La radiologia medica

, Volume 114, Issue 5, pp 811–826

Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome

  • G. Pompili
  • A. Munari
  • G. Franceschelli
  • N. Flor
  • R. Meroni
  • G. Frontino
  • L. Fedele
  • G. Cornalba
Uro-Genital Radiology/Radiologia Uro-Genitale

Abstract

Purpose

We evaluated the accuracy of magnetic resonance imaging (MRI) in young women with primary amenorrhoea with suspected Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome (congenital absence of both vagina and uterus and presence of normal ovaries).

Materials and methods

Fifty-eight women (age range 14–30 years, mean 20.9) with primary amenorrhea were studied with MRI performed with a 1.0-T superconducting magnet (Philips NT Intera). All patients were examined in the supine position using a phased-array coil (four channels). Turbo spin-echo T2-weighted images were acquired in the sagittal, axial and coronal planes with the following parameters: TR 4,750–6,686, TE 100–120, FOV 350–375, 4- to 5-mm sections with a 0.4- to 0.5-mm intersection gap and NSA 6. T1-weighted images were acquired in the axial and coronal planes (TR 470, TE 15, FOV 350, 4-mm sections with a 0.6-mm intersection gap, NSA 3). Two experienced radiologists evaluated all the examinations in consensus to assess the presence, position and morphology of vagina, uterus, ovaries and kidneys and any pelvic abnormalities. MRI results were judged on the basis of laparoscopic findings in 41 patients.

Results

MRKH syndrome was confirmed in 56 patients with 100% sensitivity and specificity. MRI identified bilateral Müllerian buds in 34/56 (61%) and unilateral in 10/56 (18%) patients. MRI sensitivity was 81.42%, and there was good agreement with laparoscopy (k=0.55) and full agreement in the identification of cavitation between MRI and intraoperative sonography. Both ovaries were visualised in 54 patients, with regular morphology in 46 (82.1%), polycystic in 10 (17.8%), pelvic in 47 (83.6%) and extrapelvic in eight (14.5%). We found associated abnormalities of the upper urinary tract in six patients (solitary kidney in four and ptosis in two).

Conclusions

MRI is a useful diagnostic tool in the preoperative evaluation of MRKH syndrome and is less expensive and invasive than laparoscopy. Strong cooperation between radiologists and surgeons is highly recommended.

Keywords

Magnetic resonance (MR) MRKH, Mayer Rokitansky Kuster Hauser Syndrome Utero-vaginal agenesis Müllerian anomalies Primary amenorrhea 

La risonanza magnetica nel management prechirurgico della sindrome di Mayer-Rokitansky-Kuster-Hauser

Abstract

Obiettivo

Valutare l’accuratezza della RM in donne con amenorrea primaria, con sospetta sindrome Mayer-Rokitansky-Kuster-Hauser (MRKH) (assenza congenita di vagina e utero con presenza di ovaie normali).

Materiali e metodi

Cinquantotto donne (14–30 anni, media 20,9), con amenorrea primaria, sono state studiate con RM mediante magnete superconduttivo 1,0 T. Tutte le pazienti sono state esaminate in posizione supina con bobina multicanale (4 canali). Immagini turbo spin-echo T2 pesate sono state acquisite sui piani sagittale, assiale e coronale con i seguenti parametri: TR 4750–6686, TE 100–120, FOV 350–375, sezioni di 4–5 mm con interspazio di 0,4–0,5 mm i, NSA 6. Immagini T1 pesate sono state acquisite sui piani assiale e coronale (TR 470, TE 15, FOV 350, sezioni di 4 mm di spessore con interspazio di 0,6 mm, NSA 3). Due radiologi esperti hanno valutato gli esami in consenso per stabilire la presenza, sede e morfologia di vagina, utero, ovaie, reni ed eventuali ulteriori malformazioni pelviche. I risultati della RM sono stati confrontati con quelli laparoscopici in 41 pazienti.

Risultati

La sindrome MKRH è stata confermata in 56 pazienti con sensibilità e specificità del 100%. La RM ha identificato residui mülleriani in 34/56 (61%) e monolaterali in 10/56 (18%) pazienti. La sensibilità della RM è stata dell’81,42% e c’è stato un buon accordo con la laparoscopia (k Cohen 0,55) e un completo accordo nella identificazione delle cavitazione dei residui tra RM e ecografia intraoperatoria. Gli annessi bilaterali sono stati visualizzati in 54 pazienti, con regolare morfologia in 46 (82,1%), policistica in 10 (17,8%); sede pelvica in 47 (83,6%), extrapelvica in 8 (14,5%). Abbiamo riscontrato anomalie dell’apparato urinario superiore in 6 pazienti (monorene in 4, ptosico in 2).

Conclusioni

La RM si è dimostrata utile metodica nella valutazione prechirurgica della sindrome MRKH ed è meno costosa ed invasive della laparoscopia. Una forte cooperazione tra radiologo e chirurgo è fortemente raccomandata.

Parole chiave

Risonanza magnetica (RM) MRKH, sindrome di Mayer Rokitansky Kuster Hauser Agenesia utero-vaginale Anomalie mülleriane Amenorrea primaria 

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Copyright information

© Springer-Verlag Italia 2009

Authors and Affiliations

  • G. Pompili
    • 1
    • 4
  • A. Munari
    • 1
  • G. Franceschelli
    • 1
  • N. Flor
    • 1
  • R. Meroni
    • 2
  • G. Frontino
    • 3
  • L. Fedele
    • 3
  • G. Cornalba
    • 1
  1. 1.Servizio di Radiologia Diagnostica e InterventisticaUniversità di Milano, Ospedale San PaoloMilanoItaly
  2. 2.Servizio di RadiologiaIstituto HumanitasBergamoItaly
  3. 3.Reparto di Ginecologia OstetriciaClinica MangiagalliMilanoItaly
  4. 4.MilanoItaly

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