A case report on the management of congenital urethroperineal fistula in an adolescent, a rare congenital anomaly.

Background: Congenital posterior urethra perineal fistula (CUPF) is a rare congenital GU anomaly with abnormal epithelium-lined communication between the posterior urethra and perineum. Some consider it to be a variant of Y-type urethral duplicate with ventral hypoplastic urethra and have suggested its adoption to the Eiffmans classification.

Methods: An 18-year-old male patient presented with urine dribbling from the perineum since childhood. He has no history of urinary tract infection and surgical procedure and instrumentation. There was normal external genitalia with meatus positioned at the tip of the glans; there was a small opening on the posterior scrotal wall near the perineum that admits a 6fr NG tube. VCUG showed a fistula from the prostatic urethra to the perineum. He was managed with near-total excision of the fistula tract, with no recurrence on a 3-month follow-up.

Conclusions: Patients with this rare condition present with dribbling from perineum during voiding, underwear wetting and recurrent UTI. Voiding CUG is a diagnostic procedure of choice for patients with CUPF. MRI can be used if there is interest in detail anatomy of the fistula tract and its relationship to the surrounding structures and is also helpful if other modalities show a non-conclusive result. In managing this patient, differentiating the dominant and functional urethra is of clinical significance. Complete or partial excision, electrofulgration of the fistula tract are options. Conclusions: Voiding CUG is a diagnostic procedure of choice for patients with CUPF and partial excision of the fistula tract is an option of management with satisfactory result.