Prune belly syndrome: current perspectives.

• Type of Material: Article
• Authors: Angela M Arlen, Yale University; Cayce Nawaf, Yale University; Andrew J. Kirsch, Emory University
• Language: English
• Date: 2019
• Publisher: Dove Medical Press
• Publication Version: Final Published Version
• Copyright Statement: Copyright © 2019 Arlen et al.
• License: Creative Commons Attribution-Noncommercial 3.0 Unported
• Final Published Version (URL): http://dx.doi.org/10.2147/PHMT.S188014
• Title of Journal or Parent Work: Pediatric Health, Medicine and Therapeutics
• ISSN: 1179-9927
• Volume: 10
• Start Page: 75
• End Page: 81
• Keywords: prune-belly syndrome; cryptorchidism; abdominoplasty; urinary tract dilation; abdominal wall laxity
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

Prune belly syndrome (PBS) is a rare but morbid congenital disease, classically defined by a triad of cardinal features that includes cryptorchidism, urinary tract dilation and laxity of the abdominal wall musculature. Children often require numerous surgical interventions including bilateral orchidopexy as well as individually tailored urinary tract and abdominal wall reconstruction. Along with the classic features, patients with PBS often experience gastrointestinal, orthopedic, and cardiopulmonary comorbidities.