title: Long-term surgical outcome of masculinizing genitoplasty in large cohort of patients with disorders of sex development.
abstract: OBJECTIVE
  We evaluated the results of masculinizing genitoplasty in a large cohort of patients with disorders of sex development treated at a single public tertiary center.
  
  
  METHODS
  We evaluated 52 patients with 46,XY and 7 with 46,XX disorders of sex development with proximal hypospadias and genital ambiguity reared as males who had undergone surgery between 1965 and 2008. Mean +/- SD followup was 14.1 +/- 9.2 years and median age at last examination was 22 years, with 38 patients having reached adulthood. Morphological result and urinary stream were evaluated by a physician. Urinary and sexual symptoms, and satisfaction with surgical results were assessed by questionnaire.
  
  
  RESULTS
  Mean penile length at diagnosis was compared between 46,XY patients and showed that those with 5alpha-reductase 2 deficiency had the shortest penile length (-5.4 +/- 1.2 SD). At the last clinical evaluation following surgical and hormonal treatment mean +/- SD penile length in 38 adults was 7.5 +/- 2.1 cm (range 4 to 12), corresponding to -4.3 +/- 1.3 SD (-6.5 to -1.5). All but 2 patients had penile length less than -2 SD. At that time mean penile length remained shorter in patients with 5alpha-reductase 2 deficiency (-5.4 +/- 1 SD) compared to those with testosterone production deficiency or indeterminate disorders of sex development (p <0.05). There was no statistical difference between mean penile length before and after treatment in all etiological groups (p >0.05). Morphological results were good in 43% of patients, fair in 54% and poor in 3%. The most common complications were urethral fistula (51%) and urethral stenosis (22%). Dribbling after voiding was the most frequent urinary symptom. Satisfaction with surgical results was reported by 89% of patients. Among adults 87% were sexually active, with 64% reporting normal sexual activity.
  
  
  CONCLUSIONS
  Most patients with 46,XY disorders of sex development were satisfied with long-term results of masculinizing genitoplasty, although specific complaints about small penile length, sexual activity and urinary symptoms were frequent. New surgical approaches should be developed to ensure full satisfaction in adulthood among patients with disorders of sex development.
authors.affiliation: Endocrinology Division, Hormone and Molecular Genetics Laboratory LIM/42, Endocrinology Discipline, Hospital das Clinicas da FMUSP, São Paulo, Brazil. mhps_msm@yahoo.com
authors.forename: Maria Helena Palma
authors.initials: MH
authors.lastname: Sircili
authors.forename: Frederico Arnaldo de Queiroz
authors.initials: FA
authors.lastname: e Silva
authors.forename: Elaine M F
authors.initials: EM
authors.lastname: Costa
authors.forename: Vinicius N
authors.initials: VN
authors.lastname: Brito
authors.forename: Ivo J P
authors.initials: IJ
authors.lastname: Arnhold
authors.forename: Francisco Tibor
authors.initials: FT
authors.lastname: Dénes
authors.forename: Marlene
authors.initials: M
authors.lastname: Inacio
authors.forename: Berenice Bilharinho
authors.initials: BB
authors.lastname: de Mendonca
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D012734:Disorders of Sex Development / Q000601:surgery*; D005500:Follow-Up Studies; D005835:Genitalia / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D017060:Patient Satisfaction; D013997:Time Factors; D016896:Treatment Outcome; D013521:Urologic Surgical Procedures, Male / Q000379:methods; D055815:Young Adult