Surgical treatment of an 8-year-old child living with Prune Belly syndrome

Prune Belly syndrome, also called Eagle-Barret syndrome, is a rare disorder with a prevalence of 3.8 live births per 100,000 births. Its primary characteristic involves underdeveloped abdominal muscles, thus, the name "prune belly syndrome." 

This case presents a 6-year-old boy with Prune Belly syndrome. He was detected with abdominal muscle underdevelopment, ureterohydronephrosis, and associated megabladder – both confirmed at birth. 

The patient experienced recurrent urinary tract infections; the episodes usually caused fever and necessitated hospitalization. Surgical intervention was planned to offer definitive treatment; however, the treatment was delayed. 

Almost six years later, the child presented with the following anatomical alterations – bilateral megaureter, persistent urachus, cryptorchidism, hypoplasia of the rectus abdominis, and phimosis. Ureteroscopy revealed significant narrowing of the left ureter. 

The proposed surgical approach was left ureteral reimplantation, along with associated urachal excision, bilateral orchidopexy, postectomy, and Monford abdominoplasty.

 Successful postoperative results were achieved, including stable renal function, wound healing, and graft recovery. 

The study highlights the importance of early intervention in managing Prune Belly syndrome. The preferred management strategy in such cases is surgical intervention – ideally addressing cryptorchidism and phimosis between 6 and 18 months of age. Correcting urinary abnormalities and abdominoplasty are recommended by the age of 4 years.

Source: Trivilato RA, Dadalt G, Assenço DL. Journal of Human Growth and Development. 2023;33(1):124.