目的

通过腹腔镜治疗腹股沟疝女性患儿手术过程中探查内生殖器，提高对完全性雄激素不敏感综合征（CAIS）的早期诊断率。

方法

回顾性分析2012年1月至2021年12月山西省儿童医院收治的月经期间经腹腔镜治疗的536例腹股沟疝女性患儿的临床资料，术中观察盆腔及双侧内环口处情况，如有正常子宫、输卵管及卵巢，常规荷包缝合未闭内环口。本组病例中有3例盆腔没有正常内生殖器，如性腺位于内环口，将性腺牵入腹腔，荷包缝合内环口。

结果

腹腔镜手术治疗腹股沟疝女性患儿时发现无内生殖器的3例患儿进一步行染色体核型及基因检测均确诊为CAIS。

结论

CAIS是一种罕见疾病，腹股沟疝女性患儿的CAIS发生率较高，腹腔镜治疗腹股沟疝女性患儿，术中常规内生殖器探查对CAIS的筛查是一种简单有效的方法，可以达到早期诊断的目的，术中检查内环口性腺情况，探查盆腔如果没有子宫及输卵管时要考虑CAIS，进一步行染色体核型及基因检测明确诊断。

Objective

To improve the early diagnosis rate of complete androgen insensitivity syndrome （CAIS） by examining internal genital organs during laparoscopic treatment of inguinal hernia in female pediatric patients.

Methods

A retrospective analysis was conducted on the clinical data of 536 female pediatric patients with inguinal hernia who underwent laparoscopic treatment during menstruation at Shanxi Children's Hospital from January 2012 to December 2021. During surgery， the condition of the pelvic cavity and bilateral internal inguinal rings was observed. If a normal uterus， fallopian tubes， and ovaries were present， standard purse-string closure of the patent internal ring was performed.

Results

In this group of cases， three patients had no normal internal genital organs in the pelvic cavity. If the gonads were located at the internal ring， they were pulled into the abdominal cavity， and the internal ring was closed with a purse-string suture. Among female pediatric patients undergoing laparoscopic surgery for inguinal hernia， three cases without internal genital organs were found. Further karyotype and genetic testing confirmed the diagnosis of CAIS in all three cases.

Conclusion

CAIS is a rare disease. The incidence of CAIS is relatively high in female pediatric patients with inguinal hernia. During laparoscopic treatment of inguinal hernia in female pediatric patients， routine intraoperative examination of internal genital organs is a simple and effective method for screening CAIS， which can achieve the goal of early diagnosis. During surgery， if gonads are found at the internal ring and no uterus or fallopian tubes are found in the pelvic cavity， CAIS should be considered， and further karyotype and genetic testing should be performed to confirm the diagnosis.