title: Clinical value of magnetic resonance imaging in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: diagnosis of associated malformations, uterine rudiments and intrauterine endometrium.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  • 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.
authors.affiliation: Department of Radiology, Diagnostic and Interventional Radiology, University Hospital Tuebingen, Hoppe-Seyler-Str. 3, 72076, Tuebingen, Germany.
authors.forename: H
authors.initials: H
authors.lastname: Preibsch
authors.forename: K
authors.initials: K
authors.lastname: Rall
authors.forename: B M
authors.initials: BM
authors.lastname: Wietek
authors.forename: S Y
authors.initials: SY
authors.lastname: Brucker
authors.forename: A
authors.initials: A
authors.lastname: Staebler
authors.forename: C D
authors.initials: CD
authors.lastname: Claussen
authors.forename: K C
authors.initials: KC
authors.lastname: Siegmann-Luz
mesh_terms: D058489:46, XX Disorders of Sex Development / Q000175:diagnosis*; D000293:Adolescent; D000328:Adult; D000013:Congenital Abnormalities / Q000175:diagnosis*; D003937:Diagnosis, Differential; D017352:Echo-Planar Imaging / Q000379:methods*; D004717:Endometrium / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D013509:Gynecologic Surgical Procedures / Q000379:methods; D006801:Humans; D010535:Laparoscopy; D009095:Mullerian Ducts / Q000002:abnormalities*; D057234:Preoperative Period; D012189:Retrospective Studies; D014621:Vagina / Q000002:abnormalities* / Q000601:surgery; D055815:Young Adult
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