Publication:

Mayer-Rokitansky-Küster-Hauser syndrome: a case report and surgical approach for neovagina construction

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital anomaly characterized by agenesis or hypoplasia of the uterus and upper two-thirds of the vagina in phenotypically normal females with a 46, XX karyotype. Despite normal ovarian function and secondary sexual characteristics, affected individuals present with primary amenorrhea. We report the case of a 21-year-old female presenting with primary amenorrhea and normal secondary sexual development. Pelvic MRI demonstrated complete uterovaginal agenesis with small fibrofatty structures suggestive of uterine buds, and normal bilateral ovaries. Hormonal profile was within normal limits, except for mildly elevated thyroid-stimulating hormone. After multidisciplinary counselling, the patient underwent neovaginoplasty using a modified McIndoe technique with an amniotic membrane graft and foam mold support. Postoperative care included gradual mold dilations and psychological support. At follow-up, the neovagina maintained adequate depth (>8 cm) and caliber, with satisfactory functional outcome and no early complications. Surgical neovaginoplasty using amniotic grafts is a safe and effective option for MRKH patients when nonsurgical methods are unsuitable. Success depends on meticulous surgical technique, structured postoperative dilation, and comprehensive psychological counseling.