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 yea's 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.