Youmans Neurological Surgery

1. Moyamoya disease
&
Adult Moyamoya disease
Youmans chapter 207&356

2. Outline
• Introduction
• Epidemiology
• Pathophysiology and etiology
• Clinical finding
• Neuroimaging
• Treatment

3. Introduction
• Moyamoya disease (MDD)
• Rare
• Stenosis-occlusion of bilateral ICAs at their terminal
portion  development peculiar moyamoya vascular at
the base of brain
• Moyamoya syndrome : unilateral ICA occlusion
• Moyamoya : distinctive finding on arteriography
• Adult : cerebral hemorrhage
• Children : cerebral ischemia, 50% in 10 years old
• Etiology unknown

4. Introduction
• 1957 Takeuchi and Shizimu
man, 29 Years old, visual disturbance since
10 Yrs, hemiconvulsive since 13 years old
angiogram : bilateral ICAs
• 1969 Suzuki and Takaku
Term Moyamoya (puff of smoke)

5. Epidemiology
• Japan, Asian, Non-asian

6. Association condition in pedriatic

7. Pathophysiology and etiology
• Not arteriosclerotic or inflammatory change
• diagnosis of MMD
– fibrocellular thickening of the intima
– irregular disruption of the internal elastic lamina
– attenuation of the media
• Pluripotent peptides and their receptors
– basic fibroblast growth factor,transforming growth
factor-,hepatocyte growth factor increased
– angiogenesis and intimal hyperplasia

8. • G internal hyperplasia
• I internal elastic
lamina disruption
• H,J : control

9. Clinical finding

10. Clinical finding
• Infarction : watershed and PCA territory
– Frontal and temporal lobe
– Hemiparesis, dysarthria, aphasia, cognitive impairment
– Seizure
– Pedriatric : mistaken for psychiatric illness or developmental
delay
– Pedriatric : TIA parcipitate by hyperventilation with
crying,exertion,dehydration,cold or fever
– Pedriatic with cerebral ischemia should be consider as a
possible Moyamota patient until prove other wise

11. Clinical finding
• Hemorrhage : intraventricular, periventricular,
intraparenchymal
– Hall mark of adult MMD
– rupture of dilated and stressed perforating arteries containing
microaneurysms,
– fibrinoid necrosis of the arterial wall in the basal ganglia
– rupture of microaneurysms in the periventricular region,
especially around the superolateral wall of the lateral ventricles
• Headache in pedriatic
• Pregnancy and delivery may increase risk for ischemic
or hemorrhagic stroke in female patients
• .

12. Clinical finding
• Saccular aneurysm
• 60% around the circle of Willis, mainly at the vertebrobasilar
territory
• 20% in peripheral arteries, such as the posterior and anterior
choroidal arteries
• 20% in the abnormal moyamoya vasculature as mentioned
earlier
• May disappear or need to surgically of repeated bleeding

13. Neuroimaging
• Cerebral angiography
– Plan of surgery
• Classification of Suzuki and Takaku
– 1) narrowing of the carotid fork
– (2) initiation of the moyamoya
– (3) intensification of the moyamoya
– (4) minimization of the moyamoya
– (5) reduction of the moyamoya
– (6) disappearance of the moyamoya

14. • Basal moyamoya
– Basal ganglia
– Thalamus
– Lenticulostriate
– Anterior choroidal artery
– Posterior choroidal artery
• Ethmoidal moyamoya
– Anterior or posterior
ethmoidal a form
Opthalmic artery
• Vault moyamoya
– Dural arteries

15. • MRI & MRA
noninvasive
• A-C : 1.5 T MRA
• B-D : 3 T MRA
Neuroimaging

16. T1 : sensitive for detect basal moyamoya
T2 : detect microbleeding 15-44%

17. Ivy sign : Leptomeningeal metastases, subarachnoid hemorrhage (SAH),
meningitis, increased inspired oxygen

18. -Xenon-enhanced computed tomography, single-photon emission computed
tomography, and positron emission tomography (PET) can be used to measure
regional CBF and metabolic distribution
- a : impair hemodynamic reserve on loading with acetazolamide
- b : postoperative improve of ACA and MCA

19. Treatment in adult
• Non-operative management
– ASA
– Calcium antagonist : empirical headache
– Steroid : involuntary movement or at the time of frequent TIA
• Surgical management : augment impair CBF
– Direct revascularization with microvascular extracranial to
intracranial(EC-IC) by pass,prefer to adult
– Indirect revascularization without microvascular anastomotic
procedure,prefer to children

20. Direct revascularization procesure using a
microvascualr technique for STA-MCA bypass
• 1967 Donaghy and Yasargil
• Donor vessel, locate by dopple sonography
– Parital, less often frontal branch
– 1 mm in diameter, 8-10 cm, free preparation
• Craniotomy
– Small, 2.5-3 cm in diameter
– Center about 6 cm above EAM(end of sylvian fissure )
• MCA
– Posterior temporal,posterior parietal a.
– 10-0,11-0 8-10 interrupted
• Advantage
– Selective supplying territories of ischemia

21. Indirect bypass techniques
• Mobilizing vascularized tissue supplied by the ECA and
placing it in contact to the brain
• Encephalomyosynangiosis(EMS)
– 1970, Karasawa
– Inappropriate cortical branch of MCA,especially children
– Gradual revascularization
– Implanting temporalis m on brain surface, secure to dura edge

22. Indirect bypass techniques
• Encephaloduroarteriosynangiosis(EDAS)
– 1979, Matsushima
– Prefer technique
– Parietal brach of STA with preservation of vascular flow
– Dissected STA is laid onto the cortical surface after having
opened the arachnoidea

23. Perioperative management
• Pt in stable clinical condition without frequent ischemic
episode
• Sufficient hydration to patient
• Normocapnia during surgery
• Preoperative evaluation of hemodynamic dysfunction
with acetazolamide with caution and surgery
perform(after 48 hrs)

24. Prognosis
• 75-80%, benign course interm of life, with or without
surgical
• After revascularization
– free of TIA and ischemic stroke
– Rebleeding during FU 30-65%
• MRI and MRA detect asymptomatic pt
• Unilateral MDD
– 7-27% progress to bilateral

25. Moyamoya disease
• What is MMD?
• What is most common symptom of MMD?
• What is pathology of MMD vessel?
• How to augmentation by surgical method for
MMD?
• How to preparation patient before operation?