Mayer-Rokitansky-Kuster- Hauser syndrome type I

A 24-year-old nulligravida woman presented with dyspareunia. 

She had a history of primary amenorrhea and had been married for 5 months. 

She was 163 cm tall and weighed 45 kg. Examination showed normal secondary sexual characteristics and hirsutism, with hair on her upper lip, chin, sternal area, and lower abdomen, scoring a 7 on the hirsutism scale. 

Auscultation revealed normal heart sounds (S1 and S2) and breath sounds. Vaginal examination revealed a vaginal opening with a short, non-patent vaginal canal of about 1 cm. The hormonal profile indicated elevated DHEA with normal levels of other hormones. Abdominal ultrasound showed the absence of a uterus but normal bilateral ovaries and kidneys. Chest X-ray and ECG were normal, with unremarkable findings for the lungs and heart.

The patient chose to undergo Davydov’s vaginoplasty. Consent was obtained, and the surgery was performed in the third week after her initial visit. 

Under general anesthesia, the procedure was approached laparoscopically. Posterior dissection created the rectovaginal space, and anterior dissection opened the vesicovaginal fold, pushing the bladder down. The peritoneal fold was dissected laterally to the infundibulopelvic ligament. An incision was made on the vaginal mucosa, and a sponge on a cotton swab marked the tissue between the vagina and peritoneum. The peritoneal folds were sutured to the vagina, and a vaginal glass mold was inserted and kept post-operatively. 

The patient was discharged after 5 days, having been instructed on mold insertion and necessary precautions. Follow-ups were scheduled at 1, 6, and 12 months. At the one-month follow-up, the patient reported no complaints and was regularly using the mold as instructed.

MRKH is a complex syndrome affecting women physically, psychologically, and sexually, requiring a multidisciplinary approach to treatment. Davydov’s vaginoplasty is an effective surgical option. For infertility, adoption, surrogacy, or uterine transplant may be considered.

Source: Chandekar A S, Shah S R, George M, et al. Indian J Obstet Gynecol Res 2024;11(2):301-303