儿童Currarino综合征合并脊髓栓系的诊断和治疗

Diagnosis and Treatment of Currarino Syndrome Combined with Tethered Spinal Cord in Children

目的：探讨合并脊髓栓系的儿童Currarino综合征的诊断和治疗。方法回顾2007年1月～2014年11月苏州大学附属儿童医院收治的6例合并脊髓栓系的Currarino综合征患儿临床表现、诊断和治疗。结果2例患儿术前诊断为Currarino综合征合并脊髓栓系，一期完成手术。另外4例在诊断脊髓栓系前已行肛门直肠畸形手术，术后因大小便功能改善效果不佳，磁共振检查发现合并脊髓栓系，二期行脊髓栓系松解术。5例手术顺利，术后大小便功能均有明显进步，1例因出现直肠瘘，先行乙状结肠造漏，直肠瘘口3个月后重新肠吻合后治愈。结论诊断Currarino综合征的患儿行核磁共振检查时应仔细观察是否合并有脊髓栓系，争取一期手术；对于未能在Currarino综合征术前明确合并脊髓栓系的病例，如术后患儿大小便功能改善不明显，应想到合并脊髓栓系的可能性，尽早手术。Currarino综合征合并脊髓栓系虽然病情复杂，但经过及时诊治仍能取得较好的预后。

Objective To study the diagnosis and treatment of Currarino syndrome combined with tethered spinal cord.Methods A retrospective study was made on clinical manifestation, diagnosis and treatment of 6 children with Currarino syndrome combined with tethered spinal cord in Children＆#39;s Hospital Affiliated to Soochow University from January 2007 to November 2014.Results2 cases was successfully made preoperative diagnosis as Currarino syndrome complicated with tethered spinal cord. 4 cases were not made the diagnosis of tethered spinal cord at the very beginning until we found the improvement of the postoperative defecation function was poor. MRI was used tofi nd combined tethered cord. They received spinal cord untethering surgery later. The postoperative defecation function was improved in 5 cases. 1 case had rectalfi stula afterfi rst operation and was cured 3 months later.Conclusion Children can be diagnosed with Currarino syndrome combined with tethered spinal cord by MR. For those cases which failed to get the preoperative diagnosis of Currarino syndrome with tethered spinal cord, if the postoperative defecation function improvement was not obvious, we should consider the possibility of tethered spinal cord. Currarino syndrome combined with tethered spinal cord is a kind of serious congenital malformations, but can still achieve good prognosis with right treatment.