A posterior-based omega-shaped flap vaginoplasty in girls with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency

OBJECTIVE: To describe a technical modification for constructing a vagina in girls with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency, using a narrower skin base but rich subcutaneous tissue, aiming to obtain both longer and larger vaginal segments with better cosmesis of the external genitalia. PATIENTS AND METHODS: From August 1997 to February 2001, 28 girls (aged 5 months to 17 years) had a neovagina constructed using a posterior-based omega-shaped flap. Twenty-six patients had a low vagina entering into the urogenital sinus and two had a high vagina that entered the urogenital sinus. In those with a high vagina the flap procedure was combined with the Passerini-Glazel technique. All the patients were scheduled for vaginal "calibrations" during the first year after surgery and, according to the result, would then undergo vaginal dilatation. RESULTS: Six children were re-operated; five had plastic surgery to correct genital folds that had regained a scrotal aspect, whereas one with a high vagina developed a urethral stricture, with urinary dribbling and infection, and had the urethra reconstructed. These six children are currently well. No hormone therapy was given to one child for 1 year who is scheduled for further surgery for a re-virilized clitoris. Two patients were lost to follow-up. Up to the last visit, 19 girls had not developed a vaginal stricture and the cosmesis of their external genitalia was deemed good. CONCLUSION: The posterior-based omega-shaped flap enabled both the construction of wider vaginal segments with a low risk of developing stenosis in those with a low vagina, and increased vaginal dimension when associated with the Passerini-Glazel technique for those with a high vagina. However, despite good cosmesis of the external genitalia, the follow-up is too short to confirm whether this technique will meet all the expectations.