Twenty-five-year experience with prune belly syndrome.

Area of Science:

• Pediatric Surgery
• Medical Genetics
• Congenital Anomalies

Background:

• Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by the triad of abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism.
• The syndrome affects males predominantly and can range in severity from mild to life-threatening.
• Early diagnosis and comprehensive management are crucial for improving outcomes.

Purpose of the Study:

• To review the clinical management, associated findings, and long-term prognosis of Prune Belly Syndrome.
• To analyze outcomes in a cohort of 30 patients diagnosed over a 25-year period.
• To identify factors influencing the prognosis of Prune Belly Syndrome.

Main Methods:

• Retrospective review of 30 cases of Prune Belly Syndrome diagnosed and managed at a university pediatric hospital.
• Data collection included prenatal diagnosis, initial presentation, surgical interventions, medical management, and patient follow-up.
• Analysis of mortality, morbidity, and long-term functional outcomes.

Main Results:

• Thirty cases of Prune Belly Syndrome were identified over a 25-year period.
• No stillborn deaths were recorded in the reviewed cases.
• The study details the spectrum of urological and gastrointestinal anomalies encountered and the surgical approaches employed.

Conclusions:

• Prune Belly Syndrome, while complex, can have a favorable prognosis with timely and appropriate multidisciplinary management.
• Aggressive surgical and medical interventions can significantly reduce morbidity and mortality.
• Continued follow-up is essential to monitor for long-term complications and optimize patient quality of life.