Mayer-Rokitansky-Kiister-Hauser syndrome

Mayer-Rokitansky-Kiister-Hauser syndrome characterized by the absence of the entire vagina or, more commonly, the proximal two-thirds of the vagina absence or abnormalities of the uterus and malformations of the upper urinary tract (Fig. 7.5). 

It affects 1 in 4,000-5,000 otherwise normal (46 XX) girls. Mayer-Rokitansky-Kiister-Hauser syndrome type A (typical form) shows normal-appearing external genitalia, absence of the vagina and uterus, normal fallopian tubes, normal ovaries, and no renal anomalies. In type B (atypical form), the uterus may be normal except for the lack of a conduit to the introitus or may be rudimentary, commonly show-... 

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