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Concordant prune belly syndrome in monozygotic twins

K C Balaji1, A Patil, P L Townes

  • 1Southern Illinois University School of Medicine, Springfield, Illinois 62702, USA.

Urology
|June 7, 2000
PubMed

Insights

Prune belly syndrome in identical twins suggests a genetic cause. With proper treatment, these patients can achieve normal growth and development, defying a poor prognosis.

Area of Science:

  • Medical Genetics
  • Developmental Biology
  • Pediatric Nephrology

Background:

  • Prune belly syndrome is a rare congenital disorder characterized by abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism.
  • The exact etiology of prune belly syndrome remains largely unknown, with theories ranging from genetic factors to intrauterine compression.

Observation:

  • This report details two cases of monozygotic twins who were both diagnosed with prune belly syndrome (PBS).
  • The twins presented with concordant manifestations of the syndrome, indicating a potential shared genetic predisposition.

Findings:

  • A 12-year follow-up of these twins demonstrated that appropriate medical and surgical interventions can lead to favorable outcomes.
  • Patients with prune belly syndrome, despite a historically poor prognosis, achieved normal growth, developmental milestones, and preserved renal function.

Implications:

  • The concordant presentation in monozygotic twins strongly supports a significant genetic component in the etiology of prune belly syndrome.
  • This case series highlights the critical role of early diagnosis and comprehensive management in improving long-term outcomes for individuals with PBS.
  • The findings offer hope and guide therapeutic strategies for affected families, emphasizing the potential for a good quality of life.

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