The prevalence of congenital cervical agenesis or dysgenesis ranges from 1/80,000 to 1/100,000, and in about 50% of these cases it coexists with congenital vaginal agenesis. This narrative review summarizes the contemporary knowledge in the field of conservative surgical restoration of the reproductive tract. The management of congenital cervical malformations aims to [1] provide relief from the obstructive symptoms, [2] establish normal sexual function, and [3] preserve the uterus for future fertility. In cases of cervical agenesis and vaginal aplasia, the surgical approach involves the creation of neovagina, the creation of neocervix, and then subsequent restoration of the continuity of the genital tract. In cases where vagina is not congenitally absent, the surgical approach involves either a direct uterovaginal anastomosis or initial creation of neocervix and then subsequent restoration of the continuity of the genital tract. The neocervix can be surgically created with small intestinal submucosa, split-thickness skin graft, full-thickness skin graft, peritoneal flap, or vaginal mucosa lined with a polytetrafluoroethylene graft. Most of the published cases report long-term menstruation and sporadic pregnancies. Conservative surgery of cervical congenital malformations could serve as a first-line treatment. Sexual function and menstruation are established in the majority of patients. Extirpatory surgery may be preserved for surgical failures after initial restoration of the continuity of uterus-cervix-vagina or in cases with more complex anatomy.

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关于先天性宫颈畸形管理的最新知识：文献综述

先天性宫颈发育不全或发育不全的患病率从 1/80,000 到 1/100,000 不等，其中约 50% 的病例与先天性阴道发育不全共存。这篇叙述性评论总结了生殖道保守手术修复领域的当代知识。先天性宫颈畸形的治疗旨在 [1] 缓解梗阻症状，[2] 建立正常的性功能，并 [3] 保留子宫以备将来生育。在宫颈发育不全和阴道发育不全的情况下，手术方法包括创建新阴道、创建新宫颈，然后恢复生殖道的连续性。在阴道不是先天性缺失的情况下，手术方法包括直接子宫阴道吻合术或初始创建新宫颈，然后恢复生殖道的连续性。新宫颈可以通过小肠黏膜下层、分层皮肤移植物、全层皮肤移植物、腹膜瓣或内衬聚四氟乙烯移植物的阴道粘膜手术创建。大多数已发表的病例报告长期月经和零星妊娠。宫颈先天性畸形的保守手术可作为一线治疗。大多数患者已建立性功能和月经。在最初恢复子宫-宫颈-阴道的连续性后或在解剖结构更复杂的情况下，可以保留摘除手术以应对手术失败。