GynecologyGynecology2079-56962079-5831Consilium Medicum3008910.26442/20795696.2018.6.180077Research ArticlePeculiarities of the genital organs in girls with aplasia of the vagina and uterus (Mayer-Rokitansky-Kyustner-Hauser syndrome)KruglyakD Adiana.kruglyak@yandex.ruMamedovaF Shf_mamedova@oparina4.ruBatyrovaZ Klinadoctor@mail.ruChupryninV Dv_chuprynin@oparina4.ruAsaturovaA Va_asaturova@oparina4.ruZaitsevN V-UvarovaE Velena-uvarova@yandex.ruLuzhinaI Amri.lab@mail.ruLunkovS Sdok-ginekolog@yandex.ruFilippovaE Ae_filippova@oparina4.ruBuralkinaN Anatalyaburalkina@yandex.ruV.I.Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology of the Ministry of Health of the Russian Federation15122018206202409042020Copyright © 2018, Consilium Medicum2018Objective - to evaluate the structural features of the external and internal genital organs in girls with aplasia of the vagina and uterus. Materials and methods. The study included 64 adolescent girls aged 15-18. The examination included a general and gynecological with an assessment of the structural features of the perineum, external genital organs and urethra, including using a specially designed measurement scheme for anatomical landmarks. An ultrasound study was performed with the aiming assessment of uterine horns and, when identifying signs of functioning, laparoscopic removal of them with histological study. In order to optimally assess the type of tissue lining the vaginal fossa zone, an analysis was carried out of the material obtained by scraping from the studied zone using liquid cytology. Results. Every third patient (34.3%) was diagnosed with urinary system pathology. In most patients, the external opening of the urethra was typical. Almost everyone had hymen (95%). Gynecological examination showed that in the vast majority of patients in the area of intended vaginal entry, was a blindly ending vaginal fossa (95.3%). The length of the fossa was on the average 1.7±0.1 cm. According to the smear scrapings from the vaginal fossa, flat cells of the superficial, intermediate, and parabasal and basal types were present. Representatives of the lactobacillus and coccal microflora were revealed. A topographic-anatomical analysis of the orientations of the perineum in girls with aplasia of the vagina showed that the depth of the vaginal fossa weakly depended from the age of the patients and did not depend on any of the suggested guide points. According to the results of instrumental methods of investigation of the pelvic organs, 68.75% of the girls had a muscular cord at the uterus place. Moreover, 22% of girls had signs of the functioning of rudimentary horns. Ovaries, as a rule, were located high, at the entrance to the small pelvis. Laparoscopy was performed in all patients with cyclic pain syndrome and closed functioning uterine rudiments, removal of the uterine horns with fallopian tubes were prefered. Histological examination of the obtained preparations was characterized by the detection of endometrial stroma and active endometrial glands. Summary. The authors emphasize that a multilateral examination of this cohort of patients is extremely important for the diagnosis and optimized treatment.patients with aplasia of the vagina and uterusvisual diagnostic methodssurgical treatmentпациентки с аплазией влагалища и маткивизуальные методы диагностикиоперативное лечение[Gell J.S. Mullerian anomalies. Semin Reprod Med 2003; 21 (4): 375-8.][Guerrier D, Mouchel T, Pasquier L, Pellerin I. The Mayer-Rokitansky-Kuster-Hauser syndrome (congenital absence of uterus and vagina) - Phenotypic manifestations and genetic approaches. J Negative Res BioMed 2006; 5 (1).][Morcel K, Camborieux L, Guerrier D. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Orphanet J Rare Dis 2007; 2: 13.][Кира Е.Ф., Политова А.К., Вязьмина К.Ю. Хирургическое лечение аплазии влагалища и шейки матки с применением робототехники. Вестн. Национального медико-хирургического Центра им. Н.И.Пирогова. 2010; 5 (2): 130-2.][Адамян Л.В., Кулаков В.И., Хашукоева А.З. Пороки развития матки и влагалища. М.: Медицина, 1998.][Вербенко А.А., Шахматова М.П. Аплазия влагалища. М.: Медицина, 1982.][Fisher K, Esham R.H, Thorneycrof I. Scolios is associated with typical l Mayer-Rokitansky-Kuster-Hauser syndrome. South Med J 2000; 93 (2): 243-6.][Мартыш Н.С. Клинико-эхографические аспекты нарушений полового развития и аномалий развития матки и влагалища у девочек. Автореф. … д-ра мед. наук. М., 1996.][Стрижакова М.А. Пороки развития влагалища и матки у девочек (клиническая лекция). Репродуктивное здоровье детей и подростков. 2005; 3: 38-44.][Уварова Е.В. Детская и подростковая гинекология. Рук-во для врачей. М.: Литтерра, 2009.][Саруханов А.Г. Отдаленные результаты кольпопоэза у подростков. Автореф. … канд. мед. наук. М., 1994; с. 22.][Угрюмова Л.Ю. Клиническая оценка эффективности различных методов кольпопоэза. Автореф. дис. … канд. мед. наук. М., 2008; с. 18.][McIndoe A. The treatment of congenital absence and obliterative conditions of the vagina. Br J Plast Surg 1950; 2 (4): 254-67.][Hayashida S.A, Soares J.M Jr, Costa E.M et al. 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: 12-6.][Reichman D.E, Laufer M.R. Congenital uterine anomalies affecting reproduction. Best Pract Res Clin Obstet Gynaecol 2010; 24 (2): 193-208.]