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Transcript

  • 1. MOYAMOYA DESEASE
  • 2. 8 yr old female patient had history of recurrent seizures and developed aphasia and quadriparesis after a seizure episode, at the age of 5 years. MRI: areas of chronic infarctsAngiogram: extensive basal collateralization - "puff of smoke"
  • 3. 8 yr old female patient had history of recurrent seizures and developed aphasia and quadriparesis after a seizure episode, at the age of 5 years. MRI: areas of chronic infarctsAngiogram: extensive basal collateralization - "puff of smoke"
  • 4. 8 yr old female patient had history of recurrent seizures and developed aphasia and quadriparesis after a seizure episode, at the age of 5 years. MRI: areas of chronic infarctsAngiogram: extensive basal collateralization - "puff of smoke"
  • 5. 8 yr old female patient had history of recurrent seizures and developed aphasia and quadriparesis after a seizure episode, at the age of 5 years. MRI: areas of chronic infarctsAngiogram: extensive basal collateralization - "puff of smoke"
  • 6. MOYAMOYAIs a progressive disease of theinternal carotid arterycharacterized by a constrictionof this vessel caused by intimalproliferation.Also MCA and the anteriorcerebral artery can be envolved.
  • 7. MOYAMOYAIs a progressive disease of theinternal carotid arterycharacterized by a constrictionof this vessel caused by intimalproliferation.Also MCA and the anteriorcerebral artery can be envolved.
  • 8. MOYAMOYAIs a progressive disease of theinternal carotid arterycharacterized by a constrictionof this vessel caused by intimalproliferation.Also MCA and the anteriorcerebral artery can be envolved.
  • 9. MOYAMOYAIs a progressive disease of theinternal carotid arterycharacterized by a constrictionof this vessel caused by intimalproliferation.Also MCA and the anteriorcerebral artery can be envolved.
  • 10. CAUSES....Hereditary linked to chromosome 17 NeurofibromatosisSecondary to other pathologies Down syndrome Sickle cell diseaseIdiopathic unknown ...& EPIDEMIOLOGY Source: Stanford Moyamoya Center
  • 11. CAUSES....Hereditary linked to chromosome 17 NeurofibromatosisSecondary to other pathologies Down syndrome Sickle cell diseaseIdiopathic unknown ...& EPIDEMIOLOGY Source: Stanford Moyamoya Center
  • 12. CAUSES....Hereditary linked to chromosome 17 NeurofibromatosisSecondary to other pathologies Down syndrome Sickle cell diseaseIdiopathic unknown ...& EPIDEMIOLOGY Source: Stanford Moyamoya Center
  • 13. CAUSES....Hereditary linked to chromosome 17 NeurofibromatosisSecondary to other pathologies Down syndrome Sickle cell diseaseIdiopathic unknown ...& EPIDEMIOLOGY Source: Stanford Moyamoya Center
  • 14. PATHOPHYSIOLOGY...Normal flow. Intima’ s overgrowth and clots cause occlusions... ...which cause the development of a fine collateral circulation. On X-rays they have the appearance of a “puff of smoke (moyamoya in japanese).
  • 15. OCCLUTIONS AND CLOTS CAUSE:Children: Adults:transient ischemic attacks (TIAs) cerebral hemorrhageischemic stroke ischemic stroke SYMPTOMES... headaches progressive cognitive or learning impairment weakness ...& SIGNS hemiplegia hemichorea paralysis
  • 16. DIAGNOSIS It is suggested by:CT MRIAngiogram
  • 17. TREATMENTSDirectSTA-MCA bybassIndirectEDAS (encephaloduroarteriosynangiosis) EMS (enchephalomyosynangiosis) Multiple burr holes
  • 18. STA-MCA BYPASS: GOLD STANDARD STEP 1 STEP 1: the patient is positioned with the head turned to the controlateral side.STEP 3 STEP 2: a doppler sound is used to mark out the course of the STA. STEP 3: an incision in the scalp and STA is isolated.
  • 19. STEP 4STEP 4: a craniotomy is performed in the midfrontal- temporal bone overlying the Sylvian fessure.STEP 5: the dura is opened and recipient vessels are identified. STEP 5 STEP 6: the recipient MCA branch is chosen besed on: diameter location orientation
  • 20. STEP 8STEP 7: dissection of arachnoid exposes this vessel.STEP 8: a temporary clip is placed on the proximal and distal part of STA donor. The distal part is cut in an oblique manner. STEP 9: anastomosis is performed under neuromicroscope. Stiches are placed. STEP 10: temporary clips are removed. STEP 9
  • 21. COMPLICATIONSSTA-MCA bypass specific: ischemic changes hypertension CSF leak Non specific: myocardical infarction pneumonia deep venous trombosis pulmonary emboli
  • 22. PROGNOSISIf untreated 73% of people willdevelope major deficit or death in 2 years after diagnosis.
  • 23. CONCLUSIONS Moyamoya needs to be treated. STA-MCA bypass: treatment of choice.EDAS, EMS, multiple burr holes: only if STA-MCA bypass can’ t be performed.
  • 24. THANK YOU!!