Prune belly syndrome

Abstract

The prune belly syndrome consists of congenital absence or deficiency of the anterior abdominal muscle, bilateral cryptorchidism and anomalies of urogenital tract. Neurological problems are common in these patients as respiratory, orthopedic, gastrointestinal and chromosomal anomalies. We have treated 17 cases of prune belly syndrome. Mortality is 23.5%: two patients died in the neonatal period of respiratory failure; one patient, aged two months, died of septicemia in postoperative period, another, 14 years old, died of respiratory failure with normal renal function. Two patients are female without urological anomalies, one male has an incomplete form with normal urinary tract. Two patients with complete syndrome are treated conservatively. Ten patients with vesicourethral reflux or ureteral dilatation for distal obstruction are treated surgically. We performed 20 ureteral reimplantations with 16 tailoring of the ureters. In three patients ureteral reimplantation failed and in these patients we performed successfully a transureteroureterostomy. All patients have adequate complete bladder emptying without surgical procedure. Renal function is normal in seven, slightly reduced in one and reduced but stable in the others. Orchidopexy was performed successfully in eleven patients (10 Fowler-Stephens procedures). Orchiectomy with testicular prosthesis was performed in a patient 12 years old. Five patients have moderate respiratory insufficiency treated with chest physiotherapy. Urinary tract abnormalities are the most common cause of morbidity and mortality in patients with prune belly syndrome. Treatment regimen must be individualized. Respiratory problems are also frequent and is mandatory a precocious chest physiotherapy.