Review Congenital Adrenal Hyperplasia - A Gender Crisis

Introduction

Congenital adrenal hyperplasia (CAH) is the most common cause of genital ambiguity in females. Sex assignment should be done as early as possible; however, in India, because of the social taboo associated with gender, these conditions are usually diagnosed late, leading to a dilemma of sex assignment.

Five cases of congenital adrenal hyperplasia are highlighted here, whose management depended on their presentation and the gender with which they wished to be raised. Out of the five cases, one patient presented with gynaecomastia and absent testis, and since the patient wished to be raised as a male, the patient was planned for a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Two patients underwent feminizing genitoplasty surgery (glans preserving reduction clitoroplasty with perineal flap vaginoplasty), and the other two underwent a simple glans preserving reduction clitoroplasty.

Conclusion

Advances in surgery have helped in improving the social life of patients with this traumatizing defect.