Abstract
This is a comprehensive overview that comprises embryology, diagnosis, classification and treatment of Müllerian duct anomaly. Despite its ill effect on obstetric outcomes, a large number of patients remain undetected. Recently the roles of magnetic resonance imaging and 3D ultrasound in diagnosis are highlighted in addition to conventional ultrasound and hysterosalpingography, etc. Corrective surgery, especially for septate uterus, is proven to have dramatic effects on obstetric prognosis, therefore accurate diagnosis and proper management cannot be overemphasized in Müllerian duct anomaly.
Figures and Tables
Fig. 1
American Society for Reproductive Medicine classification system for Müllerian fusion/absorption abnormalities (From Fertil Steril 1988;49:944-55, with permission from Elsevier) [18]. DES, diethylstilbestrol.
![kjog-54-132-g001](/upload/SynapseData/ArticleImage/2021kjog/kjog-54-132-g001.jpg)
Table 1
Diagnostic methods for the Müllerian anomalies (Reprinted from Jeon, et al. Korean J Obstet Gynecol 2010;53:626-32 [12].)
![kjog-54-132-i001](/upload/SynapseData/ArticleImage/2021kjog/kjog-54-132-i001.jpg)
Table 2
Comparison of reproductive outcome before and after hysteroscopic metroplasty for the septate uterus in selected series (From Fedele, et al. Clin Obstet Gynecol 2006;49:767-88, with permission from Lippincott Williams & Wilkins, Inc) [56]
![kjog-54-132-i002](/upload/SynapseData/ArticleImage/2021kjog/kjog-54-132-i002.jpg)
References
1. Golan A, Langer R, Bukovsky I, Caspi E. Congenital anomalies of the müllerian system. Fertil Steril. 1989. 51:747–755.
2. Green LK, Harris RE. Uterine anomalies. Frequency of diagnosis and associated obstetric complications. Obstet Gynecol. 1976. 47:427–429.
3. Heinonen PK, Saarikoski S, Pystynen P. Reproductive performance of women with uterine anomalies. An evaluation of 182 cases. Acta Obstet Gynecol Scand. 1982. 61:157–162.
4. Heinonen PK, Pystynen PP. Primary infertility and uterine anomalies. Fertil Steril. 1983. 40:311–316.
5. Raga F, Bauset C, Remohi J, Bonilla-Musoles F, Simon C, Pellicer A. Reproductive impact of congenital müllerian anomalies. Hum Reprod. 1997. 12:2277–2281.
6. Acien P. Reproductive performance of women with uterine malformations. Hum Reprod. 1993. 8:122–126.
7. Grimbizis GF, Camus M, Tarlatzis BC, Bontis JN, Devroey P. Clinical implications of uterine malformations and hysteroscopic treatment results. Hum Reprod Update. 2001. 7:161–174.
8. Simon C, Martinez L, Pardo F, Tortajada M, Pellicer A. Müllerian defects in women with normal reproductive outcome. Fertil Steril. 1991. 56:1192–1193.
9. Rackow BW, Arici A. Reproductive performance of women with müllerian anomalies. Curr Opin Obstet Gynecol. 2007. 19:229–237.
10. Li S, Qayyum A, Coakley FV, Hricak H. Association of renal agenesis and müllerian duct anomalies. J Comput Assist Tomogr. 2000. 24:829–834.
11. Chandler TM, Machan LS, Cooperberg PL, Harris AC, Chang SD. Müllerian duct anomalies: from diagnosis to intervention. Br J Radiol. 2009. 82:1034–1042.
12. Jeon GH, Park YR, Shin YJ, Kim SH, Chae HD, Kim CH, et al. Clinical characteristics of women with Müllerian anomaly: twenty years of experience at Asan Medical Center. Korean J Obstet Gynecol. 2010. 53:626–632.
13. Lin PC, Bhatnagar KP, Nettleton GS, Nakajima ST. Female genital anomalies affecting reproduction. Fertil Steril. 2002. 78:899–915.
14. 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.
15. Raga F, Bonilla-Musoles F, Blanes J, Osborne NG. Congenital Müllerian anomalies: diagnostic accuracy of three-dimensional ultrasound. Fertil Steril. 1996. 65:523–528.
16. Markham SM, Parmley TH, Murphy AA, Huggins GR, Rock JA. Cervical agenesis combined with vaginal agenesis diagnosed by magnetic resonance imaging. Fertil Steril. 1987. 48:143–145.
17. Doyle MB. Magnetic resonance imaging in müllerian fusion defects. J Reprod Med. 1992. 37:33–38.
18. 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. 1988. 49:944–955.
19. Acien P, Acien M, Sanchez-Ferrer M. Complex malformations of the female genital tract. New types and revision of classification. Hum Reprod. 2004. 19:2377–2384.
20. Grimbizis GF, Campo R. Congenital malformations of the female genital tract: the need for a new classification system. Fertil Steril. 2010. 94:401–407.
21. Homer HA, Li TC, Cooke ID. The septate uterus: a review of management and reproductive outcome. Fertil Steril. 2000. 73:1–14.
22. Fedele L, Bianchi S. Hysteroscopic metroplasty for septate uterus. Obstet Gynecol Clin North Am. 1995. 22:473–489.
23. Wood EG, Batzer FR, Corson SL. Ovarian response to gonadotrophins, optimal method for oocyte retrieval and pregnancy outcome in patients with vaginal agenesis. Hum Reprod. 1999. 14:1178–1181.
24. Rock JA, Azziz R. Genital anomalies in childhood. Clin Obstet Gynecol. 1987. 30:682–696.
25. Oppelt P, Renner SP, Kellermann A, Brucker S, Hauser GA, Ludwig KS, et al. Clinical aspects of Mayer-Rokitansky-Kuester-Hauser syndrome: recommendations for clinical diagnosis and staging. Hum Reprod. 2006. 21:792–797.
26. Pittock ST, Babovic-Vuksanovic D, Lteif A. Mayer-Rokitansky-Kuster-Hauser anomaly and its associated malformations. Am J Med Genet A. 2005. 135:314–316.
27. Laufer MR. Congenital absence of the vagina: in search of the perfect solution. When, and by what technique, should a vagina be created? Curr Opin Obstet Gynecol. 2002. 14:441–444.
28. Edmonds DK. Vaginal and uterine anomalies in the paediatric and adolescent patient. Curr Opin Obstet Gynecol. 2001. 13:463–467.
29. Roberts CP, Haber MJ, Rock JA. Vaginal creation for müllerian agenesis. Am J Obstet Gynecol. 2001. 185:1349–1352.
30. Klingele CJ, Gebhart JB, Croak AJ, DiMarco CS, Lesnick TG, Lee RA. McIndoe procedure for vaginal agenesis: long-term outcome and effect on quality of life. Am J Obstet Gynecol. 2003. 189:1569–1572.
31. Karateke A, Gurbuz A, Haliloglu B, Kabaca C, Koksal N. Intestinal vaginoplasty: is it optimal treatment of vaginal agenesis? A pilot study. Surgical method of sigmoid colon vaginoplasty in vaginal agenesis. Int Urogynecol J Pelvic Floor Dysfunct. 2006. 17:40–45.
32. O'Connor JL, DeMarco RT, Pope JC 4th, Adams MC, Brock JW 3rd. Bowel vaginoplasty in children: a retrospective review. J Pediatr Surg. 2004. 39:1205–1208.
33. Communal PH, Chevret-Measson M, Golfier F, Raudrant D. Sexuality after sigmoid colpopoiesis in patients with Mayer-Rokitansky-Kuster-Hauser Syndrome. Fertil Steril. 2003. 80:600–606.
34. Hensle TW, Shabsigh A, Shabsigh R, Reiley EA, Meyer-Bahlburg HF. Sexual function following bowel vaginoplasty. J Urol. 2006. 175:2283–2286.
35. Ismail IS, Cutner AS, Creighton SM. Laparoscopic vaginoplasty: alternative techniques in vaginal reconstruction. BJOG. 2006. 113:340–343.
36. Veronikis DK, McClure GB, Nichols DH. The Vecchietti operation for constructing a neovagina: indications, instrumentation, and techniques. Obstet Gynecol. 1997. 90:301–304.
37. Giannesi A, Marchiole P, Benchaib M, Chevret-Measson M, Mathevet P, Dargent D. Sexuality after laparoscopic Davydov in patients affected by congenital complete vaginal agenesis associated with uterine agenesis or hypoplasia. Hum Reprod. 2005. 20:2954–2957.
38. Langebrekke A, Istre O, Busund B, Sponland G, Gjonnaess H. Laparoscopic assisted colpoiesis according to Davydov. Acta Obstet Gynecol Scand. 1998. 77:1027–1028.
39. Noguchi S, Nakatsuka M, Sugiyama Y, Chekir C, Kamada Y, Hiramatsu Y. Use of artificial dermis and recombinant basic fibroblast growth factor for creating a neovagina in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome. Hum Reprod. 2004. 19:1629–1632.
40. Nahum GG. Uterine anomalies. How common are they, and what is their distribution among subtypes? J Reprod Med. 1998. 43:877–887.
41. Jayasinghe Y, Rane A, Stalewski H, Grover S. The presentation and early diagnosis of the rudimentary uterine horn. Obstet Gynecol. 2005. 105:1456–1467.
42. Fedele L, Bianchi S, Agnoli B, Tozzi L, Vignali M. Urinary tract anomalies associated with unicornuate uterus. J Urol. 1996. 155:847–848.
43. Devi Wold AS, Pham N, Arici A. Anatomic factors in recurrent pregnancy loss. Semin Reprod Med. 2006. 24:25–32.
44. Heinonen PK. Complete septate uterus with longitudinal vaginal septum. Fertil Steril. 2006. 85:700–705.
45. Breech LL, Laufer MR. Müllerian anomalies. Obstet Gynecol Clin North Am. 2009. 36:47–68.
46. Miller RJ, Breech LL. Surgical correction of vaginal anomalies. Clin Obstet Gynecol. 2008. 51:223–236.
47. Roth M, Mingin G, Dharamsi N, Psooy K, Koyle M. Endoscopic ablation of longitudinal vaginal septa in prepubertal girls: a minimally invasive alternative to open resection. J Pediatr Urol. 2010. 6:464–468.
48. Cooper AR, Merritt DF. Novel use of a tracheobronchial stent in a patient with uterine didelphys and obstructed hemivagina. Fertil Steril. 2010. 93:900–903.
49. Patton PE. Anatomic uterine defects. Clin Obstet Gynecol. 1994. 37:705–721.
50. Propst AM, Hill JA 3rd. Anatomic factors associated with recurrent pregnancy loss. Semin Reprod Med. 2000. 18:341–350.
51. Rock JA, Jones HW Jr. The clinical management of the double uterus. Fertil Steril. 1977. 28:798–806.
52. Mercer CA, Long WN, Thompson JD. Uterine unification: indications and technique. Clin Obstet Gynecol. 1981. 24:1199–1216.
53. Capraro VJ, Chuang JT, Randall CL. Improved fetal salvage after metroplasty. Obstet Gynecol. 1968. 31:97–103.
54. Musich JR, Behrman SJ. Obstetric outcome before and after metroplasty in women with uterine anomalies. Obstet Gynecol. 1978. 52:63–66.
55. Lavergne N, Aristizabal J, Zarka V, Erny R, Hedon B. Uterine anomalies and in vitro fertilization: what are the results? Eur J Obstet Gynecol Reprod Biol. 1996. 68:29–34.
56. Fedele L, Bianchi S, Frontino G. Septums and synechiae: approaches to surgical correction. Clin Obstet Gynecol. 2006. 49:767–788.
57. Tulandi T, Arronet GH, McInnes RA. Arcuate and bicornuate uterine anomalies and infertility. Fertil Steril. 1980. 34:362–364.
58. March CM, Israel R. Hysteroscopic management of recurrent abortion caused by septate uterus. Am J Obstet Gynecol. 1987. 156:834–842.
59. Perino A, Mencaglia L, Hamou J, Cittadini E. Hysteroscopy for metroplasty of uterine septa: report of 24 cases. Fertil Steril. 1987. 48:321–323.
60. Daly DC, Maier D, Soto-Albors C. Hysteroscopic metroplasty: six years' experience. Obstet Gynecol. 1989. 73:201–205.
61. 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. 59:768–772.
62. Cararach M, Penella J, Ubeda A, Labastida R. Hysteroscopic incision of the septate uterus: scissors versus resectoscope. Hum Reprod. 1994. 9:87–89.
63. Pabuccu R, Atay V, Orhon E, Urman B, Ergun A. Hysteroscopic treatment of intrauterine adhesions is safe and effective in the restoration of normal menstruation and fertility. Fertil Steril. 1997. 68:1141–1143.
64. Valle RF. Hysteroscopic treatment of partial and complete uterine septum. Int J Fertil Menopausal Stud. 1996. 41:310–315.
65. Grimbizis G, Camus M, Clasen K, Tournaye H, De Munck L, Devroey P. Hysteroscopic septum resection in patients with recurrent abortions or infertility. Hum Reprod. 1998. 13:1188–1193.