Prune Belly Syndrome

Abstract

Sir.—In their report in the March 1978 issue of theJournal(132:316-317, 1978) of a patient with prune belly syndrome, Weber et al mentioned congenital cystic adenomatoid malformation of the lung as a previously undescribed accompaniment of this condition. In 1970, I saw a typical case of prune belly syndrome with polycystic malformation of the lower lobe of the right lung in a 2-month-old girl. The IVP was normal. Microscopic examination of the surgically removed right lower lobe disclosed multiple cavities lined by stratified cylindrical ciliated epithelium, resting on a loose collagenous chorion with scarce muscular fibers and rare dystrophic cartilaginous islets. Necrotic material, mucus, and polymorphonuclear leukocytes filled these cavities. A few atrophic alveoli were visible. This case was published (much too briefly!) in the French Pediatric Society's journal.1