Mayer-Rokitansky-Küster-Hauser syndrome: report of two cases

Mayer-Rokitansky-Küster-Hauser syndrome (SMRKH) is a congenital pathology characterized by aplasia or agenesis of structures that develop from the mullerian ducts in genotypically and phenotypically normal females. The prevalence of this syndrome is uncertain. There are only two population-based studies evaluating it. We present two clinical cases, in an adolescent and older adult woman. These patients require clinical, epidemiological, hormonal and imaging studies by a multidisciplinary team that includes a psychologist to support the patient’s acceptance of the situation, as well as considerations related to subsequent surgeries and reproductive consequences.