Congenital Adrenal Hyperplasia (CAH) is caused by congenital insufficiency of the enzyme 21 - hydroxylase (21-OHD) in the cortisol synthesis pathway. Because of the virilizing effects of androgens over-production, affected girls develop clitoral hypertrophy. Three patients with CAH are discussed below along with their surgical management and follow-up.

Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder affecting adrenal steroid synthesis. More than 95% of CAH cases are caused by reduced 21hydroxylase function leading to variable extent of cortisol and aldosterone deficiency in addition to androgen excess. The foundation of CAH treatment is the use of glucocorticoids. However, overtreatment leads to Cushing’s syndrome and ...