Mao Yu, Chen ShaoJi, Tang Yunman, Wang Xuejun, Qin Daorui, Chen Yuejiao
Department of Pediatric Surgery at Children's Medical Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu Sichuan, 610072, P.R.China.
Department of Pediatric Surgery at Children's Medical Center, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu Sichuan, 610072,
Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2017 Sep 15;31(9):1092-1097. doi: 10.7507/1002-1892.201705004.
To explore the indication and effectiveness of urogenital sinus surgery in feminizing genitoplasty of disorder of sex development (DSD).
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.
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.
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.
探讨泌尿生殖窦手术在性发育障碍（DSD）女性化生殖器成形术中的适应证及有效性。
回顾性分析2010年10月至2015年12月期间接受一期女性化生殖器成形术的22例DSD患者的临床资料。患者年龄为1岁2个月至21岁，中位年龄为2岁1个月。根据普拉德分类标准，外阴外观评为Ⅰ级7例，Ⅱ级6例，Ⅲ级8例，Ⅳ级1例。所有患者在女性化生殖器成形术前均行膀胱镜检查。发现19例患者阴道与尿道低位融合，3例患者高位融合。泌尿生殖窦平均长度为1.6 cm（范围0.5 - 3.0 cm）。注水阴道平均长度为4.4 cm（范围3.5 - 5.5 cm）。16例患者在阴道末端检测到宫颈，6例患者未检测到宫颈。所有患者均采用相同的阴蒂成形术和阴唇成形术步骤。采用了三种泌尿生殖窦手术方法，6例患者采用“回缩”阴道成形术，11例患者采用“皮瓣”阴道成形术，5例患者采用部分泌尿生殖窦游离术（PUM）。
所有手术均成功完成，切口均Ⅰ期愈合。所有患者随访12 - 74个月，平均30.5个月。外观评价结果为优13例（59.1%），良6例（27.3%），差3例（13.6%）。17例已训练自主排尿的患者未发现尿失禁、残余尿量、泌尿系统感染及尿道阴道瘘。
泌尿生殖窦手术是DSD女性化生殖器成形术中最关键的步骤。它可以在青春期前与阴蒂成形术和阴唇成形术一起在一期手术中完成。如果术前进行全面评估并掌握不同手术方法的原则，结果将令人满意。
