Surgical Therapy After Failed Feminizing Genitoplasty in Young Adults With Disorders of Sex Development: Retrospective Analysis and Review of the Literature.
Surgical Therapy After Failed Feminizing Genitoplasty in Young Adults With Disorders of Sex Development: Retrospective Analysis and Review of the Literature.
复制标题
患有性发育障碍的年轻人女性化生殖成形术失败后的手术治疗：回顾性分析和文献综述。
DOI：
10.1016/j.jsxm.2021.07.008
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发表时间：
2021
期刊：
影响因子：
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通讯作者：
J. Fuchs
中科院分区：
文献类型：
--
作者：
V. Ellerkamp;K. Rall;Juergen Schaefer;D. Stefanescu;D. Schoeller;S. Brucker;J. Fuchs
背景：继发性阴道狭窄可能发生在儿童生殖器畸形重建或成人阴道发育不全修复失败后。目的探讨多学科过渡障碍/性别发育差异儿科医生和妇科医生团队对这类患者的手术治疗效果。方法回顾性分析2015 - 2018年单中心阴道翻修成形术治疗的成人和女性阴道狭窄性发育障碍/差异。潜在的畸形类型，婴儿期阴道成形术的数量和手术技术，狭窄阴道的修复技术，阴道长度和口径，性交的可能性，以及临时阴道扩张。回顾文献关于推荐的手术技术翻修阴道成形术完成。为了描述手术技术，本研究的主要结果测量是阴道翻修成形术后的阴道卡尺以及性交能力。结果13例患者出现阴道狭窄，中位年龄19岁（16-31岁）。所有患者在其病史中都有一种或多种不同类型的阴道成形术，首次阴道成形术的中位年龄为15个月（0-233）。潜在解剖条件为泌尿生殖窦（n = 8），阴道发育不全（n = 2），持续性阴沟（n = 2）和阴沟外翻（n = 1）。13例患者以阴道组织狭窄导致性交障碍为主要症状。最常见的手术技术是用会阴或外侧皮瓣部分动员泌尿生殖器官（n = 10），其次是肠阴道成形术（n = 2）， 1例患者因特殊解剖条件而翻修阴道成形术失败。在平均11个月的随访中，除了一名患者外，所有患者的阴道长度和宽度都是正常的，有伴侣的患者的性交也正常。临床意义对于阴道远端狭窄的严重病例和多次阴道成形术失败后，应考虑将阴道皮瓣部分动员作为治疗的选择，而肠阴道成形术应仅用于阴道完全愈合或高位狭窄的病例。优势与局限性本研究的优势在于对几个案例的详细描述，而回顾性的特征是一个局限性。结论阴部女性化修复术后，会阴部皮瓣部分泌尿生殖道动员可提供正常的解剖结果，并可实现无问题的性交。
BackgroundSecondary vaginal stenosis may occur after reconstruction of genital malformations in childhood or after failed vaginal aplasia repair in adults.AimThis study focusses on the results of the surgical treatment of these patients in our multidisciplinary transitional disorders/differences of sex development team of pediatric surgeons and gynecologists.MethodsA retrospective analysis was carried out on adult and female identified disorders/differences of sex development patients with vaginal stenoses treated between 2015 and 2018 in a single center with revision vaginoplasty. The underlying type of malformation, the number and surgical techniques of vaginoplasties in infancy, techniques of revision of the stenotic vagina, vaginal length and caliber, possibility of sexual intercourse, and temporary vaginal dilatation. A review of literature with regard to recommended surgical techniques of revision vaginoplasties was accomplished.OutcomesTo describe the surgical technique, the main outcome measures of this study are vaginal calipers after revision vaginoplasty as well as ability for sexual intercourse.RESULTSThirteen patients presented with vaginal stenosis with a median age of 19 years (range 16–31). All patients had one or more different types of vaginoplasties in their medical history, with a median age at first vaginoplasty of 15 months (0–233). Underlying anatomical conditions were urogenital sinus (n = 8), vaginal agenesis (n = 2), persistent cloacae (n = 2), and cloacal exstrophy (n = 1). The main symptoms were disability of sexual intercourse in 13 patients due to stenotic vaginal tissue. The most frequently performed surgical technique was partial urogenital mobilization with a perineal or lateral flaps (n = 10), followed by bowel vaginoplasty (n = 2), in 1 patient a revision vaginoplasty failed due to special anatomical conditions. In a median follow-up of 11 months, all but one patient presented with physiological vaginal length and width, and normal sexual intercourse in those with a partnership.Clinical ImplicationsPerineal flap with partial urogenital mobilization should be considered as a treatment of choice in severe cases of distal vaginal stenosis and after multiple failed former vaginoplasties, while bowel vaginoplasty should be reserved only for cases of complete cicatrization or high located stenosis of the vagina.Strengths & LimitationsThe strength of this study is the detailed description of several cases while the retrospective character is a limitation.CONCLUSIONIn patients after feminizing genital repair, perineal flap with partial urogenital mobilization provides a normal anatomical outcome and allows unproblematic sexual intercourse.