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Pediatrics
Diagnosis
Genitourinary
Uterine and Ovarian Abnormalities
Müllerian Duct Anomalies
Müllerian Duct Anomalies
Sara M. O'Hara, MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Müllerian duct anomalies (MDA)

            • Synonyms

              • Uterine fusion anomalies
              • Subtype: Obstructed hemivagina & ipsilateral renal agenesis (OHVIRA syndrome)

            • Definitions

              • Abnormal development, improper fusion, or failure of resorption of müllerian (paramesonephric) duct structures
                • Müllerian agenesis or hypoplasia (class I in American Fertility Society classification system)
                  • Complete or segmental agenesis or variable degrees of uterovaginal hypoplasia
                • Unicornuate uterus (class II)
                  • Partial or complete unilateral hypoplasia
                • Uterus didelphys (class III)
                  • Duplication of uterus
                • Bicornuate uterus (class IV)
                  • Incomplete fusion of superior uterovaginal canal
                • Septate uterus (class V)
                  • Incomplete resorption of uterine septum
                • Arcuate uterus (class VI)
                  • Near-complete resorption of uterine septum

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Radiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Habiba M et al: The development of the human uterus: morphogenesis to menarche. Hum Reprod Update. 27(1):1-26, 2021
                                    2. O'Flynn O'Brien KL et al: The prevalence of müllerian anomalies in women with a diagnosed renal anomaly. J Pediatr Adolesc Gynecol. 34(2):154-60, 2020
                                    3. Pitot MA et al: Müllerian duct anomalies coincident with endometriosis: a review. Abdom Radiol (NY). 45(6):1723-40, 2020
                                    4. Yen CF et al: Laparoscopic metroplasty: reconstructive surgery for unicornuate uterus with noncommunicating, functional uterine horn. Fertil Steril. 114(5):1119-21, 2020
                                    5. Ludwin A et al: Septate uterus according to ESHRE/ESGE, ASRM and CUME definitions: association with infertility and miscarriage, cost and warnings for women and healthcare systems. Ultrasound Obstet Gynecol. 54(6):800-14, 2019
                                    6. Vilanova-Sanchez A et al: Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review. J Pediatr Adolesc Gynecol. 32(1):7-14, 2019
                                    7. Jegannathan D et al: Magnetic resonance imaging of classified and unclassified Müllerian duct anomalies: comparison of the American Society for Reproductive Medicine and the European Society of Human Reproduction and Embryology classifications. SA J Radiol. 22(1):1259, 2018
                                    8. Choussein S et al: Mullerian dysgenesis: a critical review of the literature. Arch Gynecol Obstet. 295(6):1369-81, 2017
                                    9. Olpin JD et al: MR imaging of müllerian fusion anomalies. Magn Reson Imaging Clin N Am. 25(3):563-75, 2017
                                    10. Ludwin A et al: Reliability of the European Society of Human Reproduction and Embryology/European Society for Gynaecological Endoscopy and American Society for Reproductive Medicine classification systems for congenital uterine anomalies detected using three-dimensional ultrasonography. Fertil Steril. 104(3):688-97.e8, 2015
                                    11. Schlomer B et al: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome should be redefined as ipsilateral renal anomalies: cases of symptomatic atrophic and dysplastic kidney with ectopic ureter to obstructed hemivagina. J Pediatr Urol. 11(2):77.e1-6, 2015
                                    12. Santos XM et al: The utility of ultrasound and magnetic resonance imaging versus surgery for the characterization of müllerian anomalies in the pediatric and adolescent population. J Pediatr Adolesc Gynecol. 25(3):181-4, 2012
                                    13. Marcal L et al: Mullerian duct anomalies: MR imaging. Abdom Imaging. 36(6):756-64, 2011
                                    14. Mane SB et al: Our 10-year experience of variable Müllerian anomalies and its management. Pediatr Surg Int. 26(8):795-800, 2010
                                    15. Vallerie AM et al: Update in Müllerian anomalies: diagnosis, management, and outcomes. Curr Opin Obstet Gynecol. 22(5):381-7, 2010
                                    16. Chandler TM et al: Mullerian duct anomalies: from diagnosis to intervention. Br J Radiol. 82(984):1034-42, 2009
                                    17. Junqueira BL et al: Müllerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. Radiographics. 29(4):1085-103, 2009
                                    18. Capito C et al: Pitfalls in the diagnosis and management of obstructive uterovaginal duplication: a series of 32 cases. Pediatrics. 122(4):e891-7, 2008
                                    19. Orazi C et al: Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. sonographic and MR findings in 11 cases. Pediatr Radiol. 37(7):657-65, 2007
                                    20. Troiano RN et al: Mullerian duct anomalies: imaging and clinical issues. Radiology. 233(1):19-34, 2004
                                    21. Elford KJ et al: The forgotten female: Pediatric and adolescent gynecological concerns and their reproductive consequences. J Pediatr Adolesc Gynecol. 15(2):65-77, 2002
                                    22. Lang IM et al: MR imaging of paediatric uterovaginal anomalies. Pediatr Radiol. 29(3):163-70, 1999
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Müllerian duct anomalies (MDA)

                                              • Synonyms

                                                • Uterine fusion anomalies
                                                • Subtype: Obstructed hemivagina & ipsilateral renal agenesis (OHVIRA syndrome)

                                              • Definitions

                                                • Abnormal development, improper fusion, or failure of resorption of müllerian (paramesonephric) duct structures
                                                  • Müllerian agenesis or hypoplasia (class I in American Fertility Society classification system)
                                                    • Complete or segmental agenesis or variable degrees of uterovaginal hypoplasia
                                                  • Unicornuate uterus (class II)
                                                    • Partial or complete unilateral hypoplasia
                                                  • Uterus didelphys (class III)
                                                    • Duplication of uterus
                                                  • Bicornuate uterus (class IV)
                                                    • Incomplete fusion of superior uterovaginal canal
                                                  • Septate uterus (class V)
                                                    • Incomplete resorption of uterine septum
                                                  • Arcuate uterus (class VI)
                                                    • Near-complete resorption of uterine septum

                                              IMAGING

                                              • General Features

                                                • Ultrasonographic Findings

                                                  • MR Findings

                                                    • Radiographic Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Habiba M et al: The development of the human uterus: morphogenesis to menarche. Hum Reprod Update. 27(1):1-26, 2021
                                                                      2. O'Flynn O'Brien KL et al: The prevalence of müllerian anomalies in women with a diagnosed renal anomaly. J Pediatr Adolesc Gynecol. 34(2):154-60, 2020
                                                                      3. Pitot MA et al: Müllerian duct anomalies coincident with endometriosis: a review. Abdom Radiol (NY). 45(6):1723-40, 2020
                                                                      4. Yen CF et al: Laparoscopic metroplasty: reconstructive surgery for unicornuate uterus with noncommunicating, functional uterine horn. Fertil Steril. 114(5):1119-21, 2020
                                                                      5. Ludwin A et al: Septate uterus according to ESHRE/ESGE, ASRM and CUME definitions: association with infertility and miscarriage, cost and warnings for women and healthcare systems. Ultrasound Obstet Gynecol. 54(6):800-14, 2019
                                                                      6. Vilanova-Sanchez A et al: Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review. J Pediatr Adolesc Gynecol. 32(1):7-14, 2019
                                                                      7. Jegannathan D et al: Magnetic resonance imaging of classified and unclassified Müllerian duct anomalies: comparison of the American Society for Reproductive Medicine and the European Society of Human Reproduction and Embryology classifications. SA J Radiol. 22(1):1259, 2018
                                                                      8. Choussein S et al: Mullerian dysgenesis: a critical review of the literature. Arch Gynecol Obstet. 295(6):1369-81, 2017
                                                                      9. Olpin JD et al: MR imaging of müllerian fusion anomalies. Magn Reson Imaging Clin N Am. 25(3):563-75, 2017
                                                                      10. Ludwin A et al: Reliability of the European Society of Human Reproduction and Embryology/European Society for Gynaecological Endoscopy and American Society for Reproductive Medicine classification systems for congenital uterine anomalies detected using three-dimensional ultrasonography. Fertil Steril. 104(3):688-97.e8, 2015
                                                                      11. Schlomer B et al: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome should be redefined as ipsilateral renal anomalies: cases of symptomatic atrophic and dysplastic kidney with ectopic ureter to obstructed hemivagina. J Pediatr Urol. 11(2):77.e1-6, 2015
                                                                      12. Santos XM et al: The utility of ultrasound and magnetic resonance imaging versus surgery for the characterization of müllerian anomalies in the pediatric and adolescent population. J Pediatr Adolesc Gynecol. 25(3):181-4, 2012
                                                                      13. Marcal L et al: Mullerian duct anomalies: MR imaging. Abdom Imaging. 36(6):756-64, 2011
                                                                      14. Mane SB et al: Our 10-year experience of variable Müllerian anomalies and its management. Pediatr Surg Int. 26(8):795-800, 2010
                                                                      15. Vallerie AM et al: Update in Müllerian anomalies: diagnosis, management, and outcomes. Curr Opin Obstet Gynecol. 22(5):381-7, 2010
                                                                      16. Chandler TM et al: Mullerian duct anomalies: from diagnosis to intervention. Br J Radiol. 82(984):1034-42, 2009
                                                                      17. Junqueira BL et al: Müllerian duct anomalies and mimics in children and adolescents: correlative intraoperative assessment with clinical imaging. Radiographics. 29(4):1085-103, 2009
                                                                      18. Capito C et al: Pitfalls in the diagnosis and management of obstructive uterovaginal duplication: a series of 32 cases. Pediatrics. 122(4):e891-7, 2008
                                                                      19. Orazi C et al: Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. sonographic and MR findings in 11 cases. Pediatr Radiol. 37(7):657-65, 2007
                                                                      20. Troiano RN et al: Mullerian duct anomalies: imaging and clinical issues. Radiology. 233(1):19-34, 2004
                                                                      21. Elford KJ et al: The forgotten female: Pediatric and adolescent gynecological concerns and their reproductive consequences. J Pediatr Adolesc Gynecol. 15(2):65-77, 2002
                                                                      22. Lang IM et al: MR imaging of paediatric uterovaginal anomalies. Pediatr Radiol. 29(3):163-70, 1999