Mayer-Rokitansky-Kuster-Hauser syndrome: pathologic associations

MRKH syndrome (Mullerian structures agenesis in woman) is often associated with other anomalies: kidney and bone anomalies, deafness, ovarian tumors. Functional ovarian anomalies have not been previously reported. Case reports - Five new cases of MRKH syndrome are reported. They were asso ciated with deafness in two cases, with kidney anomaly in three cases, vert ebral anomalies in one case, and four suffered from obesity and ovarian dys function (ovarian polycystics syndrome, advanced puberty, androgenic excess ). All of them had ovarian ectopy, which was responsible for false ultrason ographic diagnosis. Conclusion - An MRKH syndrome diagnosis implicates a search for other anoma lies. Ovarian functional trouble should not let MRKH syndrome remain unreco gnized. (C) 2001 Editions scientifiques et medicales Elsevier SAS.