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  • Title: [CLITOROPLASTY FOR GENITAL VIRILIZATION IN GIRLS WITH CONGENITAL ADRENAL HYPERPLASIA].
    Author: Zeng L, Zhang J, Huang G, Huang Y, Ma X, Huang L.
    Journal: Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi; 2016 Sep 08; 30(9):1134-1138. PubMed ID: 29786370.
    Abstract:
    OBJECTIVE: To investigate the effectiveness of clitoroplasty of reserving the clitoris dorsal neurovascular bundle and the ventral complete urethral plate for genital virilization in girls with congenital adrenal hyperplasia (CAH). METHODS: Between June 2000 and October 2015, 27 girls with genital virilization were treated with clitoroplasty of reserving the clitoris dorsal neurovascular bundle and the ventral complete urethral plate. The chromosomal karyotype was 46, XX in all patients, whose age ranged from 3 to 11 years (mean, 3.9 years). According to Parder's virilization scale, 5 cases were classified as stage Ⅱ, 18 cases as stage Ⅲ, and 4 cases as stage Ⅳ. The hormone tests showed decreased cortisol level and increased testosterone and 17-hydroxyprogesterone levels. They were diagnosed with non-salt-wasting CAH. RESULTS: All of the patients underwent clitoroplasty successfully. The mean operation time was 74 minutes (range, 58-95 minutes). Incision healed primarily. The patients were followed up 6 months to 10 years (median, 19 months). The external genitalia had good appearance, without necrosis of flap and the glans clitoris, abnormal sensation of the glans clitoris, or clitorism recurrence. Five girls had breast development and normal menstrual cycle during adolescence, and 1 patient who was followed up for 10 years gave birth to a son. CONCLUSIONS: Clitoroplasty of reserving the clitoris dorsal neurovascular bundle and the ventral complete urethral plate is a relatively ideal method for treating genital virilization in girls. It has less complications, good aesthetic and functional results. There are satisfactory outcomes when combining with the endocrine treatment. 目的: 探讨保留阴蒂背侧血管神经束以及腹侧完整尿道板的阴蒂成形术矫治先天性肾上腺皮质增生(congenital adrenal hyperplasia,CAH)女童外生殖器男性化畸形的疗效。. 方法: 2000年6月-2015年10月,收治27例外生殖器男性化畸形患儿,采用保留阴蒂背侧血管神经束以及腹侧完整尿道板的阴蒂成形术矫治。27例患儿染色体检查均为46,XX核型,社会性别均为女性;年龄3~11岁,平均3.9岁。按Parder分型标准:Ⅱ型5例,Ⅲ型18例,Ⅳ型4例。激素检查提示皮质醇水平正常偏低,睾酮和17-羟孕酮均升高。入院诊断为非失盐型CAH。. 结果: 17例手术均顺利完成。手术时间58~95 min,平均74 min。切口均Ⅰ期愈合。患儿均获随访,随访时间6个月~10年,中位时间19个月。外生殖器外观恢复良好,无皮瓣和阴蒂头坏死,阴蒂头感觉未见异常,无阴蒂肥大复发。末次随访时,5例进入青春期,有乳房发育和正常月经周期;1例随访10年者已婚并生育一子。. 结论: 采用保留阴蒂背侧血管神经束以及腹侧完整尿道板的阴蒂成形术矫治CAH女童外生殖器男性化畸形,术后外生殖器外观良好,阴蒂感觉未见异常;结合内分泌等综合治疗,可获得满意疗效。.
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