Congenital cystic adenomatoid malformation of the lung. Presentation of 16 cases.
Congenital cystic adenomatoid malformation (CCAM) of the lung is one of the rarest causes of neonatal distress. The principal radiological sign of CCAM is an intrapulmonary mass of soft tissue density, containing cystic areas of different sizes and shapes. The mass usually compresses the rest of the affected lung and displaces the mediastinum and heart to the opposite side, compressing the lung which is often therefore hypoplastic. If CCAM is diagnosed in utero by ultrasound, the treatment of choice is surgery as soon as possible after birth, with good survival rates. Sixteen cases of CCAM are presented, one with bilateral disease, diagnosed at different times, and one with an associated prune belly syndrome, to be added to the 405 already reported in the literature, and their clinical, radiological and pathological features are described.
Beluffi
G
G
Department of Radiodiagnosis, Pediatric Clinics of the University, IRCCS Policlinico S. Matteo, Pavia, Italy.
Brokensha
C
C
Kozlowski
K
K
Lucaya
J
J
Masel
J
J
Morris
L
L
Rosso
R
R
Stronati
M
M
Thomson
R
R
D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007234:Infant, Premature; D008168:Lung / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D011013:Pneumonectomy; D011859:Radiography; D012127:Respiratory Distress Syndrome, Newborn / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000601:surgery