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目的 进一步了解延髓血管母细胞瘤的特点, 确定治疗方法, 改进手术技巧, 预防并发症及降低死亡率。方法 回顾性地分析自1987 年2 月至1998 年12 月显微手术治疗的47 例延髓血管母细胞瘤的临床资料。结果 39 例单发的血管母细胞瘤分布在延髓脑桥背部 (5 例) 、延髓背部(22例) 及延髓颈髓背部或髓内(12 例) ; 8 例为多发的血管母细胞瘤。974 % 单发血管母细胞瘤的 M R I上可见肿瘤周边低信号的囊肿形成, 并可分成以实质肿瘤为主体和以囊肿为主体两类。 D S A 及 M R A可以显示主要的供血动脉和浓厚的肿瘤染色。39 例单发的血管母细胞瘤皆被整个摘除, 术后615 % 病人的症状显著进步; 2 例与术前相似; 11 例恶化, 其中8 例经治疗后好转, 2 例死亡。结论 延髓血管母细胞瘤在 M R I及 D S A 上有其特点, 可在术前准确地作出诊断。对这种肿瘤切忌作活检或分块切除。小的实质性的或者囊性的肿瘤手术是比较安全的; 脑干背侧大的实质性肿瘤手术后会出现病情恶化, 我们认为这是正常灌注压突破( N P P B) 现象所致; 正确地掌握这种肿瘤的手术技巧, 预防及减轻 N P P B 是降低手术死亡率的关键。
Objective To further understand the characteristics of medullary hemangioblastoma, to determine the treatment methods, improve surgical skills, prevent complications and reduce mortality. Methods The clinical data of 47 cases of medullary hemangioblastoma treated microsurgically from February 1987 to December 1998 were analyzed retrospectively. Results 39 cases of single hemangioblastoma were found in the dorsal medulla oblongata pontine pontine (n = 5), medulla oblongata (n = 22) and medulla oblongata or in the medullary cavity (n = 12). Eight patients had multiple hemangioblastomas . 97.4% of solitary hemangioblastoma M R I visible tumor around the low signal cyst formation, and can be divided into the main tumor and the cyst as the main two categories. D S A and M R A can show the main feeding artery and thick tumor staining. Thirty-nine patients with solitary hemangioblastoma were all enucleated. The symptoms of 61.5% of patients were significantly improved after operation. Two patients were similar to preoperative and 11 patients were exacerbated. Eight patients improved after treatment and two patients died. Conclusion The medullary hemangioblastoma has its own characteristics on M R I and D S A, which can be accurately diagnosed before operation. Avoid this biopsy or subtotal resection of this tumor. Small, substantive or cystic tumor surgery is safer; a substantial tumor back in the dorsal trunk of the brain can cause a worsening of the condition, which we believe is due to the phenomenon of normal perfusion pressure breakthrough (NPPB) Correct understanding of the surgical technique of this tumor and the prevention and reduction of NP P B are the key to reducing the operative mortality.