title: Prospective assessment of cosmesis before and after genital surgery.
abstract: BACKGROUND
  Little data exist about the surgical interventions taking place for children with disorders of sex development (DSD). Most studies that have evaluated cosmetic outcomes after genitoplasty have included retrospective ratings by a physician at a single center.
  
  
  OBJECTIVE
  The present study aimed to: 1) describe frequency of sex assignment, and types of surgery performed in a cohort of patients with moderate-to-severe genital ambiguity; and 2) prospectively determine cosmesis ratings by parents and surgeons before and after genital surgery.
  
  
  METHODS
  This prospective, observational study included children aged <2 years of age, with no prior genitoplasty at the time of enrollment, moderate-to-severe genital atypia, and being treated at one of 11 children's hospitals in the United States of America (USA). Clinical information was collected, including type of surgery performed. Parents and the local pediatric urologist rated the cosmetic appearance of the child's genitalia prior to and 6 months after genitoplasty.
  
  
  RESULTS
  Of the 37 children meeting eligibility criteria, 20 (54%) had a 46,XX karyotype, 15 (40%) had a 46,XY karyotype, and two (5%) had sex chromosome mosaicism. The most common diagnosis overall was congenital adrenal hyperplasia (54%). Thirty-five children had surgery; 21 received feminizing genitoplasty, and 14 had masculinizing genitoplasty. Two families decided against surgery. At baseline, 22 mothers (63%), 14 fathers (48%), and 35 surgeons (100%) stated that they were dissatisfied or very dissatisfied with the appearance of the child's genitalia. Surgeons rated the appearance of the genitalia significantly worse than mothers (P < 0.001) and fathers (P ≤ 0.001) at baseline. At the 6-month postoperative visit, cosmesis ratings improved significantly for all groups (P < 0.001 for all groups). Thirty-two mothers (94%), 26 fathers (92%), and 31 surgeons (88%) reported either a good outcome, or they were satisfied (see Summary Figure); there were no significant between-group differences in ratings.
  
  
  CONCLUSIONS
  This multicenter, observational study showed surgical interventions being performed at DSD centers in the USA. While parent and surgeon ratings were discordant pre-operatively, they were generally concordant postoperatively. Satisfaction with postoperative cosmesis does not necessarily equate with satisfaction with the functional outcome later in life.
  
  
  CONCLUSIONS
  In this cohort of children with genital atypia, the majority had surgery. Parents and surgeons all rated the appearance of the genitalia unfavorably before surgery, with surgeons giving worse ratings than parents. Cosmesis ratings improved significantly after surgery, with no between-group differences.
authors.affiliation: Department of Pediatrics, Section of Pediatric Endocrinology, University of Colorado Denver School of Medicine, 13123 East 16th Ave Box B265, Aurora 80045, CO, USA. Electronic address: Natalie.Nokoff@childrenscolorado.org.
authors.forename: N J
authors.initials: NJ
authors.lastname: Nokoff
authors.affiliation: Department of Urology, The University of Oklahoma College of Medicine, 920 Stanton L Young Blvd, WP 3150, Oklahoma City 72104, OK, USA.
authors.forename: B
authors.initials: B
authors.lastname: Palmer
authors.affiliation: Department of Psychology, Oklahoma State University, 116 North Murray, Stillwater 74078, OK, USA.
authors.forename: A J
authors.initials: AJ
authors.lastname: Mullins
authors.affiliation: Department of Pediatrics, The University of Oklahoma College of Medicine, 920 Stanton L Young Blvd, WP 3150, Oklahoma City 72104, OK, USA.
authors.forename: C E
authors.initials: CE
authors.lastname: Aston
authors.affiliation: Department of Surgery, Division of Urology, Washington University School of Medicine, 660 South Euclid Ave, Campus Box 8242, St. Louis 63110, MO, USA.
authors.forename: P
authors.initials: P
authors.lastname: Austin
authors.affiliation: Department of Urology, University of California San Francisco, 400 Parnassus Ave, San Francisco 94143, CA, USA.
authors.forename: L
authors.initials: L
authors.lastname: Baskin
authors.affiliation: Department of Urology, Weill Cornell Medicine, 525 East 68th St., Box 94, New York 10065, NY, USA.
authors.forename: K
authors.initials: K
authors.lastname: Bernabé
authors.affiliation: Department of Pediatrics, Division of Endocrinology, Harvard Medical School, 300 Longwood Ave, Boston 02115, MA, USA.
authors.forename: Y-M
authors.initials: YM
authors.lastname: Chan
authors.affiliation: Department of Urology, Northwestern University, Feinberg School of Medicine, 225 E Chicago Ave, Box 24, Chicago 60611, IL, USA.
authors.forename: E Y
authors.initials: EY
authors.lastname: Cheng
authors.affiliation: Department of Urology, Harvard Medical School, 300 Longwood Ave, Boston 02115, MA, USA.
authors.forename: D A
authors.initials: DA
authors.lastname: Diamond
authors.affiliation: Department of Pediatric Urology, Women and Children's Hospital of Buffalo, Buffalo 14222, NY, USA.
authors.forename: A
authors.initials: A
authors.lastname: Fried
authors.affiliation: Department of Urology, The University of Oklahoma College of Medicine, 920 Stanton L Young Blvd, WP 3150, Oklahoma City 72104, OK, USA.
authors.forename: D
authors.initials: D
authors.lastname: Frimberger
authors.affiliation: Department of Urology, Weill Cornell Medicine, 525 East 68th St., Box 94, New York 10065, NY, USA.
authors.forename: D
authors.initials: D
authors.lastname: Galan
authors.affiliation: Pediatric Nephrology and Urology, University of California San Francisco, 400 Parnassus Ave, San Francisco 94143, CA, USA.
authors.forename: L
authors.initials: L
authors.lastname: Gonzalez
authors.affiliation: Department of Pediatric Urology, Women and Children's Hospital of Buffalo, Buffalo 14222, NY, USA.
authors.forename: S
authors.initials: S
authors.lastname: Greenfield
authors.affiliation: Department of Urology, Perelman School of Medicine, University of Pennsylvania, 34th Street & Civic Center Blvd., Philadelphia 19104, PA, USA.
authors.forename: T
authors.initials: T
authors.lastname: Kolon
authors.affiliation: Department of Urology, The University of Oklahoma College of Medicine, 920 Stanton L Young Blvd, WP 3150, Oklahoma City 72104, OK, USA.
authors.forename: B
authors.initials: B
authors.lastname: Kropp
authors.affiliation: Department of Urology, Wayne State University School of Medicine, 3901 Beaubien, Detroit 48201, MI, USA.
authors.forename: Y
authors.initials: Y
authors.lastname: Lakshmanan
authors.affiliation: Department of Pediatric Urology, Women and Children's Hospital of Buffalo, Buffalo 14222, NY, USA.
authors.forename: S
authors.initials: S
authors.lastname: Meyer
authors.affiliation: Department of Urology, Northwestern University, Feinberg School of Medicine, 225 E Chicago Ave, Box 24, Chicago 60611, IL, USA.
authors.forename: T
authors.initials: T
authors.lastname: Meyer
authors.affiliation: Department of Psychology, Oklahoma State University, 116 North Murray, Stillwater 74078, OK, USA.
authors.forename: L L
authors.initials: LL
authors.lastname: Mullins
authors.affiliation: Department of Surgery, Division of Urology, Washington University School of Medicine, 660 South Euclid Ave, Campus Box 8242, St. Louis 63110, MO, USA.
authors.forename: A
authors.initials: A
authors.lastname: Paradis
authors.affiliation: Department of Urology, Weill Cornell Medicine, 525 East 68th St., Box 94, New York 10065, NY, USA.
authors.forename: D
authors.initials: D
authors.lastname: Poppas
authors.affiliation: Department of Pediatrics, Division of Pediatric Urology, University of Cincinnati College of Medicine, Cincinnati Children's Hospital Medical Center, MLC 5037, 3333 Burnet Ave, Cincinnati, OH, USA.
authors.forename: P
authors.initials: P
authors.lastname: Reddy
authors.affiliation: Department of Pediatrics, Division of Pediatric Urology, University of Cincinnati College of Medicine, Cincinnati Children's Hospital Medical Center, MLC 5037, 3333 Burnet Ave, Cincinnati, OH, USA.
authors.forename: M
authors.initials: M
authors.lastname: Schulte
authors.affiliation: Department of Urology, The University of Oklahoma College of Medicine, 920 Stanton L Young Blvd, WP 3150, Oklahoma City 72104, OK, USA.
authors.forename: K J Scott
authors.initials: KJ
authors.lastname: Reyes
authors.affiliation: Department of Pediatrics, Division of Endocrinology, Harvard Medical School, 300 Longwood Ave, Boston 02115, MA, USA.
authors.forename: J M
authors.initials: JM
authors.lastname: Swartz
authors.affiliation: Department of Pediatrics, The University of Oklahoma College of Medicine, 920 Stanton L Young Blvd, WP 3150, Oklahoma City 72104, OK, USA; Department of Urology, Wayne State University School of Medicine, 3901 Beaubien, Detroit 48201, MI, USA.
authors.forename: C
authors.initials: C
authors.lastname: Wolfe-Christensen
authors.affiliation: Department of Urology, Northwestern University, Feinberg School of Medicine, 225 E Chicago Ave, Box 24, Chicago 60611, IL, USA.
authors.forename: E
authors.initials: E
authors.lastname: Yerkes
authors.affiliation: Department of Urology, The University of Oklahoma College of Medicine, 920 Stanton L Young Blvd, WP 3150, Oklahoma City 72104, OK, USA; Department of Pediatrics, The University of Oklahoma College of Medicine, 920 Stanton L Young Blvd, WP 3150, Oklahoma City 72104, OK, USA.
authors.forename: A B
authors.initials: AB
authors.lastname: Wisniewski
mesh_terms: D002675:Child, Preschool; D005260:Female; D005831:Genital Diseases, Female / Q000601:surgery*; D005832:Genital Diseases, Male / Q000601:surgery*; D005835:Genitalia / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011446:Prospective Studies; D019651:Plastic Surgery Procedures / Q000379:methods*; D013519:Urogenital Surgical Procedures
keywords: Cosmesis; Disorder of sex development; Genitoplasty