title: Long-term outcomes in males with disorders of sex development.
abstract: OBJECTIVE
  Indications that the prenatal action of testosterone in the brain is an important determinant of gender development and improved reconstructive techniques have caused a shift in male gender assignments in patients with 46XY disorders of sex development. We report long-term outcome data on psychosexual development and sexual function of these individuals in a cross-sectional study.
  
  
  METHODS
  Physical status of 14 men with a mean age of 25 years with disorders of sex development was assessed by structured interview and physical examination. Psychosexual outcome was evaluated by questionnaires and compared to a control group of 46 healthy, age matched men.
  
  
  RESULTS
  A total of 13 men underwent 1 to 6 (mean 2) genital surgeries. Mean age at first surgery was 2.7 years. Mean penile length was 6.6 cm. All men reported erections and were able to experience orgasms. Ejaculatory dysfunction was reported by 7 men. Mean penile length was 7.9 cm in patients who were able to achieve penetrative intercourse and 4.9 cm in those who were not. Meatus was glanular in 5 patients, coronal in 7 and at the distal shaft in 1. Compared to controls, men with disorders of sex development were less satisfied with the appearance of the penis and scrotum but not with total body image. These patients reported decreased sexual desire and activities.
  
  
  CONCLUSIONS
  Outcome in this group of men with disorders of sex development was poor regarding penile length, ejaculation, satisfaction with external genitalia and frequency of sexual activity. Other aspects, such as overall body image and psychosexual functioning, showed no difference from controls.
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: Nina
authors.initials: N
authors.lastname: Callens
authors.forename: Jet
authors.initials: J
authors.lastname: van Kuppenveld
authors.forename: Karen
authors.initials: K
authors.lastname: Kwak
authors.forename: Stenvert L S
authors.initials: SL
authors.lastname: Drop
authors.forename: Barbara
authors.initials: B
authors.lastname: Kortmann
authors.forename: Arianne B
authors.initials: AB
authors.lastname: Dessens
authors.forename: Katja P
authors.initials: KP
authors.lastname: Wolffenbuttel
mesh_terms: D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D016009:Chi-Square Distribution; D003430:Cross-Sectional Studies; D012734:Disorders of Sex Development / Q000175:diagnosis* / Q000523:psychology / Q000628:therapy*; D004542:Ejaculation / Q000502:physiology; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010410:Penile Erection / Q000502:physiology; D011584:Psychology; D018570:Risk Assessment; D012649:Self Concept; D012725:Sexual Behavior / Q000502:physiology* / Q000523:psychology; D018709:Statistics, Nonparametric; D011795:Surveys and Questionnaires; D013739:Testosterone / Q000627:therapeutic use; D013997:Time Factors; D013519:Urogenital Surgical Procedures / Q000379:methods*; D055815:Young Adult
chemical_list: D013739:Testosterone
keywords: DSD; FSH; GD; LH; MSHQ; Male Sexual Health Questionnaire; PAIS; body image; disorders of sex development; follicle-stimulating hormone; follow-up studies; gonadal dysgenesis; luteinizing hormone; partial androgen insensitivity syndrome; sex reassignment surgery; urologic surgical procedures, male