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[Prognosis of prune belly syndrome]
G Pillion1, M A Macher, Y Aigrain
1Service de Néphrologie, Hôpital Robert-Debré, Paris.
Insights
Prune Belly syndrome (PBS) is rare, with early mortality from sepsis or renal failure. Long-term outcomes show varied renal function, with some developing end-stage renal failure despite interventions.
Area of Science:
- Pediatric Urology
- Nephrology
- Congenital Abnormalities
Context:
- Prune Belly syndrome (PBS) is a rare congenital disorder affecting males, characterized by abdominal muscle deficiency, urinary tract abnormalities, and undescended testes.
- This study reviews a 20-year experience with 14 PBS cases, analyzing mortality, renal function, and surgical management outcomes.
Purpose:
- To evaluate the long-term outcomes of Prune Belly syndrome patients.
- To assess the impact of surgical interventions on renal function and potential fertility.
Summary:
- Four out of 14 infants (29%) with PBS died neonatally due to sepsis or end-stage renal failure.
- Of the remaining 10 patients, 6 maintained normal kidney function, while 4 developed chronic or end-stage renal failure.
- Surgical management included urethral obstruction resection in 2 cases and orchidopexy in 4 children, with fertility outcomes pending for early-operated cases.
Impact:
- This research highlights the significant risk of early mortality and progressive renal deterioration in Prune Belly syndrome.
- It underscores the importance of individualized surgical approaches, particularly regarding the timing of orchidopexy for potential fertility preservation.
- Findings contribute to understanding the natural history and management strategies for this complex condition.
Abstract:
The Prune Belly syndrome (PBS) is unfrequent. Fourteen cases have been followed in our unit during the last 20 years. Four infants (29%) died during the first months of life, because of neonatal sepsis (2 cases) or end-stage renal failure (2 cases). Among the other 10 cases, 6 (43%) had normal glomerular filtration rate at a mean age of 10 years 6 months (6 months to 15 years), 4 had chronic renal failure, including 3 cases who developed end-stage renal failure at 8, 8 years 8 months and 17 years respectively. Resection of an urethral obstruction was performed in 2 cases. This surgical indication remains widely accepted, while the current tendency is to limit ureteral surgery in PBS. Orchidopexy was performed in 4 children, 3 of them less than 6 years 6 months of age. Fertility of these early operated children remains to be established, as all adults reported in the literature remain sterile when orchidopexy was not performed or was performed after age 6.