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Uterine Hypoplasia/Agenesis
Akram M. Shaaban, MBBCh; Nyree Griffin, MD, FRCR; Caroline Reinhold, MD, MSc
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Müllerian agenesis, uterovaginal agenesis
          • Definitions

            • Class I müllerian duct anomaly based on Buttram and Gibbons and American Society for Reproductive Medicine revision
            • 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 (HSG)

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      Selected References

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

                                                • Müllerian agenesis, uterovaginal agenesis
                                              • Definitions

                                                • Class I müllerian duct anomaly based on Buttram and Gibbons and American Society for Reproductive Medicine revision
                                                • 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 (HSG)

                                                  • CT Findings

                                                    • MR Findings

                                                      • Ultrasonographic Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      DIAGNOSTIC CHECKLIST

                                                                      • Consider

                                                                        • Image Interpretation Pearls

                                                                          Selected References

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