Syndrome de Mayer-Rokitansky-Kuster-Hauser : associations pathologiques

Abstract

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 associated with deafness in two cases, with kidney anomaly in three cases, vertebral anomalies in one case, and four suffered from obesity and ovarian dysfunction (ovarian polycystics syndrome, advanced puberty, androgenic excess). All of them had ovarian ectopy, which was responsible for false ultrasonographic diagnosis. Conclusion – An MRKH syndrome diagnosis implicates a search for other anomalies. Ovarian functional trouble should not let MRKH syndrome remain unrecognized.