先天性肾上腺皮质增生症外生殖器整形  

Correction of virilization of external genitalia in children with congenital adrenal hyperplasia

作　　者：夏立广[1] 潘慰吾 周伟中 钱为特 林孝坤 李仲荣[1] 陈聪德[1,2] Xia Liguang;Pan Weiwu;Zhou Weizhong;Qian Weite;Lin Xiaokun;Li Zhongrong;Chen Congde(Department of Pediatric Urology,the Second Affiliated Hospital and Yuying Children’s Hospital of Wenzhou Medical University,Wenzhou 325000,China;Wenzhou Key Laboratory of Children Genitourinary Diseases,Wenzhou 325000,China)

机构地区：[1]温州医科大学附属第二医院育英儿童医院儿童泌尿外科,温州325000 [2]温州市儿童泌尿生殖系统疾病重点实验室,温州325000

出　　处：《中华整形外科杂志》2022年第8期873-880,共8页Chinese Journal of Plastic Surgery

基　　金：浙江省自然科学基金(LY19H070001)。

摘　　要：目的总结先天性肾上腺皮质增生症(CAH)女性患儿行外生殖器整形经验。方法回顾性分析2018年1月至2020年12月温州医科大学附属第二医院育英儿童医院儿童泌尿外科CAH女性患儿的临床资料。根据外生殖器男性化程度的评估(Prader)分级标准对患儿外生殖器外观进行评级,PraderⅡ~Ⅳ级行阴蒂整形术及阴唇整形术,其中,PraderⅢ、Ⅳ级进一步行阴道整形术,根据术中尿道阴道汇合点至外生殖器开口的长度,采用部分尿生殖窦下移术(PUM)或完全尿生殖窦下移术(TUM)。术后随访外生殖器创面恢复情况、患儿排尿控制能力、家属对患儿外生殖器外观满意度。术后6个月时,医师门诊对患儿外生殖器外观进行满意度评估。结果共纳入18例女性患儿,年龄2~9岁,平均4.5岁。PraderⅡ级5例、Ⅲ级9例、Ⅳ级4例。10例患儿(PraderⅢ级9例、Ⅳ级1例)行PUM,3例PraderⅣ级患儿行TUM。术后随访6~12个月,所有患儿外生殖器创面均一期愈合;家属对患儿外生殖器外观满意度:15例满意,3例不满意;3例患儿术后2周发现存在膀胱过度活动症表现,未予以特殊处理,术后3个月时症状消失,其余患儿排尿控制能力无异常。术后6个月,医师门诊对患儿外生殖器外观评估:满意17例,不满意1例。结论根据CAH女性患儿外生殖器男性化程度及尿道阴道共同通道的长度选择手术方式,可获取较好的效果。Objective To summarize and share the experience of one-stage feminization surgery in children with congenital adrenal hyperplasia(CAH).Methods The clinical data of CAH female children in the Department of Pediatric Urology,the Second Affiliated Hospital and Yuying Children’s Hospital of Wenzhou Medical University from January 2018 to December 2020 were retrospectively analyzed.According to the Prader classification standard,the external genital virilism of children was graded.The cases of Prader gradesⅡtoⅣwere underwent clitoroplasty and labiaplasty,while the cases of PraderⅡandⅣgrades were required further vaginoplasty.Partial urogenital sinus mobilization(PUM)or total urogenital sinus mobilization(TUM)was performed according to the length of the urethrovaginal junction to the vulvar opening during the procedure.The postoperative follow-up was included the recovery of the external genitalia wound,the child’s ability to control urination,and the family members’satisfaction with the child’s external genitalia appearance.Six months after the operation,the satisfaction with the appearance of the external genitalia was evaluated in the outpatient clinic,including four aspects:clitoris,labia majora,labia minora,and vaginal appearance.Results A total of 18 female children were enrolled,ranging in age from 2 to 9 years old,with an average age of 4.5 years.There were 5 cases of Prader gradeⅡ,9 cases of gradeⅢ,and 4 cases of gradeⅣ.Ten cases(9 cases of Prader gradeⅢand 1 case of gradeⅣ)underwent PUM,and 3 cases of Prader gradeⅣunderwent TUM.All patients were followed up for 6-12 months after the operation,and all the wounds of the external genitalia of the children were healed in one stage.The satisfaction of family members to the appearance of external genitalia of children:15 cases were satisfied,3 cases were not satisfied;3 patients were found to have overactive bladder two weeks after the operation,and no special treatment was given,the symptoms disappeared three months after the procedure,

关 键 词：儿童 肾上腺皮质疾病 生殖器 男性化 外科 整形 预后 

分 类 号：R726.9[医药卫生—儿科]