Lee Sung Ryul
Department of Surgery, Damsoyu Hospital, Gangnam-gu, Seoul, Republic of Korea,
Sex Dev. 2020;14(1-6):33-39. doi: 10.1159/000514070. Epub 2021 Mar 4.
Androgen insensitivity syndrome (AIS) is a congenital condition characterized by a 46,XY karyotype but with a female phenotype caused by mutations in the androgen receptor gene located on the X chromosome. In patients with complete AIS (CAIS), preservation of the gonad is recommended until puberty, and gonadectomy can be regarded subsequently. The location of the gonads should be considered, because positions in the labia majora or inguinal canals can cause discomfort. Here, the laparoscopic reposition of gonads into the abdominal cavity in pediatric patients with CAIS is reported. From 2013 to 2019, laparoscopic inguinal hernia repair was performed in 2,061 pediatric patients with inguinal hernias aged <10 years and with female external genitalia. Among them, 11 had CAIS. Gonads located in the labia majora or inguinal canal were repositioned into the abdominal cavity. The mean age was 18.9 months (range 1-110 months). The gonads were located in the inguinal canal in 7 patients, in the labia majora in 3, and in the abdominal cavity in 1. Laparoscopic repositioning of such gonads into the abdominal cavity is feasible in pediatric patients with an inguinal hernia and CAIS.
雄激素不敏感综合征(AIS)是一种先天性疾病,其特征为染色体核型为46,XY,但因位于X染色体上的雄激素受体基因突变而表现为女性表型。对于完全性雄激素不敏感综合征(CAIS)患者,建议在青春期前保留性腺,随后可考虑性腺切除术。应考虑性腺的位置,因为位于大阴唇或腹股沟管会引起不适。本文报道了对CAIS患儿进行腹腔镜下性腺复位至腹腔的情况。2013年至2019年,对2061例年龄<10岁、患有腹股沟疝且外生殖器为女性的儿科患者进行了腹腔镜腹股沟疝修补术。其中11例患有CAIS。将位于大阴唇或腹股沟管的性腺复位至腹腔。平均年龄为18.9个月(范围1 - 110个月)。7例患者的性腺位于腹股沟管,3例位于大阴唇,1例位于腹腔。对于患有腹股沟疝和CAIS的儿科患者,通过腹腔镜将此类性腺复位至腹腔是可行的。