Urethral sex in a woman with previously undiagnosed Mayer-Rokitansky-Kuster-Hauser syndrome

Purpose: To report a case of urethral sex in a woman with previously undiagnosed Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Materials and Methods: A 32-year-old woman presented with severe pain, dysuria, and mild hematuria after each intercourse. Secondary sexual characteristics were normal. Vaginal and rectal examinations revealed an absent vagina and uterus. Further investigations showed a normal hormonal profile, a 46 XX karyotype, and a normal intravenous pyelography. Pelvic ultrasonography and magnetic resonance imaging (MRI) confirmed the absence of a uterus and the presence of bilateral ovaries. Results: A diagnosis of MRKH syndrome was made and the patient underwent a modified Vecchietti operation for the creation of a new vagina. The urethral meatus was noticeably dilated. Postoperatively, the vaginal length was six to seven cm. Long-term follow-up revealed that she was able to have normal and satisfactory vaginal intercourse without any problems. Conclusion: Urethral intercourse is documented here for the first time in a case of misdiagnosed MRKH syndrome.