Fetuses begin with two kinds of internal reproductive systems, one of them called Mullerian and the other Wolffian. In typical female development, the Wolffian structures disappear and the Mullerian structures form the uterus, Fallopian tubes, and upper part of the vagina.
Sometimes a genetic female (46,XX) will have the DSD called MRKH Syndrome (formally known as Mayer-Rokitansky-Kustner-Hauser Syndrome). In the case of MRKH, the Mullerian system does not develop in the typical fashion. A girl born with MRKH may be missing part or all of the vagina, the uterus, and the Fallopian tubes. MRKH Syndrome sometimes also involves kidney anomalies, skeletal issues, hearing problems, and in rarer cases, anomalies of the heart and of the fingers and toes.
The main support group for MRKH is the MRKH Organization. Click here for their website.
Posted in: Specific Conditions