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先天性肾上腺皮质增生症(CAH)女性患儿的早期女性化生殖器成形术-统一手术管理分析。

Early Feminizing Genitoplasty in Girls with Congenital Adrenal Hyperplasia (CAH)-Analysis of Unified Surgical Management.

机构信息

Department of Pediatric Surgery and Urology, Medical University of Silesia, 40-752 Katowice, Poland.

Department of Pediatrics and Pediatric Endocrinology, Medical University of Silesia, 40-752 Katowice, Poland.

出版信息

Int J Environ Res Public Health. 2020 May 29;17(11):3852. doi: 10.3390/ijerph17113852.

Abstract

AIM

To analyze a single-centre experience in feminizing genitoplasty in virilized girls with congenital adrenal hyperplasia (CAH).

METHODS

Review of medical records of all 46, XX CAH patients undergoing single stage feminizing genitoplasty between 2003 and 2018 was performed.

RESULTS

A total of 31 girls aged from 4 months to 10 years were included in the study. The majority (n = 26/31, 84%) were operated before 2 years of age (median 8 months). External virilization was rated as Prader 3 (n = 7/31), Prader 4 (n = 21/31) and Prader 5 (n = 3/31). The urethrovaginal confluence location was low in 19 and high in 12 girls with a percentage distribution similar in Prader 4 and 5 ( > 0.05) but significantly different in Prader 3 ( = 0.017). The follow-up ranged from 12 months to 15 years. All parents assessed the cosmetic result as satisfactory. Perioperative complications occurred in two patients and included rectal injury (n = 1/31) and prolonged bleeding (n = 1/31). Three patients developed late complications including labial atheromas (n = 2/31) and vaginal stricture requiring surgical dilatation (n = 1/31). Low confluence did not decrease the risk of complications.

CONCLUSIONS

Early feminizing genitoplasty in girls with congenital adrenal hyperplasia, irrespective of virilization severity, gives satisfactory cosmetic results and is characterized by low and acceptable surgical risk. Nevertheless, the most important determinant of the effectiveness of such management would be future patients' satisfaction.

摘要

目的

分析先天性肾上腺皮质增生症(CAH)女性化生殖器成形术的单中心经验。

方法

回顾了 2003 年至 2018 年间在单一中心接受女性化生殖器成形术的 46,XX CAH 患者的所有病历。

结果

共有 31 名女孩(年龄 4 个月至 10 岁)纳入本研究。大多数(n = 26/31,84%)在 2 岁之前接受手术(中位数 8 个月)。外部性发育不良评分(Prader)为 3 级(n = 7/31)、4 级(n = 21/31)和 5 级(n = 3/31)。19 名女孩的尿道阴道汇合位置较低,12 名女孩的尿道阴道汇合位置较高,Prader 4 级和 5 级的分布相似(>0.05),但 Prader 3 级明显不同(=0.017)。随访时间为 12 个月至 15 年。所有家长均对美容效果评估满意。两名患者发生围手术期并发症,包括直肠损伤(n = 1/31)和出血时间延长(n = 1/31)。三名患者出现晚期并发症,包括阴唇动脉粥样硬化(n = 2/31)和阴道狭窄需要手术扩张(n = 1/31)。低汇合并不会降低并发症的风险。

结论

无论女性化严重程度如何,对先天性肾上腺皮质增生症女孩进行早期女性化生殖器成形术可获得满意的美容效果,且手术风险低且可接受。然而,这种治疗效果的最重要决定因素将是未来患者的满意度。

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