Outcome Of Revascularization And Conservative Treatment On Hemorrhagic Moyamoya Disease

Part1 Long-term follow-up of adult hemorrhagic moyamoya disease between surgical and conservative treatmentObjective:Moyamoya disease is a progressive,unique cerebrovascular disease characterized by bilateral stenosis and occlusion of the terminal internal carotid artery and abnormal collateral vessels develop at the base of brain,leading to "puff of smoke"appearance on cerebral angiography,called Moyamoya disease in Japanese.Hemorrhage and ischemia are two main manifestations of Moyamoya disease.There is no consensus on treatment of hemorrhagic moyamoya disease.The purpose of this study is to investigate the long-term outcomes between cerebro-dural-arterio-synangiosis（EDAS）and conservative treatment of hemorrhagic moyamoya disease and to analyze the risk factors for rebleeding event.Methods:A total of 112 consecutive hemorrhagic moyamoya patients（86 surgically treated cases and 26 conservatively treated cases）were recruited in this study.The characteristics of the digital subtraction angiography（DSA）,clinical chart and outcomes were analyzed.Kaplan-Meier survival analysis and COX proportional hazard regression models were used to estimate the risk factors for rebleeding.Results:The average age of hemorrhage onset was 37.7±8.5 years.The ratio of male to female was 1:1.07.70 of the 112（62.5%）hemorrhagic hemispheres developed an extremely dilated anterior choroidal artery or posterior communicating artery（AChAPComA）whereas 29 hemispheres with dilated AChA-PCom A in nonhemorrhagic group.Fourteen of 86 cases（16.3%）suffered rebleeding in surgical group and 8 of 46 cases（30.8%）in conservative group.COX regression analysis revealed that the age of hemorrhage onset（OR 1.087 95%CI 1.019-1.161,P=0.012）and conservative treatment（OR 0.313 95%CI 0.104-0.949,P=0.040）were correlated with rebleeding strokes.Conclusion:The ED AS procedure can effectively reduce the chance of rebleeding event than conservative treatment.And age of hemorrhage onset is also a risk factor for rebleeding event.Dilation of the AChA-PComA and the collateral vessels derived from them are associated with the bleeding of MMD.Part2 The long-term follow-up of spontaneous collaterals after the indirct bypass of pediatric moyamoya diseaseObjective:To explore the changes of spontaneous compensatory vessels in children with moyamoya disease after encephalo-duro-arterio-synangiosis（EDAS）procedure to adulthood.Methods:A total of fifteen pediatric patients with more than 10 years angiography-based follow-up after the encephalo-duro-arterio-synangiosis（EDAS）from Jan 2003 to Aug 2009 were analyzed.Digital subtraction angiography（DSA）and dynamic susceptibility contrast perfusion weighted imaging（DSC-PWI）was conducted to evaluate the efficacy of EDAS procedure.Matsushima grades were divided into two groups（Good group,representing Matsushima stage A;Mild group representing Matsushima stages B and C）to investigate the relationship between Matsushima grades and spontaneous compensatory vessels derived from anterior choroidal artery（AChA）and posterior communicating artery（PComA）.Results:Eighteen hemispheres showed the dilation of the AChA with/without spontaneous compensatory vessels preoperatively.In the Good Matsushima grade group,1 case had no significant change and 9 cases were ameliorated.In the Mild Matsushima stage group,7 cases had no significant change and lcase was ameliorated.In the ten patients with dilation of the PComA with/without spontaneous compensatory branches in 12 hemispheres preoperatively.Three cases were ameliorated and three cases had no change in the Good Matsushima group,whereas two cases were ameliorated and four cases had no significant change in the Mild group.The Good Matsushima group showed greater amelioration in the AChA than the Mild group,indicating that the Good Matsushima group had a significant positive correlation with diminished AChA.Leptomeningeal collateral from the PCA appeared in all 32 hemispheres supplying the anterior cerebral circulation on preoperative angiography,and decreased in 24 sides of the 14 patients during the follow-up.Cerebral perfusion of all cases have improved through the DSC-PWI.There is No bleeding event during the follow-up period.Conclusion:The EDAS revascularization can recede the dilation and abnormal collateral vessels of AChA,which may reduce the incidence of further rebleeding events.Part3 The relationship between the stage progression and neoangiogenesis after encephaloduroarteriosynangiosis for moyamoya diseaseObjective To investigate the relationship between the stage progression and long-term progression of neoangiogenesis after encephaloduroarteriosynangiosis（EDAS）for moyamoya disease（MMD）.Methods A total of 78 patients who were diagnosed moyamoya disease and received EDAS procedure at the Department of Neurosurgery in the Fifth Medical Center of Chinese PLA General Hospital from December 2002 through September 2009 were enrolled.Patients who received postoperative digital subtraction angiography（DSA）at least twice which short-term（6-12 months）and long-term（follow-up periods≥8 years）follow-up angiographies was performed.The efficacy of EDAS was graded according to the description by Matsushima grade system.Results The average age at the first episode was 26.9 ± 15.0 years.The Matsushima grades in the long-term group was higher than that in the short-term group and there was no reduction in either side of the Matsushima grade in the long-term follow-up group.Disease advanced occurred in 51 hemispheres（32.7%）in the short-term group and in 26 hemispheres（16.6%）in the long-term group.Ipsilateral Suzuki stage was negatively correlated with the disease advanced.In addition,higher Suzuki stage was significantly correlated with Matsushima grade in both short-term and long-term follow-up groups.Stroke occurred in 9 of 78 patients（11.5%）during long-term follow-up.The annual recurrent stroke rate in the advanced group was significantly higher than that in the stable group.Conclusion:In patients with moyamoya disease,the efficacy of angiogenesis after indirect revascularization improves with time.The short-term progression of the Internal carotid artery（ICA）may be related to revascularization procedure,while the long-term progression is related to the natural progression of the disease.