儿童颅缝早闭症合并Chiari畸形的治疗分析  

Treatment analysis of craniosynostosis combined with Chiari malformation in children

作　　者：祝永杰[1] 王刚[1] 何俊平[1] 高喆 钱静[1] 邱德智 郑雷[1] 王新法[1] 张瑞[1] 张献礼 Zhu Yongjie;Wang Gang;He Junping;Gao Zhe;Qian Jing;Qiu Dezhi;Zheng Lei;Wang Xinfa;Zhang Rui;Zhang Xianli(Department of Neurosurgery,Children's Hospital of Nanjing Medical University,Nanjing 210000,China)

机构地区：[1]南京医科大学附属儿童医院神经外科,210000

出　　处：《中华神经外科杂志》2019年第12期1236-1239,共4页Chinese Journal of Neurosurgery

摘　　要：目的探讨儿童颅缝早闭症合并Chiari畸形的手术方法和疗效。方法回顾性分析南京医科大学附属儿童医院神经外科2017年4月至12月收治的16例儿童颅缝早闭症合并Chiari畸形患者的临床资料。16例患儿中,未合并脑积水的12例均行颅缝再造术或颅面重建术,其中1例于术后7个月再次行后颅窝成型+枕下减压术;另合并脑积水的4例患儿中,3例行右侧脑室-腹腔分流术+颅面重建术,其中1例于术后5个月再次行后颅窝成型+枕下减压术;另1例为Pfeiffer综合征,行右侧脑室-腹腔分流术+后颅窝成型+枕下减压术治疗。术后3、6、12个月对所有患儿行门诊随访,复查头颅MRI以评估脑组织发育、脑室变化及小脑扁桃体下疝的情况。结果16例患儿的手术均成功,均未出现手术相关并发症。术后复查头颅CT,15例患儿的颅腔容积均扩大,颅骨畸形均得到改善;另1例因治疗未结束未进行评估。16例患儿的随访时间为(12.1±2.0)个月(10~18个月)。复查头颅MRI显示,脑组织发育良好,小脑扁桃体位置均未见进一步下降;行分流术的4例患儿脑室均有不同程度的缩小。所有患儿均无神经功能缺失,且家长对其颅面部外观的改善均表示满意。结论颅缝早闭症合并Chiari畸形术前应全面评估,并根据具体情况选择合适的个体化治疗方案;且一期行颅面外科手术是其有效的治疗方法。Objective To explore the surgical method and outcome of craniosynostosis combined with Chiari malformation in children.Methods A retrospective analysis was conducted on the clinical data of 16 children with craniosynostosis combined with Chiari malformation who were admitted to Department of Neurosurgery,Children′s Hospital of Nanjing Medical University from April to December 2017.All cases were retrospectively analyzed and their imaging results were analyzed.Of the 16 children without hydrocephalus,12 underwent craniofacial reconstruction of cranial suture or craniofacial reconstruction,of which 1 case underwent posterior fossa reconstruction and suboccipital decompression at 7 months post operation.In 4 children with combined hydrocephalus,3 underwent routine right ventricle-peritoneal shunting and cranial reconstruction,of which 1 case was treated with posterior fossa reconstruction and suboccipital decompression at 5 months post operation.The other 1 case was Pfeiffer syndrome and underwent right ventricle-peritoneal shunting plus posterior fossa reconstruction as well as suboccipital decompression.At 3,6 and 12 months post operation,all patients were followed up at the outpatient clinic and underwent the reexamination of head MRI for assessment of brain tissue development,ventricle changes and cerebellum tonsil herniation.Results All 16 children underwent operation successfully without any surgical complications.Revealed by the postoperative skull CT,all the children′s cranial cavity volume was enlarged,and the cranial malformation was improved.The follow-up time of 16 children was(12.1±2.0)months(10-18 months).The review of head MRI showed that brain tissue developed well and the position of the cerebellar tonsils did not decline further.All children were free of neurological deficits,and parents were satisfied with the improvement in the appearance of their skulls and faces.Conclusions The combination of craniosynostosis with Chiari malformation should be fully evaluated before surgery and appropriate

关 键 词：颅缝早闭 ARNOLD-CHIARI畸形 儿童 治疗结果 

分 类 号：R65[医药卫生—外科学]