膀胱镜及逆行造影检查在泌尿生殖窦畸形患儿诊治中的应用

Endoscopy and retrograde urography in the diagnosis of urogenital sinus anomalies

目的评价膀胱镜及逆行造影检查在泌尿生殖窦(UGS)畸形患儿诊治中的应用价值。方法对2009年1月至2011年12月29例应用膀胱镜及逆行造影检查的UGS患儿的临床资料进行回顾性分析。结果 29例患儿中包括先天性肾上腺皮质增生症患者9例,男性假两性畸形5例,单纯UGS汇合8例,一穴肛畸形7例。膀胱镜检查结果:1例双侧输尿管异位开口于共同通道,3例可见精阜样结构,1例双阴道双子宫畸形以及2例直肠阴道瘘。2例阴道开口距离膀胱颈部<15 mm,共同通道>3 cm者1例,<1 cm者8例。9例阴道长度<2 cm。根据检查结果,2例行阴道游离拖出成形术;8例行后切开及皮瓣翻转术;其余19例患儿均接受了泌尿生殖窦整体游离拖出成形术,其中7例一穴肛患儿同时进行了直肠游离拖出肛门成形手术。术后平均随访9个月,目前无并发症出现。结论对于UGS畸形的患儿,术前进行膀胱镜及逆行造影的检查有助于为诊治提供精确的解剖信息,外科医师可据此采用针对性的手术方式,对于降低手术风险难度及改善患儿的预后应该会有不小的帮助。

Objective To report the application of endoscopy and retrograde urography in urogenital sinus（UGS） anomalies. Methods From 2009.1 to 2011.12, we used the cystoscope as the routine preoperative examination to 29 UGS patients, and placed a F3 calibrated catheter in each channel. The retrograde urography was taken through the catheters after the endoscopy. Results The study contained 9 CAH, 5 male pseudohermaphroditism, 8 simple confluence of urethral and vagina and 7 cloaca malformations. Bilateral ectopia of uretal orifice was observed in one case. Verumontanum could be seen in 3 cases. One patient was found as vagina and uterine duplication. There were two rectovaginal fistula in these patients. There were 2 patients whose distance from the bladder neck to the vagina was less than 15 mm. One patient＇s length of the common urogenital sinus was more than 3 cm, 8 patients were less than 1 cm. 9 patients＇ vagina were less than 2 cm. Two patients underwent the surgery to separate the urethral and vagina, then reconstruct respectively. 8 patients was taken the“Cut-back”and“Skin-flap”. The other patients underwent the total urogenital mobilization. The rectal and anus forming operations were performed on the cloaca malformations.The mean follow-up from when initially operation was 9 months.There was no noteworthy complications as we known. Conclusion It is very important to take the endoscopy and retrograde urography in UGS malformation patients. The surgeons can choose the operation approaches according to the result of examination, which can decrease the difficulty of the operation and improve the prognosis.