Background
Prune Belly Syndrome (PBS), also known as Eagle-Barrett Syndrome, is an exceedingly rare congenital disorder characterized by deficient abdominal wall musculature, genitourinary anomalies, and cryptorchidism. The literature predominantly focuses on management of urological anomalies, with abdominal wall reconstruction largely under-reported. Female presentations are exceptionally rare, and to our knowledge, this is the first documented case in Australia, offering a unique insight into surgical management of this complex condition.
Methods
A 2-year-old female with PBS presented with excess abdominal skin obstructing vesicostomy function. Intra-operative assessment found preserved anterior abdominal wall fascia and external oblique muscles. Abdominal wall reconstruction involved midline fascial plication and abdominoplasty-based skin excision with preservation of the neo-umbilicus.
Results
There was a minor complication with early post-operative soft tissue swelling requiring intermittent catheterization. At follow up, the swelling had resolved, vesicostomy function was restored, abdominal wall tension improved, and the cosmetic outcome was excellent.
Conclusion
Abdominal wall reconstruction in PBS is poorly described in the literature due to its rarity and highly variable presentations, necessitating individualized management. This case demonstrates a flexible, patient-specific approach in achieving both functional and aesthetic success, and contributes to the limited literature in this complex population.