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Prune belly syndrome

S Hassett1, G H H Smith, A J A Holland

  • 1Douglas Cohen Department of Paediatric Surgery, The Children's Hospital at Westmead, Sydney Medical School, The University of Sydney, Sydney, NSW, Australia.

Pediatric Surgery International
|December 27, 2011
PubMed

Insights

Prune Belly Syndrome (PBS) is a rare congenital disorder. This review details its pathogenesis, diagnosis, and current management strategies for affected infants.

Area of Science:

  • Pediatric Surgery
  • Congenital Abnormalities
  • Urology

Background:

  • Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by a triad of abdominal wall deficiency, urinary tract dilation, and bilateral cryptorchidism.
  • The exact pathogenesis of PBS remains debated, with various theories proposed.
  • Management of PBS is complex and requires a multidisciplinary approach.

Purpose of the Study:

  • To review the current understanding of Prune Belly Syndrome (PBS) pathogenesis and diagnosis.
  • To discuss the evolution of management strategies for PBS.
  • To examine the evidence supporting current treatment approaches for PBS.

Main Methods:

  • A comprehensive literature review was conducted using PubMed.
  • Key publications on the pathogenesis, diagnosis, and management of PBS were identified and analyzed.
  • Evidence supporting current management options was critically evaluated.

Main Results:

  • The review highlights the typical characteristics and diagnostic criteria for PBS.
  • It discusses the challenges in managing associated anomalies, including intra-abdominal testes, abdominal wall defects, and urinary system dilation.
  • Current management strategies are presented with an examination of their supporting evidence.

Conclusions:

  • Understanding the pathogenesis and natural history of PBS is crucial for effective management.
  • A multidisciplinary approach is essential for optimizing outcomes in patients with PBS.
  • Continued research is needed to refine treatment strategies and improve long-term prognosis for PBS.

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