Objective: To explore the indication and effectiveness of urogenital sinus surgery in feminizing genitoplasty of disorder of sex development (DSD).
Methods: A retrospective analysis was made on clinical data from 22 patients with DSD who underwent one stage feminizing genitoplasty between October 2010 and December 2015. The patients' age ranged from 1 year and 2 months to 21 years, with the median age of 2 years and 1 month. According to the Prader classification criteria, the appearance of vulvas were rated as grade Ⅰ in 7 cases, grade Ⅱ in 6 cases, grade Ⅲ in 8 cases, and grade Ⅳ in 1 case. Cystoscopy was applied before feminizing genitoplasty in all patients. Low confluence of vagina and urethra was found in 19 patients, while high confluence was found in 3 patients. The mean length of urogenital sinus was 1.6 cm (range, 0.5-3.0 cm). The mean length of water-filled vagina was 4.4 cm (range, 3.5-5.5 cm). Cervix was detected at the end of vagina in 16 patients, meanwhile absence of cervix was detected in 6 patients. The same procedures of clitoroplasty and labioplasty were used in all patients. Three procedures of urogenital sinus surgery were applied, as the "cut-back" vaginoplasty in 6 patients, the "flap" vaginoplasty in 11 patients, and the partial urogenital sinus mobilization (PUM) in 5 patients.
Results: All procedures were completed successfully and the incisions healed by stage Ⅰ. All patients were followed up 12-74 months, with the average of 30.5 months. The outcome of appearance evaluation was excellent in 13 patients (59.1%), good in 6 patients (27.3%), and poor in 3 patients (13.6%). Urinary incontinence, post-void residual, urinary infection, and urethrovaginal fistula were not found in 17 toilet trained patients.
Conclusion: Urogenital sinus surgery is the most critical step in feminizing genitoplasty of DSD. It can be finished in one stage procedure with clitoroplasty and labioplasty before puberty. If thoroughly evaluation before surgery is completed and the principle of different procedures is handled, the outcome will be satisfactory.
目的: 探讨尿生殖窦成形技术在性发育异常（disorder of sex development，DSD）患者外阴女性化修复治疗中的应用指征和疗效。.
方法: 回顾性分析 2010 年 10 月—2015 年 12 月，行一期外阴女性化修复的 22 例 DSD 患者临床资料。患者年龄 1 岁 2 个月～21 岁，中位年龄 2 岁 1 个月。患者外阴按照 Prader 分级标准：Ⅰ级 7 例，Ⅱ级 6 例，Ⅲ级 8 例，Ⅳ级 1 例。外阴女性化修复前均进行膀胱镜检查，示阴道高位汇入 3 例、低位汇入 19 例；尿生殖窦长度为 0.5～3.0 cm，平均 1.6 cm；注水状态下阴道长度 3.5～5.5 cm，平均 4.4 cm；16 例阴道底部可见子宫颈样结构，6 例阴道底部为盲端。所有患者采用相同阴蒂成形和阴唇成形方式；采用 3 种尿生殖窦成形方式，其中尿生殖窦切开术 6 例，会阴皮瓣嵌入式尿生殖窦成形术 11 例，部分性尿生殖窦游离术 5 例。.
结果: 手术均顺利完成，术后切口均Ⅰ期愈合。22 例患者均获随访，随访时间 12～74 个月，平均 30.5 个月。术后 6 个月，参照 Braga 等的方式评估外阴形态，其中优 13 例（59.1%）、良 6 例（27.3%）、差 3 例（13.6%）。术后 17 例能自主排尿，均无尿失禁、膀胱残余尿、尿路感染及阴道尿道瘘发生。.
结论: 尿生殖窦成形技术是 DSD 患者外阴女性化修复治疗中的关键步骤，可在青春期前与阴蒂成形和阴唇成形同期完成。术前全面评估和准确选择手术方式，能取得良好术后外观。.
Keywords: Urogenital sinus surgery; disorder of sex development; genitoplasty; vaginoplasty.