Coexisting congenital ano-urethral fistula with posterior urethral valve: a case report and literature review.

backgroundH-type anorectal malformation is a rare congenital anomaly that appears to be more prevalent in females across Asia. While anorectal malformations are known to be associated with urologic anomalies, their association with posterior urethral valves is exceedingly rare. CASE PRESENTATION: A 9-year-old male presented with a two-year history of urinary leakage per rectum and nocturnal enuresis, along with a prolonged history of recurrent urinary tract infections. The external genitalia were male and normally developed. The anal opening was orthotopic, with normal tone and adequate caliber. Voiding cystourethrography revealed a dilated posterior urethra and a recto-urethral fistula. Cystoscopic evaluation demonstrated an obstructive Type I posterior urethral valve, which was successfully ablated endoscopically. Subsequently, with the patient in the lithotomy position and a guidewire in place, the fistulous tract was mobilized. The urethral end of the fistula was identified and ligated, and the anal sphincter complex was interposed over the repair site. The anal canal was then reconstructed. The patient was discharged on postoperative day 11, with complete resolution of urinary leakage. At three months follow-up, he remained continent, with no clinical or radiological evidence of fistula recurrence.

conclusionH-type fistulas are diagnostically challenging and are often identified late in the clinical course. The coexistence of an ano-urethral fistula with a posterior urethral valve is exceptionally rare and, to our knowledge, has not been previously reported in the literature. This unique combination underscores the importance of maintaining a high index of suspicion to ensure timely diagnosis and management. CLINICAL TRIAL NUMBER: Not applicable.