Urethral and genital malformations in prune belly syndrome

Abstract

In addition to the typical triad of the prune belly syndrome, comprising abdominal wall muscle deficiency, urinary tract dilatation and cryptorchidism, anomalies of the lower urinary tract and genitalia can occur. Between January 1974 and December 1978 we evaluated and treated 19 boys with prune belly syndrome. All boys had an abnormally wide bladder neck and prostatic urethra, and 13 (68 per cent) also had malformations of the bulbous or pendulous urethra and/or corporeal bodies of the penis. Although the etiology of these findings is uncertain we assume that they are part of the same developmental disturbance that affects the remainder of the urinary system. Our description of these urethral and penile abnormalities in boys with prune belly syndrome should provide an impetus to develop a systematic method for seeking and recording these abnormalities, and for establishing their true incidence and clinical relevance.