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[Prune belly syndrome].

P Campobasso1, L Musi, A Bedogni

  • 1Divisione di Chirurgia Pediatrica, Sezione di Urologia, Ospedale Regionale di Vicenza, Italia.

La Pediatria Medica E Chirurgica : Medical and Surgical Pediatrics
|March 1, 1989
PubMed
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Prune Belly Syndrome, a congenital condition, presents significant risks from urinary tract issues and respiratory problems. Individualized treatment is crucial for managing this rare disorder affecting abdominal muscles, urogenital tract, and often involving cryptorchidism.

Area of Science:

  • Pediatric Surgery
  • Urology
  • Genetics

Background:

  • Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by the triad of abdominal muscle deficiency, cryptorchidism, and urinary tract anomalies.
  • Associated anomalies include neurological, respiratory, orthopedic, gastrointestinal, and chromosomal abnormalities, contributing to significant morbidity and mortality.

Purpose of the Study:

  • To review the clinical characteristics, management strategies, and outcomes of 17 patients with Prune Belly Syndrome.
  • To highlight the primary causes of morbidity and mortality and emphasize the need for individualized treatment approaches.

Main Methods:

  • Retrospective analysis of 17 patients diagnosed with Prune Belly Syndrome.
  • Evaluation of surgical interventions including ureteral reimplantation, ureteral tailoring, transureteroureterostomy, and orchidopexy.

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  • Assessment of conservative management and non-surgical interventions like chest physiotherapy.

    • Main Results:

    • Mortality rate was 23.5%, with respiratory failure and septicemia being primary causes.
    • Urinary tract abnormalities were the most common cause of morbidity.
    • Surgical management for vesicourethral reflux and ureteral dilatation included 20 ureteral reimplantations and 16 ureteral tailors; transureteroureterostomy was successful in cases of reimplantation failure.
    • Orchidopexy was performed in 11 patients.
    • Adequate bladder emptying was achieved in all patients without surgical intervention.
    • Renal function was preserved in most patients.
    • Respiratory insufficiency was managed with chest physiotherapy.

    Conclusions:

    • Urinary tract abnormalities and respiratory problems are the leading causes of morbidity and mortality in Prune Belly Syndrome.
    • Individualized treatment plans are essential for optimal patient outcomes.
    • Early chest physiotherapy is mandatory for managing respiratory complications.