儿童血友病神经外科疾病的治疗策略

目的探讨儿童血友病神经外科疾病的治疗方法与策略。方法回顾性分析3例血友病神经外科疾病患儿的临床资料。结果3例患儿中，血友病A、血友病B、血管性血友病各1例。其中，血友病A和B患儿均在术前已确诊，均因脑出血后致药物难治性癫痫，经多学科评估后适合外科手术治疗；在围手术期补充相应凝血因子后，手术顺利，术后未出现再出血；术后随访24~30个月，均无癫痫发作。另一例患儿因外伤后颈髓硬脊膜下出血致四肢瘫痪而急诊手术，术中出现凝血障碍，术后因肺出血影响呼吸功能给予呼吸机辅助治疗；血液检查示血管性血友病因子（vWF）抗原（2.6）、vWF活性（＜3）显著降低，确诊为血管性血友病，给予相应治疗后痊愈出院。结论儿童血友病神经外科疾病患者给予合理补充凝血因子后，可安全实施相应的外科手术治疗，并可获得满意的临床效果。

Abstract: Objective To explore the treatment methods and strategies of children hemophilia related neurosurgical diseases. Method The clinical data of 3 children with hemophilia related neurosurgical diseases were analyzed retrospectively. Results Among the 3 cases, hemophilia A, hemophilia B and von Willebrand disease were 1 case each. Among them, hemophilia A and hemophilia B children were diagnosed before operation, because of intracerebral hemorrhage caused by drug refractory epilepsy, after multidisciplinary evaluation, suitable for surgical treatment. After the supplement of coagulation factors in perioperative period, the operation was smooth and no rebleeding occurred. All patients were followed up for 24 to 30 months. Another patient had emergency operation due to quadriplegia caused by spinal cord subdural hemorrhage after trauma. During the operation, coagulation disorder occurred. After the operation, respiratory function was affected by pulmonary hemorrhage, and ventilator was given to assist treatment. Blood examination showed that Von Willebrand factor(vWF) antigen(2.6) and vWF activity(<3) were significantly decreased. The patient was diagnosed as von Willebrand disease, and was cured after corresponding treatment. Conclusion After reasonable supplement of coagulation factors in children with hemophilia related neurosurgical diseases, the corresponding surgical treatment can be safely implemented, and satisfactory clinical results can be obtained.