First experience: management of Prune Belly syndrome in Harapan Kita Women and Children’s Hospital

Abstract

Prune belly is a rare congenital syndrome, consisting of congenital abdominal wall musculature absence, urogenital tract anomalies, and cryptorchidism in male patient. The incidence of this syndrome is 1 in 35,000 to 1 in 50,000 live births and rarely occurs in females (3-5%). One step operation of ureteral re-implantation, orchidopexy, and abdominoplasty is one option of treatment in managing this condition. This was our first experience, involving a multidisciplinary approach. A 30 year old Asian Woman G4P3A0 at 33-34 weeks of gestation was referred to the Urology Department with a fetal ultrasound showing bilateral hydronephrosis and undescended testis. Prune Belly was first suspected when abnormal abdominal circumference was noted by the obstetrician while doing a fetomaternal ultrasound at the 19 weeks gestation. No intervention was undertaken during gestation. The baby delivered spontaneously at 38 weeks gestation. Testes were impalpable and the abdominal contour was suggestive of Prune Belly. CT urography showed bilateral hydronephrosis and hydroureter. Micturating cysto-urethrography showed bilateral vesicoureteral reflux while renogram showing a cortical defect. Serial follow-ip ultrasounds were done showing bilateral hydroureter with 4th grade bilateral hydronephrosis. By the age of 2 the patient underwent one step operation of ureteral reimplantation, orchidopexy, and abdominoplasty. After the operation, the patient recovered with good function and cosmetic result. Prune belly is a rare and complex congenital syndrome. From our experience at Harapan Kita Women and Children’s Hospital, one step operation involving multidisciplinary approach of ureteral re-implantation, orchidopexy, and abdominoplasty has shown a good result. Further follow up is needed to evaluate either this choice of treatment could improve patient outcome.