Complete androgen insensitivity syndrome (CAIS) is an X-linked recessive disorder resulting from an androgen receptor mutation. Affected individuals have 46,XY karyotype but female pheno-type are raised as girls.1 A short vagina and absent uterus result in infertility, likely psychosexual problems and a risk of later gonadal malignancy such that gona-dectomy is recommended.2 Presentation may be in adolescence with primary amenorrhoea, but 1% of premenstrual phenotypic girls with inguinal hernia (IH) will have CAIS.
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