Laparoscopic Removal of Uterine Remnants in Teens With Mayer-Rokitansky-Kuster-Hauser Syndrome and Pelvic Pain

Abstract

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital anomaly of the Mullerian tract characterized by agenesis of the upper 2/3 of the vagina, cervix and uterus seen in 1/4,500 females. In those with pelvic pain, the differential diagnosis includes the presence of a functioning uterine remnant, which occurs in 7-10%. MRI is the gold standard for evaluation of these anatomic variants. Our objective is to review a series of patients with MRKH syndrome requiring surgical removal of uterine remnants for pelvic pain.