Laparoscopic Repositioning of Gonads from the Labia Majora or Inguinal Canal into the Abdominal Cavity in Pediatric Complete Androgen Insensitivity Syndrome Patients with Inguinal Hernia

Abstract

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.