[Mayer-Rokitansky-Kuster-Hauser syndrome: associated pathologies].
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.


METHODS
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.


CONCLUSIONS
An MRKH syndrome diagnosis implicates a search for other anomalies. Ovarian functional trouble should not let MRKH syndrome remain unrecognized.
Raybaud
C
C
Service de pédiatrie et d'endocrinologie pédiatrique, centre hospitalier Lyon-Sud 69495 Pierre-Bénite, France.
Richard
O
O
Arzim
M
M
David
M
M
D000015:Abnormalities, Multiple; D000293:Adolescent; D000728:Androgens / Q000032:analysis; D002648:Child; D003638:Deafness / Q000209:etiology*; D005260:Female; D005836:Genitalia, Female / Q000002:abnormalities*; D006801:Humans; D007668:Kidney / Q000002:abnormalities*; D009095:Mullerian Ducts / Q000002:abnormalities*; D009765:Obesity; D010051:Ovarian Neoplasms / Q000473:pathology; D011085:Polycystic Ovary Syndrome; D011629:Puberty, Precocious; D013131:Spine / Q000002:abnormalities; D013577:Syndrome
D000728:Androgens
Syndrome de Mayer-Rokitansky-Kuster-Hauser: associations pathologiques.