Teaching Case

Newborn from healthy mother (gestational age 39 weeks 1 day) with birth weight of 2280 grams. The Apgar score 5 minutes after spontaneous delivery was 10. Physical examination showed an extremely flabby abdominal wall, with the contour of the intestines standing out through the abdominal wall.

A female neonate, born to a healthy mother (gestational age 39 weeks 1 day) with birth weight of 2280 grams is presented. The Apgar score 5 minutes after spontaneous delivery was 10. Physical examination showed an extremely flabby abdominal wall, with the contour of the intestines standing out through the abdominal wall. On ultrasonography, absence of abdominal musculature was found. The intra-abdominal organs were normal. Chest radiographs and contrastenhanced CT-scan of the thorax were performed.

Congenital cystic adenomatoid malformation (CCAM) represents an abnormal adenomatoid overgrowth of the terminal bronchioles, with subsequent failure in the maturation of the latter and suppression of alveolar growth. These pathological changes occur at approximately the 5 th or 6 th week of gestation. CCAM is slightly more common in males. It is limited to a single lobe in over 95% of cases, and bilateral in less than 2% of cases. There is no lobar predilection, although the middle lobe is less frequently affected. Clinically 85% of babies with CCAM present in the neonatal period with respiratory compromise. Manifestation may occur later in infants with progressive respiratory distress and infection. Division is made into 3 types. Type 1 lesions comprise nearly 50% of cases and consist of few, large cysts, usually one to four in number, ranging between 3 and 10 cm in diameter. Type 2, accounting for 40% of cases, consists of more numerous small to medium sized cysts, generally ranging between 0.5 and 3 cm in diameter, diffusely distributed through the affected area. Type 3, accounting for nearly 10% of cases, consist of multiple minute cysts, usually less than 2 mm in diameter and tends to involve an entire lobe or entire lung. Prognosis is best for type 1 lesions. Very rarely, CCAM is associated with abnormalities in other organ systems. In the presented case CCAM was part of a Prune-Belly syndrome, which is characterized by multiple abnormalities such as congenital absence or deficiency of abdominal wall musculature, cryptorchidism and abnormalities of the urologic tract. Radiological differential diagnosis includes diaphragmatic herniation, congenital lobar emphysema, pulmonary sequestration, bronchogenic cyst, postinflammatory cyst and pneumatocoele. Treatment of CCAM is surgical.