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Müllerian Agenesis
Akram M. Shaaban, MBBCh
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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 (llerian 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

                                        1. Herlin MK et al: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update. Orphanet J Rare Dis. 15(1):214, 2020
                                        2. Committee on Adolescent Health Care: Committee opinion: no. 562: müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 121(5):1134-7, 2013
                                        3. Hall-Craggs MA et al: Mayer-Rokitansky-Kuster-Hauser syndrome: diagnosis with MR imaging. Radiology. 269(3):787-92, 2013
                                        4. Kara T et al: MRI in the diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. Diagn Interv Radiol. 19(3):227-32, 2013
                                        5. Rousset P et al: Ultrasonography and MRI features of the Mayer-Rokitansky-Küster-Hauser syndrome. Clin Radiol. 68(9):945-52, 2013
                                        6. Yoo RE et al: Magnetic resonance evaluation of Müllerian remnants in Mayer-Rokitansky-Küster-Hauser syndrome. Korean J Radiol. 14(2):233-9, 2013
                                        7. Behr SC et al: Imaging of müllerian duct anomalies. Radiographics. 32(6):E233-50, 2012
                                        8. 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
                                        9. Giusti S et al: Diagnosis of a variant of Mayer-Rokitansky-Kuster-Hauser syndrome: useful MRI findings. Abdom Imaging. 36(6):753-5, 2011
                                        10. 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
                                        11. Pompili G et al: Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome. Radiol Med. 114(5):811-26, 2009
                                        12. Govindarajan M et al: Magnetic resonance imaging diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. J Hum Reprod Sci. 1(2):83-5, 2008
                                        13. Morcel K et al: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Orphanet J Rare Dis. 2:13, 2007
                                        14. Pittock ST et al: Mayer-Rokitansky-Kuster-Hauser anomaly and its associated malformations. Am J Med Genet A. 2005
                                        15. 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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                                        Related Differential Diagnoses
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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 (llerian 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

                                                                              1. Herlin MK et al: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update. Orphanet J Rare Dis. 15(1):214, 2020
                                                                              2. Committee on Adolescent Health Care: Committee opinion: no. 562: müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 121(5):1134-7, 2013
                                                                              3. Hall-Craggs MA et al: Mayer-Rokitansky-Kuster-Hauser syndrome: diagnosis with MR imaging. Radiology. 269(3):787-92, 2013
                                                                              4. Kara T et al: MRI in the diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. Diagn Interv Radiol. 19(3):227-32, 2013
                                                                              5. Rousset P et al: Ultrasonography and MRI features of the Mayer-Rokitansky-Küster-Hauser syndrome. Clin Radiol. 68(9):945-52, 2013
                                                                              6. Yoo RE et al: Magnetic resonance evaluation of Müllerian remnants in Mayer-Rokitansky-Küster-Hauser syndrome. Korean J Radiol. 14(2):233-9, 2013
                                                                              7. Behr SC et al: Imaging of müllerian duct anomalies. Radiographics. 32(6):E233-50, 2012
                                                                              8. 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
                                                                              9. Giusti S et al: Diagnosis of a variant of Mayer-Rokitansky-Kuster-Hauser syndrome: useful MRI findings. Abdom Imaging. 36(6):753-5, 2011
                                                                              10. 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
                                                                              11. Pompili G et al: Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome. Radiol Med. 114(5):811-26, 2009
                                                                              12. Govindarajan M et al: Magnetic resonance imaging diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. J Hum Reprod Sci. 1(2):83-5, 2008
                                                                              13. Morcel K et al: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Orphanet J Rare Dis. 2:13, 2007
                                                                              14. Pittock ST et al: Mayer-Rokitansky-Kuster-Hauser anomaly and its associated malformations. Am J Med Genet A. 2005
                                                                              15. Fedele L et al: Magnetic resonance imaging in Mayer-Rokitansky-Kuster-Hauser syndrome. Obstet Gynecol. 76(4):593-6, 1990