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Prune belly syndrome.

R W Jennings1

  • 1Department of Surgery, Harvard Medical School and Children's Hospital of Boston, Massachusetts 02115, USA.

Seminars in Pediatric Surgery
|August 19, 2000
PubMed
Summary
This summary is machine-generated.

Prune Belly Syndrome, also known as Eagle-Barrett syndrome, is a rare condition characterized by a deficient abdominal wall, urinary tract dilation, and undescended testes. It often involves other system malformations, affecting many patients.

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Area of Science:

  • Pediatric Medicine
  • Medical Genetics
  • Developmental Biology

Background:

  • Prune Belly Syndrome (PBS) presents as a lax, wrinkled abdominal wall.
  • It is frequently associated with other congenital anomalies.
  • Also known as Eagle-Barrett syndrome or triad syndrome.

Purpose of the Study:

  • To define the characteristic features of Prune Belly Syndrome.
  • To highlight associated anomalies in affected individuals.
  • To provide a comprehensive overview of this rare condition.

Main Methods:

  • Review of existing literature on Prune Belly Syndrome.
  • Analysis of case studies and clinical observations.
  • Synthesis of diagnostic criteria and associated conditions.

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Main Results:

  • Prune Belly Syndrome involves a triad of abdominal wall deficiency, urinary tract dilation, and bilateral cryptorchidism.
  • Approximately 75% of patients exhibit additional malformations.
  • Affected systems include cardiopulmonary, gastrointestinal, and orthopedic.

Conclusions:

  • Prune Belly Syndrome is a complex congenital disorder with multisystemic implications.
  • Early recognition and management are crucial for affected infants.
  • Further research is needed to understand the underlying pathogenesis.