Long-Term Evaluation of Patients Undergoing Genitoplasty Due to Disorders of Sex Development: Results from a 14-Year Follow-Up

Below:  Externalia plasty for ovotesticular DSD. (a) Before surgery. The vagina was not obvious, and the vagina and urethra shared an outlet at the top of small penis with poor development. (b) After surgery. The outlet of the vagina and urethra was exposed, and the vagina and urethra were separated. The urethra was extended for urination control and vagina exposure. Patients agreed with publication of these photos.

Below:  DSD patients. (a) 46, XX DSD. The clitoris was hypertrophic and penis-like, the gonad was ovoid, and the patient had naïve uterus; (b) 46, XY DSD. Complete female externalia was observed in appearance, the vagina and breast were well developed, and bilateral testicles were found in the abdomen. (c) Ovotesticular DSD. The penis was poorly developed, and unilateral testicle and contralateral ovary were found; (d) 46, XY. Complete gonadal dysgenesis was observed. The vagina and urethra shared an outlet and the gonads were poorly developed. Patients agreed with publication of these photos.

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.

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