Prune belly syndrome. A case report

Abstract

Prune belly syndrome is a rare congenital anomaly. It is a triad consisting of absence, deficiency or hypoplasia of wall abdominal muscles, bilateral cryptorchidy, and urinary tract (dilatation) malformation. As an example, take the case of a male baby with in utero diagnostic of megacystis, partial posterior urethral valves, and dilated ureters. After birth, the phenotypic characteristics were suggestive of prune belly syndrome. Complementary studies confirmed the absence of testes in scrotum, bilateral vesicourereteral reflux and stricture of the proximal urethra. Magnetic resonance with 3D reconstruction reported that both kidneys had alterations in their morphology with interior cysts, right renal hypoplasia, long and tortuous ureters, distended bladder with diverticulum of urachus and deficiency of abdominal muscles. There were complications like febrile urinary tract infection and secondary chronic kidney disease. Once the infectious and obstructive events were resolved the renal function was stabilized and the surgical approach was vesicostomy. The patient was discharged in stable condition, with vesicostomy management, prophylaxis for urinary tract infection and regular follow. The urinary tract reconstruction will be in the second year of life and according to evolution of his kidney function.