1、Outcome in adult patients with hemorrhagic moyamoya disease after combined extracranial-intracranial bypassTransit ischemic attack 46%Infarction 20%Hemorrhage 21%Headache 6%Epilepsy 4%Manifestation distributionH.Hoshino,Neurol Med Chir,2012Only one peak in Hemorrhagic MMD25-50 yrsM Scott,et al.N Eng
2、l J Med 2009E Kobayashi,et al.J Neurosurg,2000 H-W Nah,Stroke,2012Characteristic of MMD-ICH Ryan R W,et al.Surg Neurol Int.2012Postulated pathophysiologyDilated fragile moyamoya perforatorsDevelopment of microaneurysms Dilated branches originated from AChA or PComADilated fragile moyamoya perforator
3、s T Iwama,et al.J Clin Neurosci.1997Development of microaneurysms Ni W,et al.J Clin Neurosci.2012Dilated branches originated from AChA or PComA M Morioka,et al.Stroke.2003Modified Morika Grading SystemGrade 1:normal or slight-moderate dilation of AChA-PComA(with stenosed or occluded ICA and prolifer
4、ation around the Willis circle)(Fig A-B)Grade 2:extreme dilation of AChA-PComA(with abnormal branches beyond the choroidal fissure,serving as collateral blood supply to the anterior circulation via posterior pericallosal arteries and/or leptomeningeal collateral vessels)(Fig C)Grade 3:nonvisualizati
5、on of AChA-PComA on DSA(with occluded ICA proximal to the PComA level)(Fig D)Dilated branches originated from AChA or PComA M Morioka,et al.Stroke.2003Dilated branches originated from AChA or PComA WH Liu,et al.J Clin Neurosci.2011Medication vs.Surgery Yasagril and Donaghy(1967)Year/AuthorsNationObj
6、ectsSurgical optionConclusion2000,KawaguchiJapan22 patients11-surgery11-medication6-bypass5-EDASThe efficacy of revascularization is confirmed2004,KarasawaJapan99 patients77-surgery22-medicationSTA-MCA bypass/EMS/EDASRehemorrhage was small when the blood flow was improved in the surgical group2011,A
7、hnKorea13 pediatric patientsIndirect bypassHave a role for prevention of rebleeding and cerebral infarction 2012,LeeKorea53 patients35-surgery18-medicationindirect/direct/combined bypassNo clear evidence that revascularization prevents rebleeding in adult MMD patients2012,Bao et alChina126 patientsE
8、DASThe efficacy of EDAS for Hemorrhagic MMD is unclear2012,Liu et alChina97 patients54-surgery43-medicationSTA-MCA bypass+EDASBypass have greater efficacy at preventing rebleeding than conservative treatmentSurgical management of hemorrhagic MMDInclusion criteriaangiographically verified MMDhemorrha
9、gic attack identified by CT18-75 year agepreoperative mRs2 from one month to one year after the latest bleeding exclusion criteriawith ruptured aneurysm in the main stem of Willisother concurrent intracranial lesions life expectancy 1 yearProspective cohort studyEndpoint assessmentpPrimary end point
10、combined total of all strokes and death from surgery within 30 days after the procedurerecurrent hemorrhage afterwardpSecondary end point rebleeding on the contralateral sideischemic stroke beyond 30 daysTIA in the operative side at any timeTable1.Baseline demographics(n=113)Mean age,y37.39.46(21-58
11、).Female patients,n65Male patients,n48Type of first bleedingIVH63ICH14ICH with IVH36Total bleeding episodes1972133241Table 2.Lesion characteristics based on Suzuki grading system(n=113)CriteriaDescriptionNo.Suzuki stageINarrowing of the internal carotid artery bifurcation without collaterals0IIIniti
12、ation of moyamoya collaterals9IIIIntensification of moyamoya collaterals51IVMinimization of moyamoya collaterals30VReduction of moyamoya collaterals23VIDisappearance of moyamoya collaterals0Table 3.Distribution of AchA-PCoA extension in 113 casesNoModified Morika Grading123Hemorrhagic hemispheres113
13、39740Non-hemorrhagic hemispheres11372410STA-MCA BYPASS+ENCEPHALO-DURO-MYO SYNAGIOSISSurgical result114 surgeries performeddouble bypass performed in 60 hemispheres single bypass performed in 54 hemispheres100%patency rate by ICG 1 asymptomatic epidural hematoma How to follow up7 lost FU106 clinical
14、FU(30.33 m,16-72)106 angiographic FUModified Rankin ScaleNo of patients0671242831405264Clinical outcome for operated patients(n=106)Angiographic assessmentBypass patencyDecreased in moyamoya vesselsModified Morika Grading SystemTransdural and transpial collateralsMatsushima criteriaNo ischemic or he
15、morrhagic stroke within 30 days Five Ipsilateral rebleeding Four died of the rebleeding eventThe cumulative probability:0%at 1 year and 1.9%at 2 yearAnnual rebleeding rate:1.87%according to the person-year methodCase 1F，28 y.o sudden headache with vomitingMoyamoya with ANR ECACTA DSA:Bypass was pate
16、ntAN disappeared,moyamoya vessel decreasedPRE-BYPASSPOST-BYPASSCase 2F,52 y.o sudden headache with right-sided numbness and weaknessDSA：L-ICAL-ECAR-ECAL-STA-MCA by-pass+EDMS(2009)CT-post operation CT-2010.6.8L ECAFollowing-up DSAR ICAR ECAR-STA-MCA by-pass+EDMSCase 3M,17 y.o sudden headache with nau
17、sea and vomiting 1 month agoIntraoperative imagePostoperative CTA5 mo-DSA FU11 mo-DSA FUPRE5 mo11 moChanges in Moyamoya vesselsPRE5 mo11 moChanges in Moyamoya vessels2012-8-302012-8-30 3rd FUConclusions1.IVH is most common in hemorrhagic moyamoya disease,Suzuki grade mainly III-V stage.2.Dilated fra
18、gile moyamoya perforators、development of microaneurysms and dilated branches originated from AChA or PComA are considered to be the postulated pathophysiologies.3.Successful combined extracranial-intracranial bypass surgery can decrease the rebleeding rate(from 7.09%to 1.87%/person/year)4.For the angiographically aggravated cases,the preventive bypass surgery before rebleeding might be necessary.
