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Müllerian Duct Anomalies in Pregnancy
Anne 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 with 2 cervices
                • Bicornuate uterus (10%)
                  • Concave or heart-shaped external contour
                  • 2 horns with variable degree of 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 (subseptate)
                • 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. Akhtar MA et al: Reproductive implications and management of congenital uterine anomalies: scientific impact paper No. 62 November 2019. BJOG. 127(5):e1-13, 2020
                                  2. Karami M et al: The association between mullerian anomalies and IUGR: a meta-analysis. J Matern Fetal Neonatal Med. 32(14):2408-11, 2019
                                  3. Khazaei S et al: The association of mullerian anomalies and placenta abruption: a meta-analysis. J Matern Fetal Neonatal Med. 32(3):512-6, 2019
                                  4. Ludwin A et al: Reproductive surgery for müllerian anomalies: a review of progress in the last decade. Fertil Steril. 112(3):408-16, 2019
                                  5. Theodoridis TD et al: Surgical management of congenital uterine anomalies (including indications and surgical techniques). Best Pract Res Clin Obstet Gynaecol. 59:66-76, 2019
                                  6. Committee on Adolescent Health Care.: ACOG committee opinion No. 728: müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 131(1):e35-42, 2018
                                  7. Friedman MA et al: Screening for mullerian anomalies in patients with unilateral renal agenesis: leveraging early detection to prevent complications. J Pediatr Urol. 14(2):144-9, 2018
                                  8. Robbins JB et al: Müllerian duct anomalies: embryological development, classification, and MRI assessment. J Magn Reson Imaging. 41(1):1-12, 2015
                                  9. Sadro CT: Pictorial review of complications of uterine anomalies. Can Assoc Radiol J. 66(4):368-76, 2015
                                  10. 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
                                  11. Dreisler E et al: Müllerian duct anomalies diagnosed by saline contrast sonohysterography: prevalence in a general population. Fertil Steril. 102(2):525-9, 2014
                                  12. Paradisi R et al: The techniques and outcomes of hysteroscopic metroplasty. Curr Opin Obstet Gynecol. 26(4):295-301, 2014
                                  13. Lewis AD et al: Pregnancy complications in women with uterine duplication abnormalities. Ultrasound Q. 26(4):193-200, 2010
                                  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 with 2 cervices
                                                • Bicornuate uterus (10%)
                                                  • Concave or heart-shaped external contour
                                                  • 2 horns with variable degree of 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 (subseptate)
                                                • 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. Akhtar MA et al: Reproductive implications and management of congenital uterine anomalies: scientific impact paper No. 62 November 2019. BJOG. 127(5):e1-13, 2020
                                                                  2. Karami M et al: The association between mullerian anomalies and IUGR: a meta-analysis. J Matern Fetal Neonatal Med. 32(14):2408-11, 2019
                                                                  3. Khazaei S et al: The association of mullerian anomalies and placenta abruption: a meta-analysis. J Matern Fetal Neonatal Med. 32(3):512-6, 2019
                                                                  4. Ludwin A et al: Reproductive surgery for müllerian anomalies: a review of progress in the last decade. Fertil Steril. 112(3):408-16, 2019
                                                                  5. Theodoridis TD et al: Surgical management of congenital uterine anomalies (including indications and surgical techniques). Best Pract Res Clin Obstet Gynaecol. 59:66-76, 2019
                                                                  6. Committee on Adolescent Health Care.: ACOG committee opinion No. 728: müllerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 131(1):e35-42, 2018
                                                                  7. Friedman MA et al: Screening for mullerian anomalies in patients with unilateral renal agenesis: leveraging early detection to prevent complications. J Pediatr Urol. 14(2):144-9, 2018
                                                                  8. Robbins JB et al: Müllerian duct anomalies: embryological development, classification, and MRI assessment. J Magn Reson Imaging. 41(1):1-12, 2015
                                                                  9. Sadro CT: Pictorial review of complications of uterine anomalies. Can Assoc Radiol J. 66(4):368-76, 2015
                                                                  10. 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
                                                                  11. Dreisler E et al: Müllerian duct anomalies diagnosed by saline contrast sonohysterography: prevalence in a general population. Fertil Steril. 102(2):525-9, 2014
                                                                  12. Paradisi R et al: The techniques and outcomes of hysteroscopic metroplasty. Curr Opin Obstet Gynecol. 26(4):295-301, 2014
                                                                  13. Lewis AD et al: Pregnancy complications in women with uterine duplication abnormalities. Ultrasound Q. 26(4):193-200, 2010