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The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome comprises the combined hypoplasia of the vagina and the uterus. In recent years, variable anomalies of the development of the Mullerian duct [as classified by the American Fertility Society (AFS)] have been operated on by the laparoscopic approach. We describe here a laparoscopic extirpation of an aplastic ectopic uterus in a patient found to have a hypoplastic vagina and ectopic uterus attached to the right pelvic wall. The uterus was linked to a normotopic right Fallopian tube and right ovary which were covered with endometriosis and adhesions. While the uterine vessels and the round ligament could be demonstrated on the right side, these structures were missing on the contralateral left side of the uterus. However, a normal tube and ovary were present on the left pelvic wall. Although not explicitly described in the AFS classification, this constellation most likely corresponds to the AFS classification stage Ie.

Laparoscopic extirpation of an aplastic ectopic uterus in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome