不同年龄段先天性肾上腺皮质增生合并外生殖器男性化女童术后并发症与手术效果分析  

Analysis of postoperative complications and surgical outcomes of feminizing genitoplasties in girls with congenital adrenal hyperplasia and masculinized external genitalia across different age groups

作　　者：赵士猛 崔晓薇[2] 曹延延 齐灿[1] 刘丽君[4] 陈潇潇 刘颖业 刘蕾[5] 周云[1] Zhao Shimeng;Cui Xiaowei;Cao Yanyan;Qi Can;Liu Lijun;Chen Xiaoxiao;Liu Yingye;Liu Lei;Zhou Yun(Department of Urology,Hebei Children’s Hospital,Shijiazhuang 050031,China;Department of Pediatrics,Hebei Children’s Hospital,Shijiazhuang 050031,China;Institute of Pediatrics,Hebei Children’s Hospital,Shijiazhuang 050031,China;Department of Endocrinology,Genetics,and Metabolism,Hebei Children’s Hospital,Shijiazhuang 050031,China;Department of Ultrasound Diagnostics,Hebei Children’s Hospital,Shijiazhuang 050031,China)

机构地区：[1]河北省儿童医院泌尿外科,石家庄050031 [2]河北省儿童医院儿科,石家庄050031 [3]河北省儿童医院儿科研究所,石家庄050031 [4]河北省儿童医院内分泌遗传代谢科,石家庄050031 [5]河北省儿童医院超声诊断科,石家庄050031

出　　处：《临床小儿外科杂志》2025年第3期236-242,共7页Journal of Clinical Pediatric Surgery

基　　金：河北省重点研发计划卫生健康创新专项项目(21377710D);河北省医学科学研究课题计划(20231130)。

摘　　要：目的比较不同年龄段先天性肾上腺皮质增生(congenital adrenal hyperplasia,CAH)合并外生殖器男性化女童行女性化外生殖器成形术(feminizing genitoplasties,FG)后并发症与手术效果。方法回顾性分析河北省儿童医院2010年3月至2022年8月收治的32例CAH合并外生殖器男性化女童的临床资料,以3岁为界将患儿分为婴幼儿组和非婴幼儿组。其中婴幼儿组15例,14例采用阴蒂成形术,1例采用阴蒂+阴道成形术;非婴幼儿组17例,16例采用阴蒂成形术,1例采用阴蒂+阴道成形术。两组患儿基线资料比较,除手术年龄差异有统计学意义外(P<0.001),其余资料差异均无统计学意义(P>0.05)。观察指标包括Prader分级、手术方式、手术时长、随访时间、随访时年龄、术后早期与晚期并发症及患儿监护人对术后外生殖器的满意度。结果是否于婴幼儿期接受手术是术后外生殖器外观满意度的独立影响因素(OR=0.068,95%CI:0.005~0.849,P<0.05)。接受随访的32例患儿中,共7例(21.88%)出现术后并发症;婴幼儿组2例,其中切口出血1例,早期切口感染伴开裂、晚期外阴瘢痕1例;非婴幼儿组5例,其中切口出血3例,留置尿管合并泌尿道感染2例;两组并发症发生率差异无统计学意义(P>0.05)。两组外生殖器外观满意度比较结果显示,外观对称性、阴蒂大小、阴蒂位置、阴道口外观、阴唇外观、生殖器皮肤质量评分比较,差异均无统计学意义(P>0.05);但两组外生殖器外观满意度总得分差异有统计学意义(P<0.05)。结论是否于婴幼儿期接受手术是CAH合并外生殖器男性化女童行FG术后外生殖器外观总体满意度的独立影响因素,3岁前行FG患儿其监护人的外生殖器外观满意度评分高于3岁后手术者,且术后并发症发生率较低。Objective To compare the postoperative complications and outcomes of feminizing genitoplasties(FG)in girls with congenital adrenal hyperplasia(CAH)and masculinized external genitalia across different age groups.Methods We conducted a retrospective analysis of 32 girls with CAH and masculinized genitalia who underwent FG procedures at Hebei Children’s Hospital from March 2010 to August 2022.Patients were divided into two groups based on age:infants and young children(≤3 years)and older children group(>3 years).The infant group included 15 cases-14 underwent clitoroplasty and 1 underwent clitoroplasty plus vaginoplasty.The older group included 17 cases-16 received clitoroplasty and 1 received both clitoridoplasty and vaginoplasty.Clinical characteristics were compared between the groups,with significant differences only in surgical age;other factors showed no statistically significant differences(P>0.05).Outcome measures included Prader staging,type of surgical procedure,operation time,follow-up duration,follow-up age,early and late postoperative complications,and caregiver satisfaction with postoperative genital appearance.Results Age at surgery was an independent predictor of overall genital appearance satisfaction postoperatively(OR=0.068,95%CI:0.005-0.849,P<0.05).Among the 32 girls,7(21.88%)experienced postoperative complications:2 cases(13.33%)in the infant group had complications,including incision bleeding(1 case)and early infection with later vulvar scarring(1 case).In the older group,5 cases(29.41%)experienced complications:incision bleeding(3 cases)and catheter-associated urinary infection(2 cases).There were no statistically significant differences between groups in complication rate(P>0.05).Comparative analysis of satisfaction with genital appearance showed no significant differences between the groups for symmetry,clitoral size,clitoral position,vaginal introitus appearance,labial appearance,and genital skin quality(P>0.05).However,the total appearance satisfaction score was significantly higher in

关 键 词：先天性肾上腺皮质增生症 性别发育异常 女性化外生殖器成形术 阴蒂成形术 阴道成形术 

分 类 号：R726.9[医药卫生—儿科]