Complete androgen insensitivity syndrome: An uncommon presentation with acute abdomen

Abstract

The typical presentation of complete androgen insensitivity syndrome (CAIS) is with an inguinal hernia in an infant or a child and with primary amenorrhea in adult phenotypic female. We hereby report a case reared as a female and considered to have primary amenorrhea, who presented with acute abdomen, provisionally diagnosed to have torsion of ovary and underwent laparoscopic gonadectomy. Histopathology revealed gangrenous testicular tissue; karyotyping was 46XY. So, finally, a diagnosis of complete androgen insensitivity with torsion of intra-abdominal testis was made. We also discuss the rarity of such a presentation, risk of neoplasia, and timing of gonadectomy in such patients.