Currarino综合征的神经外科处理和效果分析  

Neurosurgical management of Currarino syndrome and its efficacy analysis

作　　者：修波[1] 李萃萃 林和璞[1] 谢晓丽 戴宜武 Xiu Bo;Li Cuicui;Lin Hepu;Xie Xiaoli;Dai Yiwu(Department of Neurosurgery,the Seventh Medical Center of the PLA General Hospital,Beijing 100700,China;Department of Pathology,the Seventh Medical Center of the PLA General Hospital,Beijing 100700,China)

机构地区：[1]解放军总医院第七医学中心神经外科,北京100700 [2]解放军总医院第七医学中心病理科,北京100700

出　　处：《中华神经外科杂志》2023年第9期875-880,共6页Chinese Journal of Neurosurgery

摘　　要：目的探讨Currarino综合征(CS)相关的神经外科疾病的处理方法和效果。方法回顾性分析2013年10月至2020年9月解放军总医院第七医学中心神经外科收治的19例CS患儿的临床资料。所有手术均采用骶部后正中入路。根据CS相关的神经外科疾病,分别采用畸胎瘤切除术(11例)、骶前脊膜膨出手术(12例)和脂肪瘤切除手术(3例)治疗。17例合并脊髓拴系者均行脊髓拴系松解术。采用门诊或互联网随访评估临床症状的改善情况,通过影像学随访评估病变的变化情况。结果10例畸胎瘤、5例骶前脊膜膨出囊达到全切除,3例脂肪瘤达到全切除或次全切除。术后骶部切口感染1例。所有患儿术后均随访2年,5例术前存在顽固性便秘的患儿症状缓解,其余14例患儿的症状无改变。末次MRI随访结果显示,10例畸胎瘤囊性包块全切除后无复发,1例有少量残留;5例骶前脊膜膨出囊全切除后无复发,7例缩小>75%;3例患儿的脂肪瘤达到全切除或次全切除;合并脊髓拴系患儿的脊髓拴系均完全松解。结论对于CS患儿,采用合适的手术方法处理CS伴随的神经外科疾患的效果满意,并发症少,复发率低,能够部分改善患儿的症状。Objective To investigate the therapies for neurosurgical lesions associated with Currarino syndrome(CS)and the outcomes.Methods A retrospective analysis was conducted on the clinical data of 19 cases of CS admitted to Department of Neurosurgery,the Seventh Medical Center of the PLA General Hospital from October 2013 to September 2020.All operations were performed via the posterior median sacral approach.Based on associated diseases,three kinds of surgical methods were adopted which included teratectomy(11 cases),surgical management of presacral meningocele(12 cases)and lipoma resection(3 cases).The 17 children with tethered cord underwent untethering surgery.Follow-up was performed by outpatient visits or internet to evaluate the improvement of clinical symptoms.Imaging follow-up was used to determine the change of lesions after procedure.Results Total resection were achieved in children who underwent neurosurgical treatment(including teratectomy,treatment of presacral mass and sacral dysplasia).total or subtotal resection was achieved in 3 cares of lipoma.Incision infection was observed in 1 child post operation.All patients were followed up for 2 years.At the last follow-up,intractable constipation was relieved in 5 cases and symptoms in the remaining 14 patients remained unchanged.MRI showed that the cystic mass of teratoma disappeared in 10 cases and 1 case had a small amount of residue.The presacral meningocele sac disappeared in 5 cases and shrunk by more than 75%in 7 cases.Lipomas were totally or subtotally removed.All tethered cord were completely released.Conclusion Appropriate treatment of neurosurgical diseases associated with CS can lead to satisfactory efficacy and partially relieve the symptoms with few postoperative complications and low rate of recurrence.

关 键 词：神经管缺损 神经外科手术 畸胎瘤 CURRARINO综合征 治疗结果 

分 类 号：R726.5[医药卫生—儿科]