经肛门治疗Currarino综合征

目的 探讨Currarino综合征(Currarino syndrome,CS)的诊断和经肛门手术治疗的经验.方法 2013年1月至2015年5月,我院共收治11例CS患儿,男2例,女9例,平均年龄11个月.其中7例肛门位置正常,但肛门直肠严重狭窄;1例患儿反复出现肛周感染及肛瘘;3例患儿可见肛门直肠畸形.其中5例患儿术前已行结肠或回肠造瘘术.术前全部患儿行肛门指诊、骶尾部正侧位片、下消化道造影及骶部核磁共振检查.术中患儿经过肛门或瘘口游离直肠,肠管拖出后,向上牵开肠管.将切口后方向左右牵开,可暴露并切除骶前肿物.术后全部患儿给予抗感染治疗,术前已有造瘘患儿术后第1天恢复饮食,无造瘘患儿术后2d恢复饮水,术后4d恢复低渣进食,无并发症患儿术后7d出院,14d扩肛.术后每3个月复查.已有造瘘患儿术后3个月行造瘘还纳术.结果 肛门位置正常患儿全部有直肠肛门严重狭窄,小指不能进入.全部患儿影像学检查可见骶骨畸形和骶前肿物.11例患儿全部顺利完成手术,术后8例恢复良好,1例患儿出现骶前及中枢神经系统感染,1例高位肛瘘,1例出现术后骶前出血.全部患儿目前恢复良好.结论 对于年龄小于3岁,肿物最大径小于5 cm的CS患儿,经肛门一次完成肛门成型和骶前肿物切除是可行的.

Transanal treatment of Currarino syndrome

Objective To explore the diagnosis and transanal pull-through for Currarino syndrome (CS).Methods From January 2013 to May 2015,2 males and 9 females were recruited.There were normal anal site with complicated anal stenosis (n =7),recurrent anal fistula (n =1),rectovestibular fistula (n =2) and perineal fistula (n =1).And 5 of them underwent colostomy or ileostomy.Digital examination,radiograph and magnetic resonance imaging (MRI) of pelvic colostogram were performed to confirm the diagnosis of CS.Transanal pull-through was extended until the finding of normal colon.Then rectum was retracted and the exposed mass excised completely.Finally an anastomosis was created between normal bowel and anus.Results All cases of normal anal sites had anal stenosis.And sacral bony malformation and presacral mass were all visualized.All operations were successful.And the outcomes were recovery (n =8),CNS infection (n =1),presacral hemorrhage (n=1) and local infection & anal fistula (n=1).Conclusions Transanal procedure is feasible for CS patients aged under 3 years.And the maximal diameter is less than 5 cm.