Class I müllerian duct anomaly (MDA) based on classification of American Society for Reproductive Medicine (ASRM)
Early developmental failure of müllerian ducts → agenesis or various degrees of hypoplasia of proximal 2/3 of vagina, cervix, and uterus
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome
Extreme presentation of müllerian agenesis with absence of proximal vagina, cervix, and uterus
2 types have been described
Typical MRKH (type I)
Isolated agenesis of uterus and vagina
Atypical MRKH (type II) or MURCS (müllerian duct aplasia, renal aplasia, and cervicothoracic somite) syndrome
IMAGING
General Features
Hysterosalpingography
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Herlin MK et al: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update. Orphanet J Rare Dis. 15(1):214, 2020
Committee on Adolescent Health Care: Committee opinion: no. 562: müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 121(5):1134-7, 2013
Hall-Craggs MA et al: Mayer-Rokitansky-Kuster-Hauser syndrome: diagnosis with MR imaging. Radiology. 269(3):787-92, 2013
Kara T et al: MRI in the diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. Diagn Interv Radiol. 19(3):227-32, 2013
Rousset P et al: Ultrasonography and MRI features of the Mayer-Rokitansky-Küster-Hauser syndrome. Clin Radiol. 68(9):945-52, 2013
Yoo RE et al: Magnetic resonance evaluation of Müllerian remnants in Mayer-Rokitansky-Küster-Hauser syndrome. Korean J Radiol. 14(2):233-9, 2013
Behr SC et al: Imaging of müllerian duct anomalies. Radiographics. 32(6):E233-50, 2012
Fiaschetti V et al: 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. J Radiol Case Rep. 6(4):17-24, 2012
Giusti S et al: Diagnosis of a variant of Mayer-Rokitansky-Kuster-Hauser syndrome: useful MRI findings. Abdom Imaging. 36(6):753-5, 2011
Lermann J et al: Comparison of different diagnostic procedures for the staging of malformations associated with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril. 96(1):156-9, 2011
Pompili G et al: Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome. Radiol Med. 114(5):811-26, 2009
Govindarajan M et al: Magnetic resonance imaging diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. J Hum Reprod Sci. 1(2):83-5, 2008
Morcel K et al: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Orphanet J Rare Dis. 2:13, 2007
Pittock ST et al: Mayer-Rokitansky-Kuster-Hauser anomaly and its associated malformations. Am J Med Genet A. 2005
Fedele L et al: Magnetic resonance imaging in Mayer-Rokitansky-Kuster-Hauser syndrome. Obstet Gynecol. 76(4):593-6, 1990
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Synonyms
Uterovaginal agenesis
Definitions
Class I müllerian duct anomaly (MDA) based on classification of American Society for Reproductive Medicine (ASRM)
Early developmental failure of müllerian ducts → agenesis or various degrees of hypoplasia of proximal 2/3 of vagina, cervix, and uterus
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome
Extreme presentation of müllerian agenesis with absence of proximal vagina, cervix, and uterus
2 types have been described
Typical MRKH (type I)
Isolated agenesis of uterus and vagina
Atypical MRKH (type II) or MURCS (müllerian duct aplasia, renal aplasia, and cervicothoracic somite) syndrome
IMAGING
General Features
Hysterosalpingography
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Herlin MK et al: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update. Orphanet J Rare Dis. 15(1):214, 2020
Committee on Adolescent Health Care: Committee opinion: no. 562: müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 121(5):1134-7, 2013
Hall-Craggs MA et al: Mayer-Rokitansky-Kuster-Hauser syndrome: diagnosis with MR imaging. Radiology. 269(3):787-92, 2013
Kara T et al: MRI in the diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. Diagn Interv Radiol. 19(3):227-32, 2013
Rousset P et al: Ultrasonography and MRI features of the Mayer-Rokitansky-Küster-Hauser syndrome. Clin Radiol. 68(9):945-52, 2013
Yoo RE et al: Magnetic resonance evaluation of Müllerian remnants in Mayer-Rokitansky-Küster-Hauser syndrome. Korean J Radiol. 14(2):233-9, 2013
Behr SC et al: Imaging of müllerian duct anomalies. Radiographics. 32(6):E233-50, 2012
Fiaschetti V et al: 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. J Radiol Case Rep. 6(4):17-24, 2012
Giusti S et al: Diagnosis of a variant of Mayer-Rokitansky-Kuster-Hauser syndrome: useful MRI findings. Abdom Imaging. 36(6):753-5, 2011
Lermann J et al: Comparison of different diagnostic procedures for the staging of malformations associated with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril. 96(1):156-9, 2011
Pompili G et al: Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome. Radiol Med. 114(5):811-26, 2009
Govindarajan M et al: Magnetic resonance imaging diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. J Hum Reprod Sci. 1(2):83-5, 2008
Morcel K et al: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Orphanet J Rare Dis. 2:13, 2007
Pittock ST et al: Mayer-Rokitansky-Kuster-Hauser anomaly and its associated malformations. Am J Med Genet A. 2005
Fedele L et al: Magnetic resonance imaging in Mayer-Rokitansky-Kuster-Hauser syndrome. Obstet Gynecol. 76(4):593-6, 1990
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