Urethral duplication II-A Y type with rectal urethra: ASTRA approach and tunica vaginalis flap for first stage repair

Abstract

_______________________________________________________________________________Introduction: Urethral duplication is a rare congenital anomaly affecting mainly boys. Generally, the duplication develops on the sagittal plane; the accessory urethra may run dorsally or ventrally to the orthotopic one. We present a patient with urethral duplication in which the orthotopic urethra was patent in the penile segment but atresic in the bulbar and prostatic segment. The patient had urinary flow from the rectum and the ectopic urethra could be well identified by anal examination. Materials and Methods: Age at surgery was 13 months. The procedure consisted of an ASTRA (anterior sagittal trans-ano-rectal) approach for dividing the urethra and rectum and was successful to move the urethra up to the perineal area. The rectum was reconstructed and the patient placed into a lithotomy position. A urethral catheter inserted in the penile urethra oriented us were the atresic urethra in bulbar area started. The scrotum was opened in the middle and the distance between the two urethral stumps proximal and distal defined the extension of no urethral tissue that consisted of 5 cm. We opened the right scrotal space and a tunica vaginalis flap was obtained and attached to the bulbar tissue for a two-stage urethroplasty strategy. Results: Patient had a nice healing and the tunica vaginalis was nicely incorporated to the adjacent tissue, having the two urethral stumps well delineated. Conclusions: ASTRA approach in combination with a two-stage urethroplasty with tunica vaginalis dorsal flap proved to be an excellent combination for a rare case of urethral Y duplication having the main urethra into the rectum.

Duplication of the urethra in an adult male presenting with scrotal fistula: a rare case report

“…The urethral duplication is a rare congenital abnormality <300 cases reported in the commune literature [ 1 ]. Embryogenesis is not well understood and various hypotheses exist, but none can explain all types of presentations; the common pathological process is supposed to result from an abnormal relationship between the lateral folds of the genital tubercle and the ventral end of the cloacal membrane, and the duplication commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally [ 3 , 4 ]. Being a congenital, mostly the diagnosis was established in childhood or adolescence but nearly never in adult age [ 5 ], which makes our case one of few exceptions, with this anomaly being observed in an adult of 32 years of age with no other related congenital abnormality.…”

Primary congenital bladder diverticula: Where does the ureter drain?

“…This patient had also an urorectal fistula and penile urethral atresia, that we treated initially by an ASTRA surgery and a tunica vaginal two-stage urethroplasty. [ 4 ]…”