Surgical treatment of intersex disorders

Abstract

Despite the progress made in understanding the factors regulating sexual differentiation, infants born with ambiguous genitalia face significant problems. The authors reviewed a group of 84 children with ambiguous genitalia managed surgically between 1986 and 1993. The most frequent condition was male pseudohermaphroditism (PM) (58%); 31% had female pseudohermaphroditism. Fifty-seven percent of patients were raised as males and 43% as females. In each group of patients, feminine and masculine reconstructive operations were performed. In only 31% of PM and 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 timing and type of vaginoplasty were determined by the point of entry of the vagina into the urogenital sinus. Of the 29 patients reared as females, 22 required perineal vaginoplasty, had pull-through vaginoplasty, and 2 had colovaginoplasty. Since 1986, we have applied Mollard's clitoroplasty, which preserves the neurovascular bundle and is important for experiencing orgasm. Seventeen percent of patients with feminization procedures experienced complications. The optimal time for masculinization procedures is 2 years of age, after obligatory testosterone treatment. If there is utriculus prostaticus (UP) type II or III, it is removed before urethroplasty. This is not done for UP types 0 and I. 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 vagina than a phallus, not taking into consideration dimensions, aesthetics, or capability of erection of the phallus. The basis of surgical treatment of intersex disorders is not to coordinate the phenotype and the genotype, but rather to form the external genital organs which will be of the appropriate appearance and which will allow functional sexuality. It is much easier to create a vagina as a passive organ than an erectile phallus with sufficient dimension. Therefore, the authors suggest that most such infants be reared as females.