Urethral duplication in children: surgical treatment and results.

PURPOSE

Urethral duplication is a rare congenital anomaly. We report the clinical presentation, imaging findings and surgical treatment in 7 boys with incomplete sagittal duplication of the urethra.

MATERIALS AND METHODS

Duplication involved hypospadias in 5 cases (group 1) and a bifid urethra with an accessory preanal tract (Y duplication) in 2 (group 2). Group 1 was treated with 1-stage urethroplasty, including marsupialization of the dorsal orthotopic urethra, ventral-to-dorsal urethrourethrostomy and penile island flap onlay repair to cover the open dorsal urethra. In contrast, group 2 was treated with 2-stage urethral reconstruction with detachment and mobilization of the accessory preanal branch in association with a scrotal tubed neourethra followed by urethroplasty, as in group 1. In all cases the dorsal penile urethra was located between the corpora cavernosa and surrounded by the tunica albuginea.

RESULTS

A urethrocutaneous fistula developed in 1 of the 5 group 1 patients. In group 2, 1 patient had recurrent penoscrotal meatal stenosis after the 1-stage procedure and 1 had a urethral diverticulum with calculi at the scrotal tubed neourethra 7 years after urethral reconstruction. Six of the 7 patients now void spontaneously through a meatus located normally at the tip of the glans. The remaining patient with a neurogenic bladder is on intermittent catheterization via appendicovesicostomy due to difficult catheterization of the irregular and sensitive neourethra.

CONCLUSIONS

While the ideal surgical management of urethral duplication anomalies remains uncertain, we used a combination of surgical techniques to correct this severe malformation.