An 8-year old female patient presented with a history of recurrent seizures since age 5 and developed aphasia and quadriparesis after a seizure. MRI showed chronic infarcts and angiogram revealed extensive basal collateralization resembling a "puff of smoke", consistent with Moyamoya disease. Moyamoya disease is a progressive narrowing of the carotid arteries due to intimal proliferation and involvement of the middle cerebral arteries. It can cause transient ischemic attacks, ischemic strokes, or hemorrhagic strokes in children and adults. Diagnosis is suggested by imaging studies and confirmed by angiogram. Treatments include direct revascularization techniques like STA-MCA bypass or indirect techniques like EDAS or EMS.

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 infarcts
Angiogram: 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 infarcts
Angiogram: 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 infarcts
Angiogram: 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 infarcts
Angiogram: extensive basal collateralization -
"puff of smoke"

6. MOYAMOYA
Is a progressive disease of the
internal carotid artery
characterized by a constriction
of this vessel caused by intimal
proliferation.
Also MCA and the anterior
cerebral artery can be envolved.

7. MOYAMOYA
Is a progressive disease of the
internal carotid artery
characterized by a constriction
of this vessel caused by intimal
proliferation.
Also MCA and the anterior
cerebral artery can be envolved.

8. MOYAMOYA
Is a progressive disease of the
internal carotid artery
characterized by a constriction
of this vessel caused by intimal
proliferation.
Also MCA and the anterior
cerebral artery can be envolved.

9. MOYAMOYA
Is a progressive disease of the
internal carotid artery
characterized by a constriction
of this vessel caused by intimal
proliferation.
Also MCA and the anterior
cerebral artery can be envolved.

10. CAUSES....
Hereditary linked to chromosome 17
Neurofibromatosis
Secondary to other pathologies Down syndrome
Sickle cell disease
Idiopathic unknown
...& EPIDEMIOLOGY
Source: Stanford Moyamoya Center

11. CAUSES....
Hereditary linked to chromosome 17
Neurofibromatosis
Secondary to other pathologies Down syndrome
Sickle cell disease
Idiopathic unknown
...& EPIDEMIOLOGY
Source: Stanford Moyamoya Center

12. CAUSES....
Hereditary linked to chromosome 17
Neurofibromatosis
Secondary to other pathologies Down syndrome
Sickle cell disease
Idiopathic unknown
...& EPIDEMIOLOGY
Source: Stanford Moyamoya Center

13. CAUSES....
Hereditary linked to chromosome 17
Neurofibromatosis
Secondary to other pathologies Down syndrome
Sickle cell disease
Idiopathic 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 hemorrhage
ischemic stroke ischemic stroke
SYMPTOMES...
headaches
progressive cognitive or learning impairment
weakness
...& SIGNS
hemiplegia hemichorea
paralysis

16. DIAGNOSIS
It is suggested by:
CT
MRI
Angiogram

17. TREATMENTS
Direct
STA-MCA bybass
Indirect
EDAS (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 4
STEP 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 8
STEP 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. COMPLICATIONS
STA-MCA bypass specific:
ischemic changes
hypertension
CSF leak
Non specific:
myocardical infarction
pneumonia
deep venous trombosis
pulmonary emboli

22. PROGNOSIS
If untreated 73% of people will
develope 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!!