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

Abstract

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.