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Surgical treatment of intersex disorders
Z Krstić1, S Perovic, S Radmanović
1Department of Pediatric Surgery, University Children's Hospital, Belgrade, Yugoslavia.
Insights
Surgical management of ambiguous genitalia in 84 children revealed male pseudohermaphroditism as the most common condition. Early diagnosis and treatment are crucial for optimal outcomes in these complex cases.
Area of Science:
- Pediatric Surgery
- Endocrinology
- Genetics
Background:
- Infants with ambiguous genitalia present complex challenges despite advances in understanding sexual differentiation.
- Surgical intervention is often required to address anatomical abnormalities.
Purpose of the Study:
- To review surgical management outcomes for children with ambiguous genitalia.
- To analyze the timing of diagnosis and treatment in relation to patient outcomes.
Main Methods:
- Retrospective review of 84 children with ambiguous genitalia who underwent surgery between 1986 and 1993.
- Analysis of diagnosis timing, assigned sex, surgical procedures, and complications.
Main Results:
- Male pseudohermaphroditism (58%) and female pseudohermaphroditism (31%) were the most frequent diagnoses.
- Diagnosis within 2 months occurred in only 31% (PM) and 60% (PF) of cases.
- Treatment initiation before age two was considered acceptable for 41% (PF) and 40% (PM) of patients.
- Vaginoplasty techniques varied based on the urogenital sinus entry point; Mollard's clitoroplasty was used for neurovascular preservation.
- Complications occurred in 17% of feminization procedures.
- Masculinization procedures are optimally timed at 2 years, following testosterone treatment, with specific management for utriculus prostaticus (UP) types.
Conclusions:
- Surgical outcomes for ambiguous genitalia depend on accurate diagnosis and timely intervention.
- Reconstructive surgery, including vaginoplasty and phalloplasty, requires careful planning based on individual anatomy.
- Preservation of neurovascular bundles during clitoroplasty is vital for potential sexual function.
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 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 vagina than a phallus, not taking into consideration dimensions, aesthetics, or capability of erection of the phallus.(ABSTRACT TRUNCATED AT 250 WORDS)