Testicular Feminization Syndrome

ABSTRACT

Testicular feminization syndrome or androgen insensitivity syndrome is a rare disorder with an incidence of 120,000-64,000 male births. The individual with complete form of this syndrome (CIAS) have female external genitalia while those with partial form (PIAS) have variable ambiguity of genitalia and often need extensive reconsructive surgery. The diagonosis should be suspected in female child with inguinal hernia or presenting with primary ammenorrohea and on examination there is no vagina with absent axillary or pubic hair. Awareness of this entity is important as with early diagonosis such disorder can be managed appropriately and accurate information can be given to parents regarding long term issues of harmone replacement therapy and fertility.