Vaginal reconstruction after initial construction of the external genitalia in girls with salt-wasting adrenal hyperplasia.

A total of 28 patients with salt-wasting adrenal hyperplasia who underwent revision of the external genitalia at an early age (mean age 21.4 months) has been followed for 12 to 20 years (mean 16.3 years). The age at initial surgery ranged from 3 weeks to 5 years. Twenty-five patients had a low and 3 had a high takeoff of the vagina from the urogenital sinus. Of these children 22 (78.5%) required further vaginal reconstructive procedures to achieve a normal vaginal outlet. Of the 22 patients requiring further repair 18 have undergone vaginal reconstructive procedures with an initial success rate of 72.2%. These long-term data suggest that if vaginal reconstructive surgery is performed in infancy details, such as placement of the perineal flap well into the vagina and careful followup of the vaginal outlet, are mandatory for success. If there is evidence of outlet narrowing, periodic dilation will be needed to prevent vaginal stenosis and further vaginal surgery.