Y DUPLICATION OF URETHRA WITH COMPLETE ATRESIA OF THE ORTHOTOPIC CHANNEL: 1-STAGE RECONSTRUCTION

Abstract

and are characterized by a stenotic orthotopic urethra and an accessory pre-anal or perineal channel. In these patients the abnormally located urethra is more normal functionally, while the orthotopic channel is atretic to a varying extent. We report a case of Y duplication with complete atresia of the orthotopic channel and describe a method of 1-stage reconstruction. CASE REPORT A 14-year-old boy presented with urine passage through the anus since birth which was independent of defecation. External urinary meatus was absent. An ectopic meatus was situated just inside the anal verge in the midline at the 12 o’clock position. An excretory urogram and voiding cystourethrogram revealed normal upper tracts and bladder. During voiding the anterior urethra was not seen while contrast material could be seen in the posterior urethra, spilling towards the anus (fig. 1). A suprapubic tract was established, and antegrade cystoscopy using rigid and flexible cystoscopes revealed normal bladder, prostatic urethra, verumontanum and membranous urethra. From the membranous urethra in the midline, a wide epithelialized channel could be seen towards the anus, while the orthotopic anterior urethra could not be identified. The patient was placed in the lithotomy position and an inverted U incision was made in the perineum. The accessory tract was posterior and opened into the anus. Despite an aggressive search, the anterior urethra could not be located. Only a hypoplastic corpus spongiosum was identified, which on division did not reveal any lumen. The accessory channel was detached from the anus, mobilized and transposed anteriorly. After mobilization it was brought near the perineoscrotal junction. A midline strip of 3 cm. scrotal skin was isolated on the ventral dartos pedicle and tubularized (fig. 2, A). This tube was anastomosed to the mobilized accessory channel over a 16Fr silicone catheter, which extended to the penoscrotal junction. A preputial island flap on a dorsal dartos pedicle was raised and subsequently tubularized over the same silicone catheter (fig. 2, B). Proximally, this tube was anastomosed to the scrotal skin tube and distally the glans was split open to receive the other end of the tube to create a neomeatus at the tip of the glans penis. Convalescence was uneventful. At 3-week followup examination revealed a small leak at the penoscrotal junction, which healed spontaneously a few days after catheter removal. The patient is completely continent and voiding well. Voiding cystourethrography showed no stricture or diverticulum. DISCUSSION