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保留神经的女性化生殖器重建术治疗生殖器男性化:25例肾上腺性征异常综合征患儿的经验

[Nerve-sparing, feminizing genital reconstruction to correct genital virilism: Experience of 25 children with adrenogenital syndrome].

作者信息

Altwein J E, Homoki J

机构信息

Urologische Abteilung, Universitätskinderklinik und Poliklinik Ulm.

出版信息

Z Kinderchir. 1989 Aug;44(4):228-33. doi: 10.1055/s-2008-1043240.

Abstract

The reconstruction of the virilized genitalia in females with adrenogenital syndrome (AGS) is carried out sparing the dorsal neurovascular bundle either through clitoral recession or reduction with simultaneous vaginoplasty and clitoroplasty. Within seven years, 25 girls aged 6 months to 16 years (mean 2 1/2 years) with AGS had a nerve-sparing genital reconstruction with resection of the clitoral shaft in one session as described by Praetorius. 23 children had a C21-defect and 1 a C11 beta-hydroxylase defect; one child had an external virilisation. All children were followed over an average period of 50 months: 21 had a good cosmetic result, the glans had a normal blood supply and was sensitive to a prick test; in 2 girls nerve integrity was demonstrated by somato-sensory evoked potentials. 2 children had small disturbing skin folds of the newly created labia and 2 girls had a somewhat scrotal appearance of the labia. The introitus caliber measured 14 F in girls operated before 6 years of age; however, a 14- and 16-year-old required intermittent bougienage.

摘要

患有肾上腺生殖器综合征(AGS)的女性患者,其男性化生殖器的重建是通过保留背侧神经血管束来进行的,可采用阴蒂退缩或缩小术,同时进行阴道成形术和阴蒂成形术。七年内,25名年龄在6个月至16岁(平均2.5岁)的AGS女童接受了保留神经的生殖器重建手术,按照普拉托里乌斯的描述,一次性切除阴蒂体。23名儿童存在21-羟化酶缺陷,1名存在11β-羟化酶缺陷;1名儿童有外部男性化表现。所有儿童平均随访50个月:21名儿童获得了良好的美容效果,阴蒂头血供正常,针刺试验敏感;2名女童通过体感诱发电位证明神经完整。2名儿童新形成的阴唇有小的干扰性皮肤褶皱,2名女童阴唇外观有点像阴囊。6岁前接受手术的女童阴道口直径为14F;然而,一名14岁和一名16岁的女童需要间歇性扩张。

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