Obstetrics and Gynecology

doi: 10.25005/2074-0581-2019-21-1-21-25
THE STRUCTURE OF CONGENITAL MALFORMATIONS OF THE FEMALE REPRODUCTIVE SYSTEM (ACCORDING TO THE TAJIK SRI OF OBSTETRICS, GYNECOLOGY, AND PERINATOLOGY)

F.B. Aminova1, D.A. Khodzhamurodova1,2, F.O. Kosimova1, F.I. Ibragimova1

1Department of Obstetrics and Gynecology, Institute of Postgraduate Education in Healthcare of the Republic of Tajikistan, Dushanbe, Republic of Tajikistan
2Department of Endocrinological Gynecology, Tajik Scientific Research Institute of Obstetrics, Gynecology, and Perinatology, Dushanbe, Republic of Tajikistan

Objective: To study the structure of congenital malformations of the reproductive organs in adolescent girls and women of early, and active reproductive age, according to their appeal to the Tajik Scientific Research Institute of Obstetrics, Gynecology, and Perinatology.

Methods: 182 teenage girls and women of reproductive age (from 14 to 26 years), with isolated and combined congenital malformations of the reproductive organs and anomalies of the urinary system, were examined. All patients were: history collection (mothers and relatives), general clinical (general and gynecological) examination, abdominal cavity examination, rectum, vaginal probing, endoscopic (hysteroscopy, diagnostic and therapeutic laparoscopy) study, evaluation of the condition of the mammary glands according to the Tanner scale, hormonal screening, genetic and urologist consultation, determination of sex chromatin, echographic methods for examining the uterus and ovaries, magnetic resonance imaging of the pelvic organs.

Results: In 29 (15.9%) patients noted different types of vagina malformations with impaired menstrual blood flow; congenital anomaly of the body and cervix were in 86 (47.3%) patients (saddle uterus – 43 (23.6%), two-horned uterus – in 23 (12.6%), single-horned uterus – 8 (4.4%), complete doubling of the uterus and cervix – in 7 (3.8%) and rudimentary functioning uterus – in 5 (2.7%) cases). Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome was detected in 22 (12.1%) patients, while in all cases female sex chromatin (46, XX) was diagnosed, 5 (2.7%) patients suffered from female false hermaphroditism and 4 (2.2%) – masculine false hermaphroditism. In 36 (19.8%) patients, genital malformations were combined with anomalies of the urinary system

Conclusions: Identified various forms of congenital malformations of reproductive organs, clinically manifested with the onset of menarche, or – sexual life. In the structure of these defects, the developmental anomaly of the uterus and cervix prevailed over other congenital malformations and accounted for 47.3% of cases.

Keywords: Congenital malformations of the vagina and uterus,, atresia of the hymen, violation of gender identification.

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References
  1. Adamyan LV, Farkhat KN, Makiyan ZN. Kompleksnyy podkhod k diagnostike, khirurgicheskoy korrektsii i reabilitatsii bol’nykh pri sochetanii anomaliy razvitiya matki i vlagalishcha s endometriozom [An integrated approach to the diagnosis, surgical correction and rehabilitation of patients with a combination of developmental anomalies of the uterus and vagina with endometriosis]. Problemy reproduktsii. 2016;22(3):84-90.
  2. Acién P, Acién M. The presentation and management of complex female genital malformations. Hum Reprod. 2016;22(1):48-69.
  3. Adamyan LV, Popryadukhin AYu, Arakelyan AS, Kozachenko IF, Farkhat KN. Aplaziya matki i vlagalishcha (sindrom Mayera-Rokitanskogo-KyusteraKhauzera) v sochetanii s endometriozom: nereshyonnye aspekty etiologii i patogeneza (obzor literatury) [Aplasia of the uterus and vagina (MayerRokitansky-Kuster-Hauser syndrome) in combination with endometriosis: unresolved aspects of etiology and pathogenesis (literature review)]. Problemy reproduktsii. 2016;22(3):8-14.
  4. Adamyan LV, Bogdanova EA, Stepanyan AA. Okulov AB, Glybina TM, Makiyan ZN, i dr. Anomalii razvitiya zhenskikh polovykh organov: voprosy identifikatsii i klassifikatsii (obzor literatury) [Anomalies of the development of the female genital organs: issues of identification and classification (literature review)]. Problemy reproduktsii. 2010;16(2):7-15.
  5. Bobkova MV, Baranova EE, Adamyan LV. Geneticheskie aspekty formirovaniya aplazii vlagalishcha i matki: istoriya izucheniya [Genetic aspects of the formation of aplasia of the vagina and uterus: a history of study]. Problemy reproduktsii. 2015;3:10-5.
  6. Behunova J, Klimcakova L, Podracka L. Urinary tract anomalies associated with MTHFR gene polymorphism C677T in girls. Kidney & Blood Pressure Research. 2011;34(6):465-71.
  7. Connor ОM. The treatment of intersex and the problem of delay: the Australian senate inquiry into intersex surgery and conflicting human rights for children. J Law Med. 2016;3:531-43.
  8. De Leo V, Musacchio MC, Di Sabatino A, Tosti C, Morgante G, Petraglia F. Present and future of recombinant gonadotropins in reproductive medicine. Curr Pharm Biotechnol. 2012;13(3):379-91.
  9. Khodzhamurodova DA, Khayridinova SS, Narzullaeva ZR, Kosimova SI. Sindrom polikistoznykh yaichnikov u zhenshchin s besplodiem, diagnostika kliniko-gormonal’nykh i novykh ekhograficheskikh kriteriev [Polycystic ovary syndrome in women with infertility, diagnosis of clinical and hormonal and new sonographic criteria]. Vesnik Avitsenny [Avicenna Bulletin]. 2015;3:47-50.
  10. Mustafaeva IR, Alieva EM, Asadova ShSh. Osobennosti patologicheskikh protsessov, metody ikh korrektsii u devochek v periode polovogo sozrevaniya v usloviyakh g. Nakhichevani [Features of pathological processes, methods for their correction in girls in the period of puberty in the conditions of the city of Nakhichevan]. Vesnik Avitsenny [Avicenna Bulletin]. 2010;4:66-71.
  11. Layman LC. The genetic basis of female reproductive disorders: etiology and clinical testing. Molecular and Cellular Endocrinology. 2013;370(1- 2):138-48.
  12. Montgomery GW, Zondervan KT, Nyholt DR. The future for genetic studies in reproduction. Molecular Human Reproduction. 2014;20(1):1-14.
  13. Nakhal RS, Creighton SM. Management of vaginal agenesis. Journal of Pediatric and Adolescent Gynecology. 2012;25(6):352-7.
  14. Nodale C, Ceccarelli S, Giuliano M, Cammarota M, D’Amici S, Vescarelli E. et al. Gene expression profile of patients with Mayer-Rokitansky-KusterHauser syndrome: new insights into the potential role of developmental pathways. PloS ONE. 2014;9(3):1010-3.
  15. Oppelt PG, Lermann J, Strick R, Dittrich R, Strissel P, Rettig I, et al. Malformations in a cohort of 284 women with Mayer-Rokitansky-KustnerHauser syndrome (MRKH). Reproductive Biology and Endocrinology. 2012;10:57-8.
  16. Swiatkowska-Freund M, Preis K. Elastography of the uterine cervix: implications for success of induction of labor. Ultrasound Obstet Gynecol. 2011;38:52-6.
  17. Sultan C, Biason-Lauber A, Philibert P. Mayer-Rokitansky-Kustner-Hauser syndrome: recent clinical and genetic findings. Gynecol Endocrinol. 2009;25(1):8-11.

Authors informations:

Aminova Farangiz Bakhtiyordzhonovna , Full-time Postgraduate Student of the Department of Obstetrics and Gynecology, Institute of Postgraduate Education in Healthcare of the Republic of Tajikistan

Khodzhamurodova Dzhamila Amonovna , Doctor of Medical Sciences, Associate Professor of the Department of Obstetrics and Gynecology, Institute of Postgraduate Education in Healthcare of the Republic of Tajikistan; Head of the Department of Endocrinological Gynecology, Tajik Scientific Research Institute of Obstetrics, Gynecology and Perinatology

Kosimova Farzona Olimdzhonovna , Full-time Postgraduate Student of the Department of Obstetrics and Gynecology, Institute of Postgraduate Education in Healthcare of the Republic of Tajikistan

Ibragimova Farangiz Ibodulloevna , Correspondence Postgraduate Student of the Department of Obstetrics and Gynecology, Institute of Postgraduate Education in Healthcare of the Republic of Tajikistan

Address for correspondence:

Aminova Farangiz Bakhtiyordzhonovna
Full-time Postgraduate Student of the Department of Obstetrics and Gynecology, Institute of Postgraduate Education in Healthcare of the Republic of Tajikistan

734026, Republic of Tajikistan, Dushanbe, Somoni Ave., 59

Tel.: +992 (985) 380700

E-mail: farangisaminova@mail.ru

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