MC Ho, KL Ng

Abstract

Complete androgen insensitivity syndrome (CAIS) is a rare X-linked disorder with a female external phenotype.¹ We report on a Chinese baby with CAIS who presented with bilateral inguinal hernias. The baby has a normal female phenotype at birth. On further evaluation, the karyotype was found to be 46, XY. Endocrine findings were compatible with androgen insensitivity syndrome. Bilateral herniotomy was performed. We would review the literature, offer an overview of the disease, and discuss the outcome with emphasis on the treatment modalities.