Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome with a single septate uterus: a novel anomaly and description of treatment options.

OBJECTIVE

To report a rare Mayer-Rokitansky-Kuster-Hauser (MRKH) anomaly with a septate midline uterus and describe management options.

DESIGN

Case report.

SETTING

Major academic medical center.

PATIENT(S): Two adolescent patients presented with pelvic pain and primary amenorrhea. Radiographic imaging showed evidence of a bicornuate uterus with two uterine horns, cervical hypoplasia, and absence of the upper vagina.

INTERVENTION(S): Owing to pain, both patients planned laparoscopy with bilateral hemihysterectomies, but in each case laparoscopy showed evidence of a single midline uterus. Continuous hormone therapy was used to suppress pain and retrograde menstruation.

MAIN OUTCOME MEASURE(S): Uterine preservation and future fertility management.

RESULT(S): Given the fertility potential, the treatment plan was to continue hormone therapy until adulthood, when the young woman could select a treatment option for herself. After 2 years, one patient elected hysterectomy at age 18.5 due to persistent pain, while the other patient continues uterine preservation.

CONCLUSION(S): We present two cases of a rare and uniquely described anomaly of MRKH with a single septate midline uterus. We recommend uterine preservation until the age of 18, at which point the patient may select her treatment option. Novel options include septal resection with subsequent assisted reproductive technology (ART) and cesarean delivery or ART and a gestational carrier.