Clinical value of magnetic resonance imaging in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: diagnosis of associated malformations, uterine rudiments and intrauterine endometrium

Eur Radiol. 2014 Jul;24(7):1621-7. doi: 10.1007/s00330-014-3156-3. Epub 2014 Apr 16.

Abstract

Objectives: The aim of this study was to evaluate the accuracy of preoperative magnetic resonance imaging (MRI) in the diagnosis of malformations associated with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and identification of uterine endometrium to optimise the clinical management.

Methods: We retrospectively reviewed 214 consecutive MRKH patients, mean age 19 years, who underwent laparoscopy-assisted neovagina creation. A total of 115 patients (53.7%) met the inclusion criterion of sufficient preoperative MRI. In 110 of them (95.7%), MRI findings were correlated with laparoscopy and associated malformations. In 39 cases (35.5%) uterine rudiments were removed and analysed histopathologically.

Results: Ten per cent (11/110) of the patients showed complete uterine agenesis. The others presented with either unilateral (n = 16; 14.5%) or bilateral (n = 83; 75.5%) uterine rudiments. MRI detection of uterine rudiments agreed in 78.2% (86/110) with laparoscopy. In 85.4% of the removed rudiments, MRI could correctly diagnose the existence of the endometrium. Compared to laparoscopy, MRI could exactly detect ovaries in 97.3% (107/110). Renal or ureteral malformations were seen in 32 cases (27.8%). In 83% of unilateral renal agenesis and unilateral rudiment, the latter was located at the side of the kidney.

Conclusions: MRI is useful for preoperative detection of MRKH-associated malformations and assessment of the endometrium to further optimise MRKH patient treatment.

Key points: • Pelvic MRI is useful for preoperative detection of MRKH-associated malformations. • MRI can diagnose uterine endometrium in MRKH patients with high precision. • Preoperative MRI can optimise clinical management of patients with MRKH syndrome.

MeSH terms

  • 46, XX Disorders of Sex Development / diagnosis*
  • Adolescent
  • Adult
  • Congenital Abnormalities / diagnosis*
  • Diagnosis, Differential
  • Echo-Planar Imaging / methods*
  • Endometrium / pathology*
  • Female
  • Follow-Up Studies
  • Gynecologic Surgical Procedures / methods
  • Humans
  • Laparoscopy
  • Mullerian Ducts / abnormalities*
  • Preoperative Period
  • Retrospective Studies
  • Vagina / abnormalities*
  • Vagina / surgery
  • Young Adult

Supplementary concepts

  • Mullerian aplasia