Abstract
Complete androgen insensitivity syndrome is characterized by normal female appearance, including external genitalia in the presence of a 46 xy karyotype. CAIS is a rare disorder, the prevalence is estimated at between 1 in 20000 and 1 in 60000 live births. However, this condition is found more frequently in the subpopulation of girls with inguinal hernias, and the incidence of CAIS has estimated to be 0.8% to 2.4% in girls with inguinal hernias. In this article two cases of CAIS have been reported.
Case 1: An 18 years old girl with chief complains of primary amenorrhea, in physical examination bilateral palpable testes in inguinal areas. Ultrasonography revealed the absence of uterus, fallopian tubes and proximal vagina. She had a 46 xy karyotype. After consultation, she underwent bilateral orchiectomy and reared as female.
Case 2: A 16 years old boy that had reared female in neonatal period, but after bilateral inguinal herniorrhaphy, five years later she reared as male. After puberty and secondary sexual development this misguided boy underwent bilateral mastectomy and he encountered with some psychosocial problems.
CAIS results in total feminization and the female role should be supported with postpubertal orchiectomy to avoid the risk of malignancy.