Maggie Dwiggins1,2, Brittany Brookner3, Kylie Fowler1,4, Padmasree Veeraraghavan3, Veronica Gomez-Lobo1,5, Deborah P Merke3,5. 1. Medstar Washington Hospital Center and Children's National Medical Center, Washington, District of Columbia. 2. Norton Children's Medical Group, Louisville, Kentucky. 3. National Institutes of Health Clinical Center, Bethesda, Maryland. 4. Children's Minnesota, Minneapolis, Minnesota. 5. Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland.
Abstract
CONTEXT: 46,XX patients with classic congenital adrenal hyperplasia (CAH) are exposed to elevated androgens in utero causing varying levels of virilization. The majority undergo feminizing genitoplasty early in life, with potential impact on sexual function and health-related quality of life (HRQoL). OBJECTIVE: We aimed to determine how sexual and lower urinary tract function, body image, and global HRQoL differs between patients with classic CAH and controls and to characterize how gynecologic anatomy contributes to outcomes. METHODS: 36 patients with classic CAH and 27 control women who were matched for age, race, and marital status underwent standardized gynecological examination and validated questionnaires. The responses were analyzed in relation to gynecological measurements, genotype, and disease status. RESULTS: Compared with controls, patients with CAH were more likely to have sexual dysfunction (P = 0.009), dyspareunia (P = 0.007), and other pelvic pain (P = 0.007); were less likely to be heterosexual (P = 0.013) or ever have been sexually active (P = 0.003); had poorer body image independent of body mass index (P < 0.001); and had worse HRQoL in the areas of general health (P = 0.03) and pain (P = 0.009). The patients with CAH had smaller vaginal calibers and perineal body lengths and larger clitoral indexes when compared with controls (P < 0.001). A larger vaginal caliber in CAH patients was associated with better overall sexual function (P = 0.024), increased sexual satisfaction (P = 0.017), less pain (P < 0.001), and greater number of sexual partners (P = 0.02). CONCLUSIONS: 46,XX patients with CAH have increased rates of sexual dysfunction, poor body image, and poor HRQoL, which is mitigated by having a larger vaginal caliber. Management aimed at optimizing vaginal caliber might improve sexual function. Published by Oxford University Press on behalf of the Endocrine Society 2020.
CONTEXT: 46,XX patients with classic congenital adrenal hyperplasia (CAH) are exposed to elevated androgens in utero causing varying levels of virilization. The majority undergo feminizing genitoplasty early in life, with potential impact on sexual function and health-related quality of life (HRQoL). OBJECTIVE: We aimed to determine how sexual and lower urinary tract function, body image, and global HRQoL differs between patients with classic CAH and controls and to characterize how gynecologic anatomy contributes to outcomes. METHODS: 36 patients with classic CAH and 27 control women who were matched for age, race, and marital status underwent standardized gynecological examination and validated questionnaires. The responses were analyzed in relation to gynecological measurements, genotype, and disease status. RESULTS: Compared with controls, patients with CAH were more likely to have sexual dysfunction (P = 0.009), dyspareunia (P = 0.007), and other pelvic pain (P = 0.007); were less likely to be heterosexual (P = 0.013) or ever have been sexually active (P = 0.003); had poorer body image independent of body mass index (P < 0.001); and had worse HRQoL in the areas of general health (P = 0.03) and pain (P = 0.009). The patients with CAH had smaller vaginal calibers and perineal body lengths and larger clitoral indexes when compared with controls (P < 0.001). A larger vaginal caliber in CAH patients was associated with better overall sexual function (P = 0.024), increased sexual satisfaction (P = 0.017), less pain (P < 0.001), and greater number of sexual partners (P = 0.02). CONCLUSIONS: 46,XX patients with CAH have increased rates of sexual dysfunction, poor body image, and poor HRQoL, which is mitigated by having a larger vaginal caliber. Management aimed at optimizing vaginal caliber might improve sexual function. Published by Oxford University Press on behalf of the Endocrine Society 2020.
Entities:
Keywords:
body image; congenital adrenal hyperplasia; genitourinary surgery; health-related quality of life; sexual function
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