title: Evaluation and treatment for ovotesticular disorder of sex development (OT-DSD) - experience based on a Chinese series.
abstract: BACKGROUND
  The aim of this study is to review and present the clinical features and process of evaluation and treatment for OT-DSD in a single center in recent years in China.
  
  
  METHODS
  Sixteen patients with OT-DSD during the past 4 years underwent the evaluation and treatment in a single center. The clinical characteristics and outcomes of surgery were analyzed.
  
  
  RESULTS
  The surgical age ranged from 17 months to 66 months with a mean age of 20 months, and the mean follow-up was 30 months (4 months to 56 months). The presentation in 11 patients was ambiguous genitalia, and the rest 5 patients were suspected to have DSD in preoperative examination before hypospadias repair. The karyotypes were 46, XX in 11 patients, 46, XX/46, XY in 3, 46, XX/47, XXY in 1, and 46, XY in 1. Initial reared sex was male in 14 patients, female in 1, and undetermined in 1. After surgery, genders were reassigned in 3 patients, while 15 patients were raised as male with testicular tissue left. Only 1 patient with ovarian tissue left was raised as female. Repair was completed in 11 males and 1 female, and stage I urethroplasty was done in 4 males. No further surgery to remove the gonads was needed for inconsonance of gender assignment. No gonadal tumors were detected.
  
  
  CONCLUSIONS
  OT-DSD is a rare and complex deformity with few systematic reports in China. It's important to establish a regular algorithm for evaluation and treatment of OT-DSD.
authors.affiliation: Department of Pediatric Surgery of Children's Medical Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu, China.
authors.forename: Yu
authors.initials: Y
authors.lastname: Mao
authors.affiliation: Department of Pediatric Surgery of Children's Medical Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu, China.
authors.forename: Shaoji
authors.initials: S
authors.lastname: Chen
authors.affiliation: Department of Burn and Plastic Surgery, West China Hospital of Sichuan University, Chengdu, China.
authors.forename: Ru
authors.initials: R
authors.lastname: Wang
authors.affiliation: Department of Pediatric Surgery of Children's Medical Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu, China.
authors.forename: Xuejun
authors.initials: X
authors.lastname: Wang
authors.affiliation: Department of Pediatric Surgery of Children's Medical Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu, China.
authors.forename: Daorui
authors.initials: D
authors.lastname: Qin
authors.affiliation: Department of Pediatric Surgery of Children's Medical Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu, China. toil112@163.com.
authors.forename: Yunman
authors.initials: Y
authors.lastname: Tang
mesh_terms: D002675:Child, Preschool; D002681:China; D012734:Disorders of Sex Development / Q000235:genetics / Q000601:surgery; D005260:Female; D006801:Humans; D007021:Hypospadias / Q000235:genetics / Q000601:surgery; D007223:Infant; D059785:Karyotype; D008297:Male; D050090:Ovotesticular Disorders of Sex Development / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D033282:Parental Consent / Q000941:ethics
keywords: Disorder of sex development; Hypospadias; Ovotestis; Urethroplasty