AbstractMRKH syndrome (class I mullerian duct anomaly) is a rare congenital disorder characterized by uterine and cervical aplasia/hypoplasia with normally functioning ovaries in genotypical normal female (46XX) due to failed/interrupted development of the mullerian duct 1,2. Patients usually present with normal secondary sexual characteristics and primary amenorrhea. In our study we reviewed the array of magnetic resonance imaging findings in MRKH syndrome and the various MR imaging appearances.
A retrospective descriptive case series of six cases was done. Out of the six cases, one patient had imaging features consistent with Mayer-Rokitansky-Kuster- Hauser syndrome type II (vertebral column anomalies) and five patients with Mayer-Rokitansky-Kuster-Hauser syndrome type I. Four patients had mullerian duct remnants evident on imaging as either and or presence of rudimentary uterine horns with cavitation, mid line triangular soft tissue structure, fibrous band. One patient had ectopic location of left ovary.
