Abstract

Background: H-type rectourethral fistula is a rare variant of anorectal malformation that affects male neonates. It is usually associated with anal stenosis but with a normal anal position and shape, so it is difficult to be diagnosed early after birth. We present this case to evaluate the functional outcome of this rare anomaly in a male patient and compare it with other reported cases with different ages at diagnosis. Case Presentation: A nine-month-old infant presented with recurrent attacks of urinary tract infection and failure to thrive. After proper evaluation, the diagnosis of an H-type rectourethral fistula with anal stenosis was confirmed, and a diverting double-barreled colostomy was performed as an initial procedure. Posterior sagittal anorectoplasty with ligation and division of fistula was successfully done, followed by colostomy closure. Now the patient is five years old and is continent for urine and stool. Conclusions: The diagnosis of H-type rectourethral fistula with anal stenosis is challenging to physicians, and a high index of suspicion is required to reach the diagnosis as the anal opening had a normal shape and location. If this condition is diagnosed and treated early, these patients will have an excellent functional prognosis, as the muscle complex and anal sphincter are well developed.

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