paediatric neurovascular malformations

VIPUL GUPTANEUROINTERVENTION SURGERY, MEDANTA THE MEDICITY

Paediatric vascular malformations…•Understanding of a disease precede its treatment•Children are not small adult.•Pediatric vascular malformation differ significantly from the adults

HOW IS IT DIFFERENT ?•Cerebral eloquence difficult to assess.•Multifocality and Multiple fistulas •Drainage affects usually the entire venous system•Different Anatomic and Physiological characteristics

ETIOLOGICAL CLASSIFICATION

Krings T, Geibprasert S, Terbrugge K. Classification and endovascular management of pediatric cerebral vascular malformations. Neurosurg Clin N Am. 2010 Jul;21(3):463-82.

Intracranial arteriovenous shunts

• PIAL AVM

• VEIN OF GALEN MALFORMATIONS

• PIAL NON GALENIC AVF

• DURAL AV SHUNT

OTHERS VASCULAR MALFORMATION

• PROLIFERATIVE ANGIOPATHY• CAMS• DVA • SINUS PERICRANII• CAPILLARY TELANGIECTASIA• CAVERNOUS ANGIOMA

PIAL AVMS• ABNORMAL ARTERIOLAR CAPILLARY NETWORK THAT EXISTS BETWEEN

THE ARTERIAL AND VENOUS CIRCULATIONS. AVM commonest cause of hemorrhage in children (30-55%).

CHF and seizures other presentation. Neonates characteristically show signs of high-output

cardiac failure because intra- cranial arteriovenous shunting results in volume over- load to the right heart

Annual risk and rate of hemorrhage 2-4% (=adults ).But cumulative risk greater (more no. of yrs to live )

overall number of multifocal AVMs in children is twice that of adults (17.2% versus 9%).

•NCCT : Hematoma, hydrocephalus and mass effect.•CECT - serpiginous enhancements . ; 25% to

30% of cases calcificaations.

•MRI : On magnetic resonance imaging, AVMs are

seen as tightly-packed tubular tortuous flow voids on T1-

and T2-weighted sequences

•localisation , size , hemorrhage and post hemorrhagic changes , ischaemic changes

Artery • Flow-related aneurysms, Number of feeder, Type of feeder

(direct vs en passage)Nidus • Number of compartments, Intranidal aneurysms, Fistulous

versus nidalVeins • Stenoses, Number of draining veins per compartment

CTA and MRA : Angioarchitecture

DSA : Gold standard . Angioarchitecture and hemodynamic features and planning treatment

hypothesized that these venous variations are more common angioarchitectural features of pediatric pial AVMs since children often present with hydrovenous disorders, like macro- cephaly, hydrocephalus, and melting-brain syndrome.

AVM- treatment options• Embolization• Radiosurgery (Gamma Knife,

LINAC, Cyberknife)• Surgery

Embolization Glue (NBCA) vs Onyx embolization

Risk of haemorrhage in AVMsPrevious hemorrhage (10-15% in 1st yr., otherwise 2-4% every year)Periventricular/intraventricular locationDeep location- basal ganglia/thalamusArterial/intranidal aneurysmsDeep venous drainageSingle venous outletVenous restriction/stenosisDelay in venous drainageFeeder from perforator or VB system

AVM management…

• Small ruptured - Embolization/surgery first choice, RS – resiudal, no option

• Small unruptured – RS , embolization/surgery- weak spot

• Large – observe if unruptured; staged embolization followed by RS

• Curative embolization with:Acrylic glueOnyx

• Complete cure rate (10-40 % ) Single pedicle small lesion with a terminal feeder

Long term effects of SRS for the pediatric popu- lation is unknown and late recurrence after AVM obliteration with SRS has been reporte

Combined endovascular & gamma therapy

PIAL NON GALEN AVF • One or more pial arteries feed directly into

the cortical vein (Single/Multi hole fistula)• Without intervening nidus • Often large varix• Fistula in subpial meningeal space• No direct involvement of embryonic MPV

Pial AVFs - presentation

• neonates - congestive heart failure (CHF) was the most common

• infants - included, seizure (36%) and macrocephaly (33%).

• children - seizure (31%) was ; Hemorrhage, headache, and neurological deficits without hemorrhage were each found in 24%

ASSOCIATED SYNDROME:• HHT • Ehlers danlos syndrome• NF1 • Klippel trenaunay weber syndrome• Encephalocranioocutanous lipomatosis• Aortic corarctation

Prognosis with conservative management poor Treatment usually attempted unless already

evidence of significant brain damage.Tt – embolization – glue ; coil and glue, balloon

assisted glue/onyx

FISTULAS

9 year old girl with seizures

DURAL ARTERIOVENOUS SHUNTS IN CHILDREN

THREE SUBTYPES :

Dural sinus malformation (DSM)

Infantile DAVS

Adult DAVS

DSM

• GAINT POUCHES / LAKES OF DURAL VENNOUS SINUS

• Associated AV shunting • M.c involved in transverse, SSS and torcula.• Total cavernous capture – favorable features• Torcular involvement and pial, straight/SSS

reflux –unfavorable feature

dural

Infantile DAVS• Infantile DAVS are the most common dural arterio- venous

shunt reported in children • These are dural, high-flow shunts that occur in the absence of

a sinus malformation; these lesions can recruit pial fistulae as well.

• Due to high flow, neonates and infants may present with heart failure as an initial sign, while older children may present with symptoms related to global venous intracranial venous congestion: macrocrania, developmental delay, hydrocephalus and/ or seizures.

INFANTILE DAVS

• High flow shunt • Occur in absence of a sinus malformation • Recruit pial fistula also• Heart failure in neonates and infants• Increased intracranial venous congestion in

older children

Adult-type DAVS • Adult-type DAVS are acquired lesions which are typically low flow. • They may develop in the setting of sinus thrombosis, trauma, prior

infection or prior craniotomy• In contrast to adults, the most common location for this adult

subtype of DAVS in children is at the cavernous sinus, while sigmoid sinus lesions are rare

• Venous and/or intracranial hypertension or hemorrhage, with the best predictors for neurologic morbidity being the presence of leptomeningeal venous reflux and symptomaticity of the lesion

• Onyx embolization -Usually curative • Cavernous – transvenous- coils, onyx

TREATMENT:(ENDOVASCULAR MAIN STAY)For regression of secondarly induced pial

fistulae.And also prevent the progression ( more

complex, multifocal and aneurysm formation).ALSO ROLE OF PALLIATIVE ENDOVASCULAR

TREATMENT SURGERY : HIGH MORTALITYRADIOSUGERY : NOT WELL DESCRIBED

Proliferative Angiopathy• Nidus: Composed of multiple arteries as an angiogenetic

response to cortical ischemia

• Interspersed normal brain parenchyma between the abnormal

vessels

BA C

D E F

CEREBRAL ANGIOGRAPHY:• Arterial feeder vessels normal size or only moderately enlarged

• Stenoses of the feeder vessels

• Extensive transdural supply (ECA branches)

• Lack of clear early venous drainage

TREATMENT PIAL SYNANGIOSIS OR BURR-HOLE THERAPY A B

• Wyburn-Mason syndrome

• Multiple AVMs in both the brain parenchyma and the facial region.

• Symptoms m.c related to the facial AVMs.

CEREBROFACIAL ARTERIO-VENOUS METAMERIC SYNDROME

• CAMS1: Corpus callosum, hypothalamus

• CAMS2: Occipital lobe and optic tract,

including the thalamus, retina and

maxilla

• CAMS3: Cerebellum, pons, and

mandible

ANGIOGRAPHIC FINDINGS

• Diffuse or proliferative type nidus

• Multiple small perforator collateral vessels supply.

• Slow AV shunting into the veins

• ECA injection to detect for potential optic,

maxillofacial, or mandibular vascular malformations• Endovascular treatment : Best option in the emergency setting • Surgical resection (whenever possible) remains the treatment of

choice for facial AVMs Geibprasert S, Pongpech S,Jiarakongmun P, Shroff M, Armstrong D, Krings T. Radiologic Assessment of Brain Arteriovenous Malformations: What Clinicians Need to Know. RadioGraphics 2010; 30:483–501

VEIN OF GALEN ANEURYSMAL MALFORMATION (VGAM)

– AVF between deep choroidal arteries/median

prosencephalic vein

• Midline location in choroidal fissure at quadrigeminal

plate/velum interpositum cisterns

– Persistent median prosencephalic vein of Markowski

(MPV): Precursor of vein of Galen

• Vein becomes aneurysmal

• Drains via falcine sinus to superior sagittal sinus

– Clinical presentation of progressively worsening cardiac

failure leading to multiorgan failure, macrocephaly,

developmental delay, seizures etc…

– ENDOVASCULAR EMBOLISATION TREATMENT OF CHOICE- trans-

arterial glue, rarely transvenous coils

IMAGING• Antenatal USG: Identified in 3rd trimester of

pregnancy– MPV appears as mildly echogenic midline mass– Arterial feeders; turbulent Doppler flow in MPV

• NECT/ CECT–

• Magnetic resonance (MR)– T1WI/T2WI

• Flow voids in arterial feeding vessels• Flow void/mixed intensity in MPV from

fast/turbulent flow• T1 Hyperintense foci within pouch:

Thrombus• T1 Hyperintense foci within brain:

Hemorrhage (rare)• Ischemia poorly seen (unmyelinated

infant brain)

– DWI• Restriction in areas of acute

ischemia/infarction

• Digital subtraction angiography (DSA)– "Choroidal" or "mural" classification

• Choroidal: Multiple feeders from pericallosal/choroidal/thalamoperforating arteries

• Mural: Few feeders from uni- or bilateral collicular/posterior choroidal arteries

•

VGAM

LVA- PRE EMB. LVA- POST EMB.

2yr, F: failure to thrive, enlarging head

Newborn with cardiac failure – indication for urgent treatment

Developmental venous anomaly• Altered venous pathways• Associations- usually solitary (75%) , except in

blue rubber bleb naevus syndrome. • 8-33% of cases- ass. ith cavernous malformations mixed vascular

malformations • Association with venous malformations of the head and neck .

PathologyHistologically cavernous malformations are composed of a "mulberry-like" cluster of dilated thin-walled capillaries, with surrounding haemosiderin (unusually ass. With DVA, mives vasc. Mal.) – angiographically occult

Cavernomas

Capillary telangiectasia • They can account for up to ~ 20 % • Vast majority of - asymptomatic and discovered incidentally • They are comprised of dilated capillaries and are interspersed with normal brain

parenchyma with a thin endothelial lining but no vascular smooth muscle of elastic fiber lining.

• Most occur in the pons, cerebellum and spinal cord.• Associations- Osler-Weber-Rendu syndrome• Capillary telangiectasias are most are located within the brainstem and pontine

regions•

• Cranial venous anomaly - communication between intracranial dural sinuses and extracranial venous structures (via an emissary transosseous vein).

• low flow vascular malformation. • frequently involves the superior sagittal sinus.• prominent on supine position•

Sinus Pericranii

Conclusion

• Paediatric vascular malformations – varied pathologies

• Interventional neuroradiologist play a critical role in diagnosis and management

• Understanding pathophysiology and team work is crucial

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