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June 1979

'Prune Belly' Syndrome-Reply

Author Affiliations

Department of Pediatrics Dysmorphology Unit University of Washington School of Medicine Seattle, WA 98195

Am J Dis Child. 1979;133(6):658. doi:10.1001/archpedi.1979.02130060098032

In Reply.—The thoughts of Drs Kroovand and Perlmutter express the predominant opinion of the past relative to the pathogenesis of the "prune belly" syndrome. Our hypothesis, that the most common pathogenesis is early urethral obstruction, will be published in detail in the near future.1 Severe early urethral obstruction results in a host of secondary biomechanical deformations as a consequence of the back pressure. The problem in bladder and urethral musculature and its partial replacement by collagen is the result of the distensile pressure. The same is true for the variable impact of gross bladder distension on abdominal musculature. These deductions are made possible by the evaluation of embryos and fetuses who had severe urethral obstruction that was lethal in fetal life, which would seem to be the most common outcome. To survive to be born and be evaluated by the urologist, the individual with severe urethral obstruction must

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