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Cystic Adenomatoid Malformation (CAM)

This document discusses congenital cystic adenomatoid malformation (CCAM) of the lung, a developmental abnormality arising in the seventh week of gestation. It presents in three subtypes and can be diagnosed prenatally via ultrasound. For hydropic fetuses, fetal therapy such as shunting or lobectomy may be considered. After birth, CCAM often presents as respiratory distress and is typically treated with surgical resection due to risk of infection and malignancy.

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Aliyah Tofani Pawelloi
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70 views38 pages

Cystic Adenomatoid Malformation (CAM)

This document discusses congenital cystic adenomatoid malformation (CCAM) of the lung, a developmental abnormality arising in the seventh week of gestation. It presents in three subtypes and can be diagnosed prenatally via ultrasound. For hydropic fetuses, fetal therapy such as shunting or lobectomy may be considered. After birth, CCAM often presents as respiratory distress and is typically treated with surgical resection due to risk of infection and malignancy.

Uploaded by

Aliyah Tofani Pawelloi
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PPT, PDF, TXT or read online on Scribd
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"

Congenital Cystic
Adenomatoid
Malformation (CCAM) of
the Lung

Dr Bental NICU Laniado Hospital


Congenital cystic adenomatoid malformation
(CCAM) of the lungs is a developmental
abnormality (hamartoma) arising at ~ the seventh
week of gestation from overgrowth of the terminal
respiratory bronchioles modified by
intercommunicating cysts (Stocker et al. 1977
).Occurrence is sporadic unrelated to race,
maternal age, or familial genetic history.
In the fetus CCAM presents in association
with hydramnios, hydrops, prematurity, and
stillbirth.
Etiology/Pathophysiology

Congenital hamartomatous lesion of the


lung
Pathology
Cystic Adenomatoid Malformation
Lower lobe of lung.
Black arrow points to large cyst; yellow areas to smaller
cysts.
White arrows highlight pleural surface.
Portion of lung adjacent to pleura has normal apearance;
remainder shows enlarged spaces.
Pathology
There
are 3 subtypes, all of which lack
normal bronchial communications:

Type I - multiple large air or fluid filled


cysts, usually greater than 2.0 cm in
diameter.


AP and lateral chest radiographs show a well
defined cystic mass in the left lung. Type 1.
PA chest radiograph shows a cystic mass in the left
upper lobe
Lung windows from an unenhanced CT scan of
the chest in the same patient better demonstrate
the cystic nature of the left lung mass.
Type 1
Type II - variably sized less bulky lesion
with smaller cysts.

Type III - bulky mass composed of
multiple tiny, microscopic cysts resembling
bronchi that involves the entire lobe.
Adenomatous hyperplasia with an increase
in terminal bronchiolar structures, as well as
a polypoid arrangement of mucosal
epithelium is seen.
Autopsy gross photograph showing the
abnormally enlarged left lung containing
multiple, small cysts.
Bilateral CCAM
Differential Diagnosis
Congenital lobar emphysema
Bronchogenic cyst
Bronchopulmonary sequestration
Prior infection with pneumatocele formation
Diaphragmatic hernia

Congenital Lobar Emphysema (CLE)


Supine chest radiograph showing a large
ANTENATAL DIAGNOSIS

Type I CCAM appears as large cystic spaces


usually in the upper or middle lung .
Type II appears as multiple small cysts < 1 cm on
a slightly echogenic background.
Type III appears as an echogenic mass with no
cystic structure visible.
US
of the fetus showing a large
mass in the thorax
ANTENATAL DIAGNOSIS
Polyhydramnios- Due to esophageal
compression (Donn et al., 1981), or
increased fetal lung fluid production by the
abnormal tissue (Krous et al., 1980)
Fetal hydrops -From venocaval obstruction
and cardiac compression secondary to
mediastinal shift)
Ascites
USshowing integumentary edema
and ascites
FETAL THERAPY
Following a targeted ultrasound
amniocentesis and cardiac echo are
recommended to evaluate the fetus for
additional anomalies
Prenatal MRI (?).
FETAL THERAPY
HYDROPIC FETUS
In the hydropic fetus with an isolated lesion
prior to 32 weeks' thoracoamniotic shunting
is particularly useful for alleviating
mediastinal compression in predominantly
cystic CAM (Nicolaides K and Azar GB,
1990, Thorpe-Beeston JG 1994) .
FETAL THERAPY
In cases not amenable to drainage (multiple small
cysts or Type III) referral for fetal lobectomy
should be considered .
The hydropic fetus > 32 weeks' is better served by
ex utero lobectomy(Adzick NS, 1993).
The major complications associated with surgical
interventions are premature labor and preterm
rupture of membranes
FETAL THERAPY
NONHYDROPIC FETUS
The nonhydropic fetus with isolated CCAM
occupying < 50% of the thorax appear to do
reasonably well and may be followed with
serial exams (Dommergues M et al 1997).
Postnatal Clinical
Presentation
The most common presentation is acute
respiratory distress in the newborn in the
first few hours of life. Alternatively, it can
present at several months or several years of
age as recurrent pneumonias.
Supine chest radiograph at one hour of life shows
a solid mass in the left hemithorax causing
mediastinal shift to the right, evidenced by the
position of the endotracheal tube.
Supine chest radiograph at three hours of life in the same
patient shows a multiseptated cystic mass in the left
hemithorax causing mediastinal shift to the right.
The chest-computerized tomograph demonstrates a right
lower lobe congenital cystic adenomatoid malformation
(CCAM) in a 6-week-old infant who presented with
tachypnea. The most striking feature is the solitary
enlarged cyst surrounded by a number of microcysts. This
lesion was resected without complication
POSTNATAL MANAGEMENT

A significant number of CCAMs either


decrease in size or resolve in utero
spontaneously (Bromley B et al 1995 ) .
However, Winters et al have demonstrated
persistent abnormalities after birth in
cases of "disappearing" fetal lung masses.
POSTNATAL MANAGEMENT
CT scan in the postnatal period is therefore
recommended as the findings are often subtle on
radiograph(Winters et al.,1997).
Frequent infectious complications and malignant
change in CCAM argue for resection of these
tumors. (Granata et al ,1998, Kaslovsky RA et al
1997, d'Agostino S et al 1997, Ribet ME et al
1995)

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