手术治疗颅骨血友病性假性肿瘤一例并文献复习  

Surgical management of cranial hemophilic pseudotumor: case report and literature review

作　　者：陈川[1] 王辉[1] 叶卓鹏[1] 罗伦[1] 常艳宇 郭英[1] 

机构地区：[1]中山大学附属第三医院神经外科,广州510630 [2]中山大学附属第三医院神经内科

出　　处：《中华脑科疾病与康复杂志（电子版）》2014年第4期42-45,共4页Chinese Journal of Brain Diseases and Rehabilitation（Electronic Edition）

摘　　要：目的探讨颅骨血友病性假性肿瘤的手术治疗策略。方法参考既往文献报道,对1例罕见颅骨血友病性假性肿瘤病例采取手术治疗,并对既往世界范围内报道的7例病例的病史特点和治疗经验进行归纳总结。结果在围手术期及术中予凝血因子Ⅷ替代治疗改善凝血功能。术中彻底清除陈旧性血肿及受损骨质,颅骨缺损处予钛网修补。组织学检查提示标本为陈旧性血块组织。术后动态复查CT显示受压移位小脑、脑干逐步复位。患者头晕、步态不稳症状消失。既往报道了7例颅骨血友病性假性肿瘤,其中有6例(85.7%)采取了手术治疗。围手术期均予凝血因子Ⅷ、冷沉淀和(或)血浆替代治疗,预后良好。结论外科手术是治疗颅骨血友病性假性肿瘤的有效手段,其手术干预的要点在于围手术期凝血因子的补充、凝血因子水平的监测以及术中清除血肿后残腔的处理。Objective To explore the surgical treatment strategy of hemophilic pseudotumors occurring in the cranium. Methods Referring to previous foreign reports, a rare case of cranial hemophilic pseudotumor with surgical procedures were managed,then the case characteristic and treatment experience of 7 reported foreign cases were summarized. Results Coagulation factorVl~ replacement was performed before, during and after surgery. The hematoma was totally evacuated, and the eroded bone was removed and replaced by titanium skull. Histologic analysis of the lesion disclosed old blood coagulum. The repeated postoperative CT scans revealed gradual restoration of dislocated cerebellum and brain stem, and the patient got free of dizzy and walking lability. Only 7 cases of this tumor occurring in the cranium had been previously reported, of which 6 cases （85.7%） received surgical treatment. All cases received coagulation factor replacement with factor Ⅶ, cryoprecipitate and/or fresh frozen plasma, and were given excellent prognosis. Conclusion Surgical treatment is a practical management option of cranial hemophilic pseudotumor. Supplement of factors, monitoring factor level in perioperative period and appropriate management of hematoma cavity are of critical importance for surgical intervention.

关 键 词：颅骨 外科手术 血友病性假性肿瘤 

分 类 号：R651.1[医药卫生—外科学]