Cover Image




Androgen Insensitivity Syndrome, previously called as Testicular feminisation syndrome, is an X-linked recessive rare disorder. It was initially described by Morris in 1953. It can be either complete (CAIS), mild (MAIS) or partial androgen insensitivity (PAIS) depending on the receptors response to testosterone. Partial androgen insensitivity  syndrome describes a variety of disorders that result in   androgen action less severe than those associated with  complete androgen sensitivity. The individual is  phenotypically female and genotypically male (XY) a male pseudohermaphrodite. The condition is suspected when the individual is evaluated for primary amenorrhoea, infertility, or when unilateral or bilateral inguinal hernia is diagnosed in females or lack of virilisation in males at puberty. Treatment is directed towards gender assignment, psychological support, gonadectomy, vaginoplasty and hormonal therapy. Here we present a case of a 25year old female with partial androgen sensitivity syndrome with primary amenorrhoea with normal secondary sexual characters with clitoromegaly and testis in labia majora. She was managed surgically by Reduction clitoroplasty with bilateral gonadectomy. Post operatively patient was started on estrogen replacement therapy with Tab.Premarin 0.625mg daily.


Full Text:



a) Achermann JC, Jameson JL (2006). "Disorders of sexual differentiation". In Hauser SL, Kasper DL, Fauci AS, Braunwald E, Longo DL. Harrison's endocrinology. New York: McGraw-Hill Medical Pub. Division. pp. 161–172. ISBN 0-07-145744-5

b) Hughes IA, Houk C, Ahmed SF, Lee PA (July 2006). "Consensus statement on management of intersex disorders". Arch. Dis. Child. 91 (7): 554–63. doi: 10.1136/adc.2006.098319. PMC 2082839.PMID 16624884

c) Hughes IA (February 2008). "Disorders of sex development: a new definition and classification". Best Pract. Res. Clin. Endocrinol. Metab. 22 (1): 119–34.doi:10.1016/j.beem.2007.11.001. PMID 18279784

d) Zucker KJ (February 2002). "Intersexuality and gender identity differentiation". J Pediatr Adolesc Gynecol 15 (1):

–13. doi:10.1016/S1083-3188(01)00133-4. PMID 11888804

a) Evans BA, Hughes IA, Bevan CL, Patterson MN, Gregory JW (June 2007). "Phenotypic diversity in siblings with partial androgen insensitivity syndrome". Arch. Dis. Child. 76 (6): 529–31. doi: 10. 1136/ adc. 76.6. 529. PMC 1717223. PMID 9245853.

b) Creighton SM, Minto CL, Steele SJ (July 2001). "Objective cosmetic and anatomical outcomes at adolescence of feminising surgery for ambiguous genitalia done in childhood".Lancet 358 (9276):124–5. doi:10.1016/S0140-6736(01)05343-50 PMID 11463417


  • There are currently no refbacks.

Creative Commons License
This work is licensed under a Creative Commons Attribution-NoDerivatives 4.0 International License.

An Initiative of The Tamil Nadu Dr MGR Medical University