Ⅰ型神经纤维瘤病相关巨大丛状神经纤维瘤破裂出血急诊处理及围术期管理策略

Emergency management and perioperative strategies for intra-tumoral hemorrhage in neurofibromatosis type 1-related giant plexiform neurofibroma

目的综述Ⅰ型神经纤维瘤病(neurofibromatosis type 1,NF1)相关巨大丛状神经纤维瘤(plexiform neurofibroma,PNF)破裂出血的急诊处理及围术期管理策略,提供一种系统处理流程,以提高巨大PNF治疗效果和患者生存质量。方法回顾巨大PNF破裂出血的相关治疗文献,并结合临床实践经验,总结当前巨大PNF破裂出血诊断方法、治疗方案及围术期综合管理措施。结果通过实施综合诊疗策略(包括早期诊断、影像学评估、急诊超选择性动脉栓塞术与手术切除相结合),不仅能够有效控制急性出血,还可以降低手术中大出血风险,减少术后并发症,从而显著提高治疗成功率和患者生存质量。结论对于NF1相关的巨大PNF破裂出血,在多学科团队合作下采用急诊超选择性动脉栓塞术结合手术切除治疗,可有效提高治疗成功率,快速控制出血、缩小瘤体以及降低患者死亡率。未来研究需要继续关注巨大PNF破裂出血患者治疗后长期生存质量及进一步优化治疗方案。

Objective To review the emergency management and perioperative strategies for ruptured neurofibromatosis type 1(NF1)-related giant plexiform neurofibroma(PNF),providing a systematic treatment protocol to improve the therapeutic outcomes and quality of life for patients with giant PNF.Methods The literature on the management of giant PNF rupture and hemorrhage was reviewed,and the diagnosis,treatment,and perioperative management were summarized based on clinical experiences.Results By implementing an integrated diagnostic and treatment strategy that includes early diagnosis,imaging evaluation,emergency ultra-selective arterial embolization combined with surgical excision,acute hemorrhage can be effectively controlled while also reducing the risk of major intraoperative bleeding and minimizing postoperative complications.As a result,this approach significantly improves treatment success rates and patient quality of life.Conclusion For ruptured NF1-related giant PNF,employing emergency ultra-selective arterial embolization combined with surgical excision,under the collaboration of a multidisciplinary team,can effectively improve treatment success rates,rapidly control bleeding,reduce tumor size,and lower mortality.Future research should focus on assessing the long-term quality of life of patients treated for ruptured and hemorrhaging giant PNF and on further optimizing treatment protocols.