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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。
Objective To investigate the indications and curative effects of urogenital sinus surgery on vulvar feminization in patients with disorder of sex development (DSD). Methods The clinical data of 22 DSD patients with vulvar feminization repaired from October 2010 to December 2015 were retrospectively analyzed. Patients aged 1 year and 2 months to 21 years old, the median age of 2 years and 1 month. According to Prader grading standards of patients with genitals: Ⅰ grade in 7 cases, Ⅱ grade in 6 cases, Ⅲ grade in 8 cases, Ⅳ grade in 1 case. Vulva feminization before the repair of cystoscopy, showed high invagination in 3 cases, 19 cases of low entry; urine genital sinus length of 0.5 ~ 3.0 cm, an average of 1.6 cm; water state, vaginal length 3.5 ~ 5.5 cm, the average 4.4 cm. Cervix-like structures were seen in 16 cases at the bottom of the vagina and 6 cases at the bottom of the vagina. All patients used the same clot forming and labia forming; using three kinds of urogenital sinus formation, including 6 cases of urogenital sinus incision, perineal flap embedded genitourinary sinus angioplasty in 11 cases, partial urogenital sinus surgery 5 cases. Results All the operations were successfully completed and all incisions healed in the first stage. Twenty-two patients were followed up for 12-74 months with an average of 30.5 months. Six months after operation, the morphology of the vulva was evaluated with reference to Braga et al., Of which 13 cases were excellent (59.1%), 6 (27.3%) good and 3 (13.6%) poor. Postoperative 17 cases of spontaneous urination, no urinary incontinence, bladder residual urine, urinary tract infection and vaginal urethral fistula occurred. Conclusions Urogenital sinus surgery is a key step in the feminization of vulva for DSD patients. It can be performed simultaneously with clitoris formation and labia forming before puberty. Preoperative comprehensive assessment and accurate selection of surgical methods, can achieve a good postoperative appearance.