Moyamoya disease is a rare cerebrovascular disorder caused by blocked arteries in the basal ganglia region of the brain. This leads to the formation of tiny collateral vessels that resemble a "puff of smoke", giving the disease its name. Vein of Galen malformations are also rare abnormalities where arteries in the brain connect directly to veins instead of capillaries, causing high pressure blood flow into the veins. Both conditions can cause symptoms like headaches, seizures, weakness and cognitive decline. Diagnosis involves MRI, angiogram or CTA imaging. Treatment may include medication, surgery such as cerebral bypass, or endovascular procedures to occlude arterial feeders or veins.

1. MOYAMOYA DISEASE
AND
VEIN OF GALEN MALFORMATION

2. Moyamoya disease is a rare, progressive
cerebrovascular disorder caused by blocked
arteries at the base of brain in the area called
basal ganglia.
The moyamoya means “puff of smoke” in
Japanese & look of tiny vessels formed to
compensate for blockage and it usually
affected both side of brain.
MOYAMOYA DISEASE

4. Narrowing of
internal
carotid
arteries
Origin of
moyamoya
vessels at the
base of the
brain
Increased
collateral
vessels from
the scalp
Complete
blockage
of internal
carotid
arteries
Narrowing of
internal
carotid artery
PROGRESSION OF THE DISEASE

6. Headache
Seizures
Weakness, numbness or paralysis in your face
typically on one side of body
Visual disturbance
Aphasia (difficulty in speaking)
Developmental delay
Involuntary movement
Cognitive decline
SYMPTOMS

7. oThe exact cause is unknown and inheritance.
oIt occurs in patients with neurofibromatosis
type 1, sickle cell disease and Down’s
syndrome.
oIt may also occur patients who undergone
radiation or radiotherapy to head and neck
such as pituitary tumors and
craniopharyngiomas.
CAUSES

8.  MRI – is performed by injecting a contrast
agent into the blood stream so the arteries
of the brain can be seen.
 Angiogram.
 CTA – this type of test show the picture of
both blood vessels (angiography) and soft
tissues (computed tomography).
diagnosis

9. medication:
Those experiencing
stroke and TIA, medication such
as aspirin reduce the risk of
stroke.
treatment

10. Is generally recommended for moyamoya
patients with recurrent or progressive TIAs
or stroke.
SURGICAL TREATMENT

11. Procedure is a direct method of
revascularization.
 The most common bypass procedure, the
STA-MCA (superficial, temporal artery to
middle cerebral artery) may achieve
instant improvement in blood flow.
Cerebral bypass

12.  A vein of galen malformation (VOGM) is a
type of rare blood vessel abnormality inside
the brain.
 In VOGM misshapen arteries in the brain
connect directly with veins instead of
connecting with capillaries which help slow
blood flow.
 It cause a rush of high pressure blood into
the veins.
Vein of galen malformation

15. Pure internal fistula
Fistula between thalamoperforators
and vein of galen
Mixed form (most common type)
types

16. Refusal
of feed
Respirator
y distress
Hydroce
phalus
sunset
sign
Cyanosis
Cardiac
failure
Seizures
Clinical features

17. They have multiple fistulas, up to 25%
of their cardiac output passes through
the fistulas causing high output
congestive cardiac failure.
It depends on size of shunt, adequacy
of venous drainage, complexity of
arterial supply.
neonates

18. It usually have single fistula with a
smaller shunt.
They present with macrocephaly or with
hydrocephaly.
Cerebral venous hypertension may also
present with delayed milestone.
Infants & children

19. It usually have low flow fistula.
They present with headache and
seizures.
Subarachnoid haemorrhage and
intracerebral haemorrhage can occur in
this scenario.
Older children's & adults

20. Ultrasound
• Pulsatile flow it helps in differentiating VOGMs
from other midline cystic lesions.
• In antenatal ultrasonography the hydrocephalus
and cardiac dysfunction can also be obtained.
• In postnatal Doppler ultrasonography can be
used to demonstrate the hemodynamic changes
associated with the malformation.
diagnosis

21. CT
COUMPUTED
TOMOGRAP
HY
•CT scan of brain usually demonstrates
a well defined multilobulated,
intensely enhancing lesion.
•Dilatation of the ventricular system,
periventricular white matter
hypodensities, cerebral atrophy are
common.

22. MRI
•Location of fistula,
presence of any nidus,
venous sac & venous
drainage can demonstrate
and thrombosis also
depicted on MRI.

23. ANGIOGRAPHY
•It remains the gold standard
for the evaluation of VOGMs.
•It demonstrate small feeders
supplying of fistula, venous
drainage of normal brain &
arteriovenous shunt.

24. SURGERY ?
Similarly ventricular shunting may
worsen the cerebral venous hypertension
and should be avoided before elimination of
the arteriovenous shunt. This procedure is
not tolerated by infants.
TREATMENT

25. The timing of endovascular management is
determined by the clinical presentation.
Venous and arterial embolization is possible,
depending on the number of feeders, and
controversy persists in regards to the
optimal approach. Options include:
> arterial feeder & fistula occlusion.
> transtorcular or transverse
embolization of the dilated vein.
endovascular