Objective: Early genital surgery has been the routine practice in virilized girls with severe forms of CAH for many years. During the last decade studies have shown genital surgery to have unsatisfactory effects on genital sensation and sexuality, and the current practice with early surgery has been questioned by patients and support groups as well as by clinicians and researchers. As surgery has been postponed in only few girls, published data on the effect of hydrocortisone treatment on clitoral size are sparse. We aimed to investigate the effect of hydrocortisone treatment on clitoral size during the first year of life.
Methods: Six girls with CAH due to 21-hydroxylase deficiency were investigated at birth before start of treatment with hydrocortisone, and when vaginoscopy was performed at a mean age of 0.7 years (range 0.4 1.0). Clitoral length and width were measured and the clitoral index was calculated from the product of the length and the width, and expressed as square millimeters. Anthropometric measurements were taken and body surface area (BSA) was calculated. To evaluate clitoral size in relation to general growth clitoral index per square meter BSA was calculated. Hydrocortisone and fludrocortisone dosages at time of vaginoscopy were recorded.
Results: The changes in clitoral length (26.3 mm (range 19.032.0) vs. 25.7 mm (range 12.035.0), P=0.674), width (10.3 mm (range 8.015.0) vs. 7.5 mm (range 5.010.0), P=0.066) and clitoral index (273.3 mm2 (range 152384) vs. 201.7 mm2 (range 60320), P=0.116) were not significant. There was a significant reduction in clitoral index per m2 BSA (1264 mm2/m2 (range 7091909) vs. 534 mm2/m2 (range 140967), P<0.05). The mean Hydrocortisone dose was 11.2 mg/m2 BSA/day, (range 9.312.6), and the mean Fludrocortisone dose was 199.0 microgram/m2 BSA/day (range 58453).
Conclusion: In this small observational study Hydrocortisone treatment with regular recommended dosages was sufficient to prevent clitoral growth during the first year of life. Clitoral index showed a significant decrease per square meter body surface area, indicating that clitoromegaly becomes less marked as the girls grow.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology