Three cases of prune belly syndrome at the Lagos State University Teaching Hospital, Ikeja

Area of Science:

• Pediatrics
• Urology
• Medical Genetics

Background:

• Prune belly syndrome (PBS) is a rare congenital disorder affecting 2.5 to 3.8 per 100,000 live births globally.
• PBS is characterized by a triad of abdominal muscle deficiency, urinary tract anomalies, and cryptorchidism.

Observation:

• Three cases of PBS are presented, all referred after birth with characteristic lax abdominal wall and congenital anomalies of the kidney and urinary tract.
• Associated anomalies included an absent foot, cryptorchidism, and deranged renal function in one patient.
• Radiological findings revealed hydronephrosis, hydroureters, and bladder changes on ultrasound, with vesicoureteric reflux noted on voiding cystourethrogram in one case.

Findings:

• Clinical manifestations, laboratory results, and radiological characteristics of PBS were documented in the reported cases.
• Urinary tract infections were managed with antibiotics based on sensitivity testing.
• Management challenges were identified, including parental demands for discharge against medical advice and strong cultural beliefs.

Implications:

• There is a critical need to increase awareness of Prune belly syndrome among healthcare providers and the public.
• Effective PBS management requires addressing cultural beliefs and improving parent education to prevent discharge against medical advice.
• A multidisciplinary approach is essential for optimal diagnosis and long-term care of patients with Prune belly syndrome.