Atypical presentation of the Mayer-Rokitansky-Kuster-Hauser syndrome: roleof ultrasonography

We present three cases with atypical presentation of the Mayer-Rokitansky-K uster-Hauser syndrome observed by US between August 1994 and December 1997. In two cases we observed complete absence of the uterine. In the last case , an uterus remnant was present. In all cases, both ovaries were normal and the three women reported "normal" sexual activity. An ectopic kidney was p resent in one patient with complete absence of the uterus. Atypical forms o f Mayer-Rokitansky-Kuster-Hauser syndrome with normal vagina can be diagnos ed at US. In the cases presented here, US allowed a clear description of th e anatomy and provided a comprehensive evaluation of the patients.