Modern methods of surgical treatment of obstructive genital malformations in girls

Abstract

Congenital anomalies of female genital tract in children and adolescents are more commonly detected at the age of menarche with several symptoms caused by obstruction. These diseases are rare, but without timely diagnostics and management they can cause a number of serious complications. This article is aimed to review modern approaches to diagnostics and management of this pathology and to highlight some aspects that require further research.

A literature search was conducted using the following databases: MEDLINE, Google Scholar, eLIBRARY, international guidelines and clinical recommendations. Key words that were used for search were: «congenital malformations», «distal vaginal atresia», «imperforated hymen», «OHVIRA», «transverse vaginal septum», «hematocolpos», «obstructive uterovaginal anomalies», «surgical treatment in pediatrics». International clinical trials which had been analysed by the authors were included in the review.

They have explored the current literature related to the following obstructive malformations: distal vaginal atresia, transverse vaginal septum, imperforated hymen and Herlin–Werner–Wunderlich syndrome.

Main conclusions are: further studies with a larger sample of patients, longer follow-up, long-term outcomes and effective preventive measures against complications. Another unresolved question is the age which can be recommended for surgical treatment of obstructive malformations. Potentials for developing new algorithms of earlier diagnostics are also among research priorities.

Keywords

congenital malformations / obstructive anomalies of female genital tract / surgical treatment of the congenital malformations

References

[1] Grimbizis GF, Gordts S, Di Spiezio Sardo A, et al. The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies. Human Rep (Oxford, England). 2013;28(8):2032–2044. doi: 10.1093/humrep/det098

[2] Grimbizis G.F., Gordts S., Di Spiezio Sardo A., et al. The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies // Human Rep (Oxford, England). 2013. Vol. 28, N 8. P. 2032–2044. doi: 10.1093/humrep/det098

[3] Ludwin A, Ludwin I. Comparison of the ESHRE-ESGE and ASRM classifications of Müllerian duct anomalies in everyday practice. Human Rep (Oxford, England). 2015;30(3):569–580. doi: 10.1093/humrep/deu344

[4] Ludwin A., Ludwin I. Comparison of the ESHRE-ESGE and ASRM classifications of Müllerian duct anomalies in everyday practice // Human Rep (Oxford, England). 2015. Vol. 30, N 3. P. 569–580. doi: 10.1093/humrep/deu344

[5] Acién P, Acién MI. The history of female genital tract malformation classifications and proposal of an updated system. Human Rep Update. 2011;17(5):693–705. doi: 10.1093/humupd/dmr021

[6] Acién P., Acién M.I. The history of female genital tract malformation classifications and proposal of an updated system // Human Rep Update. 2011. Vol. 17, N 5. P. 693–705. doi: 10.1093/humupd/dmr021

[7] Adamyan LV, Kuznetsova MV, Popryaduhin AY, et al. Genetic aspects of malformations of female genital organs: A modern view of the problem. Russ J Human Rep. 2023;29(4-2):23–28. EDN: JXBBWH doi: 10.17116/repro20232904223

[8] Адамян Л.В., Кузнецова М.В., Попрядухин А.Ю., и др. Генетические аспекты пороков развития женских половых органов: современный взгляд на проблему // Проблемы репродукции. 2023. Vol. 29, N 4-2. P. 23–28. EDN: JXBBWH doi: 10.17116/repro20232904223

[9] Maciel C, Bharwani N, Kubik-Huch RA, et al. MRI of female genital tract congenital anomalies: European Society of Urogenital Radiology (ESUR) guidelines. Eur Radiol. 2020;30(8):4272–4283. EDN: NTWYEB doi: 10.1007/s00330-020-06750-8

[10] Maciel C., Bharwani N., Kubik-Huch R.A., et al. MRI of female genital tract congenital anomalies: European Society of Urogenital Radiology (ESUR) guidelines // Eur Radiol. 2020. Vol. 30, N 8. P. 4272–4283. EDN: NTWYEB doi: 10.1007/s00330-020-06750-8

[11] Management of Acute Obstructive Uterovaginal Anomalies: ACOG Committee Opinion, Number 779. Obstetrics and gynecology. 2019;133(6):e363–e371. doi: 10.1097/AOG.0000000000003281

[12] Management of Acute Obstructive Uterovaginal Anomalies: ACOG Committee Opinion, Number 779 // Obstetrics Gynecology. 2019. Vol. 133, N 6. P. e363–e371. doi: 10.1097/AOG.0000000000003281

[13] Akhtar MA, Saravelos SH, Li TC, Jayaprakasan K. Royal College of Obstetricians and Gynaecologists. Reproductive implications and management of congenital uterine anomalies: Scientific impact paper No. 62 November 2019. BJOG. 2020;127(5):e1–e13. doi: 10.1111/1471-0528.15968

[14] Akhtar M.A., Saravelos S.H., Li T.C., Jayaprakasan K.; Royal College of Obstetricians and Gynaecologists. Reproductive implications and management of congenital uterine anomalies: scientific impact paper No. 62 November 2019 // BJOG: An international journal of obstetrics and gynaecology. 2020. Vol. 127, N 5. P. e1–e13. doi: 10.1111/1471-0528.15968

[15] Valle RF, Ekpo GE. Hysteroscopic metroplasty for the septate uterus: Review and meta-analysis. J Minimally Invasive Gynecol. 2013;20(1):22–42. doi: 10.1016/j.jmig.2012.09.010

[16] Valle R.F., Ekpo G.E. Hysteroscopic metroplasty for the septate uterus: Review and meta-analysis // J Minimally Invasive Gynecol. 2013. Vol. 20, N 1. P. 22–42. doi: 10.1016/j.jmig.2012.09.010

[17] Chandler TM, Machan LS, Cooperberg PL, et al. Mullerian duct anomalies: From diagnosis to intervention. Brit J Radiol. 2009;82(984):1034–1042. doi: 10.1259/bjr/99354802

[18] Chandler T.M., Machan L.S., Cooperberg P.L., et al. Mullerian duct anomalies: From diagnosis to intervention // The British J Radiol. 2009. Vol. 82, N 984. P. 1034–1042. doi: 10.1259/bjr/99354802

[19] Tanitame K, Tanitame N, Urayama S, et al. Congenital anomalies causing hemato/hydrocolpos: Imaging findings, treatments, and outcomes. Jpn J Radiol. 2021;39(8):733–740. EDN: NCSPIY doi: 10.1007/s11604-021-01115-7

[20] Tanitame K., Tanitame N., Urayama S., et al. Congenital anomalies causing hemato/hydrocolpos: Imaging findings, treatments, and outcomes // Jpn J Radiol. 2021. Vol. 39, N 8. P. 733–740. EDN: NCSPIY doi: 10.1007/s11604-021-01115-7

[21] Michala L, Aslam N, Conway GS, Creighton SM. The clandestine uterus: Or how the uterus escapes detection prior to puberty. BJOG. 2010;117(2):212–215. doi: 10.1111/j.1471-0528.2009.02413.x

[22] Michala L., Aslam N., Conway G.S., Creighton S.M. The clandestine uterus: Or how the uterus escapes detection prior to puberty // BJOG. 2010. Vol. 117, N 2. P. 212–215. doi: 10.1111/j.1471-0528.2009.02413.x

[23] Kresowik J, Ryan GL, Austin JC, van Voorhis BJ. Ultrasound-assisted repair of a unique case of distal vaginal agenesis. Fertility Sterility. 2007;87(4):976.e9–976.e9.76E12. EDN: LTFPRH doi: 10.1016/j.fertnstert.2006.06.062

[24] Kresowik J., Ryan G.L., Austin J.C., van Voorhis B.J. Ultrasound-assisted repair of a unique case of distal vaginal agenesis // Fertility Sterility. 2007. Vol. 87, N 4. P. 976.e9–976.e9.76E12. EDN: LTFPRH doi: 10.1016/j.fertnstert.2006.06.062

[25] Adair L, Georgiades M, Osborne R, Ng T. Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: Common presentation of an unusual variation. J Radiol Case Reports. 2011;5(1):1–8. doi: 10.3941/jrcr.v5i1.572

[26] Adair L., Georgiades M., Osborne R., Ng T. Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: Common presentation of an unusual variation // J Radiol Case Rep. 2011. Vol. 5, N 1. P. 1–8. doi: 10.3941/jrcr.v5i1.572

[27] Eksioglu AS, Maden HA, Cinar G, Yildiz TY. Imperforate hymen causing bilateral hydroureteronephrosis in an infant with bicornuate uterus. Case Rep Urol. 2012;2012:102683.

[28] Eksioglu A.S., Maden H.A., Cinar G., Yildiz T.Y. Imperforate hymen causing bilateral hydroureteronephrosis in an infant with bicornuate uterus // Case Rep Urol. 2012. Vol. 2012. P. 102683.

[29] Bischoff A, Alaniz VI, Trecartin A, Peña A. Vaginal reconstruction for distal vaginal atresia without anorectal malformation: Is the approach different? Pediatr Surg Int. 2019;35(9):963–966. EDN: VFDEFF doi: 10.1007/s00383-019-04512-2

[30] Bischoff A., Alaniz V.I., Trecartin A., Peña A. Vaginal reconstruction for distal vaginal atresia without anorectal malformation: Is the approach different? // Pediatr Surg Int. 2019. Vol. 35, N 9. P. 963–966. EDN: VFDEFF doi: 10.1007/s00383-019-04512-2

[31] Ugur MG, Balat O, Ozturk E, et al. Pitfalls in diagnosis and management of distal vaginal agenesis: 10-year experience at a single centre. Eur J Obstet Gynecol Reprod Biol. 2012;163(1):85–90. doi: 10.1016/j.ejogrb.2012.03.024

[32] Ugur M.G., Balat O., Ozturk E., et al. Pitfalls in diagnosis and management of distal vaginal agenesis: 10-year experience at a single centre // Eur J Obstet Gynecol Reprod Biol. 2012. Vol. 163, N 1. P. 85–90. doi: 10.1016/j.ejogrb.2012.03.024

[33] Dural O, Ugurlucan FG, Yasa C, et al. A case of distal vaginal agenesis presenting with recurrent urinary tract infection and pyuria in a prepubertal girl. J Pediatr Adolesc Gynecol. 2017;30(1):e7–e10. doi: 10.1016/j.jpag.2016.08.007

[34] Dural O., Ugurlucan F.G., Yasa C., et al. A case of distal vaginal agenesis presenting with recurrent urinary tract infection and pyuria in a prepubertal girl // J Pediatr Adolesc Gynecol. 2017. Vol. 30, N 1. P. e7–e10. doi: 10.1016/j.jpag.2016.08.007

[35] Priyatini T, Moegni F, Hidayah GN, et al. Case series: Interdigitating "Y" flap for transverse vaginal septum management. Int J Surg Case Rep. 2022;(100):107601. doi: 10.1016/j.ijscr.2022.107601

[36] Priyatini T., Moegni F., Hidayah G.N., et al. Case series: Interdigitating "Y" flap for transverse vaginal septum management // Int J Surg Case Rep. 2022. N 100. P. 107601. doi: 10.1016/j.ijscr.2022.107601

[37] Bobkova MV, Puchko TK, Adamyan LV. Reproduction in women with congenital uterus and vagina anomalies. Russ J Human Rep. 2018;24(2):42–53. EDN: XPLZFJ doi: 10.17116/repro201824242-53

[38] Бобкова М.В., Пучко Т.К., Адамян Л.В. Репродуктивная функция у женщин с пороками развития матки и влагалища // Проблемы репродукции. 2018. Т. 24, № 2. С. 42–53. EDN: XPLZFJ doi: 10.17116/repro201824242-53

[39] Williams CE, Nakhal RS, Hall-Craggs MA, et al. Transverse vaginal septae: Management and long-term outcomes. BJOG. 2014;121(13):1653–1658. doi: 10.1111/1471-0528.12899

[40] Williams C.E., Nakhal R.S., Hall-Craggs M.A., et al. Transverse vaginal septae: Management and long-term outcomes // BJOG. 2014. Vol. 121, N 13. P. 1653–1658. doi: 10.1111/1471-0528.12899

[41] Singh S, Biswas M, Dey M, et al. A rare case of low transverse vaginal septum. Int J Reprod Contracept Obstet Gynecol. 2015;4(6):2103–2106.

[42] Singh S., Biswas M., Dey M., et al. A rare case of low transverse vaginal septum // Int J Reprod Contracept Obstet Gynecol. 2015. Vol. 4, N 6. P. 2103–2106.

[43] Abdelrahman HM, Feloney MP. Imperforate hymen. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2022.

[44] Abdelrahman H.M., Feloney M.P. Imperforate hymen. В кн.: StatPearls. Treasure Island (FL): StatPearls Publishing, 2022.

[45] Lee KH, Hong JS, Jung HJ, et al. Imperforate hymen: A comprehensive systematic review. J Clin Med. 2019;8(1):56. doi: 10.3390/jcm8010056

[46] Lee K.H., Hong J.S., Jung H.J., et al. Imperforate hymen: A comprehensive systematic review // J Clin Med. 2019. Vol. 8, N 1. P. 56. doi: 10.3390/jcm8010056

[47] Batyrova ZK, Uvarova EV, Kumykova ZK, et al. Congenital disorders of genital organs with menstrual blood outflow defect: Diagnostics, treatment and prevention of complications. Pediatric Pharmacol. 2019;16(6):349–352. EDN: XZIZZU doi: 10.15690/pf.v16i6.2072

[48] Батырова З.К., Уварова Е.В., Кумыкова З.Х., и др. Аномалии развития половых органов с нарушением оттока менструальной крови: диагностика, лечение и профилактика осложнений // Педиатрическая фармакология. 2019. Т. 16, № 6. С. 349–352. EDN: XZIZZU doi: 10.15690/pf.v16i6.2072

[49] Cetin C, Soysal C, Khatib G, et al. Annular hymenotomy for imperforate hymen. J Obstet Gynaecol Res. 2016;42(8):1013–1015. doi: 10.1111/jog.13010

[50] Cetin C., Soysal C., Khatib G., et al. Annular hymenotomy for imperforate hymen // J Obstet Gynaecol Res. 2016. Vol. 42, N 8. P. 1013–1015. doi: 10.1111/jog.13010

[51] Acar A, Balci O, Karatayli R, et al. The treatment of 65 women with imperforate hymen by a central incision and application of Foley catheter. BJOG. 2007;114(11):1376–1379. doi: 10.1111/j.1471-0528.2007.01446.x

[52] Acar A., Balci O., Karatayli R., et al. The treatment of 65 women with imperforate hymen by a central incision and application of Foley catheter // BJOG. 2007. Vol. 114, N 11. P. 1376–1379. doi: 10.1111/j.1471-0528.2007.01446.x

[53] Parker PB, Edwards JR, Bochnakova TN, Lee DM. Image-guided transvaginal and transabdominal drain placement for surgical navigation in a patient with tubo-ovarian abscess and obstructed hemivagina and ipsilateral renal anomaly syndrome. J Pediatr Adolesc Gynecol. 2022;35(1):94–97. EDN: UEIYTI doi: 10.1016/j.jpag.2021.07.006

[54] Parker P.B., Edwards J.R., Bochnakova T.N., Lee D.M. Image-guided transvaginal and transabdominal drain placement for surgical navigation in a patient with tubo-ovarian abscess and obstructed hemivagina and ipsilateral renal anomaly syndrome // J Pediatr Adolesc Gynecol. 2022. Vol. 35, N 1. P. 94–97. EDN: UEIYTI doi: 10.1016/j.jpag.2021.07.006

[55] Kudela G, Wiernik A, Drosdzol-Cop A, et al. Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: One clinical center case series and the systematic review of 734 cases. J Pediatr Urol. 2021;17(5):653.e1–653.e9. doi: 10.1016/j.jpurol.2021.06.023

[56] Kudela G., Wiernik A., Drosdzol-Cop A., et al. Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: One clinical center case series and the systematic review of 734 cases // J Pediatr Urol. 2021. Vol. 17, N 5. P. 653.e1–653.e9. doi: 10.1016/j.jpurol.2021.06.023

[57] Arakelyan AS, Gus AI, Adamyan LV, et al. The role of 3d echographic diagnostics in the choice of tactics for reconstructive plastic surgery in case of abnormalities of the development of the uterus and vagina. Russ J Human Reproduct. 2021;27(4):32–42. EDN: TCIGLZ doi: 10.17116/repro20212704132

[58] Аракелян А.С., Гус А.И., Адамян Л.В., и др. Роль 3D-эхографической диагностики в выборе тактики реконструктивно-пластических операций при аномалиях развития матки и влагалища // Проблемы репродукции. 2021. Т. 27, № 4. С. 32–42. EDN: TCIGLZ doi: 10.17116/repro20212704132

[59] Kriplani A, Dalal V, Kachhawa G, et al. Minimally invasive endoscopic approach for management of OHVIRA syndrome. J Obstet Gynaecol India. 2019;69(4):350–355. EDN: PKWBRG doi: 10.1007/s13224-019-01240-4

[60] Kriplani A., Dalal V., Kachhawa G., et al. Minimally invasive endoscopic approach for management of OHVIRA syndrome // J Obstet Gynaecol India. 2019. Vol. 69, N 4. P. 350–355. EDN: PKWBRG doi: 10.1007/s13224-019-01240-4

[61] Akhapkina ES, Batyrova ZK, Сhuprynin VD, et al. Features of the management of girls with anomalies of the genitourinary system. Gynecology. 2021;23(3):245–249. EDN: UTYBOS doi: 10.26442/20795696.2021.3.200951

[62] Ахапкина Е.С., Батырова З.К., Чупрынин В.Д., и др. Особенности ведения девочек с аномалиями развития мочеполовой системы // Гинекология. 2021. Т. 23, № 3. С. 245–249. EDN: UTYBOS doi: 10.26442/20795696.2021.3.200951

[63] Cheng C, Subedi J, Zhang A, et al. Vaginoscopic incision of oblique vaginal septum in adolescents with OHVIRA syndrome. Sci Rep. 2019;9(1):20042. doi: 10.1038/s41598-019-56471-2

[64] Cheng C., Subedi J., Zhang A., et al. Vaginoscopic incision of oblique vaginal septum in adolescents with OHVIRA syndrome // Sci Rep. 2019. Vol. 9, N 1. P. 20042. doi: 10.1038/s41598-019-56471-2

[65] Ludwin A, Ludwin I, Bhagavath B, et al. Virginity-sparing management of blind hemivagina in obstructed hemivagina and ipsilateral renal anomaly syndrome. Fertil Steril. 2018;110(5):976–978. doi: 10.1016/j.fertnstert.2018.07.007

[66] Ludwin A., Ludwin I., Bhagavath B., et al. Virginity-sparing management of blind hemivagina in obstructed hemivagina and ipsilateral renal anomaly syndrome // Fertil Steril. 2018. Vol. 110, N 5. P. 976–978. doi: 10.1016/j.fertnstert.2018.07.007

RIGHTS & PERMISSIONS

Eco-Vector