SURGICAL-TREATMENT OF INTERSEX DISORDERS

Despite the progress made in understanding the factors regulating sexu al differentiation, infants born with ambiguous genitalia face signifi cant problems, The authors reviewed a group of 84 children with ambigu ous genitalia managed surgically between 1986 and 1993. The most frequ ent condition was male pseudohermaphroditism (PM) (58%); 31% had femal e pseudohermaphroditism. Fifty-seven percent of patients were raised a s males and 43% as females. In each group of patients, feminine and ma sculine reconstructive operations were performed. In only 31% of PM an d 60% of PF cases was the diagnosis made within the first 2 months of life. In 41% of PF and 40% of PM patients, treatment was begun before the second year of life, which we consider an acceptable time. The tim ing and type of vaginoplasty were determined by the point of entry of the vagina into the urogenital sinus. Of the 29 patients reared as fem ales, 22 required perineal vaginoplasty, had pull through vaginoplasty , and 2 had colovaginoplasty. Since 1986, we have applied Mollard's cl itoroplasty, which preserves the neurovascular bundle and is important for experiencing orgasm. Seventeen percent of patients with feminizat ion procedures experienced complications. The optimal time for masculi nization procedures is 2 years of age, after obligatory testosterone t reatment. If there is utriculus prostaticus (UP) type II or III, it is removed before urethroplasty. This is not done for UP types 0 and 1. In PM cases, the number of feminization and masculinization operations was 2.1 and 4.05 per patient, respectively. It is easier to make a va gina than a phallus, not taking into consideration dimensions, aesthet ics, or capability of erection of the phallus. The basis of surgical t reatment of intersex disorders is not to coordinate the phenotype and the genotype, but rather to form the external genital organs which wil l be of the appropriate appearance and which will allow functional sex uality. It is much easier to create a vagina as a passive organ than a n erectile phallus with sufficient dimension. Therefore, the authors s uggest that most such infants be reared as females. Copyright (C) 1995 by W.B. Saunders Company.