Mayer-Rokitansky-Kuster-Hauser syndrome type II: A rare case.
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Date
2013-01
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Abstract
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 4th 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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Keywords
Dandy Walker malformation, mayer-rokitansky-kuster-hauser syndrome type II, mullerian agenesis, renal agenesis
Citation
Pai Anand, Shakir Mohammad. Mayer-Rokitansky-Kuster-Hauser syndrome type II: A rare case. Indian Journal of Human Genetics. 2013 Jan; 19(1): 113-115.