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Good outcome in prune-belly syndrome despite associated severe anomalies
I Zelikovic1, S Dabbagh, A L Friedman
1Department of Pediatrics, University of California, Davis School of Medicine.
Insights
A boy with prune-belly syndrome (PBS) and severe anomalies achieved a good outcome with early intervention. Neonatal kidney function after urinary diversion, not associated anomalies, is key for PBS prognosis.
Area of Science:
- Pediatric Surgery
- Nephrology
- Medical Genetics
Background:
- Prune-belly syndrome (PBS) is a congenital disorder characterized by abdominal muscle deficiency, urinary tract anomalies, and cryptorchidism.
- Associated anomalies, such as urethral stenosis, oligohydramnios, imperforate anus, and fistulas, often confer a poor prognosis.
Observation:
- A case report of a 4.5-year-old boy with PBS, urethral stenosis, oligohydramnios, imperforate anus, and a vesicosigmoid fistula.
- The patient underwent vesicostomy and divided transverse colostomy postnatally, with infection prophylaxis and bicarbonate supplementation.
Findings:
- Despite severe congenital anomalies, the patient achieved a favorable outcome.
- The in utero vesicosigmoid fistula may have acted as a natural urinary diversion, mitigating pressure-induced renal damage.
- Neonatal renal function post-urinary diversion, reflecting kidney dysplasia, appears to be the primary prognostic determinant in PBS.
Implications:
- Early surgical intervention and supportive care can lead to positive outcomes in complex PBS cases.
- The degree of congenital kidney dysplasia, assessed via neonatal renal function, is a critical factor for predicting prognosis in prune-belly syndrome.
- This case highlights the potential protective role of certain fetal urinary anomalies in mitigating renal damage in PBS.
Abstract:
A boy aged 4.5 years with prune-belly syndrome and associated urethral stenosis, oligohydramnios, imperforate anus and vesicosigmoid fistula 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.