrosis.
❑ Duplication of the gallbladder occurs rarely.
❑ Gallbladder ectopia may occur with a transverse, intrahepatic, left-
sided, or retroplaced location.
❑ Multiseptate gallbladder, characterized by the presence of multiple
septa dividing the gallbladder lumen, is another rare congenital
anomaly of the gallbladder.
Biliary dyskinesia is a motility disorder of the biliary tract that may
cause biliary pain in children, often in association with nausea and fatty
food intolerance.
 European classification (ROME) ICD-10
E. Functional gallbladder and sphincter of
Oddi disorders Functional gallbladder
disorders
E1. Biliary Pain
E1a. Functional gallbladder disorder
E1b. Functional biliary sphincter of Oddi
disorder Functional sphincter of
Oddi disorder: biliary,
E2. Functional pancreatic sphincter of Oddi pancreatic
disorder
Pain located in the epigastrium and/or right upper quadrant and all of
the following:
1. Builds up to a steady level and lasting 30 minutes or longer
2. Occurring at different intervals (not daily)
3. Severe enough to interrupt daily activities or lead to an emergency
department visit
4. Not significantly (<20%) related to bowel movements
5. Not significantly (<20%) relieved by postural change or acid
suppression
Supportive Criteria
The pain may be associated with:
1. Nausea and vomiting
2. Radiation to the back and/or right infrasubscapular region
3. Waking from sleep
1. Biliary pain
2. Absence of gallstones or other structural pathology

Supportive Criteria
1. Low ejection fraction (<40%) on gallbladder scintigraphy
2. Normal liver enzymes, conjugated bilirubin, and amylase/lipase
 Assessment of Gallbladder Emptying
❑ Cholecystokinin-stimulated cholescintigraphy (CCK-CS). The test
involves the intravenous administration of technetium 99m (Tc
99m)labeled hepatobiliary iminodiacetic acid analogs. These
compounds are readily excreted into the biliary tract, and are
concentrated in the GB.

FGBD is often diagnosed by a low gallbladder

ejection fraction at CCK-CS.
❑ Ultrasound scanning is useful in assessment of liver size and
consistency, as well as gallbladder size.
GB length normally varies from 1,5-5,5 cm (average 3 cm) in infants to
4-8 cm in adolescents; Width ranges from 0,5-2,5 cm for all ages.
❑ GB emptying can be assessed with ultrasound scanning after
cholecystokinin or fatty meal stimulation. GB stones should be
excluded by US.
❑ Endoscopic ultrasound is more sensitive for detecting small stones
and biliary sludge, and can also detect small tumors, and changes of
chronic pancreatitis.

❑ Magnetic resonance cholangiopancreatography

❑ Computed tomography scanning
❑ Esophageal manometry, gastric emptying tests, and transit studies may be required if
symptoms suggest alternative dysfunctional syndromes.
EGD – esophagogastroduodenoscopy
EUS – endoscopic ultrasonography
❑ Symptoms suggestive of FGBD often resolve spontaneously.
❑ Patients may respond to reassurance and medical treatments such as
antispasmodics, neuromodulators, or ursodeoxycholic acid .
❑ Cholecystectomy is considered when these methods fail, and symptoms
are severe.
Dysfunction of the biliary sphincter of Oddi is commonly considered in
patients with biliary-type pains after cholecystectomy, when stones and
other pathology are excluded.
1. Criteria for biliary pain
2. Elevated liver enzymes or dilated bile duct, but not both
3. Absence of bile duct stones or other structural abnormalities

Supportive Criteria
1. Normal amylase/lipase
2. Abnormal sphincter of Oddi manometry
3. Hepatobiliary scintigraphy
Aberrant sphincter physiology leads to biliary pain by increased
resistance to bile outflow and subsequent rise in intrabiliary pressure.
❑ The initial diagnostic approach should consist of a careful history and
physical examination, followed by standard liver and pancreas blood
tests, upper endoscopy, and abdominal imaging.
❑ Ultrasound or computed tomography scanning may be used initially,
MRCP or EUS provide more complete information.
❑ EUS is the best way to rule out duct stones and pathology of the
papilla.

N.B. Regular narcotic use can cause biliary dilation, although usually associated with
normal liver enzymes.
❑ The drainage dynamics of the bile duct have been tested after
stimulation with a fatty meal or injection of CCK and measuring any
dilatation of the duct with abdominal or endoscopic ultrasound.

❑ Hepatobiliary scintigraphy involves intravenous injection of a

radionucleotide and deriving time-activity curves for its excretion
throughout the hepatobiliary system. This technique has been used to
assess the rate of bile flow into the duodenum and to look for any
evidence of obstruction.
❑ Endoscopic retrograde cholangiopancreatography and sphincter of
Oddi manometry. ERCP should be reserved for patients who need
sphincter manometry or endoscopic therapy, such as those with strong
objective evidence for biliary obstruction.
❑ Manometry technique. ERCP allows measurement of both the biliary
and pancreatic sphincters, but the method is imperfect. The standard
upper limit of normal for baseline biliary sphincter pressure is 35-40
mm Hg. Normal pancreatic sphincter pressures are accepted as similar
to those of the bile duct.
1. Documented recurrent episodes of pancreatitis (typical pain with
amylase or lipase >3 times normal and/or imaging evidence of acute
pancreatitis)
2. Other etiologies of pancreatitis excluded
3. Negative endoscopic ultrasound
4. Abnormal sphincter manometry
❑ Measuring the size of the pancreatic duct by MRCP or EUS before and
after an intravenous injection of secretin.
❑Medical therapy. Nifedipine, phosphodiesterase type-5 inhibitors,
trimebutine, hyoscine butylbromide, octreotide, and nitric oxide have
been shown to reduce basal sphincter pressures in SOD. H2 antagonists,
gabexate mesilate, ulinastatin, and gastrokinetic agents also showed
inhibitory effects on sphincter motility. Amitriptyline, as a
neuromodulator, also has been used along with simple analgesics.
❑ Transcutaneous electrical nerve stimulation and acupuncture also
have been shown to reduce SO pressures, but their long-term efficacy has
not been evaluated.
❑ Endoscopic therapy: sphincterotomy.
❑ Surgical therapy. Surgical sphincteroplasty can be performed
primarily or after failed endoscopic therapy.
Acute acalculous cholecystitis is uncommon in children and is usually
caused by infection. Pathogens include streptococci (groups A and B),
Gram-negative organisms, particularly Salmonella and Leptospira
interrogans. Parasitic infestation with Ascaris or Giardia lamblia may be
found.

Calculous cholecystitis may rarely follow abdominal trauma or burn

injury or is associated with a systemic vasculitis, such as periarteritis
nodosa.
❑ Clinical features include right upper quadrant or epigastric pain,
nausea, vomiting, fever, and jaundice. Right upper quadrant
guarding and tenderness are present. Pain may radiate to an area
just below the right scapula.
❑ Ultrasonography discloses an enlarged, thick-walled gallbladder,
without calculi.
❑ Serum alkaline phosphatase activity and direct-reacting bilirubin
levels are elevated. Leukocytosis is usual.
❑ Patients may recover with treatment of systemic and biliary infection.
❑ Cholecystectomy is required in patients who fail to improve with
conservative management.
❑ Cholecystostomy drainage is an alternative approach in a critically
ill patient.
Cholelithiasis is relatively rare in otherwise healthy children, occurring
more commonly in patients with various predisposing disorders.

In children:
❑ >70% of gallstones are the pigment type,
❑ 15-20% are cholesterol stones,
❑ the remainder are composed of a mixture of cholesterol, organic
matrix, and calcium bilirubinate.

Black pigment gallstones, composed mostly of calcium bilirubinate and glycoprotein matrix, are
a frequent complication of chronic hemolytic anemias.
Brown pigment stones form mostly in infants as a result of biliary tract infection.
 Conditions Associated with Cholelithiasis:
❑ Biliary dyskinesia
❑ Chronic hemolytic disease (sickle cell anemia, spherocytosis, thalassemia, Gilbert
disease)
❑ Ileal resection or disease, сystic fibrosis, сirrhosis, сholestasis, Crohn disease,
obesity, insulin resistance
❑ Prolonged parenteral nutrition
❑ Prematurity with complicated medical or surgical course
❑ Prolonged fasting or rapid weight reduction
❑ Treatment of childhood cancer
❑ Abdominal surgery
❑ Pregnancy
❑ Sepsis
❑ Genetic (ABCB4, ABCG5/G8) progressive familial intrahepatic cholestasis
❑ Cephalosporins (prolonged use of high-dose ceftriaxone, a third-generation cephalosporin,
has been associated with the formation of calcium-ceftriaxone salt precipitates (biliary
pseudolithiasis) in the gallbladder.
Cholelithiasis in premature infants is often asymptomatic and may
resolve spontaneously.

Brown pigment stones are found in infants with obstructive jaundice and infected intra-
and extrahepatic bile ducts.
These stones are usually radiolucent, owing to a lower content of calcium phosphate
and carbonate and a higher amount of cholesterol than in black pigment stones.
 TYPES OF GALLSTONES
Types of gallstones that can form in the gallbladder include:
CHOLESTEROL GALLSTONES. The most common type of gallstone, called a
cholesterol gallstone, often appears yellow in color. These gallstones are composed
mainly of undissolved cholesterol, but may contain other components.

PIGMENT GALLSTONES. These dark brown or black stones form when your bile
contains too much bilirubin.
❑ More than 50% of patients with gallstones have symptoms, and 18% present with a
complication as the first indication of cholelithiasis, such as pancreatitis, choledocholithiasis
or acute calculous cholecystitis.
❑ The most important clinical feature of cholelithiasis is recurrent abdominal pain, which is
often colicky and localized to the right upper quadrant.
❑ Pain may radiate to an area just below the right scapula.
❑ An older child may have intolerance for fatty foods.
❑ Jaundice occurs more commonly in children than adults.
❑ A plain X-ray of the abdomen may reveal opaque calculi, but radiolucent (cholesterol)
stones are not visualized.
❑ Ultrasonography is the method of choice for gallstone detection.

❑ Hepatobiliary scintography is a valuable adjunct in that failure to visualize the gallbladder

provides evidence of cholecystitis.
❑ Cholecystectomy. Laparoscopic cholecystectomy is routinely performed in
symptomatic infants and children with cholelithiasis.
❑ Common bile duct stones are unusual in children, occurring in 2-6% of cases with
cholelithiasis, often in association with obstructive jaundice and pancreatitis.
❑ Operative cholangiography should be done at the time of surgery, however, to
detect unsuspected common duct calculi.
❑ Endoscopic retrograde cholangiography with extraction of common duct stones is
an option before laparoscopic cholecystectomy in older children and adolescents.