OBJECTIVE: Girls with congenital adrenal hyperplasia show a variable degree of genital masculinization at birth. Antenatal dexamethazone treatment for the mother is known to reduce the severity of this condition. There are however few data on the effect of postnatal steroid therapy on the cosmetic appearance of the external genitalia. PATIENTS AND METHOD: We report the appearance of the external genitalia of three girls with classical congenital adrenal hyperplasia followed up by steroid therapy alone. RESULTS: Growth of the labia majora and a relative reduction in clitoral size improved the appearance of the external genitalia in these three girls. CONCLUSION: There seems to be improvement in the external appearance of genitalia with postnatal steroid therapy. This could allay parental concerns and help in the planning of corrective surgery peripubertally with the informed consent of the child.
OBJECTIVE:Girls with congenital adrenal hyperplasia show a variable degree of genital masculinization at birth. Antenatal dexamethazone treatment for the mother is known to reduce the severity of this condition. There are however few data on the effect of postnatal steroid therapy on the cosmetic appearance of the external genitalia. PATIENTS AND METHOD: We report the appearance of the external genitalia of three girls with classical congenital adrenal hyperplasia followed up by steroid therapy alone. RESULTS: Growth of the labia majora and a relative reduction in clitoral size improved the appearance of the external genitalia in these three girls. CONCLUSION: There seems to be improvement in the external appearance of genitalia with postnatal steroid therapy. This could allay parental concerns and help in the planning of corrective surgery peripubertally with the informed consent of the child.
Authors: Raoul C M Hennekam; Judith E Allanson; Leslie G Biesecker; John C Carey; John M Opitz; Eric Vilain Journal: Am J Med Genet A Date: 2013-05-06 Impact factor: 2.802