Korean J Fertil Steril.  2003 Jun;30(2):179-184.

A Case of Mayer-Rokitansky-Kuster-Hauser Syndrome Accompanying Endometriosis

Affiliations
  • 1Department of Obstetrics and Gynecology, College of Medicine, University of Ulsan, Asan Medical Center, Seoul, Korea. chnkim@amc.seoul.kr

Abstract

The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome refers to a condition which presents as normal female secondary sex characteristics, normal external genitalia, congenital absence of the internal vagina, usually a rudimentary uterus in the form of bilateral noncanaliculated muscular buds, and normal tubes and ovaries with normal cytogenetic and endocrine evaluation, frequent association of renal, skeletal and other congenital anomalies. However, rarely, whole uterus or a segment of uterus may be present, but lacking a conduit to the introitus. If a partial endometial cavity is present in a segment of uterus, cyclic abdominal pain may be a complaint and furthermore endometiosis can be developed. Recently, we experienced a case of MRKH syndrome with the segments of uterus accompanying endometriosis in young woman. We present this case with a brief review of literatures.

Keyword

MRKH Syndrome; Endometriosis

MeSH Terms

Abdominal Pain
Cytogenetics
Endometriosis*
Female
Genitalia
Humans
Ovary
Sex Characteristics
Uterus
Vagina
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