Mayer-Rokitansky-Küster-Hauser syndrome complicated by hematometra and hematosalpinx treated with laparoscopic uterine drain placement: a case report

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a reproductive tract malformation occurring in ~1 in 5000 female births. It is characterized by congenital agenesis or marked hypoplasia of the Mullerian ducts derivative structures. About 3% of MRKH patients have a functioning uterus. A 12-year-old otherwise healthy female presented with persistent cyclic severe right lower quadrant pain. She was found to have MRKH syndrome with a right-sided functioning uterus and hematometra and ipsilateral hematosalpinx. She subsequently underwent diagnostic cystoscopy, vaginoscopy, and pelvic laparoscopy. Laparoscopic drainage of the hematometra and hematosalpinx, and hysterostomy catheter placement and uterine fixation to anterior abdominal wall were performed. Symptoms fully resolved post-operatively. A computed tomography scan 2 weeks later demonstrated resolution of the hematometra and significant improvement in the hematosalpinx. MRKH syndrome with a functioning uterus may present as cyclic abdominal pain in adolescent female patients. Laparoscopic uterine drain placement is a management option.