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Growing up with clitoromegaly: experiences of North American women with congenital adrenal hyperplasia

  • Author Footnotes
    1 Members of the “Steering Committee of the Life with Congenitial Adrenal Hyperplasia Study Group”.
    Konrad M. Szymanski
    Correspondence
    Correspondence to: K.M. Szymanski, Division of Pediatric Urology, Riley Hospital for Children at IU Health, 705 Riley Hospital Dr., Suite 4230, Indianapolis, IN 46202, USA, Tel.: +1 317 944 7446; fax.: +1 317 944 7481
    Footnotes
    1 Members of the “Steering Committee of the Life with Congenitial Adrenal Hyperplasia Study Group”.
    Affiliations
    Division of Pediatric Urology, Riley Hospital for Children at IU Health, Indianapolis, IN, USA
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  • Author Footnotes
    1 Members of the “Steering Committee of the Life with Congenitial Adrenal Hyperplasia Study Group”.
    Paul Kokorowski
    Footnotes
    1 Members of the “Steering Committee of the Life with Congenitial Adrenal Hyperplasia Study Group”.
    Affiliations
    Division of Pediatric Urology, Children’s Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
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  • Author Footnotes
    2 Members of the “Members of the Life with Congenital Adrenal Hyperplasia Study Group”.
    Luis H. Braga
    Footnotes
    2 Members of the “Members of the Life with Congenital Adrenal Hyperplasia Study Group”.
    Affiliations
    Division of Pediatric Urology, McMaster Children’s Hospital, McMaster University, Hamilton, ON, Canada
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  • Author Footnotes
    2 Members of the “Members of the Life with Congenital Adrenal Hyperplasia Study Group”.
    Heather Frady
    Footnotes
    2 Members of the “Members of the Life with Congenital Adrenal Hyperplasia Study Group”.
    Affiliations
    Division of Pediatric Urology, Riley Hospital for Children at IU Health, Indianapolis, IN, USA
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  • Author Footnotes
    2 Members of the “Members of the Life with Congenital Adrenal Hyperplasia Study Group”.
    Benjamin Whittam
    Footnotes
    2 Members of the “Members of the Life with Congenital Adrenal Hyperplasia Study Group”.
    Affiliations
    Division of Pediatric Urology, Riley Hospital for Children at IU Health, Indianapolis, IN, USA
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  • Author Footnotes
    2 Members of the “Members of the Life with Congenital Adrenal Hyperplasia Study Group”.
    Devon J. Hensel
    Footnotes
    2 Members of the “Members of the Life with Congenital Adrenal Hyperplasia Study Group”.
    Affiliations
    Division of Adolescent Medicine, Department of Pediatrics, Indiana University School of Medicine and Department of Sociology, Indiana University Purdue University Indianapolis, Indianapolis, IN, USA
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  • on behalf ofLife with Congenital Adrenal Hyperplasia Study Group
  • Author Footnotes
    1 Members of the “Steering Committee of the Life with Congenitial Adrenal Hyperplasia Study Group”.
    2 Members of the “Members of the Life with Congenital Adrenal Hyperplasia Study Group”.

      Summary

      Introduction

      To describe experiences of clitoromegaly in women with congenital adrenal hyperplasia (CAH).

      Methods

      CAH females (46XX, ≥16 years old) from the United States and Canada were eligible for a cross-sectional online survey (2019–2020) if reporting clitoromegaly (life-long: “growing up with a larger than average clitoris,” secondary: “clitoris grew over weeks or months”). A multidisciplinary team and women with CAH drafted questions assessing net effects of clitoromegaly on 10 activities and 10 life domains. Fisher's exact test was used to compare net effect (positive-negative) vs. no effect (Bonferroni p = 0.05/10 = 0.005).

      Results

      Of 97 women with CAH enrolled, 53 women (55%, median age: 36 years, advocacy group recruitment: 81%) reported recognizing clitoromegaly at median 11–13 years old, with 21% identifying it in adulthood. There was no difference in self-reported timing or clitoral shape between life-long or secondary clitoromegaly (p ≥ 0.06). There were no net positive effects of clitoromegaly. Rather, clitoromegaly had net negative effects on 7/10 activities (p ≤ 0.003) and no net effect (neutral) on 3 (Table). Women were less likely to wear tight clothing, change clothes in public locker rooms and play group sports. Women reported net negative effects for most romantic activities (dating, any sexual activity, pain-free sexual activity, having a partner see their genitalia, p=<0.003), but did not report a net effect on pleasurable sexual activity (p = 0.12).
      Clitoromegaly had net negative effects in 9/10 life domains (p < 0.001) and neutral on job self-perception (p = 0.25). Few women reported any positive impact (2–6%). However, 49–59% of women experienced poor self-esteem, anxiety, gender self-perception and body image, while 36% felt “down or depressed.” Also, 21–23% experienced negative self-perception as friends and parents, 42–47% reported negative effects on plans for romantic and sexual relationships. Responses did not differ with advocacy group membership (p ≥ 0.02).

      Discussion

      Our findings support qualitative and case series evidence that clitoromegaly has a negative psychological outcome on women with CAH. Clitoromegaly may add to the burden of living with a chronic endocrine disease. Women with positive and negative experiences had the same opportunity to participate. Since we could not assess objective clitoral size, baseline virilization and exact nature of any childhood clitoral procedures, these data cannot be used to estimate the impact of specific clitoral size or effectiveness of early clitoral treatments.

      Conclusions

      Clitoromegaly appears to be common among women with CAH. While experiences of clitoromegaly vary between women, the overall experience is negative in multiple social, romantic, and emotional activities and domains.

      Keywords

      Abbreviations:

      CAH (congenital adrenal hyperplasia), DSD (differences of sex development), IQR (interquartile range)
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      Linked Article

      • Commentary to Growing up with clitoromegaly: Experiences of North American women with congenital adrenal hyperplasia
        Journal of Pediatric UrologyVol. 18Issue 6
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          In the conversation surrounding medical necessity in genital surgery, the psychologic impact of variations in genitalia is heavily debated [1]. Many advocates for delaying surgery purport that psychologic impact is a result of outdated societal norms that should no longer exist, holding firm that bodily autonomy should supersede the potential negative psychological impact of variations in genitalia. Answering the question of whether there is or is not a detrimental impact (or potential for one) to one’s personal psyche by delaying surgical reconstruction is complicated at best, as individual experiences, cultural backgrounds, and underlying diagnoses are as unique as the genitalia itself.
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