Asian Research Journal of Gynaecology and Obstetrics

Background: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital malformation affecting phenotypically normal 46,XX females. It is characterised by primary failure of Müllerian duct development, resulting in congenital aplasia of the uterus and the upper two-thirds of the vagina, while ovarian endocrine function remains normal. Patients usually present during adolescence with primary amenorrhoea. Delayed diagnosis until later adult life in a married woman may, however, lead to particular anatomical, surgical, and psychosexual difficulties related to vaginal agenesis and coital dysfunction.

Case Presentation: This case concerns a 35-year-old married woman who presented with a history of primary amenorrhoea. However, she was able to cohabit and engage in intercourse involving vaginal penetration. She was well nourished, with normal secondary sexual characteristics, including Tanner stage 5 breast development and Tanner stage 5 pubic hair, together with normal external genitalia. She had complete vaginal atresia with a 1 cm dimple in the vaginal mucosa. Abdominal ultrasonography revealed absence of the uterus, cervix, and vagina, with normally positioned bilateral kidneys, consistent with Mayer-Rokitansky-Küster-Hauser Type 1. Haematological and biochemical profiles were within normal limits. A 12-lead ECG showed no abnormalities. The patient underwent McIndoe vaginoplasty under spinal anaesthesia. The rectovesical space was developed by sharp and blunt dissection to create a neovaginal canal measuring 9.5 cm in length and 3.5 cm in width. The neovaginal canal was grafted with a split-thickness skin graft harvested from the right lateral thigh and packed with a foam-dressed mould. The postoperative course was uneventful. The skin graft healed well, with >95% graft take and no signs of haematoma, infection, or dehiscence. The mould was removed on postoperative day 7, and the patient achieved a compliant neovaginal canal measuring 8.5 cm in depth, admitting two fingers, with maintenance of normal coitus at 3 and 6 months postoperatively.

Conclusion: McIndoe vaginoplasty using an autologous split-thickness skin graft remains a simple and practical surgical option for adults with severe obstructive MRKH syndrome associated with coital obstruction. Careful understanding of the appropriate surgical planes, adequate dissection, proper graft stenting, and consistent postoperative dilatation can contribute to an acceptable functional outcome.