Restoring normal anatomy in female patients with atypical genitalia

Abstract Female patients with congenital adrenal hyperplasia (CAH) have varying degrees of atypical genitalia secondary to prenatal and postnatal androgen exposure. Surgical treatment is focused on restoring normal genitalia anatomy by bringing the vagina to the normal position on the perineum, separating the distal vagina from the urethra, forming a normal introitus and preserving sexual function of the clitoris by accepting moderate degrees of hypertrophy as normal and strategically reducing clitoral size only in the most severely virilized patients. There remains a need for continued monitoring of patients as they go through puberty with the possibility of additional surgery for vaginal stenosis. Anatomically based surgery and refinement in surgical techniques with acceptance of moderate degrees of clitoral hypertrophy as normal should improve long-term outcomes.