1-Stage feminizing genitoplasty: 8 years of experience with 49 cases.

摘要

PURPOSE: We retrospectively evaluated the results of a modified technique for 1-stage feminizing genitoplasty in children with congenital adrenal hyperplasia (CAH) and other rare intersex conditions. MATERIALS AND METHODS: Between 1991 and 1998, 47 infants and 2 adolescents underwent 1-stage feminizing genitoplasty at our hospital. Of the patients 44 had CAH, 2 had the partial androgen insensitivity syndrome and 3 were true hermaphrodites. In 3 patients we used the Passerini-Glazel technique, while in 46 our technique of feminizing genitoplasty was used, which consists of maximal mobilization of the urogenital sinus en bloc including the vagina and urethra via the perineal approach. Two lateral plates built from dorsal split phallic and preputial skin together with the dorsal split urogenital sinus are used to construct the vaginal introitus and labia minora, and the scrotal folds are used to construct the labia majora. Reduction clitoroplasty is done according to the technique of Kogan et al. Mean age plus or minus standard deviation was 0.9 +/- 0.3 years of the patients with CAH and 13 +/- 2.3 of the remainder. Preoperative information, including status of the internal genitalia, anatomical length of the vagina site of communication between the vagina and urogenital sinus, in relation to the pelvic floor, was obtained by transabdominal pelvic ultrasound. RESULTS: Operating time ranged between 120 and 180 minutes, average hospitalization period was 4 to 5 days and mean followup was 4.7 +/- 2.6 years. Preoperative ultrasound provided the correct data regarding the vaginal and internal genitalia anatomy in all cases, and the exact communication site between the vagina and urogenital sinus was demonstrated in 93%. Complications consisted of intraoperative rectal injury in 1 case and mild wound infection of the buttocks area in 3. In 1 case total clitoris loss was observed. All patients who underwent modified genitoplasty have had successful cosmetic and early functional results. Two patients presented with repeat clitoromegalia due to inadequate adrenal suppression. CONCLUSIONS: Our adapted technique of 1-stage feminizing genitoplasty provides good cosmetic and functional results. The functional results of this operation should be reevaluated after puberty and in adulthood.