Cystic Adenomatoid Malformation
Gross Ian
Congenital cystic adenomatoid malformation (CCAM) is believed to be the result of an early focal arrest in lung development. Cystic areas replace the normal bronchi and alveoli in the affected part of the lung. In the majority of cases, the lesion is confined to one lobe and there is a connection, which may be tortuous, to the airway. The blood supply is derived from the pulmonary circulation (and not from an aberrant artery as is the case with pulmonary sequestration). Three types have been described based on the size and number of the cysts:
Type I: Made up of a few large cysts
Type II: Numerous small or medium-sized cysts
Type III: Multiple tiny cysts
CLINICAL MANIFESTATIONS AND COMPLICATIONS
The clinical presentation in the perinatal period varies considerably. The lesion may be diagnosed antenatally by ultrasound; types I and II lesions appear as cystic echolucent masses, whereas type III lesions appear as a large solid mass that may be associated with hydrops due to mediastinal shift and obstruction of the vena cava. Occasionally, the lesion shrinks in size prior to delivery. After birth, infants with symptomatic CCAM present with respiratory distress. The severity varies considerably depending on the size of the lesion and the presence and extent of pulmonary hypoplasia.
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