• Мюллерова агенезия (синдром Майера–Рокитанского–Кустера–Хаузера — МРКХ): диагностика, менеджмент и лечение Номер 728 • Январь 2018 года (Заменяет мнение комитета в номере 562, май 2013 года)

Мюллерова агенезия (синдром Майера–Рокитанского–Кустера–Хаузера — МРКХ): диагностика, менеджмент и лечение Номер 728 • Январь 2018 года (Заменяет мнение комитета в номере 562, май 2013 года)

HEALTH OF WOMAN. 2018.3(129):132–137

Комитет по охране здоровья подростков, Североамериканское общество педиатрии и подростковой гинекологии одобряет этот документ. Этот документ был разработан Комитетом Американского колледжа акушеров и гинекологов по охране здоровья подростков в сотрудничестве с членом комитета Энн-Мари Эмис Оэлшлагер, MD.
Сокращенный вариант Адаптировано – С.А. Шурпяк

СПИСОК ЛИТЕРАТУРЫ

1. Fontana L, Gentilin B, Fedele L, Gervasini C, Miozzo M. Genetics of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Clin Genet 2017;91:233–46. https://doi.org/10.1111/cge.12883; PMid:27716927

2. Sarpel U, Palmer SK, Dolgin SE. The incidence of complete androgen insensitivity in girls with inguinal hernias and assessment of screening by vaginal length measurement. J Pediatr Surg 2005;40:133–6; discussion 136–7. https://doi.org/10.1016/j.jpedsurg.2004.09.012; PMid:15868573

3. DeebA,HughesIA.Inguinalherniainfemaleinfants:acue to check the sex chromosomes? BJU Int 2005;96:401–3. https://doi.org/10.1111/j.1464-410X.2005.05639.x; PMid:16042738

4. Patel V, Casey RK, Gomez-Lobo V. Timing of gonadectomy in patients with complete androgen insensitivity syndrome-current recommendations and future directions. J Pediatr Adolesc Gynecol 2016;29:320–5. https://doi.org/10.1016/j.jpag.2015.03.011; PMid:26428189

5. Lee PA, Nordenstrom A, Houk CP, Ahmed SF, Auchus R, Baratz A, et al. Global disorders of sex development update since 2006: perceptions, approach and care. Global DSD Update Consortium [published erratum appears in Horm Res Paediatr 2016;85:180]. Horm Res Paediatr 2016;85:158–80. https://doi.org/10.1159/000442975; PMid:26820577

6. Kim SM, Rhee JH. A case of 17 alpha-hydroxylase defi- ciency. Clin Exp Reprod Med 2015;42:72–6. https://doi.org/10.5653/cerm.2015.42.2.72; PMid:26161337 PMCid:PMC4496435

7. Costa-Santos M, Kater CE, Auchus RJ. Two prevalent CYP17 mutations and genotype-phenotype correlations in 24 Brazilian patients with 17-hydroxylase deficiency. Brazilian Congenital Adrenal Hyperplasia Multicenter Study Group. J Clin Endocrinol Metab 2004;89:49–60. https://doi.org/10.1210/jc.2003-031021; PMid:14715827

8. Preibsch H, Rall K, Wietek BM, Brucker SY, Staebler A, Claussen CD, et al. Clinical value of magnetic resonance imaging in patients with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome: diagnosis of associated malformations, uterine rudiments and intrauterine endometrium. Eur Radiol 2014;24:1621–7. https://doi.org/10.1007/s00330-014-3156-3; PMid:24737529

9. Michala L, Aslam N, Conway GS, Creighton SM. The clan- destine uterus: or how the uterus escapes detection prior to puberty. BJOG 2010;117:212–5. https://doi.org/10.1111/j.1471-0528.2009.02413.x; PMid:20002397

10. Laufer MR. Struturalab normaliti esof the femalereproductive tract. In: Emans SJ, Laufer MR, editors. Pediatric and adolescent gynecology. 6th ed. Philadelphia (PA): Wolters Kluwer; Lippincott Williams & Wilkins; 2012. p. 177–237.

11. Cho MK, Kim CH, Oh ST. Endometriosis in a patient with Rokitansky-Kuster-Hauser syndrome. J Obstet Gynaecol Res 2009;35:994–6. https://doi.org/10.1111/j.1447-0756.2009.01025.x; PMid:20149055

12. OppeltP,RennerSP,KellermannA,BruckerS,HauserGA, Ludwig KS, et al. Clinical aspects of Mayer-Rokitansky-Kuester-Hauser syndrome: recommendations for clinical diagnosis and staging. Hum Reprod 2006;21:792–7. https://doi.org/10.1093/humrep/dei381; PMid:16284062

13. Kapczuk K, Iwaniec K, Friebe Z, Kedzia W. Congenital malformations and other comorbidities in 125 women with Mayer-Rokitansky-Kuster-Hauser syndrome. Eur J Obstet Gynecol Reprod Biol 2016;207:45–9. https://doi.org/10.1016/j.ejogrb.2016.10.014; PMid:27825026

14. Rall K, Eisenbeis S, Henninger V, Henes M, Wallwiener D, Bonin M, et al. Typical and atypical associated findings in a group of 346 patients with Mayer-Rokitansky-Kuester- Hauser Syndrome. J Pediatr Adolesc Gynecol 2015;28: 362–8. https://doi.org/10.1016/j.jpag.2014.07.019; PMid:26148785

15. Breech L. Gynecologic concerns in patients with anorectal malformations. Semin Pediatr Surg 2010;19:139–45. https://doi.org/10.1053/j.sempedsurg.2009.11.019; PMid:20307850

16. Patterson CJ, Crawford R, Jahoda A. Exploring the psychological impact of Mayer-Rokitansky-Kuster-Hauser syndrome on young women: an interpretative phenom- enological analysis. J Health Psychol 2016;21:1228–40. https://doi.org/10.1177/1359105314551077; PMid:25293965

17. Ernst ME, Sandberg DE, Keegan C, Quint EH, Lossie AC, Yashar BM. The lived experience of MRKH: sharing health information with peers. J Pediatr Adolesc Gynecol 2016;29:154–8. https://doi.org/10.1016/j.jpag.2015.09.009; PMid:26453829

18. PolandML, EvansTN.Psychologicaspectsofvaginalagen- esis. J Reprod Med 1985;30:340–4. PMid:4009551

19. Friedler S, Grin L, Liberti G, Saar-Ryss B, Rabinson Y, Meltzer S. The reproductive potential of patients with Mayer-Rokitansky-Kuster-Hauser syndrome using ges- tational surrogacy: a systematic review. Reprod Biomed Online 2016;32:54–61. https://doi.org/10.1016/j.rbmo.2015.09.006; PMid:26626805

20. PetrozzaJC,GrayMR,DavisAJ,ReindollarRH.Congenital absence of the uterus and vagina is not commonly transmitted as a dominant genetic trait: outcomes of surrogate pregnancies. Fertil Steril 1997;67:387–9. https://doi.org/10.1016/S0015-0282(97)81927-9

21. Carson SA, Simpson JL, Malinak LR, Elias S, Gerbie AB, Buttram VC Jr, et al. Heritable aspects of uterine anomalies. II. Genetic analysis of müllerian aplasia. Fertil Steril 1983;40:86–90. https://doi.org/10.1016/S0015-0282(16)47182-7

22. Brannstrom M, Johannesson L, Bokstrom H, Kvarnstrom N, Molne J, Dahm­Kahler P, et al. Livebirth after uterus transplantation. Lancet 2015;385:607–16. https://doi.org/10.1016/S0140-6736(14)61728-1; https://doi.org/10.1016/S0140-6736(15)61098-4

23. Johannesson L, Kvarnstrom N, Molne J, Dahm­Kahler P, Enskog A, Diaz­Garcia C, et al. Uterus transplantation trial: 1­year outcome. Fertil Steril 2015;103:199–204. https://doi.org/10.1016/j.fertnstert.2014.09.024; PMid:25439846

24. Willemsen WN, Kluivers KB. Long­term results of vaginal construction with the use of Frank dilation and a peritoneal graft (Davydov procedure) in patients with Mayer­ Rokitansky­Kuster syndrome. Fertil Steril 2015;103: 220–7.e1. https://doi.org/10.1016/j.fertnstert.2014.10.014; PMid:25455533

25. Gargollo PC, Cannon GM Jr, Diamond DA, Thomas P, Burke V, Laufer MR. Should progressive perineal dilation be considered first line therapy for vaginal agenesis? J Urol 2009;182:1882–9. https://doi.org/10.1016/j.juro.2009.03.071; PMid:19695600

26. Roberts CP, Haber MJ, Rock JA. Vaginal creation for mül­ lerian agenesis. Am J Obstet Gynecol 2001;185:1349–52; discussion 1352–3. https://doi.org/10.1067/mob.2001.119075; PMid:11744908

27. Edmonds DK, Rose GL, Lipton MG, Quek J. Mayer­ Rokitansky­Kuster­Hauser syndrome: a review of 245 consecutive cases managed by a multidisciplinary approach with vaginal dilators. Fertil Steril 2012;97:686–90. https://doi.org/10.1016/j.fertnstert.2011.12.038; PMid:22265001

28. Patel V, Hakim J, Gomez­Lobo V, Oelschlager AA. Providers’ experiences with vaginal dilator training for patients with vaginal agenesis. J Pediatr Adolesc Gynecol 2017. Available at: http://www.sciencedirect.com/science/ article/pii/S1083318817302656. Retrieved September 14, 2017.

29. Adeyemi­Fowode OA, Dietrich JE. Assessing the experience of vaginal dilator use and potential barriers to ongoing use among a focus group of women with Mayer­ Rokitansky­Kuster­Hauser Syndrome. J Pediatr Adolesc Gynecol 2017;30:491–4. https://doi.org/10.1016/j.jpag.2017.02.002; PMid:28216131

30. Oelschlager AM, Debiec K, Appelbaum H. Primary vaginal dilation for vaginal agenesis: strategies to anticipate chal­ lenges and optimize outcomes. Curr Opin Obstet Gynecol 2016;28:345–9. https://doi.org/10.1097/GCO.0000000000000302; PMid:27454852

31. Callens N, De Cuypere G, De Sutter P, Monstrey S, Weyers S, Hoebeke P, et al. An update on surgical and non­surgical treatments for vaginal hypoplasia. Hum Reprod Update 2014;20:775–801. https://doi.org/10.1093/humupd/dmu024; PMid:24899229

32. McVearry ME, Warner WB. Use of physical therapy to augment dilator treatment for vaginal agenesis. Female Pelvic Med Reconstr Surg 2011;17:153–6. https://doi.org/10.1097/SPV.0b013e31821bcd83; PMid:22453790

33. Moen MH. Vaginal agenesis treated by coital dilatation in 20 patients. Int J Gynaecol Obstet 2014;125:282–3. https://doi.org/10.1016/j.ijgo.2014.01.007; PMid:24630858

34. Callens N, Weyers S, Monstrey S, Stockman S, van Hoorde B, van Hoecke E, et al. Vaginal dilation treatment in women with vaginal hypoplasia: a prospective one­year follow­up study. Am J Obstet Gynecol 2014;211:228.e1–12. https://doi.org/10.1016/j.ajog.2014.03.051; PMid:24681288

35. Michala L, Strawbridge L, Bikoo M, Cutner AS, Creighton SM. Lower urinary tract symptoms in women with vaginal agenesis. Int Urogynecol J 2013;24:425–9. https://doi.org/10.1007/s00192-012-1870-4; PMid:22797462

36. 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;14:441–4. https://doi.org/10.1097/00001703-200210000-00001; PMid:12401969

37. Brucker SY, Gegusch M, Zubke W, Rall K, Gauwerky JF, Wallwiener D. Neovagina creation in vaginal agenesis: development of a new laparoscopic Vecchietti­based procedure and optimized instruments in a prospective comparative interventional study in 101 patients. Fertil Steril 2008;90:1940–52. https://doi.org/10.1016/j.fertnstert.2007.08.070; PMid:18061172

38. Borruto F, Chasen ST, Chervenak FA, Fedele L. The Vecchietti procedure for surgical treatment of vaginal agenesis: comparison of laparoscopy and laparotomy. Int J Gynaecol Obstet 1999;64:153–8. https://doi.org/10.1016/S0020-7292(98)00244-6

39. AdamyanLV.Laparoscopicmanagementofvaginalaplasia with or without functional noncommunicating rudimentary uterus. In: Arregui ME, Fitzgibbons RJ Jr, Katkhouda N, McKernan JB, Reich H, editors. Principles of laparoscopic surgery: basic and advanced techniques. New York (NY): Springer­Verlag; 1995. p. 646–51.

40. Davydov SN, Zhvitiashvili OD. Formation of vagina (col­ popoiesis) from peritoneum of Douglas pouch. Acta Chir Plast 1974;16:35–41.

41. Adamyan LV. Therapeutic and endoscopic perspectives. In: Nichols DH, Clarke­Pearson DL, editors. Gynecologic, obstetric, and related surgery. 2nd ed. St. Louis (MO): Mosby; 2000. p. 1209–17.

42. Allen LM, Lucco KL, Brown CM, Spitzer RF, Kives S. Psychosexual and functional outcomes after creation of a neovagina with laparoscopic Davydov in patients with vaginal agenesis. Fertil Steril 2010;94:2272–6. https://doi.org/10.1016/j.fertnstert.2010.02.008; PMid:20236638

43. Workowski KA, Bolan GA. Sexually transmitted diseases treatment guidelines, 2015. Centers for Disease Control and Prevention [published erratum appears in MMWR Recomm Rep 2015;64:924]. MMWR Recomm Rep 2015;64(RR­03):1–137. PMid:26042815 PMCid:PMC5885289

44. Frega A, Scirpa P, Sopracordevole F, Biamonti A, Bianchi P, De Sanctis L, et al. Impact of human papillomavirus infection on the neovaginal and vulval tissues of women who underwent surgical treatment for Mayer­Rokitansky­Kuster­Hauser syndrome. Fertil Steril 2011;96:969–73. https://doi.org/10.1016/j.fertnstert.2011.07.1099;PMid:21820652

45. Human papillomavirus vaccination. Committee Opinion No. 704. American College of Obstetricians and Gynecologists. Obstet Gynecol 2017;129:e173–8. https://doi.org/10.1097/AOG.0000000000002052; PMid:28346275