[Prune-belly syndrome and therapeutic problems]

Area of Science:

• Pediatric Surgery
• Urology
• Medical Genetics

Background:

• Prune-Belly syndrome (PBS) is a congenital disorder characterized by abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism.
• Early diagnosis, often prenatal, is crucial for managing PBS and mitigating potential complications.

Observation:

• This study presents the disease progression and therapeutic challenges in four boys diagnosed with PBS.
• Prenatal diagnosis was achieved in three cases, with interventions including amniotic fluid supplementation and an abdominal-amniotic shunt.
• Delivery methods varied, with two born naturally and two via cesarean section at 31 weeks due to intrauterine infection risk.

Findings:

• Postnatal observations revealed urinary infections and renal function issues in all patients.
• Surgical interventions included percutaneous nephrostomy, cutaneocystostomy, and orchidopexy.
• One patient died, and two experienced chronic kidney disease (II degree).

Implications:

• Children with PBS necessitate ongoing, multidisciplinary medical care.
• Staged surgical treatment is vital for preserving renal function and enhancing the quality of life for individuals with PBS.