Mayer-Rokitansky-Kuster-Hauser Syndrome diagnosed by Magnetic Resonance Imaging. Role of Imaging to identify and evaluate the uncommon variation in development of the female genital tract.

Authors

  • Valeria Fiaschetti
  • Amedeo Taglieri
  • Vito Gisone
  • Irene Coco
  • Giovanni Simonetti

DOI:

https://doi.org/10.3941/jrcr.v6i4.992

Keywords:

Mayer-Rokitansky-Kuster-Hauser, pelvic magnetic resonance, amenorrhea, vaginoplasty, Mí¼llerian abnormalities, infertility

Abstract

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a spectrum of Mí¼llerian duct anomalies characterized by congenital aplasia of the uterus and of the upper part (2/3) of the vagina, in young women presenting otherwise with normal endocrine status. The ovaries and fallopian tubes are present. It is one of the most common causes of primary amenorrhea and affects at least 1 out of 4500 women. Its penetrance varies, as does the involvement of other organ systems and itcan be isolated (type I) or associated with other malformations (type II). The MRKH syndrome usually remains undetected until the patient presents with primary amenorrhea despite normal development of secondary sexual characteristics, so imaging evaluation can demonstrate in one setting, non invasively, the anomalies in development of genital tract. We report a case of MRKH syndrome in a 16-year-old woman who presented with primary amenorrhea, stressing the role and benefit of imaging in the differential diagnosis.

Author Biographies

Valeria Fiaschetti

Department of Diagnostic Imaging, Molecular Imaging,  Interventional Radiology and Radiation Therapy to the University Hospital "Tor Vergata", Rome.

Amedeo Taglieri

Department of Diagnostic Imaging, Molecular Imaging,  Interventional Radiology and Radiation Therapy to the University Hospital "Tor Vergata", Rome.

Vito Gisone

Department of Diagnostic Imaging, Molecular Imaging,  Interventional Radiology and Radiation Therapy to the University Hospital "Tor Vergata", Rome.

Irene Coco

 

Department of Diagnostic Imaging, Molecular Imaging,  Interventional Radiology and Radiation Therapy to the University Hospital "Tor Vergata", Rome.

Giovanni Simonetti

Department of Diagnostic Imaging, Molecular Imaging,  Interventional Radiology and Radiation Therapy to the University Hospital "Tor Vergata", Rome.

Published

2012-04-15

Issue

Section

Obstetric & Gynecologic Radiology