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The prune-belly syndrome: current insights
R S Sutherland1, R A Mevorach, B A Kogan
1Department of Urology, University of California School of Medicine, San Francisco 94143-0738, USA.
Insights
Prune-belly syndrome involves distinct physical findings, but its cause and management are debated. This review covers pathogenesis, fetal/neonatal diagnosis, and management, alongside associated anomalies impacting long-term prognosis.
Area of Science:
- Pediatric Surgery
- Developmental Biology
- Medical Genetics
Background:
- Prune-belly syndrome (PBS) is a congenital disorder characterized by a triad of abdominal muscle deficiency, urinary tract anomalies, and cryptorchidism.
- While physical findings are recognized, the underlying etiology and optimal treatment strategies remain subjects of ongoing research and debate.
Purpose of the Study:
- To review the current understanding of prune-belly syndrome pathogenesis.
- To characterize fetal and neonatal diagnosis and management approaches for PBS.
- To discuss associated anomalies and their influence on treatment and prognosis throughout childhood and beyond.
Main Methods:
- Literature review focusing on pathogenesis, diagnosis, and management of prune-belly syndrome.
- Analysis of associated anomalies and their impact on patient outcomes.
- Synthesis of current evidence for clinical decision-making.
Main Results:
- Pathogenesis of PBS involves complex genetic and environmental factors affecting fetal development.
- Early diagnosis through prenatal ultrasound and prompt postnatal management are crucial for improving outcomes.
- Associated anomalies significantly influence the morbidity and long-term prognosis in affected individuals.
Conclusions:
- A comprehensive understanding of PBS pathogenesis is evolving.
- Multidisciplinary management tailored to individual patient needs, considering associated anomalies, is essential.
- Further research is needed to refine treatment protocols and improve long-term outcomes for prune-belly syndrome patients.
Abstract:
The prune-belly syndrome comprises a constellation of well-established physical findings, yet the cause and management remain controversial. This review focuses on the current understanding of its pathogenesis and characterizes the fetal and neonatal diagnosis and management. Other associated anomalies are discussed to understand better the factors affecting treatment and prognosis as these patients grow into childhood and beyond.