Paediatric neurovascular malformations: classification and management

1. VIPUL GUPTA
NEUROINTERVENTION SURGERY, MEDANTA THE MEDICITY

2. 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

3. 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.

4. Intracranial arteriovenous
shunts
• PIAL AVM
• VEIN OF GALEN MALFORMATIONS
• PIAL NON GALENIC AVF
• DURAL AV SHUNT

5. OTHERS VASCULAR MALFORMATION
• PROLIFERATIVE ANGIOPATHY
• CAMS
• DVA
• SINUS PERICRANII
• CAPILLARY TELANGIECTASIA
• CAVERNOUS ANGIOMA

6. 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%).

7. •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

8. Artery
• Flow-related aneurysms, Number of feeder, Type of feeder
(direct vs en passage)
Nidus
• Number of compartments, Intranidal aneurysms, Fistulous
versus nidal
Veins
• 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.

9. AVM- treatment options
• Embolization
• Radiosurgery (Gamma Knife, LINAC,
Cyberknife)
• Surgery
Embolization
Glue (NBCA) vs Onyx embolization

10. Risk of haemorrhage in AVMs
Previous hemorrhage (10-15% in 1st
yr.,
otherwise 2-4% every year)
Periventricular/intraventricular location
Deep location- basal ganglia/thalamus
Arterial/intranidal aneurysms
Deep venous drainage
Single venous outlet
Venous restriction/stenosis
Delay in venous drainage
Feeder from perforator or VB system

11. 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

12. • Curative embolization with:
Acrylic glue
Onyx
• Complete cure rate (10-40 % )
 Single pedicle
small lesion with a terminal feeder

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

16. Combined endovascular & gamma therapy

17. 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

18. 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%

19. 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

20. FISTULAS

21. 9 year old girl with seizures

24. DURAL ARTERIOVENOUS SHUNTS
IN CHILDREN
THREE SUBTYPES :
Dural sinus malformation (DSM)
Infantile DAVS
Adult DAVS

25. 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

27. dural

29. 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.

30. 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

31. 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

32. 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 MORTALITY
RADIOSUGERY : NOT WELL DESCRIBED

36. 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

37. 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
THERAPYA B

38. • 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

39. 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

40. 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

41. – 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

42. 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
–

43. • 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

44. • 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
•

45. VGAM
LVA- PRE EMB. LVA- POST EMB.
2yr, F: failure to thrive, enlarging head

46. Newborn with cardiac failure – indication for urgent treatment

48. 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 .

49. Pathology
Histologically
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

50. 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
•

53. • 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

54. 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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56. Thank You