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Prune belly syndrome: A rare case report.

Siddinath Gyawali¹, Balkrishna Gyawali¹, Bhumika Ghimire¹

¹Institute of Medicine, Tribhuvan University Teaching Hospital Kathmandu Nepal.

Clinical Case Reports

|June 19, 2024

Summary

Early diagnosis of prune belly syndrome is crucial in infants with omphalocele. This condition requires careful evaluation for associated anomalies like kidney absence and cryptorchidism for timely intervention.

Keywords:

congenital disorder cryptorchidism omphalocele prune belly syndrome

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Area of Science:

Pediatric Surgery
Neonatology
Medical Genetics

Background:

Omphalocele, a congenital abdominal wall defect, can be associated with various syndromes.
Prune Belly Syndrome (PBS) is a rare congenital disorder characterized by the triad of abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism.

Observation:

A male neonate presented with an omphalocele on the 13th day of life.
Clinical evaluation revealed congenital absence of the left kidney and bilateral cryptorchidism.

Findings:

The patient was diagnosed with Prune Belly Syndrome based on the observed clinical features.
The infant showed a positive response to abdominoplasty.
A 'wait and watch' approach was adopted for managing the bilateral cryptorchidism.

Implications:

This case highlights the importance of scrutinizing for PBS components in neonates with omphalocele.
Early identification facilitates timely management and can improve patient outcomes.
Comprehensive evaluation is essential for diagnosing complex congenital anomalies like PBS.