Indian Journal of Human Genetics
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Year : 2013  |  Volume : 19  |  Issue : 1  |  Page : 113-115

Mayer-Rokitansky-Kuster-Hauser syndrome type II: A rare case

Department of General Medicine, Aarupadai Veedu Medical College and Hospital, Puducherry, India

Correspondence Address:
Anand Pai
Department of General Medicine, Aarupadai Veedu Medical College, Kirumampakam - 607 402, Puducherry
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-6866.112928

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Mayer-Rokitansky-Kuster-Hauser (MRKH) is a malformation complex comprising absent vagina and absent or rudimentary uterus. MRKH syndrome may be attributed to an initial affection of the intermediate mesoderm consequently leading (by the end of the 4 th week of fetal life) to an alteration of the blastema of the cervicothoracicsomites and the pronephricducts. These latter subsequently induce the differentiation of the mesonephric and then the Wolffian and Mullerian ducts. There are very sparse such cases reported. We present a case of type II MRKH or Mullerian renal cervical somite association (i.e., Mullerian duct aplasia, renal dysplasia, and cervical somite anomalies).

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