Gynecology

Purpose: The presentations of 17β-hydroxysteroid dehydrogenase type 3 deficiency, 5α-reductase type 2 deficiency, and complete androgen insensitivity snydrome can be clinically similar. However, individuals with 17β-hydroxysteroid dehydrogenase type 3 and 5α-reductase type 2 deficiencies will undergo pubertal virilization and should undergo gonadectomy prior to the age of maturation if reared in the female gender. The decision of when to perform gonadectomy among individuals with complete androgen insensitivity remains controversial. Proponents of delayed gonadectomy suggest that endogenous estrogen results in natural breast development and improved bone health. Proponents of early gonadectomy suggest that individuals are at increased risk of gonadal malignancy if the testes are left in situ. Methods: Two sisters were initially diagnosed with complete androgen insensitivity snydrome as young children after testes were discovered during hernia surgery. A decision was made to delay go...