Diagnosis and treatment of female pseudohermaphroditism caused by congenital adrenal hyperplasia (report of 4 cases) / 中华泌尿外科杂志

ABSTRACT

Objective To report the effect of hormone replacement plus orthopedic surgery for 4 patients with female pseudohermaphroditism caused by congenital adrenal hyperplasia. Methods Four patients with female pseudohermaphroditism caused by congenital adrenal hyperplasia were included.On admission their ages were 11,15,14 and 20 years,respectively.The diagnoses were confirmed by medical histories,ultrasonography,serum levels of hormones and chromosome analysis.They all underwent clitoroplasty and vaginoplasty with perineal skin flap. Results After operation the patients received estrogen and progestogen for a short time to adjust the menstrual cycle and hydrocortisone replacement(10 to 20 mg,twice a day) for their lifetime.Normal menstruation and well-developed breasts were observed in all the patients.No constriction occurred in the reconstructed vaginas. Conclusions This disease can be easily misdiagnosed as hypospadias with bilateral cryptorchidism.Therefore,in addition to careful physical examination,the measurement of serum hormones,ultrasonography of the abdomen,and chromosome analysis are necessary.Proper utilization of the perineal skin flap for reconstruction of the clitoris and vagina as well as careful perioperative managements in combination with hormone replacement therapy can greatly contribute to satisfactory results.