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Table 2 Review of cosmetic and functional outcome studies of feminizing genitoplasty in girls and women with congenital adrenal hyperplasia due to 2-hydroxylase deficiency conducted from 2000–present.

From: An Evidence-Based Model of Multidisciplinary Care for Patients and Families Affected by Classical Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Dependent Measure Sample size; age range () at participation Earliest surgery dates; age range () of earliest surgery Summary Reference
Cosmetic appearance     
  27; 14.0–33.0 1970s–1980s; 2.4–9.9
(i) combination of clitoral excision and reduction as well as fortunoff flap or pull through vaginoplasty
(i) participants () who experienced vaginal stenosis secondary to their vaginoplasty were less satisfied than the authors with the appearance of their genitalia [61]
  21; 7.0–19.0 1970s–1980s; 0.8–10.4
(i) majority received clitoral reduction and fortunoff vaginoplasty
(i) 24% had a "good" outcome as determined by the authors
(ii) most required more than a single-stage genitoplasty
[68]
  23; 21.0–71.0 1950s–1990s; 0.3–20.0
(i) majority received clitoral excision and fortunoff vaginoplasty
(i) author ratings of the appearance of the genitals were better than self-ratings [62]
  15; 12.0–25.0 1980s–1990s; 2.0–2.0
(i) combination of clitoral excision, reduction and recession as well as V-Y vaginoplasty and vaginal pull-through
(i) Equally good cosmetic outcome for participants who had surgery prior to 2 years of age versus after 2
(ii) Better cosmetic outcome for planned one-stage surgery than planned multistage
(iii) Better cosmetic outcome when the procedures were performed at an institution and by a surgeon with expertise in DSD
[69]
  24; 2.0–17.0 1990s–2000s; 0.1–16.0
(i) all received partial urogenital sinus mobilization and clitoral resection with preservation of neuron-vascular bundle and glanular reloaction, half received glanular reduction
(i) 87.5% had a "good" outcome as determined by the authors [67]
  35; 18.0–43.0 1950s–1990s; 0.5–18.0
(i) combination of clitoral excision and resection as well as fortunoff or pull-through vaginoplasty
(i) majority had a "good" outcome as determined by the authors and self-report
(ii) mean genital surgeries 2
[63]
  49; 18.0–63.0 1950s–2000s; 0.5–20.0
(i) combination of clitoral excision, recession and resection as well as simple cleavage and fortunoff vaginoplasty
(i) majority of women receiving clitoral surgery rated their clitoral size as "normal" versus majority of women not receiving surgery rates their clitoris as "too big"
(ii) unsatisfactory appearance attributed to size/location of clitoris and scar tissue in women who had received genitoplasty
(iii) mean genital surgeries 2
[64]
Sexual function     
  27; 14.0–33.0 1970s–1980s; 2.4–9.9
(i) combination of clitoral excision and reduction as well as fortunoff flap or pull through vaginoplasty
(i) women born with greater masculinization of their genitalia (Prader III or greater) are more likely to develop intravaginal stenosis if they received a single-stage vaginoplasty prior to puberty
(ii) participants () who experienced vaginal stenosis secondary to their vaginoplasty were less satisfied with their degree of vaginal opening than examiners
[61]
  23; 21.0–71.0 1950s–1990s; 0.3–20.0
(i) majority received clitoral excision and fortunoff vaginoplasty
(i) women born with greater genital masculinization (salt-losing or SL) reported poorer sexual function than those with less masculinization (simple-virilizing or SV) following genitoplasty
(ii) SL women had a shorter vagina than SV women following genitoplasty
[62]
  41; 16.0–46.0 1960s–2000s; infancy–puberty
(i) combination of clitoral excision and resection as well as fortunoff or pull-through vaginoplasty
(i) 1 in 6 women born with less masculinized genitalia who did not receive genitoplasty were unable to have sexual intercourse versus 3 in 29 women born with more masculinized genitalia who did receive genitoplasty
(ii) women born with more masculinized genitalia were more likely to a 2-stage genitoplasty
[65]
  27; 0.1–19.0 1990s–2000s; 0.1–19.0
(i) all received nerve sparing ventral clitoral reduction
(i) no large dorsal nerves visualized in excised erectile tissue following clitoral reduction [70]
  35; 18.0–43.0 1950s–1990s; 0.5–18.0
(i) combination of clitoral excision and resection as well as perineal or pull-through vaginoplasty
(i) women born with greater genital masculinization (Prader IV-V) prior to genitoplasty have fewer sex partners and fewer episodes of vaginal penetration than less masculinized women (Prader I–III) or controls
(ii) Prader IV-V women experience more difficulties with pain, lubrication, and orgasm than Prader I–III women or controls
[63]
  63; 2.0–19.3 1985–2000; 0.2–19.6
(i) combination of fortunoff flap, pull-through and vaginal replacement
(i) 27% developed vaginal strictures, 5% developed major complications including fistulas and diversion colitis
(ii) the complication rate for fortunoff flap vaginoplasties performed in prepubertal patients was higher than that of flap procedures performed postpubertally
[71]
  28; 17.0–39.0 1970s–2000s; 4.0–16.0
(i) combination of clitoral excision and reduction; type of vaginoplasty not reported
(i) CAH women who had received genitoplasty had less frequent intercourse and greater penetration difficulty and anorgasmia than CAH women who had not received genitoplasty ()
(ii) CAH women who had received genitoplasty were less sensitive to temperature and vibration following clitoral stimulation than controls
(iii) Other measures of sexual function and avoidance did not differ between women with CAH who had received genitoplasty versus those that did not and controls
[66]
  49; 18.0–63.0 1950s–2000s; 0.5–20.0
(i) combination of clitoral excision, recession, and resection as well as simple cleavage or dorsal flap vaginoplasty
(i) 20.4% women were dissatisfied with their genitoplasty
(ii) those who had received clitoral excision or multiple clitoral surgeries reported the least clitoral sensitivity
(iii) 45.6% women reported the vaginal introitus as "too small" or "tight" whether they received vaginoplasty or not
[64]