Korean J Urol.  2001 Sep;42(9):984-990.

The Surgical Management of the Ambiguous Genitalia in 16 Cases

Affiliations
  • 1Department of Urology, College of Medicine, Kyungpook National University, Daegu, Korea.

Abstract

PURPOSE: A change in gender assignment after 2 years of age is associated with severe psychological problems for the child and family. It is important that a definitive diagnosis be determined as quickly as possible. The treatment of ambiguous genitalia will be different by individual difference. We reviewed 16 cases of ambiguous genitalia patients with the object of encouraging early diagnosis and proper treatment individually.
MATERIALS AND METHODS
We reviewed retrospectively 16 patients with ambiguous genitalia who were surgically managed at our department. Diagnostic workup included chromosomal analysis, blood and urine steroid measurement, hormonal study and radiologic study. The patients consisted of female pseudohermaphroditism in five cases, male pseudohermaphroditism in nine cases, true hermaphroditism and mixed gonadal dysgenesis in one case in each. The groups were analyzed according to karyotype, sex of rearing, age at diagnosis, age at operation, op procedure, post op complication and follow up.
RESULTS
Five cases of female pseudohermaphroditism were raised as female in three cases and male in two cases, re-assigned and surgically corrected as four females and one male. Nine cases of male pseudohermaphroditism were raised as female in six cases and male in three cases, re-assigned and surgically corrected as three females and six males. One case of true hermaphroditism was surgically corrected as male. One case of mixed gonadal dysgenesis was surgically corrected as female and then given hormonal therapy. Four patients had sex conversion after 2 years of age.
CONCLUSIONS
Though early diagnosis and treatment are most important, most patients were diagnosed and treated after 2 years of age. A continuous effort should be made to educate parents and alert attending physicians so that early diagnosis and treatment of these patients could be made as soon as possible.

Keyword

Ambiguous genitalia; Hermaphroditism; Surgical management

MeSH Terms

46, XX Disorders of Sex Development
46, XY Disorders of Sex Development
Child
Diagnosis
Disorders of Sex Development*
Early Diagnosis
Female
Follow-Up Studies
Gonadal Dysgenesis, Mixed
Humans
Individuality
Karyotype
Male
Ovotesticular Disorders of Sex Development
Parents
Retrospective Studies
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