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Gender Dysphoria and Gender Change in Androgen Insensitivity or Micropenis

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Abstract

This review article answers three questions relevant to the medical management and care of individuals born with complete androgen insensitivity syndrome (CAIS), partial androgen insensitivity syndrome (PAIS), or a micropenis: (1) Do any of these individuals reassign themselves from their initial gender assignment? (2) Do more reassign than the ones who do not? (3) Is there evidence of gender dysphoria in those who do not self-initiate reassignment? Reviewed were all articles on CAIS, PAIS, and micropenis cited in K. J. Zucker (1999) plus articles published through 2004. There were no documented cases of gender change in individuals with CAIS (N= 156 females) or micropenis (N= 89: 79 males, 10 females). Nine (9.1%) out of 99 individuals with PAIS changed gender. Thus, self-initiated gender reassignment was rare. Gender dysphoria also appears to be a rare occurrence. The best predictor of adult gender identity in CAIS, PAIS, and micropenis is initial gender assignment.

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References

  • Assael, M., Lancet, M., & Shani, A. (1976). Incomplete testicular feminization syndrome: Clinical and psychiatric aspects. Psychiatria Clinic, 9, 118–129.

    Google Scholar 

  • Beheshti, M., Hardy, B. E., Churchill, B. M., & Daneman, D. (1983). Gender assignment in male pseudohermaphrodite children. Urology, 22, 604–607.

    Article  PubMed  Google Scholar 

  • Bin-Abbas, B., Conte, F. A., Grumbach, M. M., & Kaplan, S. L. (1999). Congenital hypogonadotropic hypogonadism and micropenis: Effect of testosterone treatment on adult penile size—Why sex reversal is not indicated. Journal of Pediatrics, 134, 579–583.

    PubMed  Google Scholar 

  • Burstein, S., Grumbach, M. M., & Kaplan, S. L. (1979). Early determination of androgen-responsiveness is important in the management of microphallus. Lancet, 301, 983–986.

    Article  Google Scholar 

  • Costa, E. M. F., Mendonca, B. B., Inacio, M., Arnhold, I. J. P., Silva, F. A. Q., & Lodovici, O. (1997). Management of ambiguous genitalia in pseudohermaphrodites: New perspectives on vaginal dilation. American Society for Reproductive Medicine, 67, 229–232.

    Google Scholar 

  • Dessens, A. B., Slijper, F. M. E., & Drop, S. L. S. (2005). Gender dysphoria and gender change in chromosomal females with congenital adrenal hyperplasia. Archives of Sexual Behavior, 34, 389–397.

    Google Scholar 

  • Dessouky, N. M. (2003). Gender assignment for children with intersex problems: An Egyptian perspective. Saudi Medical Journal, 24(Suppl. 5), S51–52.

    Google Scholar 

  • Diamond, M., & Sigmundson, H. K. (1997). Management of intersexuality: Guidelines for dealing with persons with ambiguous genitalia. Archives of Pediatrics & Adolescent Medicine, 151, 1046–1050.

    Google Scholar 

  • Diamond, M., & Watson, L. A. (2004). Androgen insensitivity syndrome and Klinefelter’s syndrome: Sex and gender considerations. Child and Adolescent Psychiatric Clinics of North America, 13, 623–640.

    Article  PubMed  Google Scholar 

  • Egan, S. K., & Perry, D. G. (2001). Gender identity: A multidimensional analysis with implications for psychosocial adjustment. Developmental Psychology, 37, 451–463.

    Article  PubMed  Google Scholar 

  • Gooren, L., & Cohen-Kettenis, P. T. (1991). Development of male gender identity/role and a sexual orientation towards women in a 46,XY subject with an incomplete form of the androgen insensitivity syndrome. Archives of Sexual Behavior, 20, 459–470.

    Article  PubMed  Google Scholar 

  • Hannema, S. E., Scott, I. S., Hodapp, J., Martin, H., Coleman, N., Schwabe, J. W., et al. (2004). Residual activity of mutant androgen receptors explains wolffian duct development in the complete androgen insensitivity syndrome. Journal of Clinical Endocrinology & Metabolism, 89, 5815–5822.

    Google Scholar 

  • Hines, M., Ahmed, F. S., & Hughes, I. A. (2003). Psychological outcomes and gender related development in complete androgen insensitivity syndrome. Archives of Sexual Behavior, 32, 93–101.

    Article  PubMed  Google Scholar 

  • Hinman, Jr., F. (1972). Microphallus: Characteristics and choice of treatment from a study of 20 cases. Journal of Urology, 107, 499–505.

    PubMed  Google Scholar 

  • Hooper, H. T., Figueiredo, B. C., Pavan-Senn, C. C., De Lacerda, L., Sandrini, R., Mengarelli, J. K., et al. (2004). Concordance of phenotypic expression and gender identity in a large kindred with a mutation in the androgen receptor. Clinical Genetics, 65, 183–190.

    Article  PubMed  Google Scholar 

  • Houk, C. P., Dayner, J., & Lee, P. A. (2004). Genital ambiguity with a Y chromosome: Does gender assignment matter? Journal of Pediatric Endocrinology and Metabolism, 17, 825–839.

    PubMed  Google Scholar 

  • Husmann, D. A. (2004). The androgen insensitive micropenis: Long-term follow-up into adulthood. Journal of Pediatric Endocrinology & Metabolism, 17, 1037–1041.

    Google Scholar 

  • Imperato-McGuinley, J., Pichardo, M., Gautier, T., Voyer, D., & Bryden, M. P. (1991). Cognitive abilities in androgen-insensitive subjects: Comparison with control males and females from the same kindred. Clinical Endocrinology, 34, 341–347.

    PubMed  Google Scholar 

  • Lee, P. A., & Houk, C. P. (2004). Outcome studies among men with micropenis. Journal of Pediatric Endocrinology & Metabolism, 17, 1043–1053.

    Google Scholar 

  • Lee, P. A., Mazur, T., Danish, R., Amrhein, J. A., Blizzard, R. M., Money, J., et al. (1980). Micropenis. I. Criteria, etiologies, and classification. Johns Hopkins Medical Journal, 145, 156–163.

    Google Scholar 

  • Madden, J. D., Walsh, P. C., MacDonald, P. C., & Wilson, J. D. (1975). Clinical and endocrinologic characterization of a patient with the syndrome of incomplete testicular feminization. Journal of Clinical Endocrinology and Metabolism, 41, 751–760.

    PubMed  Google Scholar 

  • Masica, D. N., Money, J., & Ehrhardt, A. A. (1971). Fetal feminization and female gender identity in the testicular feminizing syndrome of androgen insensitivity. Archives of Sexual Behavior, 1, 131–142.

    Article  Google Scholar 

  • Mazur, T., Sandberg, D. E., Perrin, M. A., Gallagher, J. A., & MacGillivray, M. H. (2004). Male pseudohermaphroditism: Long-term quality of life outcome in five 46,XY individuals reared female. Journal of Pediatric Endocrinology and Metabolism, 17, 809–823.

    PubMed  Google Scholar 

  • Melo, K. F. S., Mendonca, B. B., Billerbeck, A. E., Costa, E. M. F., Inacio, M. I. F., Silva, F. A. Q., et al. (2003). Clinical, hormonal, behavioral and genetic characteristics of androgen insensitivity syndrome in a Brazilian cohort: Five novel mutations in the androgen receptor gene. Journal of Clinical Endocrinology and Metabolism, 88, 3241–3250.

    Article  PubMed  Google Scholar 

  • Meyer-Bahlburg, H. F. L., & Blizzard, R. M. (2004). Research on intersex: Summary of a planning workshop. Endocrinologist, 14, 59–69.

    Article  Google Scholar 

  • Migeon, C. J., Wisniewski, A. B., Brown, T. R., Rock, J. A., Meyer-Bahlburg, H. F. L., Money, J., et al. (2002a). 46,XY intersex individuals: Phenotypic and etiological classification, knowledge of condition, and satisfaction with knowledge in adulthood. Pediatrics, 110(3), e32.

    Article  Google Scholar 

  • Migeon, C. J., Wisniewski, A. B., Gearhart, J. P., Meyer-Bahlburg, H. F. L., Rock, J. A., Brown, T. R., et al. (2002b). Ambiguous genitalia with perineoscrotal hypospadias in 46,XY individuals: Long-term medical, surgical and psychosexual outcomes. Pediatrics, 110(3), e31.

    Article  Google Scholar 

  • Minto, C. L., Liao, L., Woodhouse, C. R. J., Ransley, P. G., & Creighton, S. M. (2003). The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: A cross sectional study. Lancet, 361, 1252–1257.

    Article  PubMed  Google Scholar 

  • Money, J. (1984a). Part I: Childhood coping and adult follow-up of micropenis syndrome in a case with female sex assignment. International Journal of Family Psychiatry, 50, 317–339.

    Google Scholar 

  • Money, J. (1984b). Part II: Transsexual versus homosexual coping in micropenis syndrome with male sex assignment. International Journal of Family Psychiatry, 50, 341–373.

    Google Scholar 

  • Money, J., & Ehrhardt, A. A. (1972). Man and woman, boy and girl: The differentiation and dimorphism of gender identity from conception to maturity. Baltimore, MD: Johns Hopkins Press.

    Google Scholar 

  • Money, J., Ehrhardt, A. A., & Masica, D. (1968). Fetal feminization induced by androgen insensitivity in the testicular feminizing syndrome: Effect on marriage and maternalism. Johns Hopkins Medical Journal, 123, 105–114.

    PubMed  Google Scholar 

  • Money, J., Lehne, G. K., & Pierre-Jerome, F. (1985). Micropenis: Gender, erotosexual coping strategy and behavioral health in nine pediatric cases followed to adulthood. Comprehensive Psychiatry, 26, 29–42.

    Article  PubMed  Google Scholar 

  • Money, J., & Mazur, T. (1977). Microphallus: The successful use of a prosthetic phallus in a 9 year old boy. Journal of Sex & Marital Therapy, 3, 187–196.

    Google Scholar 

  • Money, J., Mazur, T., Abrams, C., & Norman, B. F. (1981). Micropenis, family mental health, and neonatal management: A report of 14 patients reared as girls. Journal of Preventive Psychiatry, 1, 17–27.

    Google Scholar 

  • Money, J., & Norman, B. F. (1988). Pedagogical handicap associated with micropenis and other CHARGE syndrome anomalies of embryogenesis: Four 46,XY cases reared as girls. American Journal of Psychotherapy, 42, 354–379.

    PubMed  Google Scholar 

  • Money, J., & Ogunro, C. (1974). Behavioral sexology: Ten cases of genetic male intersexuality with impaired prenatal and pubertal androgenization. Archives of Sexual Behavior, 3, 181–205.

    Article  PubMed  Google Scholar 

  • Morris, J. M., & Mahesh, V. B. (1963). Further observations on the syndrome, “testicular feminization”. American Journal of Obstetrics & Gynecology, 87, 731–748.

    Google Scholar 

  • Random House College Dictionary. (1988). New York: Random House.

  • Reilly, J. M., & Woodhouse, C. R. J. (1989). Small penis and the male sexual role. Journal of Urology, 142, 569–572.

    PubMed  Google Scholar 

  • Slijper, F. M. E., Drop, S. L. S., Molenaar, J. G., & de Muinck Keizer-Schrama, S. M. P. E. (1998). Long-term psychological evaluation of intersex children. Archives of Sexual Behavior, 27, 125–144.

    Article  PubMed  Google Scholar 

  • Slijper, F. M. E., Frets, P. G., Boehmer, A. L. M., Drop, S. L. S., & Niermeijer, M. F. (2000). Androgen insensitivity syndrome (AIS): Emotional reactions of parents and adult patients to the clinical diagnosis of AIS and its confirmation by androgen receptor gene mutation analysis. Hormone Research, 53, 9–15.

    Article  Google Scholar 

  • Slob, A. K., van der Werff ten Bosch, J. J., van Hall, E. V., de Hong, F. H., Weijmar Schultz, W. C. M., & Eikelbloom, F. A. (1993). Psychosexual functioning in women with testicular feminization: Is androgen replacement therapy preferable to estrogen? Journal of Sex and Marital Therapy, 19, 201–208.

    PubMed  Google Scholar 

  • Sobel, V., & Imperato-McGinley, J. (2004). Gender identity in XY intersexuality. Child and Adolescent Psychiatric Clinics of North America, 13, 609–622.

    Article  PubMed  Google Scholar 

  • Teter, J., & Boczkowski, K. (1966). Testicular feminization with and without clitoral enlargement. American Journal of Obstetrics & Gynecology, 94, 813–819.

    Google Scholar 

  • Wisniewski, A. B., Migeon, C. J., Gearhart, J. D., Rock, J. A., Berkovitz, G. D., Plotnick, L. P., et al. (2001). Congenital micropenis: Long term medical, surgical and psychosexual follow-up of individuals raised male or female. Hormone Research, 56, 3–11.

    Article  PubMed  Google Scholar 

  • Wisniewski, A. B., Migeon, C. J., Meyer-Bahlburg, H. F. L., Gearhart, J. P., Berkovitz, G. D., Brown, T. R., et al. (2000). Complete androgen insensitivity syndrome: Long term medical, surgical and psychosexual outcome. Journal of Clinical Endocrinology and Metabolism, 85, 2664–2669.

    Article  PubMed  Google Scholar 

  • Zucker, K. J. (1999). Intersexuality and gender identity differentiation. Annual Review of Sex Research, 10, 1–69.

    PubMed  Google Scholar 

  • Zucker, K. J. (2005). Measurement of psychosexual differentiation. Archives of Sexual Behavior, 34, 375–388.

    Google Scholar 

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Correspondence to Tom Mazur Psy.D..

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Mazur, T. Gender Dysphoria and Gender Change in Androgen Insensitivity or Micropenis. Arch Sex Behav 34, 411–421 (2005). https://doi.org/10.1007/s10508-005-4341-x

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