Evaluation and treatment for ovotesticular disorder of sex development (OT-DSD) - experience based on a Chinese series

BMC Urology

Research Article

Ru Wang¹  Daorui Qin²  Yunman Tang²  Shaoji Chen²  Xuejun Wang²  Yu Mao² 

[1] Department of Burn and Plastic Surgery, West China Hospital of Sichuan University, Chengdu, China;Department of Pediatric Surgery of Children’s Medical Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People’s Hospital, Chengdu, China;

关键词: Ovotestis;    Hypospadias;    Urethroplasty;    Disorder of sex development;   

DOI  :  10.1186/s12894-017-0212-8

 received in 2016-09-06, accepted in 2017-03-20,  发布年份 2017

来源: Springer

【 摘 要 】

BackgroundThe 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.MethodsSixteen 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.ResultsThe 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.ConclusionsOT-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.

【 授权许可】

CC BY   
© The Author(s). 2017