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Müllerian Duct Anomalies in Pregnancy
Anne M. Kennedy, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Müllerian duct anomaly (MDA)
            • Definitions

              • Spectrum of congenital uterine malformations
                • Agenesis/hypoplasia (10%)
                  • Combined agenesis of uterus, cervix, and upper portion of vagina most common
                    • Presents as primary amenorrhea
                • Unicornuate uterus (20%)
                  • Single uterine horn; may have rudimentary horn
                • Uterus didelphys (5%)
                  • 2 separate, noncommunicating horns
                  • 2 cervices
                • Bicornuate uterus (10%)
                  • Concave or heart-shaped external contour
                  • 2 horns with variable fusion
                  • May have 1 cervix (bicornis unicollis) or 2 cervices (bicornis bicollis)
                • Septate uterus (55%)
                  • Normal external contour
                  • Septum may extend for variable lengths
                    • Complete (to external os) or partial
              • Arcuate uterus
                • Argued whether this should be classified as congenital anomaly or anatomic variant
                • Smooth, broad, fundal indentation into cavity with normal external contour

            IMAGING

            • Ultrasonographic Findings

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  Selected References

                                  1. Robbins JB et al: Müllerian duct anomalies: embryological development, classification, and MRI assessment. J Magn Reson Imaging. 41(1):1-12, 2015
                                  2. Sadro CT: Pictorial review of complications of uterine anomalies. Can Assoc Radiol J. 66(4):368-76, 2015
                                  3. Acién P et al: Reproductive outcome in uterine malformations with or without an associated unilateral renal agenesis. J Reprod Med. 59(1-2):69-75, 2014
                                  4. Dreisler E et al: Müllerian duct anomalies diagnosed by saline contrast sonohysterography: prevalence in a general population. Fertil Steril. 102(2):525-9, 2014
                                  5. Paradisi R et al: The techniques and outcomes of hysteroscopic metroplasty. Curr Opin Obstet Gynecol. 26(4):295-301, 2014
                                  6. Lewis AD et al: Pregnancy complications in women with uterine duplication abnormalities. Ultrasound Q. 26(4):193-200, 2010
                                  7. Olpin JD et al: Imaging of müllerian duct anomalies. Clin Obstet Gynecol. 52(1):40-56, 2009
                                  8. Reichman D et al: Pregnancy outcomes in unicornuate uteri: a review. Fertil Steril. 91(5):1886-94, 2009
                                  9. Shulman LP: Müllerian anomalies. Clin Obstet Gynecol. 51(2):214-22, 2008
                                  10. Erez O et al: Trial of labor and vaginal birth after cesarean section in patients with uterine Müllerian anomalies: a population-based study. Am J Obstet Gynecol. 196(6):537, 2007
                                  11. Rackow BW et al: Reproductive performance of women with müllerian anomalies. Curr Opin Obstet Gynecol. 19(3):229-37, 2007
                                  12. Troiano RN et al: Mullerian duct anomalies: imaging and clinical issues. Radiology. 233(1):19-34, 2004
                                  13. Gell JS: Mullerian anomalies. Semin Reprod Med. 21(4):375-88, 2003
                                  14. Salim R et al: Reproducibility of three-dimensional ultrasound diagnosis of congenital uterine anomalies. Ultrasound Obstet Gynecol. 21(6):578-82, 2003
                                  15. Scarsbrook AF et al: MRI appearances of mullerian duct abnormalities. Clin Radiol. 58(10):747-54, 2003
                                  16. Kupesic S et al: Screening for uterine abnormalities by three-dimensional ultrasound improves perinatal outcome. J Perinat Med. 30(1):9-17, 2002
                                  17. Homer HA et al: The septate uterus: a review of management and reproductive outcome. Fertil Steril. 73(1):1-14, 2000
                                  18. Li S et al: Association of renal agenesis and mullerian duct anomalies. J Comput Assist Tomogr. 24(6):829-34, 2000
                                  19. Propst AM et al: Anatomic factors associated with recurrent pregnancy loss. Semin Reprod Med. 18(4):341-50, 2000
                                  20. No authors listed: The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions. Fertil Steril. 49(6):944-55, 1988
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Pathology

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Müllerian duct anomaly (MDA)
                                            • Definitions

                                              • Spectrum of congenital uterine malformations
                                                • Agenesis/hypoplasia (10%)
                                                  • Combined agenesis of uterus, cervix, and upper portion of vagina most common
                                                    • Presents as primary amenorrhea
                                                • Unicornuate uterus (20%)
                                                  • Single uterine horn; may have rudimentary horn
                                                • Uterus didelphys (5%)
                                                  • 2 separate, noncommunicating horns
                                                  • 2 cervices
                                                • Bicornuate uterus (10%)
                                                  • Concave or heart-shaped external contour
                                                  • 2 horns with variable fusion
                                                  • May have 1 cervix (bicornis unicollis) or 2 cervices (bicornis bicollis)
                                                • Septate uterus (55%)
                                                  • Normal external contour
                                                  • Septum may extend for variable lengths
                                                    • Complete (to external os) or partial
                                              • Arcuate uterus
                                                • Argued whether this should be classified as congenital anomaly or anatomic variant
                                                • Smooth, broad, fundal indentation into cavity with normal external contour

                                            IMAGING

                                            • Ultrasonographic Findings

                                              • MR Findings

                                                • Imaging Recommendations

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      • Staging, Grading, & Classification

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Demographics

                                                            • Natural History & Prognosis

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  Selected References

                                                                  1. Robbins JB et al: Müllerian duct anomalies: embryological development, classification, and MRI assessment. J Magn Reson Imaging. 41(1):1-12, 2015
                                                                  2. Sadro CT: Pictorial review of complications of uterine anomalies. Can Assoc Radiol J. 66(4):368-76, 2015
                                                                  3. Acién P et al: Reproductive outcome in uterine malformations with or without an associated unilateral renal agenesis. J Reprod Med. 59(1-2):69-75, 2014
                                                                  4. Dreisler E et al: Müllerian duct anomalies diagnosed by saline contrast sonohysterography: prevalence in a general population. Fertil Steril. 102(2):525-9, 2014
                                                                  5. Paradisi R et al: The techniques and outcomes of hysteroscopic metroplasty. Curr Opin Obstet Gynecol. 26(4):295-301, 2014
                                                                  6. Lewis AD et al: Pregnancy complications in women with uterine duplication abnormalities. Ultrasound Q. 26(4):193-200, 2010
                                                                  7. Olpin JD et al: Imaging of müllerian duct anomalies. Clin Obstet Gynecol. 52(1):40-56, 2009
                                                                  8. Reichman D et al: Pregnancy outcomes in unicornuate uteri: a review. Fertil Steril. 91(5):1886-94, 2009
                                                                  9. Shulman LP: Müllerian anomalies. Clin Obstet Gynecol. 51(2):214-22, 2008
                                                                  10. Erez O et al: Trial of labor and vaginal birth after cesarean section in patients with uterine Müllerian anomalies: a population-based study. Am J Obstet Gynecol. 196(6):537, 2007
                                                                  11. Rackow BW et al: Reproductive performance of women with müllerian anomalies. Curr Opin Obstet Gynecol. 19(3):229-37, 2007
                                                                  12. Troiano RN et al: Mullerian duct anomalies: imaging and clinical issues. Radiology. 233(1):19-34, 2004
                                                                  13. Gell JS: Mullerian anomalies. Semin Reprod Med. 21(4):375-88, 2003
                                                                  14. Salim R et al: Reproducibility of three-dimensional ultrasound diagnosis of congenital uterine anomalies. Ultrasound Obstet Gynecol. 21(6):578-82, 2003
                                                                  15. Scarsbrook AF et al: MRI appearances of mullerian duct abnormalities. Clin Radiol. 58(10):747-54, 2003
                                                                  16. Kupesic S et al: Screening for uterine abnormalities by three-dimensional ultrasound improves perinatal outcome. J Perinat Med. 30(1):9-17, 2002
                                                                  17. Homer HA et al: The septate uterus: a review of management and reproductive outcome. Fertil Steril. 73(1):1-14, 2000
                                                                  18. Li S et al: Association of renal agenesis and mullerian duct anomalies. J Comput Assist Tomogr. 24(6):829-34, 2000
                                                                  19. Propst AM et al: Anatomic factors associated with recurrent pregnancy loss. Semin Reprod Med. 18(4):341-50, 2000
                                                                  20. No authors listed: The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions. Fertil Steril. 49(6):944-55, 1988