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Prune Belly Syndrome

ROLAND WALBAUM, MD
Am J Dis Child. 1979;133(3):332. doi:10.1001/archpedi.1979.02130030108024.
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Sir.—In their report in the March 1978 issue of the Journal (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

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