Mayer-Rokitansky-Küster-Hauser (MRKH)
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare disorder that affects women. It is characterized by the failure of the uterus and the vagina to develop properly in women who have normal ovarian function and normal external genitalia. Women with this disorder develop normal secondary sexual characteristics during puberty (e.g., breast development and pubic hair), but do not have a menstrual cycle (primary amenorrhea). Often, the failure to begin the menstrual cycle is the initial clinical sign of MRKH syndrome. CASE STUDY: A 13-year-old woman with cyclic abdominal pain. We diagnosed MRKH syndrome with cyclic pain due to a hematometra in a functioning right horn associated to a right hematosalpinx and a nonfunctioning left horn. interventions: Reestablishing uterovaginal continuity with excision of the left rudimentary horn via a total laparoscopic procedure. Main outcome: Restoring regular menstruation. Result: After total laparoscopic uterovaginal anastomosis and excision of the left nonfunctioning horn, the patient's menstruation resumed 3 months later, and regular, unimpeded menstrual flow was still present at the 2-year-follow-up evaluation. Conclusion(s) Total laparoscopic reestablishment of uterovaginal continuity in MRKH syndrome with a functioning horn is a valuable alternative to the currently recommended treatment (laparotomy and radical excision of the rudimentary uterus). By: https://www.fertstert.org/article/S0015-0282%2807%2903914-3/fulltext
