Work

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

Year: 2025

Type: article

Abstract: 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.

Source: Journal of Surgical Case Reports

DOI: 10.1093/jscr/rjaf797

Authors: Uma V Mahajan, Marla Sacks, Carlos Reck, Varag Abed, Harry Zinn, Francisca T Velcek

Institutions: State University of New York, SUNY Downstate Health Sciences University

Language: English

Topic: Gynecological conditions and treatments

Subfield: Obstetrics and Gynecology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $1,000