Psychosexual and social consequences in a woman with undiagnosed Mayer-Rokitansky-Küster-Hauser syndrome.

PURPOSE: To report a woman with devastating psychosexual and social consequences as a result of undiagnosed Mayer-Rokitansky- Küster-Hauser syndrome (MRKH) syndrome.

MATERIALS AND METHODS: An 18-year-old woman was referred after being divorced for "absent vagina and uterus". On examination, secondary sexual characteristics were normal. Vaginal and rectal examinations revealed absent vagina and uterus. Investigations showed normal hormonal profile, 46 XX karyotype, and normal intravenous pyelography. Pelvic ultrasonography and magnetic resonance imaging (MRI) confirmed the absence of the uterus and presence bilateral ovaries.

RESULTS: A diagnosis of MRKH syndrome was made and she underwent successful modified laparoscopic Vecchietti operation for creation of a new vagina. Vaginal dilators were used after the surgery. Two years of follow up confirmed that vaginal length was about ten cm.

CONCLUSION: The proper diagnosis, counseling, and prompt treatment of MRKH syndrome can prevent tragic consequences.