OBJECTIVE: To present a single series of Y-type duplication with an analysis of the presenting features, the management and outcome, as complete Y-type urethral duplication in the male is a rare congenital anomaly that presents many challenges. PATIENTS AND METHODS: We retrospectively reviewed patient charts, identifying those with urethral duplication, and then those with complete Y-type duplication. The age at presentation, investigations, management and outcomes were reviewed. RESULTS: Of 49 patients with a diagnosis of urethral duplication, 13 had the complete Y-type. Three were lost to follow-up and were therefore excluded from the study. The median (range) age at presentation was 2 months (birth to 10 years). All patients had other comorbidities, with anorectal malformations in seven, renal agenesis in four, sacral agenesis/partial agenesis in two, and unilateral/bilateral cryptorchidism in four. Presenting features included passage of urine perineally or rectally in seven, and dribbling from the orthotopic urethral meatus in one. The diagnosis was confirmed by micturating cysto-urethrography. A mean of 14 procedures was required to achieve a final complete repair, and included urethral reconstruction, repair of fistulae, urethral dilatation, and evaluative cystoscopy. Excluding cystoscopy and urethral dilatation, a mean (range) of 3 (1-5) reconstructive procedures was required in each patient. The median follow-up was 8 years. Five boys developed urethral strictures and one developed multiple fistulae. The best outcomes were in boys who had a staged urethral reconstruction. CONCLUSION: Due to the strong association with other congenital anomalies we recommend that all patients should be evaluated thoroughly, including a detailed physical examination, renal tract ultrasonography and spinal radiography. Surgical management remains a significant challenge, requiring many procedures with unpredictable outcomes, the goals of which should be to maintain continence and reconstruct the urethra with good cosmesis. The optimum management scheme must be individualized in this rare condition.
OBJECTIVE: To present a single series of Y-type duplication with an analysis of the presenting features, the management and outcome, as complete Y-type urethral duplication in the male is a rare congenital anomaly that presents many challenges. PATIENTS AND METHODS: We retrospectively reviewed patient charts, identifying those with urethral duplication, and then those with complete Y-type duplication. The age at presentation, investigations, management and outcomes were reviewed. RESULTS: Of 49 patients with a diagnosis of urethral duplication, 13 had the complete Y-type. Three were lost to follow-up and were therefore excluded from the study. The median (range) age at presentation was 2 months (birth to 10 years). All patients had other comorbidities, with anorectal malformations in seven, renal agenesis in four, sacral agenesis/partial agenesis in two, and unilateral/bilateral cryptorchidism in four. Presenting features included passage of urine perineally or rectally in seven, and dribbling from the orthotopic urethral meatus in one. The diagnosis was confirmed by micturating cysto-urethrography. A mean of 14 procedures was required to achieve a final complete repair, and included urethral reconstruction, repair of fistulae, urethral dilatation, and evaluative cystoscopy. Excluding cystoscopy and urethral dilatation, a mean (range) of 3 (1-5) reconstructive procedures was required in each patient. The median follow-up was 8 years. Five boys developed urethral strictures and one developed multiple fistulae. The best outcomes were in boys who had a staged urethral reconstruction. CONCLUSION: Due to the strong association with other congenital anomalies we recommend that all patients should be evaluated thoroughly, including a detailed physical examination, renal tract ultrasonography and spinal radiography. Surgical management remains a significant challenge, requiring many procedures with unpredictable outcomes, the goals of which should be to maintain continence and reconstruct the urethra with good cosmesis. The optimum management scheme must be individualized in this rare condition.
Authors: Salvatore Arena; Carmela Arena; Maria Grazia Scuderi; Giuseppe Sanges; Francesco Arena; Vincenzo Di Benedetto Journal: Pediatr Surg Int Date: 2007-06-19 Impact factor: 1.827