Mayer-Rokitansky-Küster-Hauser Syndrome with Imperforate Anus: A Rare Association and an Innovative Surgical Management.

ABSTRACT

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is one of the rare disorder of Mullerian agenesis leading to non-development of uterus and vagina. Its association with anorectal malformation is rare. We report a case of MRKH syndrome with recto-vestibular in a female child. The child had undergone a sigmoid loop colostomy in the neonatal period. On clinical examination of the perineum, a fistula was present in the vestibule just below the urethral opening, but no vaginal canal was found. It was managed with an innovative surgical technique, preserving the fistula and lower rectum to function as vagina.