Mayer-Rokitansky-Kuster-Hauser Syndrome: Surgical Management of Two Cases

The Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome is a rare anomaly characterized by congenital aplasia of the uterus and vagina in women showing normal development of secondary sexual characters and normal 44 XX karyotype. We report our experience in the management of two patients with congenital absence of the vagina due to the MRKH syndrome. The first case was a 24-year-old student, who presented with primary amenorrhea, uterovaginal agenesis, right pelvi-ureteric junction obstruction, and left renal agenesis. The second patient was a 24-year-old housewife, who presented with primary amenorrhea and inability to achieve penetrative sexual intercourse. She had vaginal atresia and a grossly hypoplastic uterus. Both had successful sigmoid colovaginoplasty and are sexually active. Vaginal reconstruction using the sigmoid colon saw an immediate and satisfactory outcome in both patients.