Pathophysiology and management of urinary incontinence in case of distal penile epispadias.

Abstract

We evaluated boys with distal epispadias and urinary incontinence to determine the cause, and designed a simplified bladder neck reconstruction to restore urinary continence. Six boys with epispadias of the glans or distal penile shaft whose incontinence persisted after successful single stage epispadias repair were evaluated with cystoscopy and urodynamics after failed attempts at toilet training. Surgical management--simplified bladder neck reconstruction--involved suprapubic excision of an identified deformity of the roof of the bladder neck and posterior urethra, followed by reapproximation of the remaining normal bladder neck and posterior urethral tissues. All boys displayed a characteristic deformity of the roof of the bladder neck and posterior urethra, which extended distally through the membranous urethral sphincter toward the urethral meatus. Five of the 6 boys were treated surgically, and promptly achieved normal continence and urinary control that remained durable through a mean followup of 9.6 years. Histologically, the roof deformity exhibited abnormalities including attenuation and reduction of smooth muscle. The meatus is not the only site involved in distal epispadias, which presents as a field defect that deforms the roof of the urethra distal to the bladder neck. Incontinence in distal epispadias has a dual etiology, namely anatomical dilatation and distortion of the bladder neck and posterior urethra, and histological abnormality of the roof tissues. These conditions combine to affect adversely coaptive and constrictive functions of the posterior urethra and urinary sphincter. Excision and reapproximation of the roof deformity appears to be proof in principle of the cause of incontinence, and offers an alternative approach to treating incontinence in these patients without the need for more invasive surgical procedures.