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Twenty seven years Experience in theTwenty seven years Experience in the

Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)

The fourth annual

International Neurosurgery Conference27-30 December 2008

Quintana Leonidas , Massaro Paolo, Gonzalez Francisco, Yokota Patricio, Segovia Miguel

Department of Neurosurgery- Valparaso University - Faculty of Medicine

Chile

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ChronicChronic cerebrovascularcerebrovascularocclusiveocclusive

diseasedisease

Arteritis (Arteritis ( endarteritisendarteritis))

GeneticGenetic immunologicalimmunological basebase

NameName: cigarette smoke in the air: cigarette smoke in the air

MoyamoyaMoyamoya ininjapanesejapanese

Twenty seven years Experience in theTwenty seven years Experience in the

Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)

Jiro Suzuki

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StagesStages ofofprogressionprogression

(( SuzukisSuzukis ClassicationClassication:: ArchArch NeurolNeurol ,, VolVol 20, 28820, 288--299,1969)299,1969)

1

2

3

4

5

6

PROGRESSIVE STENOSIS OF INTRACRANIAL INTERNAL CAROTID ARTERIES , ACA & MCA

PROGRESSIVE APPEARANCE OF A MESH OF VESSELS OF SMALL DIAMETER IN THE REGION

OF BASAL GANGLIA, AS A FORM OF COLLATERAL CIRCULATION

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ISCHEMIC FORMISCHEMIC FORM

ChildsChilds

T.I.AT.I.A

InfarctionInfarction

CerebralCerebral atrophyatrophy

HEMORRHAGIC FORMHEMORRHAGIC FORM

YoungYoung adultadult

VentricularVentricularhemorrhagehemorrhage

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What is the meaning of moyamoya vessels?

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VASCULAR ENDOTHELIAL GROWTH FACTOR EXPRESSION AND ANGIOGENESIS

INDUCED BY CHRONIC CEREBRAL HYPOPERFUSION IN RAT BRAINJian Hai, M.D., Ph.D. Shi-Ting Li, M.D., Qi Lin, M.S., Qing-Gang Pan, M.S., Fei Gao, Ph.D., Mei-Xiu Ding, M.D.

Neurosurgery 53:963-972, 2003Conclusions: These results demonstrated that chronic cerebral hypoperfusion could

Induce sustained up-regulation of VEGF mRNA and protein expression in rat brain,

which was correlated with angiogenesis.

Enhanced brain angiogenesis in chronic cerebral hypoperfusion after

administration of plasmid human vascular endothelial growth factor in

combination with indirect vasoreconstructive surgeryNOBORU KUSAKA, M.D., KENJI SUGIU, M.D., KOJI TOKUNAGA, M.D.,ATSUSHI KATSUMATA, M.D., AYUMI NISHIDA, M.D.,

KATSUNARI NAMBA, M.D.,HIROFUMI HAMADA, M.D., HIROYUKI NAKASHIMA, M.D., AND ISAO DATE, M.D.

J Neurosurg 103:882890, 2005

Conclusions. In rat models of chronic cerebral hypoperfusion, administration of

phVEGF combined with indirect vasoreconstructive surgery significantly increased

capillary density in the brain. The authors results indicate that administration of

phVEGF may be an effective therapy in patients with chronic cerebral hypoperfusion,such as those with moyamoya disease.

Chronic ischemia Genetic mech. mRNA VEGF Angiogenesis

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ISCHEMIA

HYPOXIA

cell elected

ANGIOGENESIS

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15 cases ( 7 males, 8 females. Age : 6 46 y.o ;media : 25,4 y. old)

Name Age Gender StrokeType Moyamoya clinical pattern

R.A. 6 M Ischemic TypicalL.M. 8 M Ischemic Typical

J.N. 9 F Ischemic Typical

Y.M. 11 M Ischemic Typical

L.O. 15 M Ischemic Atypical (unilat)

F.G. 16 M Ischemic TypicalF.C. 16 F Ischemic Typical

Ischemic cases7 cases ; 6 typical , 1 atypical

Twenty seven years Experience in theTwenty seven years Experience in the

Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)

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15 cases ( 7 males, 8 females. Age : 6 46 y.o ;media : 25,4 y. old)

Name Age Gender StrokeType Moyamoya clinical pattern

J.G. 18 F Hemorrhagic Atypical

L.E. 25 F Hemorrhagic TypicalH.N. 36 M Hemorrhagic Atypical (unilat)

V.S. 37 F Hemorrhagic Atypical (unilat)

R.C. 42 F Hemorrhagic Atypical (unilat)

J.G. 46 F Hemorrhagic Atypical (unilat)F.M. 46 M Hemorrhagic Typical

C.R. 51 F Hemorrhagic Typical

Hemorrhagic cases

8 cases ; 3 typical , 5 atypical

Twenty seven years Experience in theTwenty seven years Experience in the

Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)

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Angiographical criteria to diagnose the Moyamoya Disease(Research committee on progressive occlusive disease of the circle of Willis,Ministry of

Health and Welfare of Japan 1978)

1-Stenosis or occlusions at the terminal portion of intracranial ICA

and/or the proximal portion of the ACA and/or the MCA.

2- Abnormal vascular mesh, the moyamoya vessels, observed inthe neighborhood of the above mentioned areas in the arterial

phase.

3- Above mentioned findings are recognized bilaterally.

We observed 6 atypical cases ( CUASI MOYAMOYA )

or Akim Moyamoya ( 40%)

4 cases Hemorrhagic Stroke unilaterally moyamoya vessels

1 case Hemorrhagic Stroke early occlusion of cervical ICA bilat.

1 case Ischemic Stroke unilaterally moyamoya vessels

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CASE N 6 J.G. 18 years old , female

Mild headache, left hemiparesis , with complete recovery.

AORTIC ARCH RIGHT CERVICAL LEFT CEREVICAL

ICA ICA

RIGHT ICA AP LEFT ICA AP VERTEBRAL

CASE N 2 J N 9 ld f l

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CASE N 2 J.N. , 9 years old, female

Difficult learning, speech disturbance, right progressive

hemiparesis, focal convulsions.

VERTEBRAL LAT. VERTEBRAL AP.

RIGHT ICA AP RIGHT ICA LATERAL LEFT ICA AP LEFT ICA LATERAL

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ENCEPHALO-DURO-ARTERIO-SINANGIOSIS ( EDAS )

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ENCEPHALO-DURO-ARTERIO-GALEO-SINANGIOSIS

BURR HOLES PERIOSTIAL -SINANGIOSIS1

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2 ENCEPHALO-DURO-ARTERIO-GALEO-SINANGIOSISBURR HOLES PERIOSTIAL -SINANGIOSIS

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ANGIOGRAPHICAL CONTROL ( 6-12 monthes )

RIGHT ICA LAT. PREOP. RIGHT ICA LATERAL RIGHT ICA LATERAL

POSTOP. 1 POSTOP. 2

LEFT ICA LAT. PREOP. LEFT ICA LATERAL LEFT ICA LATERAL

POSTOP. 1 POSTOP. 2

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SURGICAL TREATMENT

10 first operations (1982- 2000) : EDAS

5 last operations (2001- 2007) :EDAS + wide galeal flap

Encephalo-duro-galeo-

arterio- sinangiosis

+burr holes and periostialsinangiosis

MEDICAL TREATMENT

Vasodilators drugsCorticosteroids

Platelets antiaggregants

Nootropics drugs

NO UTILITY

DEMONSTRATED

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TABLE FOR FUNCTIONAL EVALUATION

I Return to a normal life

II Activity with mild limitations

III Activity with severe limitationsIV Vegetative state

V Died

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ANALYSIS OF FUNCTIONAL RESULTS

ACCORDING WITH THE INITIAL DAMAGE

Ischemic cases Hemorrhagic cases Grade

2 TIA 1 small ICH ; 1 IVH I

3 Infarction (2 temp;1 front.) 1 IVH; 1IVH+ lobar ICH II

1 Hemispheric Infarction 2 severe IVH

2 IVH + basal ganglia ICH III

1 Bilateral multiple infarctions IV

Initial clinical presentation and time of evolution

of Moyamoya Disease is strongly related

with the final outcome

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RESULTS AT 6 TO 12 MONTHS AFTER OPERATIONClnical results

I Return to normal life 4 cases

II Activity with mild limitations 5 cases

III Activity with severe limitations 5 casesIV Vegetative state 1 case

V Death 0 cases

Angiographical results

14/15 Presented signs of effective revascularization1/15 Not presented signs of effective revascularization

The revascularization signs were present between 6-12 months

after the operation, and the donnor artery with wider caliber

EXCELLENTEXCELLENT

OR GOODOR GOOD

BADBAD

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OUTCOMEFollow up between 2 27 years

I Return to a normal life 4 cases

II Activity with mild limitations 5 casesIII Activity with severe limitations 5 cases

IV Vegetative state 0 cases

V Died 1 cases

Related with:

1- Early Diagnosis

2- Effective treatment

EXCELLENTEXCELLENT

OR GOODOR GOOD

POORPOOR

CONCLUSIONS

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CONCLUSIONS

1- THE PATTERN OF MOYAMOYA DISEASE IS SOMEWHATDIFFERENT IN OUR EXPERIENCE, FROM THE JAPANESE

EXPERIENCE , WITH MANY CASES CATALOGUED AS CUASI

MOYA MOYA,PERHAPS WE SHOULD SAY WESTERN

MOYAMOYA.AND ITS MORE FREQUENTLY SEEN, IN OUR EXPERIENCE, IN THE

HEMORRHAGIC FORM OF MOYA MOYA DISEASE.

2- OUR EXPERIENCE SHOWS THAT THE MOST IMPORTANTPOINTS RELATED WITH THE CLINICAL RESULTS ARE THE

INITIAL NEUROLOGICAL DAMAGE, AND THE EARLY DIAGNOSIS

AND TREATMENT

3-WE EMPLOYED THE WIDELY USED TECHNIQUE OF EDAS, AND

LATER, EDAS PLUS WIDER GALEAL FLAP, AND BURR HOLES,

WITH GOOD ANGIOGRAPHICAL RESULTS AND RELATIVE GOOD

CLINICAL RESULTS.

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Twenty seven years Experience in theTwenty seven years Experience in the

Treatment of Moyamoya Disease (1982Treatment of Moyamoya Disease (1982--2007)2007)

The fourth annual

International Neurosurgery Conference27-30 December 2008

Quintana Leonidas , Massaro Paolo, Gonzalez Francisco, Yokota Patricio, Segovia Miguel

Department of Neurosurgery- Valparaso University - Faculty of Medicine

Chile

THANK YOU VERY MUCH !!!THANK YOU VERY MUCH !!!