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Congenital Uterine Anomalies

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Ultrasound Imaging in Reproductive Medicine

Abstract

Congenital uterine anomalies are the most common anomaly of the female reproductive tract, affecting 3–8 % of fertile and infertile women. Uterine anomalies are traditionally classified as hypoplasia or agenesis, unicornuate, didelphys, bicornuate, septate and arcuate, and diethylstilbestrol exposed. These anomalies are associated with normal reproductive outcomes and adverse reproductive outcomes such as pregnancy loss and preterm delivery and are not typically associated with infertility. Although females with obstructive uterine anomalies tend to present during adolescence due to pelvic pain or dysmenorrhea, nonobstructive uterine anomalies may not be identified until a woman presents for evaluation of first or second trimester pregnancy loss, preterm delivery, or infertility. Imaging modalities commonly employed to evaluate congenital uterine anomalies include two-dimensional ultrasonography (2DUS), three-dimensional ultrasonography (3DUS), hysterosalpingography, saline-infusion ultrasonography, and magnetic resonance imaging (MRI). As 3DUS is increasingly utilized in pelvic imaging, this modality is becoming the next level of testing after 2DUS when a uterine anomaly is suspected. For more complex congenital anomalies, additional imaging such as MRI may be warranted. Detailed imaging of the reproductive tract is essential to make the correct diagnosis of a uterine anomaly, determine if surgical intervention is recommended, and educate the patient about the reproductive implications of the diagnosis.

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Abbreviations

2DUS:

Two-dimensional ultrasonography

3DUS:

Three-dimensional ultrasonography

CT:

Computed tomography

DES:

Diethylstilbestrol

HSG:

Hysterosalpingography

MA:

Müllerian anomalies

MR:

Magnetic resonance

MRI:

Magnetic resonance imaging

MRKH:

Mayer-Rokitansky-Küster-Hauser syndrome

RPL:

Recurrent pregnancy loss

SIS:

Saline-infusion sonography

References

  1. Raga F, Bauset C, Remohi J, Bonilla-Musoles F, Simon C, Pellicer A. Reproductive impact of congenital müllerian anomalies. Hum Reprod. 1997;12:2277–81.

    Article  CAS  PubMed  Google Scholar 

  2. Simon C, Martinez L, Pardo F, Tortajada M, Pellicer A. Müllerian defects in women with normal reproductive outcome. Fertil Steril. 1991;56:1192–3.

    CAS  PubMed  Google Scholar 

  3. Grimbizis GF, Camus M, Tarlatzis BC, Bontis JN, Devroey P. Clinical implications of uterine malformations and hysteroscopic treatment results. Hum Reprod Update. 2001;7:161–74.

    Article  CAS  PubMed  Google Scholar 

  4. Saravelos SH, Cocksedge KA, Li TC. Prevalence and diagnosis of congenital uterine anomalies in women with reproductive failure: a critical appraisal. Hum Reprod Update. 2008;14:415–29.

    Article  PubMed  Google Scholar 

  5. Chan YY, Jayaprakasan K, Zamora J, Thornton JG, Raine-Fenning N, Coomarasamy A. The prevalence of congenital uterine anomalies in unselected and high-risk populations: a systematic review. Hum Reprod Update. 2011;17:761–71.

    Article  CAS  PubMed  Google Scholar 

  6. Nahum GG. Uterine anomalies: how common are they, and what is their distribution among subtypes? J Reprod Med. 1998;43:877–87.

    CAS  PubMed  Google Scholar 

  7. Kupesic S, Kurjac A, Skenderovic S, Bjelos D. Screening for uterine abnormalities by three-dimensional ultrasound improves perinatal outcomes. J Perinat Med. 2002;30:9–17.

    PubMed  Google Scholar 

  8. Propst AM, Hill JA. Anatomic factors associated with recurrent pregnancy loss. Semin Reprod Med. 2000;18:341–50.

    Article  CAS  PubMed  Google Scholar 

  9. Hammoud AO, Gibson M, Mathew Peterson C, Kerber RA, Mineau GP, Hatasaka H. Quantification of the familial contribution to Müllerian anomalies. Obstet Gynecol. 2008;111:378–84.

    Article  PubMed  Google Scholar 

  10. Oppelt P, von Have M, Paulsen M, Strissel PL, Strick R, Brucker S, et al. Female genital malformations and their associated abnormalities. Fertil Steril. 2007;87:335–42.

    Article  PubMed  Google Scholar 

  11. Troiano RN, McCarthy SM. Müllerian duct anomalies: imaging and clinical issues. Radiology. 2004;233:19–34.

    Article  PubMed  Google Scholar 

  12. Chang AS, Siegel CL, Moley KH, Ratts VS, Odem RR. Septate uterus with cervical duplication and longitudinal vaginal septum: a report of five new cases. Fertil Steril. 2004;81:1133–6.

    Article  PubMed  Google Scholar 

  13. Pavone ME, King JA, Vlahos N. Septate uterus with cervical duplication and a longitudinal vaginal septum: a müllerian anomaly without a classification. Fertil Steril. 2006;85:e9–10.

    Article  PubMed  Google Scholar 

  14. Haddad B, Louis-Sylvestre C, Poitout P, Paniel BJ. Longitudinal vaginal septum: a retrospective study of 202 cases. Eur J Obstet Gynecol Reprod Biol. 1997;74:197–9.

    Article  CAS  PubMed  Google Scholar 

  15. Ergun A, Pabuccu R, Atay V, Kücük T, Duru NK, Güngör S. Three sisters with septate uteri: another reference to bidirectional theory. Hum Reprod. 1997;12:140–2.

    Article  CAS  PubMed  Google Scholar 

  16. The American Fertility Society classification of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions. Fertil Steril. 1988;49:944–55.

    Google Scholar 

  17. Bermejo C, Ten Martinez P, Cantarero R, Diaz D, Perez Pedregosa J, Barron E, et al. Three-dimensional ultrasound in the diagnosis of Müllerian duct anomalies and concordance with magnetic resonance imaging. Ultrasound Obstet Gynecol. 2010;35:593–601.

    Article  CAS  PubMed  Google Scholar 

  18. Deutch TD, Abuhamad AZ. The role of 3-dimensional ultrasonography and magnetic resonance imaging in the diagnosis of müllerian duct anomalies: a review of the literature. J Ultrasound Med. 2008;27:413–23.

    PubMed  Google Scholar 

  19. Woelfer B, Salim R, Banerjee S, Elson J, Regan L, Jurkovic D. Reproductive outcomes in women with congenital uterine anomalies detected by three-dimensional ultrasound screening. Obstet Gynecol. 2001;98:1099–103.

    Article  CAS  PubMed  Google Scholar 

  20. Jayasinghe Y, Rane A, Stalewski H, Grover S. The presentation and early diagnosis of the rudimentary uterine horn. Obstet Gynecol. 2005;105:1456–67.

    Article  PubMed  Google Scholar 

  21. Lin PC, Bhatnagar KP, Nettleton GS, Nakajima ST. Female genital anomalies affecting reproduction. Fertil Steril. 2002;78:899–915.

    Article  PubMed  Google Scholar 

  22. Fedele L, Zamberletti D, Vercellini P, Dorta M, Candiani GB. Reproductive performance of women with unicornuate uterus. Fertil Steril. 1987;47:416–9.

    CAS  PubMed  Google Scholar 

  23. Vercellini P, Daguati R, Somigliana E, Vigano P, Lanzani A, Fedele L. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review. Fertil Steril. 2007;87:719–24.

    Article  PubMed  Google Scholar 

  24. Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril. 2007;87:918–22.

    Article  PubMed  Google Scholar 

  25. Fedele L, Ferrazzi E, Dorta M, Vercellini P, Candiani GB. Ultrasonography in the differential diagnosis of ‘double’ uteri. Fertil Steril. 1988;50:361–4.

    CAS  PubMed  Google Scholar 

  26. Candiani GB, Ferrazzi E, Fedele L, Vercellini P, Dorta M. Sonographic evaluation of uterine morphology: a new scanning technique. Acta Eur Fertil. 1986;17:345–8.

    CAS  PubMed  Google Scholar 

  27. Salim R, Jurkovic D. Assessing congenital uterine anomalies: the role of three-dimensional ultrasonography. Best Pract Res Clin Obstet Gynaecol. 2004;18:29–36.

    Article  PubMed  Google Scholar 

  28. Wu MH, Hsu CC, Huang KE. Detection of congenital müllerian duct anomalies using three-dimensional ultrasound. J Clin Ultrasound. 1997;25:487–92.

    Article  CAS  PubMed  Google Scholar 

  29. Fedele L, Bianchi S, Frontino G. Septums and synechiae: approaches to surgical correction. Clin Obstet Gynecol. 2006;49:767–88.

    Article  PubMed  Google Scholar 

  30. Nawroth F, Rahimi G, Nawroth C, Foth D, Ludwig M, Schmidt T. Is there an association between septate uterus and endometriosis? Hum Reprod. 2006;21:542–4.

    Article  PubMed  Google Scholar 

  31. Evans TN, Poland ML, Boving RL. Vaginal malformations. Am J Obstet Gynecol. 1981;141:910–20.

    CAS  PubMed  Google Scholar 

  32. Fedele L, Bianchi S, Frontino G, Ciappina N, Fontana E, Borruto F. Laparoscopic findings and pelvic anatomy in Mayer-Rokitansky-Küster-Hauser syndrome. Obstet Gynecol. 2007;109:1111–5.

    Article  PubMed  Google Scholar 

  33. Oppelt P, Renner SP, Kellermann A, Brucker S, Hauser GA, Ludwig KS, et al. Clinical aspects of Mayer-Rokitansky-Küster-Hauser syndrome: recommendations for clinical diagnosis and staging. Hum Reprod. 2006;21:792–7.

    Article  PubMed  Google Scholar 

  34. Pittock ST, Babovic-Vuksanovic D, Lteif A. Mayer-Rokitansky-Küster-Hauser anomaly and its associated malformations. Am J Med Genet. 2005;135A:314–6.

    Article  Google Scholar 

  35. Braun P, Grau FV, Pons RM, Enguix DP. Is hysterosalpingography able to diagnose all uterine malformations correctly? A retrospective study. Eur J Radiol. 2005;53:274–9.

    PubMed  Google Scholar 

  36. Mazouni C, Girard G, Deter R, Haumonte JB, Blanc B, Bretelle F. Diagnosis of müllerian anomalies in adults: evaluation of practice. Fertil Steril. 2008;89:219–22.

    Article  PubMed  Google Scholar 

  37. Sanfilippo JS, Wakim NG, Schikler KN, Yussman MA. Endometriosis in association with uterine anomaly. Am J Obstet Gynecol. 1986;154:39–43.

    Article  CAS  PubMed  Google Scholar 

  38. Acien P. Reproductive performance of women with uterine malformations. Hum Reprod. 1993;8:122–6.

    CAS  PubMed  Google Scholar 

  39. Ludmir J, Samuels P, Brooks S, Mennuti MT. Pregnancy outcome of patients with uncorrected uterine anomalies managed in a high-risk obstetric setting. Obstet Gynecol. 1990;75:906–10.

    CAS  PubMed  Google Scholar 

  40. Acien P. Incidence of müllerian defects in fertile and infertile women. Hum Reprod. 1997;12:1372–6.

    Article  CAS  PubMed  Google Scholar 

  41. Homer HA, Li TC, Cooke ID. The septate uterus: a review of management and reproductive outcome. Fertil Steril. 2000;73:1–14.

    Article  CAS  PubMed  Google Scholar 

  42. Fedele L, Bianchi S, Marchini M, Franchi D, Tozzi L, Dorta M. Ultrastructural aspects of endometrium in infertile women with septate uterus. Fertil Steril. 1996;65:750–2.

    CAS  PubMed  Google Scholar 

  43. Fedele L, Bianchi S. Hysteroscopic metroplasty for septate uterus. Obstet Gynecol Clin North Am. 1995;22:473–89.

    CAS  PubMed  Google Scholar 

  44. Andrews MC, Jones Jr HW. Impaired reproductive performance of the unicornuate uterus: intrauterine growth retardation, infertility, and recurrent abortion in five cases. Am J Obstet Gynecol. 1982;144:173–6.

    CAS  PubMed  Google Scholar 

  45. Moutos DM, Damewood MD, Schlaff WD, Rock JA. A comparison of the reproductive outcome between women with a unicornuate uterus and women with a didelphic uterus. Fertil Steril. 1992;58:88–93.

    CAS  PubMed  Google Scholar 

  46. Candiani GB, Fedele L, Zamberletti D, De Virgiliis D, Carinelli S. Endometrial patterns in malformed uteri. Acta Eur Fertil. 1983;14:311–8.

    CAS  PubMed  Google Scholar 

  47. Golan A, Langer R, Wexler S, Segev E, Niv D, David MP. Cervical cerclage: its role in the pregnant anomalous uterus. Int J Fertil. 1990;35:164–70.

    CAS  PubMed  Google Scholar 

  48. Ghi T, Casadio P, Kuleva M, Perrone AM, Savelli L, Giunchi S, et al. Accuracy of three-dimensional ultrasound in diagnosis and classification of congenital uterine anomalies. Fertil Steril. 2009;92:808–13.

    Article  PubMed  Google Scholar 

  49. Faivre E, Fernandez H, Deffieux X, Gerviase A, Frydman R, Levaillant JM. Accuracy of three-dimensional ultrasonography in differential diagnosis of septate and bicornuate uterus compared with office hysteroscopy and pelvic magnetic resonance imaging. J Minim Invasive Gynecol. 2012;19:101–6.

    Article  PubMed  Google Scholar 

  50. Reuter KL, Daly DC, Cohen SM. Septate versus bicornuate uteri: errors in imaging diagnosis. Radiology. 1989;172:749–52.

    CAS  PubMed  Google Scholar 

  51. Nicolini U, Bellotti M, Bonazzi B, Zamberletti D, Candiani GB. Can ultrasound be used to screen uterine malformations? Fertil Steril. 1987;47:89–93.

    CAS  PubMed  Google Scholar 

  52. Caliskan E, Ozkan S, Cakiroglu Y, Sarisoy HT, Corakci A, Ozeren S. Diagnostic accuracy of real-time 3D sonography in the diagnosis of congenital müllerian anomalies in high-risk patients with respect to the phase of the menstrual cycle. J Clin Ultrasound. 2010;38:123–7.

    PubMed  Google Scholar 

  53. Goldberg JM, Falcone T, Attaran M. Sonohysterographic evaluation of uterine abnormalities noted on hysterosalpingography. Hum Reprod. 1997;12:2151–3.

    Article  CAS  PubMed  Google Scholar 

  54. Troiano RN. Magnetic resonance imaging of müllerian duct anomalies of the uterus. Top Magn Reson Imaging. 2003;14:269–79.

    Article  PubMed  Google Scholar 

  55. Pellerito JS, McCarthy SM, Doyle MB, Glickman MG, DeCherney AH. Diagnosis of uterine anomalies: relative accuracy of MR imaging, endovaginal sonography, and hysterosalpingography. Radiology. 1992;183:795–800.

    CAS  PubMed  Google Scholar 

  56. Letterie GS, Haggerty M, Lindee G. A comparison of pelvic ultrasound and magnetic resonance imaging as diagnostic studies for müllerian tract abnormalities. Int J Fertil Menopausal Stud. 1995;40:34–8.

    CAS  PubMed  Google Scholar 

  57. Fedele L, Dorta M, Brioschi D, Massari C, Candiani GB. Magnetic resonance evaluation of double uteri. Obstet Gynecol. 1989;74:844–7.

    CAS  PubMed  Google Scholar 

  58. Carrington BM, Hricak H, Nuruddin RN, Secaf E, Laros Jr RK, Hill EC. Müllerian duct anomalies: MR imaging evaluation. Radiology. 1990;176:715–20.

    CAS  PubMed  Google Scholar 

  59. Jurkovic D, Geipel A, Gruboeck K, Jauniaux E, Natucci M, Campbell S. Three-dimensional ultrasound for the assessment of uterine anatomy and detection of congenital anomalies: a comparison with hysterosalpingography and two-dimensional sonography. Ultrasound Obstet Gynecol. 1995;5:233–7.

    Article  CAS  PubMed  Google Scholar 

  60. Raine-Fenning N, Fleischer AC. Clarifying the role of three-dimensional transvaginal sonography in reproductive medicine: an evidence-based appraisal. J Exp Clin Assist Reprod. 2005;2:10.

    Article  PubMed Central  PubMed  Google Scholar 

  61. Salim R, Woelfer B, Backow M, Regan L, Jurkovic D. Reproducibility of three-dimensional ultrasound diagnosis of congenital uterine anomalies. Ultrasound Obstet Gynecol. 2003;21:578–82.

    Article  CAS  PubMed  Google Scholar 

  62. Raga F, Bonilla-Musoles F, Blanes J, Osborne NG. Congenital müllerian anomalies: diagnostic accuracy of three-dimensional ultrasound. Fertil Steril. 1996;65:523–8.

    CAS  PubMed  Google Scholar 

  63. Reichman DE, Laufer MR. Congenital uterine anomalies affecting reproduction. Best Pract Res Clin Obstet Gynaecol. 2010;24:193–208.

    Article  PubMed  Google Scholar 

  64. Marcus S, al-Shawaf T, Brinsden P. The obstetric outcome of in vitro fertilization and embryo transfer in women with congenital uterine malformation. Am J Obstet Gynecol. 1996;175:85–9.

    Article  CAS  PubMed  Google Scholar 

  65. Taylor E, Gomel V. The uterus and fertility. Fertil Steril. 2008;89:1–16.

    Article  PubMed  Google Scholar 

  66. Dabirashrafi H, Bahadori M, Mohammad K, Alavi M, Moghadami-Tabrizi N, Zandinejad K, et al. Septate uterus: new idea on the histologic features of the septum in this abnormal uterus. Am J Obstet Gynecol. 1995;172:105–7.

    Article  CAS  PubMed  Google Scholar 

  67. Heinonen PK, Saarikoski S, Pystynen P. Reproductive performance of women with uterine anomalies: an evaluation of 182 cases. Acta Obstet Gynecol Scand. 1982;61:157–62.

    Article  CAS  PubMed  Google Scholar 

  68. Jacobsen LJ, DeCherney A. Results of conventional and hysteroscopic surgery. Hum Reprod. 1997;12:1376–81.

    Article  CAS  PubMed  Google Scholar 

  69. Pabuccu R, Gomel V. Reproductive outcome after hysteroscopic metroplasty in women with septate uterus and otherwise unexplained infertility. Fertil Steril. 2004;81:1675–8.

    Article  PubMed  Google Scholar 

  70. Fedele L, Arcaini L, Parazzini F, Vercellini P, Di Nola G. Reproductive prognosis after hysteroscopic metroplasty in 102 women: life-table analysis. Fertil Steril. 1993;59:768–72.

    CAS  PubMed  Google Scholar 

  71. Mollo A, De Franciscis P, Colacurci N, Cobellis L, Perino A, Venezia R, et al. Hysteroscopic resection of the septum improves the pregnancy rate of women with unexplained infertility: a prospective controlled trial. Fertil Steril. 2009;91:2628–31.

    Article  PubMed  Google Scholar 

  72. Rackow BW, Arici A. Reproductive performance of women with müllerian anomalies. Curr Opin Obstet Gynecol. 2007;19:229–37.

    Article  PubMed  Google Scholar 

  73. Choe JK, Baggish MS. Hysteroscopic treatment of septate uterus with neodymium-YAG laser. Fertil Steril. 1992;57:81–4.

    CAS  PubMed  Google Scholar 

  74. Daly DC, Maier D, Soto-Albors C. Hysteroscopic metroplasty: six years’ experience. Obstet Gynecol. 1989;73:201–5.

    CAS  PubMed  Google Scholar 

  75. Tomazevic T, Ban-Frangez H, Ribic-Pucelj M, Premru-Srsen T, Verdenik I. Small uterine septum is an important risk variable for preterm birth. Eur J Obstet Gynecol Reprod Biol. 2007;135:154–7.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Beth W. Rackow MD .

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Rackow, B.W. (2014). Congenital Uterine Anomalies. In: Stadtmauer, L., Tur-Kaspa, I. (eds) Ultrasound Imaging in Reproductive Medicine. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9182-8_9

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