Diagnosis and Treatment Approaches in Vaginal Agenesis

Year 2024, Volume: 26 Issue: 2, 91 - 94, 30.08.2024

Alper Başbuğ

https://doi.org/10.18678/dtfd.1531224

Abstract

The development of the female genital system is a complex process that depends on a series of events involving cellular differentiation, migration, fusion, and recanalization. Failure of any of these processes results in congenital anomalies. Developmental anomalies can occur at various stages, resulting in conditions that impact both the urinary and reproductive systems. In younger patients, such malformations can significantly affect their overall health and quality of life, including aspects such as fertility, sexual function, and psychological well-being. The psychosexual effects of vaginal agenesis should not be overlooked, and clinical care primarily involves comprehensive counseling and support through open communication with the patient. For adult patients, treatment for vaginal agenesis typically starts with therapeutic counseling and education, with non-invasive vaginal dilation being recommended as the first-line approach, or surgery if necessary. Consequently, managing these issues often requires a multidisciplinary approach, engaging specialists such as urologists, gynecologists, endocrinologists, and geneticists, among others. Early detection and timely intervention can greatly enhance the outlook for individuals with these conditions. Besides considering the patient's expectations, the surgeon's experience plays a crucial role in selecting the appropriate surgical technique. This is because the success of the initial surgery is critical to the effectiveness of any subsequent procedures if required. In this review, the evaluation and treatment of vaginal agenesis, which constitutes an important part of congenital anomalies of the vagina, were discussed.

Keywords

Müllerian canal, vaginal agenesis, vaginoplasty

Vajinal Agenizde Tanı ve Tedavi Yaklaşımları

Abstract

Kadın genital sisteminin gelişimi, hücresel farklılaşma, göç, füzyon ve yeniden kanalizasyonu içeren bir dizi olaya bağlı olan karmaşık bir süreçtir. Bu süreçlerden herhangi birinin başarısızlığı doğumsal anomalilerle sonuçlanır. Gelişimsel anomaliler çeşitli aşamalarda ortaya çıkabilir ve hem üriner hem de üreme sistemlerini etkileyen durumlarla sonuçlanabilir. Genç hastalarda, bu tür malformasyonlar doğurganlık, cinsel işlev ve psikolojik refah gibi hususlar da dahil olmak üzere genel sağlıklarını ve yaşam kalitelerini önemli ölçüde etkileyebilir. Vajinal agenezinin psikoseksüel etkileri göz ardı edilmemelidir ve klinik bakım öncelikle hastayla açık iletişim yoluyla kapsamlı danışmanlık ve desteği içerir. Yetişkin hastalar için vajinal agenezi tedavisi tipik olarak terapötik danışmanlık ve eğitimle başlar, ilk basamak yaklaşım olarak invazif olmayan vajinal dilatasyon veya gerekirse cerrahi önerilir. Sonuç olarak, bu sorunların yönetimi genellikle ürologlar, jinekologlar, endokrinologlar ve genetikçiler gibi uzmanları içeren multidisipliner bir yaklaşım gerektirir. Erken teşhis ve zamanında müdahale, bu durumlara sahip bireylerin görünümünü büyük ölçüde iyileştirebilir. Hastanın beklentilerini göz önünde bulundurmanın yanı sıra, cerrahın deneyimi de uygun cerrahi tekniğin seçilmesinde çok önemli bir rol oynar. Bunun nedeni, ilk ameliyatın başarısının, gerekirse sonraki prosedürlerin etkinliği açısından kritik olmasıdır. Bu derlemede, vajinanın konjenital anomalilerinin önemli bir bölümünü oluşturan vajinal agenezinin değerlendirilmesi ve tedavisi tartışılmıştır.

Keywords

Müllerian kanal, vajinal agenezi, vajinoplasti

References

• P A A, Arbor TC, Krishan K. Embryology, sexual development. 2023 Aug 28. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024.
• Yi P, Niu HL, Gao Q, Wang FH, Jia W, Chen ZR, et al. Pathologic features on gonadal changes of sexual developmental disorders in children. Zhonghua Bing Li Xue Za Zhi. 2018;47(7):531-5. Chinese.
• Matemanosak P, Peeyananjarassri K, Klangsin S, Wattanakumtornkul S, Dhanaworavibul K, Choksuchat C, et al. Clinical features and management of women with Mayer-Rokitansky-Küster-Hauser syndrome in a Thai population. Obstet Gynecol Sci. 2024;67(3):314-22.
• Pietzsch M, Schönfisch B, Höller A, Koch A, Staebler A, Dreser K, et al. A cohort of 469 Mayer-Rokitansky-Küster-Hauser syndrome patients-associated malformations, syndromes, and heterogeneity of the phenotype. J Clin Med. 2024;13(2):607.
• Chmel R Jr, Pastor Z, Mužík M, Brtnický T, Nováčková M. Syndrome Mayer-Rokitansky-Küster-Hauser - uterine and vaginal agenesis: current knowledge and therapeutic options. Ceska Gynekol. 2019;84(5):386-92.
• Herlin MK, Petersen MB, Brännström M. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update. Orphanet J Rare Dis. 2020;15(1):214.
• Le TTQ, Le NTH, Vu TA, Nguyen HH, Vuong LN. Perception of having children through surrogacy in individuals with MRKH in Vietnam: a qualitative study. Front Psychol. 2024;15:1372405.
• Goldwyn RM. History of attempts to form a vagina. Plast Reconstr Surg. 1977;59(3):319-29.
• Abbe R. New method of creating a vagina in a case of congenital absence. Med Rec. 1898;54(24):836-8.
• Gargollo PC, Cannon GM Jr, Diamond DA, Thomas P, Burke V, Laufer MR. Should progressive perineal dilation be considered first line therapy for vaginal agenesis? J Urol. 2009;182(4 Suppl):1882-9.
• Herlin M, Bay Bjørn AM, Jørgensen LK, Trolle B, Petersen MB. Treatment of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome in Denmark: a nationwide comparative study of anatomical outcome and complications. Fertil Steril. 2018;110(4):746-53.
• Martens L, Tannenbaum L, Van Kuijk SMJ, Notten KJB, Kluivers KB. Laparoscopic Davydov vs. laparoscopic Vecchietti neovaginoplasty in women with Mayer-Rokitansky-Küster-Hauser syndrome; a systematic review and meta-analysis. Fertil Steril. 2024;121(4):679-92.
• Harmanli OH, Grody MH. Laparoscopic Vecchietti procedure: improving on an indispensable method Novel variations on an essential technique may benefit surgeons and their patients. Am J Obstet Gynecol. 2008;199(6):713.e1-2.
• Yang X, Zhu L, Wang YJ, Tong B, Zhong S, Yang C, et al. Comparison of the modified laparoscopic Vecchietti and Davydov colpoplasty techniques in Mayer-Rokitansky-Küster-Hauser syndrome: A long-term follow-up analysis. J Obstet Gynaecol Res. 2022;48(7):1930-7.
• Rall K, Schickner MC, Barresi G, Schönfisch B, Wallwiener M, Wallwiener CW, et al., Laparoscopically assisted neovaginoplasty in vaginal agenesis: a long-term outcome study in 240 patients. J Pediatr Adolesc Gynecol, 2014;27(6):379-85.
• 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-Küster syndrome. Fertil Steril. 2015;103(1):220-7.e1.
• Skinner B, Quint EH, Nonobstructive reproductive tract anomalies: a review of surgical management. J Minim Invasive Gynecol. 2017;24(6):909-14.
• Sinha A, Amrita, Sinha M, Biswas P. Anatomical and functional evaluation of modified abbe-mcindoe vaginoplasty at a tertiary care hospital. Niger J Clin Pract. 2024;27(5):643-6.
• Chaudhary A, Acharya S, Dahal A, Basnet R, Basnet A, Chaudhary A, et al Mayer-Rokitansky-Küster-Hauser syndrome managed with McIndoe's vaginoplasty: a case series and literature review. Ann Med Surg (Lond). 2024;86(6):3206-10.
• Candiani M, Fedele F, Ruffolo AF, Di Fatta S, Salvatore S, Parazzini F. Histological Features of neovaginal epithelium after vaginoplasty in Mayer-Rokitansky-Küster-Hauser syndrome. J Pediatr Adolesc Gynecol. 2024;37(3):353-9.
• Teng Y, Zhu L, Chong Y, Zeng A, Liu Z, Yu N, et al. The modified McIndoe technique: A scar-free surgical approach for vaginoplasty with an autologous micromucosa graft. Urology. 2019;131:240-4.
• Robinson IS, Cripps CN, Bluebond-Langner R, Zhao LC. Operative management of complications following intestinal vaginoplasty: A case series and systematic review. Urology. 2023;180:105-12.