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Published on: October 2, 2018
Testicular feminization: the androgen insensitivity syndrome
R Shah1, M M Woolley, G Costin
1Division of Pediatric, Children's Hospital of Los Angeles, CA 90027.
Journal of Pediatric Surgery
|June 1, 1992
Summary
Testicular feminization (TF) is an androgen insensitivity syndrome. Early diagnosis and raising patients as female, with early gonadectomy, improves outcomes and avoids complications from reconstructive surgery.
Area of Science:
- Endocrinology
- Genetics
- Pediatric Surgery
Background:
- Testicular feminization (TF) is a condition of androgen insensitivity, presenting in complete (CTF) and incomplete (ITF) forms.
- Patients often present with ambiguous genitalia or are diagnosed incidentally during hernia repair or family history screening.
Purpose of the Study:
- To review the clinical presentation, diagnosis, and management of 21 patients with TF over 24 years.
- To evaluate the outcomes of different sex assignments and surgical interventions in TF patients.
Main Methods:
- Retrospective review of 21 patients with a 46,XY karyotype diagnosed with TF.
- Analysis of presenting symptoms, diagnostic methods, sex assignment, surgical interventions (gonadectomy, genital reconstruction), and long-term outcomes.
Main Results:
- Patients presented with ambiguous genitalia, inguinal hernias, or through family history.
- Complete TF patients were phenotypically female; ITF patients showed variable external genitalia.
- Patients raised as females with early gonadectomy had better outcomes than those raised as males undergoing reconstructive surgery.
Conclusions:
- Early diagnosis of TF through careful evaluation of ambiguous genitalia and inguinal hernias in females is crucial.
- Raising TF patients as female with early gonadectomy is recommended due to poor results of genital reconstructive surgery in males.
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