MAYER-ROKITANSKY-KUSTER-HAUSER SYNDROME (CLINICAL CASE)
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Abstract
The Mayer-Rokitansky-Custer-Hauser syndrome (SMRCX) is a violation of the intrauterine development of the Pmuller ducts, leading to aplasia of the vagina and uterus. It is diagnosed in 1 out of 4500-5000 newborn girls. The etiology of the syndrome has not been sufficiently studied. According to world statistics, the role of hereditary factors in the formation of malformations of the reproductive system ranges from 10 to 25 %.
This article describes a rare clinical case of MRC syndrome. Considering the optimal chosen management tactics of the patient, a favorable outcome was obtained for the woman.
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ACOG Committee Opinion Summary No. 728. Summary: Mullerian agenesis: diagnosis, management, and treatment. Obstet Gynecol. 2018; 131(1): 196-197. DOI: 10.1097/AOG.0000000000002452
Londra L, Chuong FS, Kolp L. Mayer-Rokitansky-Kuster-Hauser syndrome: a review. Int J Womens Health. 2015; 7: 865-870. DOI: 10.2147/IJWH.S75637
Willemsen WN, Kluivers KB. Long-term results of vaginal construction with the use of Frank dilation and a peritoneal graft (Davydov procedure) in patients with Mayer-Rokitansky-Kuster syndrome. Fertil Steril. 2015; 103(1): 220-227. DOI: 10.1016/j.fertnstert.2014.10.014
Paluch L, Nawrocka-Laskus E, Wieczorek J, Mruk B, Frel M, Walecki J. Use of ultrasound elastography in the assessment of the musculoskeletal system. Pol J Radiol. 2016; 81: 240-246. DOI: 10.12659/PJR.896099
Nowicki A, Dobruch-Sobczak K. Introduction to ultrasound elastography. J Ultrason. 2016; 16(65): 113-124. DOI: 10.15557/JoU.2016.0013
Benedetti PP, Maffucci D, Ceccarelli S, Vescarelli E, Perniola G, Muzii L, Marchese C. Autologous in vitro cultured vaginal tissue for vaginoplasty in women with Mayer-Rokitansky-Kuster-Hauser syndrome: anatomic and functional results. Minim Invasive Gynecol. 2015; 22(2): 205-211. DOI: 10.1016/j.jmig.2014.09.012
Torres-de la Roche LA, Devassy R, Gopalakrishnan S, de Wilde MS, Herrmann A, Larbig A, De Wilde RL. Plastic neo-vaginal construction in Mayer-Rokitansky-Küster-Hauser syndrome: an expert opinion paper on the decision-making treatment process. GMS Interdiscip Plast Reconstr Surg DGPW. 2016; 5: 1-5. DOI: 10.3205/iprs000087
Nodale C, Vescarelli E, D’Amici S, Maffucci D, Ceccarelli S, Monti M, et al. Characterization of human vaginal mucosa cells for autologous in vitro cultured vaginal tissue transplantation in patients with MRKH syndrome. Biomed Res Int. 2014; 2014: 201518. DOI: 10.1155/2014/201518
Khoder WY, Stief CG, Burgmann M, Burges A. Laparoscopic reconstruction of an iatrogenic perforation of the neovagina and urinary bladder by a neovaginal dilator in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome. Int Urogynecol J. 2015; 26(7): 1083-1087. DOI: 10.1007/s00192-014-2609-1
Ushakova GA, Yelgina SI. Methodological approach in preventing disturbances formation in girls' reproductive system. Pediatric and Adolescent Reproductive Health. 2008; (6): 29-35. Russian (Ушакова Г.А., Елгина С.И. Методология и организация профилактики нарушений формирования репродуктивной системы девочек //Репродуктивное здоровье детей и подростков. 2008. № 6. С. 29-35)
Hayashida SA, Soares JM, Costa EM, da Fonseca AM, Maciel GAR, Mendonça BB, Baracat EC. The clinical, structural, and biological features of neovaginas: a comparison of the Frank and the McIndoe techniques. Eur J Obstet Gynecol Reprod Biol. 2015; 186: 12-16. DOI: 10.1016/j.ejogrb.2014.12.025
Uvarova EV, Davtyan GM, Buralkina NA, Kruglyak DA. Vagina formation by a complex non-surgical colpopoiesis in patients with Mayer-Rokitansky-Kustner-Hauser syndrome. Pediatric and adolescent reproductive health. 2014; (6): 40-49. Russian (Уварова Е.В., Давтян Г.М., Буралкина Н.А., Кругляк Д.А. Формирование неовлагалища путем комплексного неоперативного кольпопоэза у пациенток с синдромом Майера-Рокитанского-Кюстнера-Хаузера //Репродуктивное здоровье детей и подростков. 2014. № 6. C. 40-49)
Veropotvelyan PN, Tsehmistrenko IS, Veropotvelyan NP, Gatselyuk SV. Strategic view on the risk factors of genital prolapse and methods of its correction. Medical aspects of women's health. 2016; (3): 66-74. Ukrainian (Веропотвелян П.Н., Цехмистренко И.С., Веропотвелян Н.П., Гацелюк С.В. Стратегический взгляд на факторы риска пролапса гениталий и способы их коррекции //Медицинские аспекты здоровья женщины. 2016. № 3. C. 66-74)