Download - 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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