关键词: 颅内出血, 脑室出血, 烟雾病病, 脑血管造影

Abstract: 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.

Key words: intraventricular haemorrhage, intracerebral haemorrhage, moyamoya disease, cerebral angiography