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Prune Belly Syndrome Associated With Congenital Cystic Adenomatoid Malformation of the Lung

MICHEL L. WEBER, MD, FRCP; GUY RIVARD, MD; GILLES PERREAULT, MD, FRCP
Am J Dis Child. 1978;132(3):316-317. doi:10.1001/archpedi.1978.02120280100022.
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The first case of prune belly syndrome was reported in 1839. Since then, more than 150 cases have been described1-6 and an extensive review of the literature was made in 1950 by Silverman and Huang.7

This syndrome affects mostly boys and associates abdominal wall muscle deficiency, undescended testes, and urinary tract malformations. Other anomalies occur. The prognosis is poor and many patients die at an early age of urinary tract infection, renal failure, or pulmonary complications. Management includes the use of an abdominal binder and medical or surgical care of urinary and gastrointestinal problems.

To our knowledge, no case of this syndrome associated with cystic adenomatoid malformation of the lung has yet been published. This report describes this occurrence in one child.

Report of a Case.—A 7-day-old male infant was admitted to the hospital with the complaint of mild respiratory difficulties. Birth weight was 3,030 gm; length,

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