Razi Journal of Medical Sciences

  Background & Aim:Many factors such as chromosomes, gonads, hormones and enzymes influence the development and differentiation of embryonic external genitalia. Any defect in these factors leads to intersex condition, a term used to describe discrepancy between external and internal genitals. Physical and emotional complications arising from genital ambiguity may be prevented by appropriate assessment and planning. This study investigated methods of treating patients with genital ambiguity and compared the results of two-stage surgery with one-stage surgery in the more common form of disorders of sexual differentiation that is female pseudohermaphroditism due to adrenal hyperplasia.

  Patients and Method: A retrospective study was employed to evaluate the records of 65 patients undergone genital reconstructive surgery in Ali Asghar Children's Hospital, Tehran Children's Hospital, and Seyed-al-Shohada charity Hospital. 50 patients had female pseudohermaphroditism with adrenal hyperplasia origin and 15 patients had other types of intersex disorders. The timing of performing one-stage surgery was between 3 and 6 months of age and for the two-stage type of surgery, the second procedure was performed when the patient was between 5 and 6 years of age. Forty-eight patients with female pseudohermaphroditism underwent clitoroplasty, labioplasty and vaginoplasty to change the masculine external genitalia to feminine. In one-stage group, these procedures were completed concurrently, while in two-stage type, clitoroplasty and labioplasty were done at the age of 3 to 6 months and vaginoplasty was performed when the patient was 5 to 6 years of age.

  Results: 36 out of 48 cases of female pseudohermaphroditism due to the adrenal gland hyperplasia underwent one-stage surgery. 12 cases had a two-stage surgery in which clitoroplasty and labioplasty were performed at the age of 3-6 months and vaginoplasty when the patient was between 5 and 6 years of age. Vaginal opening stenosis being the most common post-surgical complication was not observed in 41. 7% of one-stage surgery group and 75% of two-stage type. The obtained difference was statistically significant (p-value=0. 047).

  Conclusion: In comparison to one-stage surgery, vaginal opening stenosis was not observed in two-stage surgery therefore, two-stage surgery is considered a better method to treat patients with genital ambiguity.