Mayer-Rokitansky-Küster-Hauser syndrome: Rare case Diagnosis and Management

Abstract

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital disorder characterized by vaginal agenesis or hypoplasia, often associated with uterine anomalies. We present a case of a 35-year-old woman with Type 1 MRKH syndrome, who underwent McIndoe vaginoplasty to create a functional vagina. The patient presented with primary amenorrhea and difficulty with sexual intercourse. Preoperative evaluation confirmed vaginal agenesis, and the patient underwent McIndoe vaginoplasty using a split-thickness skin graft. Postoperative follow-up showed successful creation of a functional vagina, with satisfactory anatomical and functional outcomes. This case highlights the effectiveness of McIndoe vaginoplasty in creating a functional vagina in patients with MRKH syndrome, improving their quality of life and sexual function.