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Pediatric Nephrology

, Volume 2, Issue 4, pp 512–514 | Cite as

Good outcome in prune-belly syndrome despite associated severe anomalies

  • Israel Zelikovic
  • Shermine Dabbagh
  • Aaron L. Friedman
  • David T. Uehling
  • Russell W. Chesney
Brief Report
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Abstract

A boy aged 4.5 years with prune-belly syndrome (PBS) and associated urethral stenosis, oligohydramnios, imperforate anus and vesicosigmoid fustula is described. In contrast to the anticipated poor prognosis, vesicostomy and divided transverse colostomy performed after birth followed by prophylaxis of infection and bicarbonate supplementation have resulted in a good outcome. The vesicosigmoid fistula might have served in utero as a “natural diversion” protecting from pressure-induced renal damage. It is suggested that the main determinant of prognosis in PBS is the presence and degree of kidney dysplasia at birth as reflected by the neonatal renal function after performance of an indicated urinary diversion procedure rather than the presence of severe associated anomalies.

Key words

Prune-belly syndrome Urethral stenosis Oligohydramnios Imperforate anus 

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Copyright information

© IPNA 1988

Authors and Affiliations

  • Israel Zelikovic
    • 1
  • Shermine Dabbagh
    • 2
  • Aaron L. Friedman
    • 2
  • David T. Uehling
    • 3
  • Russell W. Chesney
    • 1
  1. 1.Department of PediatricsUniversity of California, Davis School of MedicineDavisUSA
  2. 2.Department of Pediatrics, Division of UrologyUniversity of Wisconsin, Center for Health SciencesMadisonUSA
  3. 3.Department of Surgery, Division of UrologyUniversity of Wisconsin, Center for Health SciencesMadisonUSA

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