Complete Testicular Feminization Syndrome: a Case Report.
- Author:
Ma Hae CHO
1
;
Eun Sook NAM
Author Information
1. Pyungchon Daehang Surgical Clinic, Anyang, Korea. mcho1@chol.com
- Publication Type:Case Report
- Keywords:
Testicular feminization syndrome;
Androgen insensitivity syndrome;
Inguinal hernia
- MeSH:
Adolescent;
Amenorrhea;
Androgen-Insensitivity Syndrome*;
Child;
Diagnosis;
Dihydrotestosterone;
Female;
Genitalia;
Hernia, Inguinal;
Humans;
Infant;
Karyotype;
Ligation;
Male;
Ovary;
Testis;
Testosterone;
Uterus
- From:Journal of the Korean Association of Pediatric Surgeons
2006;12(1):47-52
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Testicular feminization syndrome (TFS) is a genetic disorder due to androgen insensitivity of the target organs. The most common clinical presentation of complete TFS is inguinal hernia in the infant or primary amenorrhea in the adolescence. A 7-year old phenotypically female patient was seen with a complaint of a right inguinal mass. Under the diagnosis of right inguinal hernia, high ligation was performed. Six months later, the patient showed a left inguinal mass. On operation, the mass looked like a testis. The external genitalia were normal female, but a uterus and ovary were not identified. Chromosome study showed a 46, XY karyotype and the levels of serum testosterone and dihydrotestosterone were increased after HCG stimulation. The patient was diagnosed as complete TFS and underwent bilateral gonadectomy 6 months later.
