Sánchez-Martín R, Molina E, Cerdá J, Navascués J A, Barrientos G, Romero R, Vázquez J
Servicio de Cirugía Pediátrica, Hospital Infantil Gregorio Marañón, Madrid.
Cir Pediatr. 1999 Apr;12(2):83-7.
The presence of ambiguous external genitals can be related with a number of nosologic entities. We can find the female form with hypertrophic clitoris and vaginal hypoplasia in a wide variety of phenotype presentations. We have two young girls with ambiguous external genitals, where we notice hypertrophic clitoris and vaginal hypoplasia, with the diagnosis of mixed gonadal dysgenesis and androgen insensitivity syndrome (Morri's Syndrome). They have been operated several times: gonadectomy, genitoplasty, vaginoplasty and mammoplasty. We have made a neovagina, using a segment of sigma and preserving the walls of the former vagina. The surgical technique we used, consisted in the abdominoperineal descent of the segment of sigmoid colon and anastomosis with the back wall of the former vagina and the perineum; after that, we split the intervaginal septum, linking these two structures and reaching a lonely introitus vaginae. The hormonal treatment for the girl with mixed gonadal dysgenesis with estrogen and progestogen induces normal menstruations; we only use estrogen for the patient with Morri's Syndrome. The histology showed that there were dysgenetic gonads in one of the patients and testicular tissue without gonadal elements. Both girls have had a good evolution, with no problems and an excellent aesthetic and functional result.
外生殖器模糊的情况可能与多种疾病实体相关。我们能在多种表型表现中发现具有肥大阴蒂和阴道发育不全的女性形态。我们有两名外生殖器模糊的年轻女孩，她们存在肥大阴蒂和阴道发育不全的情况，诊断为混合性性腺发育不全和雄激素不敏感综合征（莫里斯综合征）。她们已经接受了多次手术：性腺切除术、生殖器成形术、阴道成形术和乳房成形术。我们使用一段乙状结肠制作了一个新阴道，并保留了原阴道的壁。我们采用的手术技术包括乙状结肠段的腹会阴下移并与原阴道后壁和会阴进行吻合；之后，我们切开阴道间隔，连接这两个结构，形成一个单一的阴道口。对于患有混合性性腺发育不全的女孩，使用雌激素和孕激素进行激素治疗可诱导正常月经；对于患有莫里斯综合征的患者，我们仅使用雌激素。组织学检查显示，其中一名患者有发育不全的性腺，另一名患者有不含性腺成分的睾丸组织。两名女孩术后恢复良好，没有出现问题，在美观和功能方面都取得了极佳的效果。
