Abstract
Purpose. To summarize the experience in treating patients with genitoplasty due to disorders of sex development in China. Methods. The operative procedures, gender of rearing, surgical outcome, and psychosocial and family adjustments of 262 patients were reviewed retrospectively. Results. At initial diagnosis, the mean age was 14.3 ± 2.8 years (range: 2–38 years). There were 96 children, 133 adolescents, and 33 adults. Follow-up was done every 6 months. Patients with female sex assignment had no urinary incontinence or voiding difficulty. Five patients underwent the second surgery (3%); vaginal dilation was performed in 35 patients with postoperative vaginal stenosis; 12 patients (7.4%) were unsatisfactory with the outcome. For patients with male sex assignment, the median length of penis was 2.2 cm in prepubertal patients, 4.2 cm in pubertal patients, and 5.0 cm in adults; 39 patients developed postvoid dribbling (39%); 21 patients underwent a second surgery (21%); urethral dilation was done in 28 patients (28%) due to urethral stricture; 38 patients were unsatisfactory with the outcome (38%). In addition, 136 patients (83%) with female sex assignment and 54 (54%) with male sex assignment had favorable psychosocial adjustment. Conclusions. Patients with male sex assignment have more surgical complications and difficulties in psychosocial adjustment as compared to those with female sex assignment.
Highlights
Disorders of sexual development (DSD) refer to congenital conditions with atypical development of chromosomal, gonadal, or anatomical sex [1]
The patients were classified by karyotype, signs of androgen effects (XY-DSD and XX-DSD as suggested by the consensus statement of European Society for Paediatric Endocrinology (ESPE)/LWSPE conference) according to methods in study of Lux et al [9] (Table 1)
Among DSD-XX-PF patients, the assigned sex was female in 41 patients and male in 8; among DSD-XX-P-M patients, the assigned sex was female in 2 patients and male in 25; among DSD-XX-C-F patients, the assigned sex was female in 9 patients and male in 2; among DSD-XY-P-F patients, the assigned sex was female in 66 patients and male in 6; among DSD-XY-C-F patients, the assigned sex was female in 43 patients and male in 11; among DSD-XY-P-M patients, the assigned sex was female in 2 patients and male in 47 (Table 1)
Summary
Disorders of sexual development (DSD) refer to congenital conditions with atypical development of chromosomal, gonadal, or anatomical sex [1]. The sex chromosomes of sperm and egg determine the gonad (testes or ovaries) by which sex hormones are secreted. These hormones bind to corresponding receptors determining the development of sexual characteristics. Any abnormality in this process may cause DSD which includes the loss or mutation of SRY gene in chromosome Y resulting in abnormal sexual differentiation and development. Chromosomes X, 11, 17, and 9 are regarded to involve in the regulation of this process [2,3,4,5,6]. In 2006, the European Society for Paediatric Endocrinology (ESPE) and the Lawson Wilkins Paediatric
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
