出血型Moyamoya病临床分析

目的回顾性分析首都医科大学附属北京天坛医院神经外科15年来收治的82例出血型烟雾病(Moyamoya)患者的临床资料,以提高对出血型Moyamoya病的进一步认识。方法全部患者均经脑血管造影检查确诊为Moyamoya病。根据CT检查及脑血管造影将其分为3组。A组:10例(12%),脑血管造影显示合并有动脉瘤。B组:38例(46%),CT显示为脑内出血(ICH),脑血管造影无动脉瘤。C组:34例(42%),CT显示为脑室出血(IVH),无动脉瘤或ICH。手术治疗49例患者,保守治疗33例。回顾性分析患者的临床及影像学资料并对其恢复情况做随访调查。结果共随访64例患者,其中手术治疗者42例,保守治疗者22例。疗效优良者46例,良好者9例,1例恢复差,8例死亡。有10例患者在随访期间在初次出血后0.3~8年间发生再出血,再出血率为12%,82例患者中有2例死于初次出血,死亡率为2.4%,6例患者死于再出血,死亡率为60%。结论出血是Moyamoya患者的重要死亡原因,再出血是出血型Moyamoya病的主要死亡原因。其预后主要取决于颅内出血部位、出血量和有无再次出血及治疗时机等。

Clinical Analysis of the Haemorrhagic Type Moyamoya Disease

Objective To analyse cases of haemorrhagic type moyamoya disease during 15 years period and elucidate the clinical features of this disease.Methods Eighty-two patients with haemorrhagic type moyamoya disease,50 males and 32 females,with average aged of 36.6 years,diagnosed with digital subtraction angiography,were divided into 3 groups according to CT scan: Group A consisted of 10 cases(12%) with aneurysms;Group B consisted of 38 cases(46%) with intracerebral haemorrhage(ICH) without aneurysms;Group C consisted of 34 cases(42%) with intraventricular haemorrhage(IVH) without aneurysms or ICH.Surgical treatment was performed in 49 patients and conservative treatment was done in 33 patients.Results Sixty-four patients were followed up from 1990 to 2005 over a period ranging from 0.6 to 15 years(mean 5.8 years).The outcomes were excellent in 46,good in 9,poor in 1 case,and 8 deaths.During the follow-up period,recurrent bleeding occurred in 10 cases.The interval to recurrent bleeding ranged from 0.3～8 years.The rate of recurrent bleeding was 12%.Two patients died of the initial intracranial hemorrhage,The rate of death was 2.4%.Six patients died of recurrent bleeding.The mortality of which was 60%.Conclusion Intracerebral haemorrhage is the important cause of death in patients with moyamoya disease,and recurrent bleeding is the most common cause of death in patients with the haemorrhagic type moyamoya disease.Long-term preventive measures against recurrent bleeding is necessary and a long-term prospective study with a large number of patients with haemorrhagic moyamoya disease is required to determine whether bypass surgery will prevent recurrent bleeding of haemorrhagic type moyamoya disease.