Mayer-Rokitansky-Kuster- Hauser Syndrome Type I

A 24-year-old nulligravida married woman presented with dyspareunia and a history of primary amenorrhea.

The lady had been married for 5 months. She was 163 cm tall and weighed 45 kg. 

On examination, she had normal secondary sexual characteristics and hirsutism, with hair on her upper lip, chin, sternal area, and lower abdomen – scoring 7 on the hirsutism scale. Her heart and breath sounds were normal. A vaginal examination showed a short, non-patent vaginal canal about 1 cm in length. Hormonal tests indicated elevated DHEA levels, while other hormones were normal. An abdominal ultrasound revealed the absence of the uterus but normal bilateral ovaries and kidneys. Chest X-ray and ECG were normal.

The patient chose to undergo Davydov’s vaginoplasty. Consent was obtained, and surgery was performed three weeks after the initial visit under general anesthesia with a laparoscopic approach.

The procedure involved creating a rectovaginal space and opening the vesicovaginal fold. The bladder was pushed down, and peritoneal folds were dissected lateral to the infundibulopelvic ligament. An incision was made on the vaginal mucosa, and a sponge on a cotton swab was placed to mark the bridging tissue between the vagina and peritoneum. The peritoneal folds were sutured to the vagina, and a vaginal glass mold was inserted post-operatively. 

The patient was discharged five days later after being taught how to self-insert the mold and the necessary precautions. Follow-up appointments were scheduled for 1, 6, and 12 months. At the one-month follow-up, the patient had no complaints and was regularly using the mold as instructed.

MRKH syndrome is a rare and complex condition affecting females physically, psychologically, and sexually, requiring a multidisciplinary treatment approach. Patients should be counseled and provided with appropriate treatment options. Davydov’s vaginoplasty is an effective surgical treatment. Adoption, surrogacy, and possibly uterine transplant can be considered to combatinfertility.

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