Two forms of Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome are recognized: isolated uterovaginal agenesis and associated with extragenital malformations, including several well-recognized syndromes. Trichorhinophalangeal syndrome (TRPS) is a rare autosomal dominant condition characterized by facial dysmorphism, ectodermal and skeletal features. TRPS comprises TRPSI (caused by a heterozygous pathogenic variant in TRPS1), TRPSII (caused by contiguous gene deletion of TRPS1, RAD21, and EXT1). Genital anomalies occur particularly in TRPSII. We present a case of rare combination TRPSII with MRKH syndrome. Delayed diagnosis resulted to prolonged pain syndrome and repeated surgery. Recognition of genital anomalies in TRPS allows timely referral diagnosis and appropriate care by paediatrician and adolescent gynaecologists.

Vydelyayut dva varianta sindroma Majera–Rokitanskogo–Kyustera–Hauzera (MRKKH): tip I, pri kotorom nablyudaetsya izolirovannaya aplaziya matki i vlagalishcha, i tip II, pri kotorom imeyut mesto soputstvuyushchie ekstragenital'nye poroki razvitiya, v ramkah nekotoryh sindromal'nyh sostoyanij. Sindrom Langera–Gideona ili trihorinofalangeal'nyj sindrom (TRPS) — redkoe autosomno-dominantnoe zabolevanie, harakterizuyushcheesya licevym dicmorfizmom i anomaliyami kozhi, nogtej i volos. Vydelyayut dva tipa trihorinofalangeal'nogo sindroma: TRPSI, obuslovlennyj patogennym variantom gena TRPSI, i TRPSII, obuslovlennyj deleciej s vovlecheniem genov TRPS1, RAD21 i EXT. Kak pravilo, poroki razvitiya polovyh organov vstrechayutsya pri tipe II. Predstavleno klinicheskoe nablyudenie TRPSII v sochetanii s MRKKH. Otsrochennaya diagnostika poroka razvitiya polovyh organov privela k dlitel'nomu bolevomu sindromu u pacientki s krajne otyagoshchennym anamnezom i provedeniyu neodnokratnyh hirurgicheskih vmeshatel'stv. Svoevremennoe obnaruzhenie sochetaniya anomalij polovyh organov u devochek s TRPS pozvolyaet ne tol'ko ustanovit' diagnoz, no i okazat' kvalificirovannuyu pomoshch' s uchastiem ginekologa detej i podrostkov, s cel'yu minimizacii vozmozhnyh oslozhnenij.

Anti-Mullerian hormone (AMH) is well known as one of the key factors in reproductive development and the formation of sexual characteristics in the embryonic period in both sexes. In women, AMH is produced by granulosa cells of the preantral and early antral follicles of the ovaries and is a key biochemical marker of ovarian reserve. Recently, the role of AMH and its transmembrane receptor AMHRII as possible pathogenetic links in a number of gynecological diseases has been actively studied. The ability of AMH to cause regression of the Müllerian duct in male embryos suggests its inhibitory role for a number of benign and malignant gynecological tumors, as well as endometriosis. In this connection, active scientific research in this direction is currently underway. A number of studies have shown that AMH causes apoptosis of human endometrial stromal cells and endometriosis cells in vitro, and is also involved in the development of autophagy processes in endometriosis. The above studies demonstrate the important role of AMH in cell apoptosis in endometriosis, and indicate its therapeutic potential for a wide range of gynecological diseases. It is important to note that AMH, as a representativemember of the TGF-β superfamily, has high affinity and specificity for the AMHRII receptor, which. This fact makes further study of the function of AMH and AMHRII relevant both for assessing their effectinfluence on the processes of folliculogenesis, and reproductive aging processes, and for developing new targeting targeted therapy strategies therapy for a wide range of gynecological diseases, including endometriosis.

The article is devoted to the problems of gynecology of children and adolescents. The historical information about the formation of specialized gynecological medical care for girls in Russia from the first thematic publications at the end of the XIX Century to modern days is given. The achievements of gynecologists in diagnostics and treatment of main diseases of reproductive system that represent major problematics within the medical community are observed as well, such as: the norm and the pathology of vaginal and vulva microbiocenosis; eligibility of establishing of a diagnoses of polycystic ovary syndrome and genital endometriosis in menstruating adolescent girls; relapses of abnormal uterine bleeding in adolescents; the quality of life and the prevention of surgical complications in adolescent girls with defects in the development of uterus and vagina; the possibility of rehabilitation of a follicular reserve in girls with premature ovarian exhaustion.

Introduction. About 30 to 50% of women with endometriosis complain of infertility. The incidence of infertility in all localizations of endometriosis is about 3-4 times higher than that in the general population. Objective of the study : estimate the hormonal profile of patients with deep infiltrative endometriosis (DIE) based on the study of the level of AMH, FSH, LH, progesterone, estradiol, prolactin in the serum of peripheral blood, conduct a comparative analysis of the level of these hormones between groups of patients with DIE and tuboperitoneal infertility. Materials and methods : the main study group consisted of 99 patients of reproductive age (from 18 to 40 years) with DIE, the comparison group consisted of 18 patients with tuboperitoneal infertility. The patients of the main group were divided into 2 subgroups: subgroup IA - DIE with colorectal endometriosis (n = 63), subgroup IB - DIE without colorectal endometriosis (n = 36). AH patients underwent determination of the level of AMH, LH, FSH, progesterone, estradiol, prolactin in the serum of peripheral blood before surgery. Results and discussion : a significant decrease in AMH level was revealed in patients with DIE. The level of AMH in the main and comparison groups was 1.80 and 3.28 ng/ml, respectively (p = 0.01). At the same time, subgroup IA has a more decreased AMH level (1.37 ng/ml) than in the comparison group (p = 0.003). In subgroup IB (the median AMH level - 2.50 ng/ml) were no significant differences with the comparison group (p = 0.14). It was also noted that in the group of patients with colorectal endometriosis (subgroup IA) the percentage of patients with reduced ovarian reserve (AMH 0.01 - 1.0 ng / ml) was significantly higher than in the comparison group (30.2% versus 5, 6%, p = 0.03), there were no significant differences between the subgroups. There were no significant differences in the level of LH, FSH, E2, prolactin in serum between the groups (p> 0.05). Assessment of the level of anti-mullerian hormone, as the most accurate marker of ovarian reserve, revealed a significant decrease in AMH levels in patients with DIE, more decreased in the group of patients with colorectal endometriosis. Every 3rd patient with DIE had an AMH level of less than 1, which is a risk factor for a “poor” response to ovarian stimulation according to the Bologna criteria. Conclusion. A significant decrease in the level of serum AMH, along with a high percentage of previous surgeries for endometriosis, including ovarian resections, may be one of the factors in a decrease in the reproductive potential of patients with DIE.

Длительно существующее перекручивание придатков матки с некрозом: важность своевременной МРТ-диагностики. Серия клинических случаевАктуальность.Перекручивание придатков матки (ППМ) занимает 5-е место среди всех неотложных гинекологических состояний.Подозрение на ППМ требует немедленной диагностики и срочного хирургического лечения.Своевременная постановка диагноза и проведение деторсии способствуют полноценному восстановлению нарушенного венозного оттока и лимфодренажа тканей яичника, препятствуя развитию выраженной ишемии и некроза.Применение современных визуальных методов диагностики представляется крайне важным инструментом в сохранении фертильности у таких пациенток

В последние годы эндометриоз признается одним из наиболее распространенных гинекологических заболеваний, ассоциированных с женским бесплодием. Механизмы влияния глубокого инфильтративного эндометриоза на репродуктивный потенциал до сих пор не до конца ясны. В настоящее время нет единого алгоритма при выборе первой линии лечения пациенток с бесплодием и колоректальным эндометриозом. Неоднозначны и мнения разных авторов в вопросе влияния хирургического лечения эндометриоза на улучшение фертильности. В связи с этим решение касательно необходимости проведения и объема хирургического лечения должно быть принято после тщательного информирования пациенток обо всех преимуществах и рисках, связанных как с выполнением оперативного вмешательства, так и с отказом от операции.

Introduction. Adenomyosis remains one of the significant challenges in modern gynecology and affects a large number of women of reproductive age. The clinical presentation of adenomyosis is variable, with severity of symptoms depending on the form of the disease. In case of the occurrence of severe pelvic pain, dysmenorrhea and dyspareunia, the disease disrupts social functions and leads to a decrease in the patient’s quality of life.Aim. To study clinical and anamnestic features and patient’s quality of life with diffuse and nodular forms of adenomyosis.Materials and methods. The study included 126 patients with various forms of adenomyosis (45 patients with nodular adenomyosis (NAM), 81 with stage III – IV DAM). The control group included 20 patients with tubo-peritoneal factor of infertility, without adenomyosis based on the ultrasound and hysteroscopy findings. All patients underwent a comprehensive examination, including general clinical, instrumental and laboratory tests. The study included a comparative analysis of the obtained data and determination of the clinical and anamnestic characteristics of the patients with stage III – IV DAM as compared with the patients with NAM. We also studied the quality of life of patients with NAM and DAM on the basis of a specialized questionnaire on the study of the quality of life of patients with endometriosis – Endometriosis Health Profile, ENR-5+6. Statistical analysis and data processing were conducted using Microsoft Excel (version 16), Statistica 10.0, StatPlus 7.3, GraphPad Prism 9 software. Data processing was performed in accordance with the guidelines for medical and biological research.Results and discussion. As compared with patients with nodular adenomyosis, the patients with stage III-IV diffuse adenomyosis are characterized by an older age (42.1 (4.6) and 34.6 (6.2) years, respectively; p <0.001), have a high prevalence of somatic (96 and 77%, respectively; p < 0.05) and gynecological pathology (79 and 51%, respectively; p < 0.001) in past medical history, a higher frequency of intrauterine manipulations (hysteroscopy, separate diagnostic curettage) (73 and 42%, respectively; p < 0.001) and medical abortions (52 and 28%, respectively, p < 0.001). Complaints of heavy menstruation, bloody vaginal discharge between periods, and intestinal symptoms had a special place among the complaints of such patients. Moderate to severe anemia, as a consequence of heavy menstrual bleeding, was also a frequent concomitant pathology.Conclusions. Stage III-IV DAM is characterized by a more severe course and marked symptoms as compared with NAM, and significantly affects the patient’s quality of life.

лечение пациенток, редактирование статьи после рецензирования, одобрение Пирогова М.М. — отбор и обследование пациенток, обработка данных; Васильченко О.Н. — отбор и обследование пациенток, обработка данных; Чупрынин В.Д. — лечение пациенток, проверка критически важного in patients with various rate of trophoblast invasion. 11(166): 29–34. DOI: 10.31550/1727-2378-2019-166-11-29-34 Materials and Methods: 98 pregnant women diagnosed with placenta ingrowth were analysed. Diagnoses: placenta accretа, placenta increta, placenta percreta were confirmed with morphological study in 28 (28,6%), 64 (65,3%) and 6 (6,1%) patients. Age, previous uterine surgeries, information about previous pregnancies, blood loss, uterine extirpation, ligation of internal iliac vessels were assessed. All pregnant women with suspected placenta ingrowth diagnosed had to undergo surgery (lower midline incision), bottom caesarean section, comprehensive compression hemostasis, metroplasty. Study Results: the cumulative blood loss has grown: in placenta accreta it was 975 mL, in placenta increta — 1,300 mL, and in placenta percreta — 2,200 mL (p = 0.048). Uterectomy was indicated in patients with more marked placenta ingrowth: 9,4% vs 33,3% (p = 0,038), and in placenta accreta it was not needed at all. The frequency of ligation of internal iliac vessels also increased with increasing depth of trophoblast invasion: 7,1%, 14,1%, 50% (p = 0,026). Conclusion: the relation between blood loss and the rate of trophoblast invasion was identified, making it possible to predict and design an optimal surgery plan for delivery by patients with placenta ingrowth. The issue needs further studying in order to improve the outcome for patients with severe pathology.

The article highlights modern ideas about the etiology and pathogenesis of pelvic prolapse, risk factors, clinical manifestations, surgical methods of correction.

Purpose of research. To analyze reproductive outcomes and relapses of endometriosis in women after surgical treatment of colorectal endometriosis depending on hormonal therapy.Materials and methods. The article includes data on reproductive outcomes and recurrence of endometriosis in women undergoing surgical treatment for colorectal endometriosis in the surgical department of Kulakov National Medical Research Centre for Obstetrics, Gynaecology and Perinatology from 2014 to 2016. As an anti-relapse therapy, the first group of women received dienogest (DNG) at a dose of 2 mg/day, the second group included women who received a combined oral contraceptive containing DNG 2 mg + ethinyl estradiol (EE) 0.03 mg for the period of rehabilitation or prior to planning of pregnancy. The comparison group consisted of women who did not receive hormonal drugs in the postoperative period. Data on side effects and tolerability of the drug are also described.Results. During the follow up period from 12 to 48 months, 51 patients were selected for this study, 14 patients were included in the first group, 18 patients were in the second group, 19 patients were in the comparison group. There were no differences in the overall rate of pregnancy depending on the choice of tactics of postoperative management. The highest number of pregnancies (92.3%) occurred during the first year after surgery, which suggests the effect of the duration of hormone therapy on the probability of pregnancy. The lowest number of relapses was registered in the group of long-term monotherapy of DNG. In the group of DNG monotherapy a greater number of side effects were noted, which in most cases did not cause significant discomfort to patients.Conclusion. Long-term administration of DNG at a dose of 2 mg/day after surgical treatment of colorectal endometriosis is an effective means of preventing relapses with good tolerability. However, in some women planning a pregnancy, it may be rational to abandon hormone therapy in favor of earlier pregnancy planning.

The article highlights the concept of the mechanisms of formation of intrauterine synechia, which represents an important medical and social problem. Its relevance is due to the violation of the reproductive function of women and in severe forms of the disease has irreversible consequences. Surgical intervention remains the leading method of treatment for such patients. However, in complex clinical situations, its application is associated with the formation of a vicious circle, aggravating the severity of the pathological process. Therefore, today, high hopes are placed on the application of methods for the prevention of the disease and its relapse. Our data on this issue allow us to optimize the use of non-surgical high-tech methods for the prevention of Asherman’s syndrome. The use of anti-adhesive resorbable gel Antiadhesin is described and its pathogenetic application is justified.

Objective - 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.

The goal is to assess the sensitivity and specificity of transvaginal ultrasound (USI) in the diagnosis of localization of foci of deep infiltrative endometriosis. Materials and methods. The study included 142 patients with deep infiltrative endometriosis, who underwent surgery in the surgical department of the V.I.Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology of the Ministry of Health of Russian Federation in the period 2014-2016. Criteria for inclusion in the study: informed agreement of the patient, the reproductive age (from 18 to 44 years), deep infiltrative endometriosis with a confirmed histological conclusion. Exclusion criteria were malignant tumors of the pelvis and abdomen, refusal to undergo surgical treatment of deep infiltrative endometriosis, and malformations of the urogenital system. Results. Transvaginal echography in identifying the localization of foci of deep infiltrative endometriosis is highly sensitive in the diagnosis of colorectal endometriosis (88.9%) and endometriosis of the bladder (96%) with a relatively low specificity of the study (35%). The sizes of endometrial infiltrates of the intestine, retrocervical endometriosis, and bladder intraoperatively were large in comparison with the data obtained with transvaginal ultrasound. When assessing the accuracy of the diagnostic method, it was found that transvaginal ultrasound evaluates retrocervical endometriosis with an accuracy of 41.5%, endometriosis of the intestine - 37.7%, bladder - 54.8%. Conclusion. Transvaginal echography, based on a quantitative assessment of the characteristic curve by calculating the area under it, the size of the infiltrate and their localization, showed that our study have middle diagnostic quality and visualization of DIE lesions with enough specify.

Among ovarian tumors, germinogenic tumors occur in 25.9% of cases, develop from the polypotent highly specialized germinogenic epithelium of the gonads. Most germinogenic tumors are mature (benign) cystic teratomas (95%), malignant germinogenic tumors occur in only 3-5% of cases. The only reasonable method of treating these tumors is surgery, which is associated with losses of the ovarian reserve. The article presents the clinical and anamnestic characteristics of girls aged 7 to 17 years with ovarian teratomas, describes the structure of the surgical interventions (access, volume of operations) with the analysis of the histological conclusion of the remote formations.

The article presents a clinical case of cryopreservation of ovarian tissue in a patient 15 years with the bulk formation of a single ovary, is made on the basis of V.I.Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology of the Ministry of Health of the Russian Federation.

Objective: to study the prevalence of different types of human papilloma virus (HPV) in cervix and anal canal in patients with HPV-associated cervical intraepithelial neoplasia (CIN) of varying severity.Material and methods: the clinical examination, colposcopy; high resolution anoscopy; cytology; typing of HPV.Results. The study involved 204 women, mean age 30±1.2 years. By results of inspection are formed 2 groups: 1 - 92 (45%) with CIN I-III, 2 - 112 (55%) without CIN. In group 1 in the cervical canal were detected HPV 16, 68 (р