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)To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of Pediatric UrologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Baseline characteristics of infants with atypical genital development: phenotypes, diagnoses, and sex of rearing.J Endocr Soc. 2019; 3: 264-272https://doi.org/10.1210/js.2018-00316
- Participation of adults with disorders/differences of sex development (DSD) in the clinical study dsd-LIFE: design, methodology, recruitment, data quality and study population.BMC Endocr Disord. 2017; 17: 52https://doi.org/10.1186/s12902-017-0198-y
- Syndrome-related stigma in the general social environment as reported by women with classical congenital adrenal hyperplasia.Arch Sex Behav. 2017; 46: 341-351https://doi.org/10.1007/s10508-016-0862-8
- Stigma associated with classical congenital adrenal hyperplasia in women's sexual lives.Arch Sex Behav. 2018; 47: 943-951https://doi.org/10.1007/s10508-017-1003-8
- Influence of genotype and hyperandrogenism on sexual function in women with congenital adrenal hyperplasia.J Sex Med. 2019; 16: 1529-1540https://doi.org/10.1016/j.jsxm.2019.07.009
- Type of mutation and surgical procedure affect long-term quality of life for women with congenital adrenal hyperplasia.J Clin Endocrinol Metab. 2008; 93: 380-386https://doi.org/10.1210/jc.2007-0556
- Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients.J Clin Endocrinol Metab. 2010; 95: 5110-5121https://doi.org/10.1210/jc.2010-0917
- Poor compliance and increased mortality, depression and healthcare costs in patients with congenital adrenal hyperplasia.Eur J Endocrinol. 2018; 178: 309-320https://doi.org/10.1530/EJE-17-0895
SB 201 California: Medical procedures: treatment or intervention: sex characteristics of a minor. 2019-2020 ed2020.
- An act relating to health care; establishing conditions for the performance on a child of any surgical procedure to assign anatomical sex; providing for disciplinary action against certain providers of health care for any violation of those conditions; and providing other matters properly relating thereto.2017 (ed2017)
- An act concerning a person's intersex status or characteristics.2019
- Promoting the human rights of and eliminating discrimination against intersex people.2017
- Human.Rights.Office. United nations for intersex awareness: intersex babies are perfect just as they are.2020
Government.of.Malta. Gender identity, Gender Expression and Sex Characteristics Act.
- Portugal approves law to boost transgender rights, protect intersex infants.2018
- What do patients and parents say about parents' ability to choose early genital surgery for girls with congenital adrenal hyperplasia?.J Urol. 2021; 205: 1770-1777https://doi.org/10.1097/JU.0000000000001573
- Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex.J Pediatr Urol. 2021; 17 (e1- e9 https://doi.org/10.1016/j.jpurol.2020.09.009): 210
- Patient and caregiver attitudes toward disorders of sex development nomenclature.J Urol. 2020; 204 (101097JU0000000000001076): 835-842https://doi.org/10.1097/JU.0000000000001076
- United States census bureau.https://www.census.gov/en.htmlDate: 2020
- Partnership and sexuality in adult female patients with congenital adrenal hyperplasia. First results of a cross-sectional quality-of-life evaluation.J Steroid Biochem Mol Biol. 1993; 45: 123-126https://doi.org/10.1016/0960-0760(93)90131-f
Article info
Publication history
Published online: May 30, 2022
Accepted:
May 19,
2022
Received in revised form:
March 25,
2022
Received:
December 3,
2021
Identification
Copyright
© 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
ScienceDirect
Access this article on ScienceDirectLinked Article
- Commentary to Growing up with clitoromegaly: Experiences of North American women with congenital adrenal hyperplasiaJournal of Pediatric UrologyVol. 18Issue 6
- PreviewIn 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.
- Full-Text
- Preview
