title: Long-term followup of a large cohort of patients with ovotesticular disorder of sex development.
abstract: OBJECTIVE
  We present the followup of a large cohort of patients with ovotesticular disorder of sex development treated at a single tertiary center.
  
  
  METHODS
  We reviewed the records of 20 patients with ovotesticular disorder of sex development. We retrospectively evaluated clinical and surgical characteristics. A prospective study was also performed, including evaluation of surgical results, gonadal function, sexual activity and voiding symptoms of these patients during adulthood.
  
  
  RESULTS
  All patients had ambiguous genitalia, including 18 with a 46,XX karyotype and 2 with a 46,XX/46,XY karyotype. Gender assignment at birth was male in 13 patients and female in 7. Three females were later reassigned to the male gender. Bilateral gonadectomy was performed in 10 patients. Testicular tissue was preserved in 8 males and ovarian tissue was preserved in 2 females. Average followup was 25 years (range 4 to 46). Puberty started spontaneously in 14 patients between ages 11 and 14 years. Seven patients showed spontaneous puberty after conservative gonadal surgery and 4 required hormonal replacement during adulthood. The most frequent complications in males were urethral fistula in 6 and late urethral stenosis in 3. Two patients with urethral stenosis had symptoms 10 years postoperatively. One female presented with temporary dyspareunia. In adulthood 8 males and 2 females reported sexual activity. All male patients reported orgasm and 2 reported ejaculation.
  
  
  CONCLUSIONS
  Male gender assignment was more prevalent. Long-term followup revealed adequate pubertal development and sexual activity. Complications involving the urethra developed frequently in male patients.
authors.affiliation: Division of Urology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil; Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil. Electronic address: mhps_msm@yahoo.com.
authors.forename: Maria Helena Palma
authors.initials: MH
authors.lastname: Sircili
authors.affiliation: Division of Urology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Francisco Tibor
authors.initials: FT
authors.lastname: Denes
authors.affiliation: Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Elaine Maria Frade
authors.initials: EM
authors.lastname: Costa
authors.affiliation: Division of Urology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Marcos Giannetti
authors.initials: MG
authors.lastname: Machado
authors.affiliation: Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Marlene
authors.initials: M
authors.lastname: Inacio
authors.affiliation: Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Rosana Barbosa
authors.initials: RB
authors.lastname: Silva
authors.affiliation: Division of Urology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Miguel
authors.initials: M
authors.lastname: Srougi
authors.affiliation: Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Berenice Bilharinho
authors.initials: BB
authors.lastname: Mendonca
authors.affiliation: Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Sorahia
authors.initials: S
authors.lastname: Domenice
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D050090:Ovotesticular Disorders of Sex Development / Q000601:surgery*; D011627:Puberty; D012189:Retrospective Studies; D055815:Young Adult
keywords: disorders of sex development; follow-up studies; ovary; sex reassignment procedures; testis