[Diagnosis and treatment of ovotesticular disorders of sex development in children].
OBJECTIVE
To investigate the diagnosis and treatment of ovotesticular disorders of sex development (DSD) in children.


METHODS
We reviewed the clinical data of 9 cases of ovotesticular DSD admitted in our department from 1988 to 2007.


RESULTS
The patients ranged in age from 9 months to 9 years, 7 raised as males and 2 as females. As for the karyotype, 4 cases were 46,XX, 2 were 46,XX/46,XY, 1 was 46,XY, and the other 2 had no karyotype data. All of them presented with obscure external genitalia: perineal or penoscrotal hypospadias with or without cryptorchidism in males and hypertrophy of the clitoris in females. They were diagnosed with ovotesticular DSD by gonad biopsy and underwent genitoplasty.


CONCLUSIONS
The gender assignment of the ovotesticular DSD patient was chiefly based on the development of external genitalia, dominant gonad, karyotype and the parent's will. Laparoscopic technology is recommended in gonad biopsy and orchiopexy during the treatment of ovotesticular DSD.
Lu
Liang-Sheng
LS
Department of Surgery, Children's Hospital Affiliated to Fudan University, Shanghai 201102, China.
Ruan
Shuang-Sui
SS
Bi
Yun-Li
YL
Lu
Yi-Qun
YQ
Wang
Xiang
X
Ge
Lin-Juan
LJ
Xiao
Xian-Min
XM
D002648:Child; D002675:Child, Preschool; D012734:Disorders of Sex Development / Q000175:diagnosis* / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D010535:Laparoscopy; D008297:Male; D012189:Retrospective Studies; D046468:Sexual Development