Journal of South Asian Federation of Obstetrics and Gynaecology

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VOLUME 15 , ISSUE 6 ( November-December, 2023 ) > List of Articles

CASE REPORT

An Atypical Case of Complete Androgen Insensitivity Syndrome Presenting in Adulthood

Sakshi Dinesh Firke, Gajanan Patil, Kamlesh Chaudhari

Keywords : Androgen insensitivity syndrome, Case report, Sex differentiation disorder, Testosterone

Citation Information : Firke SD, Patil G, Chaudhari K. An Atypical Case of Complete Androgen Insensitivity Syndrome Presenting in Adulthood. J South Asian Feder Obs Gynae 2023; 15 (6):721-724.

DOI: 10.5005/jp-journals-10006-2332

License: CC BY-NC 4.0

Published Online: 04-12-2023

Copyright Statement:  Copyright © 2023; The Author(s).


Abstract

Aim and background: A report about case presenting at atypical age, adulthood in a female; later diagnosed to be complete androgen insensitivity syndrome. Virtue of this case is to give message for practicing and budding gene experts, gynecologist, about early diagnosis for prenatal screening of fetus and prevention of malignancy. Case description: A 23-year-old phenotypically female came with a chief complaint of primary amenorrhea, and examination and investigations were carried out. Later case was diagnosed as complete androgen insensitivity syndrome and managed with orchidectomy with achievement of satisfactory vaginal length with serial vaginal lengthening and dilation followed by estrogen supplements and psychological counseling. Conclusion: Collaboration of experts and sympathetic handling via psychologist, endocrinologist, and gynecologist counseled for current gender acceptance, sexual functioning, and quality of life are backbones. Clinical significance: Androgen insensitivity syndrome otherwise known as testicular feminization syndrome is a rare inherited, sex differentiation disorder. It is a testosterone hormone-resistant disorder usually presenting in their childhood to puberty period range. The most concerning negative outcome for women is malignant alterations within the gonads, but their removal to prevent cancer is contested. Main three-pronged treatment focuses on orchidectomy, interventions in external genitalia reconstruction, and suitable post operatively hormonal therapy of estrogen. Action plan for achieving fertility is not yet stabilized.


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