title: Severity of virilization is associated with cosmetic appearance and sexual function in women with congenital adrenal hyperplasia: a cross-sectional study.
abstract: BACKGROUND
  Women with the classical form of congenital adrenal hyperplasia (CAH) are born with different degrees of virilization of the external genitalia. Feminizing surgery is often performed in childhood to change the appearance of the genitalia and to enable penile-vaginal intercourse later in life. There are suggestions that this affects sexual functioning.
  
  
  OBJECTIVE
  The aim is to study the anatomical, surgical, cosmetic, and psychosexual outcomes in women with CAH.
  
  
  METHODS
  Forty women with CAH, aged over 15 years, from two referral centers for management of Disorders of Sex Development in the Netherlands were included. Physical and functional status were assessed by a gynecological interview and examination. Sexual functioning was assessed with the Female Sexual Function Index and Female Sexual Distress Scale-Revised scales and compared with a reference group.
  
  
  METHODS
  Surgery performed, anatomy, cosmetic score, sexual function and distress.
  
  
  RESULTS
  Thirty-six of the 40 women had undergone feminizing surgery; 25 women (69%) underwent more than one operation. Resurgery was performed in seven of the 13 (54%) women who had had a single-stage procedure. Anatomical assessment showed reasonable outcomes. Multiple linear regression showed that only level of confluence had a significant effect on cosmetic outcome, the impact depending on the number of surgeries performed. Cosmetic evaluations did not differ between the women and the gynecologists. Only 20 women had experience of intercourse. Eight women reported dyspareunia; seven women reported urinary incontinence. The women's perceived sexual functioning was less satisfactory than in the reference group, and they reported more sexual distress.
  
  
  CONCLUSIONS
  The level of confluence was the major determinant for cosmetic outcome; the impact depended on the number of surgeries performed. Fifty-four percent of the women required resurgery after a single-stage procedure in childhood. Anatomical assessment showed reasonable outcomes. The women evaluated their sexual functioning and functional outcome less favorable than the reference group, and they experienced less often sexual intercourse.
authors.affiliation: Department of Pediatrics, Division of Pediatric Endocrinology, Erasmus MC-Sophia, Rotterdam, The Netherlands. y.vanderzwan@erasmusmc.nl
authors.forename: Yvonne G
authors.initials: YG
authors.lastname: van der Zwan
authors.forename: Eefje H C C
authors.initials: EH
authors.lastname: Janssen
authors.forename: Nina
authors.initials: N
authors.lastname: Callens
authors.forename: Katja P
authors.initials: KP
authors.lastname: Wolffenbuttel
authors.forename: Peggy T
authors.initials: PT
authors.lastname: Cohen-Kettenis
authors.forename: Marjan
authors.initials: M
authors.lastname: van den Berg
authors.forename: Stenvert L S
authors.initials: SL
authors.lastname: Drop
authors.forename: Arianne B
authors.initials: AB
authors.lastname: Dessens
authors.forename: Catharina
authors.initials: C
authors.lastname: Beerendonk
mesh_terms: D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000523:psychology / Q000601:surgery*; D000328:Adult; D003075:Coitus; D003430:Cross-Sectional Studies; D004414:Dyspareunia / Q000209:etiology; D004954:Esthetics; D005260:Female; D005836:Genitalia, Female / Q000601:surgery*; D013509:Gynecologic Surgical Procedures; D006801:Humans; D016014:Linear Models; D008875:Middle Aged; D012086:Reoperation / Q000706:statistics & numerical data; D012720:Severity of Illness Index; D013315:Stress, Psychological / Q000209:etiology; D014549:Urinary Incontinence / Q000209:etiology; D055815:Young Adult