MOYAMOYA DESEASE
8 yr old female patient had history of recurrent seizures and developed aphasia and                quadriparesis after a s...
8 yr old female patient had history of recurrent seizures and developed aphasia and                quadriparesis after a s...
8 yr old female patient had history of recurrent seizures and developed aphasia and                quadriparesis after a s...
8 yr old female patient had history of recurrent seizures and developed aphasia and                quadriparesis after a s...
MOYAMOYAIs a progressive disease of theinternal carotid arterycharacterized by a constrictionof this vessel caused by inti...
MOYAMOYAIs a progressive disease of theinternal carotid arterycharacterized by a constrictionof this vessel caused by inti...
MOYAMOYAIs a progressive disease of theinternal carotid arterycharacterized by a constrictionof this vessel caused by inti...
MOYAMOYAIs a progressive disease of theinternal carotid arterycharacterized by a constrictionof this vessel caused by inti...
CAUSES....Hereditary   linked to chromosome 17                                                NeurofibromatosisSecondary   ...
CAUSES....Hereditary   linked to chromosome 17                                                NeurofibromatosisSecondary   ...
CAUSES....Hereditary   linked to chromosome 17                                                NeurofibromatosisSecondary   ...
CAUSES....Hereditary   linked to chromosome 17                                                NeurofibromatosisSecondary   ...
PATHOPHYSIOLOGY...Normal flow.                      Intima’ s overgrowth and clots                            cause occlusi...
OCCLUTIONS AND CLOTS CAUSE:Children:                                                          Adults:transient ischemic at...
DIAGNOSIS             It is suggested by:CT                                   MRIAngiogram
TREATMENTSDirectSTA-MCA bybassIndirectEDAS (encephaloduroarteriosynangiosis)                 EMS (enchephalomyosynangiosis...
STA-MCA BYPASS: GOLD STANDARD                                                      STEP 1 STEP 1: the patient is positione...
STEP 4STEP 4: a craniotomy is performed in the  midfrontal- temporal bone overlying           the Sylvian fessure.STEP 5: ...
STEP 8STEP 7: dissection of arachnoid exposes this vessel.STEP 8: a temporary clip is placed on the proximal          and ...
COMPLICATIONSSTA-MCA bypass specific:  ischemic changes  hypertension  CSF leak                              Non specific:  ...
PROGNOSISIf untreated 73% of people willdevelope major deficit or death   in 2 years after diagnosis.
CONCLUSIONS            Moyamoya needs to be treated.        STA-MCA bypass: treatment of choice.EDAS, EMS, multiple burr h...
THANK YOU!!
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  • marta salis skullbase

    1. 1. MOYAMOYA DESEASE
    2. 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. 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. 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. 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. 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. 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. 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. 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. 10. CAUSES....Hereditary linked to chromosome 17 NeurofibromatosisSecondary to other pathologies Down syndrome Sickle cell diseaseIdiopathic unknown ...& EPIDEMIOLOGY Source: Stanford Moyamoya Center
    11. 11. CAUSES....Hereditary linked to chromosome 17 NeurofibromatosisSecondary to other pathologies Down syndrome Sickle cell diseaseIdiopathic unknown ...& EPIDEMIOLOGY Source: Stanford Moyamoya Center
    12. 12. CAUSES....Hereditary linked to chromosome 17 NeurofibromatosisSecondary to other pathologies Down syndrome Sickle cell diseaseIdiopathic unknown ...& EPIDEMIOLOGY Source: Stanford Moyamoya Center
    13. 13. CAUSES....Hereditary linked to chromosome 17 NeurofibromatosisSecondary to other pathologies Down syndrome Sickle cell diseaseIdiopathic unknown ...& EPIDEMIOLOGY Source: Stanford Moyamoya Center
    14. 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. 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. 16. DIAGNOSIS It is suggested by:CT MRIAngiogram
    17. 17. TREATMENTSDirectSTA-MCA bybassIndirectEDAS (encephaloduroarteriosynangiosis) EMS (enchephalomyosynangiosis) Multiple burr holes
    18. 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. 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. 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. 21. COMPLICATIONSSTA-MCA bypass specific: ischemic changes hypertension CSF leak Non specific: myocardical infarction pneumonia deep venous trombosis pulmonary emboli
    22. 22. PROGNOSISIf untreated 73% of people willdevelope major deficit or death in 2 years after diagnosis.
    23. 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. 24. THANK YOU!!

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