2002 Volume 38 Issue 7 Pages 1069-1073
Mayer-Rokitansky-Kuter-Hauser (MRKH) syndrome is a rare disorder and has an occurence rate of 1 in 4, 000-5, 000 live female births. The typical form of MRKH syndrome is characterized by congenital absence or rudimentary vagina. Patients with MRKH syndrome have a 46XX karyotype and normal secondary sex characteristics with a normal appearance of the external genitalia. We encountered a 1-year-and 6-month-old girl who had underwent cut-back procedure for her initial diagnosis as a superficial anorectal malformation in another hospital. She was diagnosed with vaginal agenesis and MRKH syndrome with intermediate anomaly of anorectal malformation during the correction of her anteriorally located anus. We simultaneously operated on the anomalies by posterior sagittal anorectoplasty and vaginoplasty, and separated the rectovaginal fistula.
