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Quiz Prize Rules Current Rankings DIAGNOSIS:
Congenital Cystic Adenomatoid MalformationThe plain radiograph of the chest reveals multiple cystic lucencies in the left lung with contralateral herniation of the left lung and shift of the mediastinum and trachea to the right side. The fundic gas bubble is noted in its place below the diaphragm.
HRCT of the lung shows multilocular, thin-walled, variable-sized (about 1-4 cm), cystic lesions in the left lung surrounded by normal lung parenchyma. No evidence of air-fluid levels is seen. There is contralateral herniation of the left lung with shift of the mediastinum to the right side.
The above features are highly suggestive of congenital cystic adenomatoid malformation.
Congenital cystic adenomatoid malformation (CCAM) is a rare pulmonary anomaly. It is a hamartomatous lesion characterized by a cessation of normal bronchiolar maturation, resulting in cystic overgrowth of terminal bronchioles and alveoli leading ultimately to large masses which communicate with the tracheo-bronchial tree and have feeding vascularization from the pulmonary (bronchial) circulation.
It usually occurs in children less than 3 years of age, in boys more than girls, and is usually unilateral.
The usual presentation is with life-threatening respiratory distress or a history of recurrent chest infections. Rarely, some patients may be asymptomatic.
The classification of CCAM with CT:
Type I - most common. Variable-sized cysts with at least one dominant cyst (>2 cm). Excellent prognosis.
Type II - smaller uniform cysts (upto 2 cm), commonly associated with anomalies (renal, cardiac, intestinal and skeletal)
Type III - least common. Microcysts appearing solid upon visual inspection. Poor prognosis is secondary to respiratory compromise and associated congenital abnormalities.
Differential diagnosis: Congenital lobar emphysema, bronchopulmonary sequestration, congenital diaphragmatic hernia, bronchogenic cyst, prior infection with pneumatocele formation.
CCAM is treated with lobectomy, which may be done on an emergency basis depending on the clinical status. Some cases have been operated on prenatally. Treatment can be postponed if the patient is asymptomatic.
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PubMed References for Congenital Cystic Adenomatoid Malformation
At any given time this link will provide references from Pubmed, including the most recent updates. Retrievals are designed to include articles available free and also Indian studies.
The following radiologists have answered this quiz correctly.
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Abhang Apte
Abhilash Jayaram
Ajitsinh Jemalbhai Vahder
Alex Daniel
Alli A
Amarnath C
Amit Kaur
Anil Dasyam
Anoop Kumar Pandey
Anubhav Khandelwal
Arvind Bhan
Ashish Gupta
Ashwin Kumar Patil
Banuprakash S
Bharat Madhavaram
bhuvana devendran
Bikramjit Singh
Chary Ds
Chaudhari N
Chethan T N
David Nucci
Davis Chiramel
Debabrata Das
Deepak B
Denis Regent
dipti khajanchi
Edmund Moses
Fiaz Parakot
Ganesh Agrawal
Gayathri sivagurunathan
Gilles Genin
Harsh Sadana
Hasmukh J Prajapati
Hiral Shah
I Venkatraman
Jaykuumar Nair
Jaywant Akolkar
Jeshil Shah
Jitendra Ashtekar
Jjm Reddy
Julie Arora
K.Shivashankar
Kabilan Chokkappan
Kajal Shah
Kiran Parmar
Kolamala Usharani
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lovkesh mittal
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poonam sherwani
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sunali desai
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sushila pai
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