Prune belly syndromeの2剖検例と発生病因

Abstract

Two autopsy cases of prune belly syndrome are reported. In Japan, 111 cases have been reported. Abdominal muscle deficiency or hypoplasia, urinary tract abnormalities and cryptorchidism are usually recognized. Case 1 was a female stillborn at 33 weeks' gestation and case 2 was a male stillborn at 21 weeks' gestation. Both cases had anomalies of genitourinary and digestive system. There was anatomic obstruction of external urethral orifice in case 1 and overriding urethra in case 2 which meant functional obstruction. In relation to the pathogenesis, we propose the important role of the urethral obstruction, whatever anatomic or functional. Further examination of the lower urinary tract including serial section analysis might be necessary to identify the pathogenesis of prune belly syndrome.