Good outcome in prune-belly syndrome despite associated severe anomalies |
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Authors: | Israel Zelikovic Shermine Dabbagh Aaron L Friedman David T Uehling Russell W Chesney |
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Institution: | (1) Department of Pediatrics, University of California, Davis School of Medicine, Davis, California, USA;(2) Department of Pediatrics, Division of Urology, University of Wisconsin, Center for Health Sciences, Madison, Wisconsin, USA;(3) Department of Surgery, Division of Urology, University of Wisconsin, Center for Health Sciences, Madison, Wisconsin, USA;(4) Pediatric Research Laboratory, 956 Court Avenue, Room B310, 38163 Memphis, TN, USA |
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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.Supported in part by grants from the National Institute of Health AM 37223-01 and the Medical School and Graduate School Research Committees of the University of Wisconsin and a Research Career Development Award KO4 AM 00421 (RWC), by the Pearl M. Stetler Foundation (SD) and by a National Kidney Foundation fellowship (IZ) |
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Keywords: | Prune-belly syndrome Urethral stenosis Oligohydramnios Imperforate anus |
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