II. Definitions

  1. Cerebral Arteriovenous Malformation
    1. AV Malformations (AVMs) are abnormal direct connections between arteries and veins, bypassing capillaries
    2. AVMs Results in dilated arteries at risk of bleeding, in this case in the brain resulting in Intracerebral Hemorrhage

III. Epidemiology

  1. Prevalence (unruptured and ruptured): 10 to 18 per 100,000 adults

IV. Findings: Presentations

  1. Asymptomatic in 85% of patients
  2. Intracranial Hemorrhage (>40% of cases, esp. children)
    1. Represents 10% of Intracranial Hemorrhage cases
    2. Intracerebral Hemorrhage is most common, but Subarachnoid Hemorrhage may occur
  3. Seizure (>15% of cases)
    1. More common in large or multiple, superficial lesions

V. Symptoms

  1. Headache
  2. Neurologic deficits

VI. Imaging

  1. CT Head
    1. Performed in acute presentation suspicious for Intracranial Hemorrhage
    2. However, low Test Sensitivity for AV Malformation
  2. CT Angiogram Brain
    1. "Bag of Worm" appearance of tortuous, dilated arteries to nidus and draining vein
  3. MRI Brain (preferred)
    1. T2 Weighted images are most sensitive at identifying flow voids in the region of AV Malformation

VII. Grading: Spetzler-Martin

  1. Nidus Size
    1. Score 1: <3 cm
    2. Score 2: 3-6 cm
    3. Score 3: >6 cm
  2. Importance (Eloquence) of Adjacent Brain Regions at Risk (score one point for any of the following)
    1. Brainstem
    2. Cerebellar Peduncles
    3. Thalamus
    4. Hypothalamus
    5. Language centers
    6. Sensorimotor centers
    7. Primary visual cortex
  3. Venous Drainage (score one point for venous drainage to deep veins )
    1. Basal veins
    2. Internal cerebral veins
    3. Precentral cerebellar veins

IX. Management

  1. Consult Neurosurgery
  2. Surgical Intervention
    1. Indications
      1. Young patients with at least one high risk feature for Intracranial Hemorrhage
      2. Refractory Seizures
    2. Primary Procedures
      1. Open microsurgical excision
    3. Adjunctive or Alternative Procedures
      1. Radiotherapy
      2. Endovascular embolization
  3. Medical management
    1. Seizure Prophylaxis
    2. Headache Management

X. Complications

  1. Intracranial Hemorrhage
  2. Seizure Disorder
  3. Intracranial Mass Effect
  4. Progressive neurologic deficits

XI. References

  1. Bokhari (2021) Arteriovenous Malformation of the Brain, Stat Pearls, Treasure Island, accessed 3/30/2022
    1. https://www.ncbi.nlm.nih.gov/books/NBK430744/
  2. Derdeyn (2017) Stroke 48:e200-24 +PMID:28642352 [PubMed]

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Ontology: Arteriovenous Malformations, Cerebral (C0917804)

Definition (NCI) An abnormal connection between arteries and veins characterized by the absence of intervening capillaries in the brain. Signs and symptoms include headaches, bruit upon head examination, seizures, and bleeding. When bleeding occurs, the signs and symptoms are similar to those in stroke.
Concepts Congenital Abnormality (T019)
MSH D002538
ICD10 Q28.2
SnomedCT 204499006, 234142008
English Arteriovenous Malformation, Cerebral, Cerebral Arteriovenous Malformations, Malformation, Cerebral Arteriovenous, Malformations, Cerebral Arteriovenous, AVM-Cerebral arterioven malfrm, Cerebral arteriovenous malform, Cong.cerebral A-V malformation, Arteriovenous malformation of cerebral vessels, ARTERIOVENOUS MALFORMATIONS OF THE BRAIN, CEREBRAL ARTERIOVENOUS MALFORMATIONS, BAVM, cerebral arteriovenous malformation (diagnosis), cerebral AV malformation, cerebral AVM, cerebral arteriovenous malformation, Cerebrovascular arteriovenous malformation, Cerebral arteriovenous malformations, AVM - Cerebral arteriovenous malformation, Cerebral arteriovenous malformation, Congenital cerebral arteriovenous malformation, Cerebral arteriovenous malformation (disorder), Cerebral Arteriovenous Malformation, Arteriovenous Malformations, Cerebral
Italian Malformazione arterovenosa cerebrovascolare, Malformazioni arterovenose cerebrali, Malformazioni artero-venose cerebrali
French Malformation artérioveineuse cérébrale, Malformations artérioveineuses cérébrales, Malformation artérioveineuse cérébrovasculaire, Malformations artério-veineuses cérébrales, Malformations artérioveineuses des vaisseaux cérébraux, Malformations des vaisseaux cérébraux
Japanese 脳血管動静脈奇形, ノウケッカンドウジョウミャクキケイ
Czech arteriovenózní malformace mozku, Cerebrovaskulární arteriovenózní malformace
Portuguese Malformações Arteriovenosas Cerebrais, Malformação arteriovenosa cerebrovascular
Spanish Malformaciones Arteriovenosas Cerebrales, MAV - malformación arteriovenosa cerebral, malformación arteriovenosa cerebral (trastorno), malformación arteriovenosa cerebral, Malformación arteriovenosa cerebrovascular
German Arteriovenöse Fehlbildungen, zerebrale, Arteriovenoese Fehlbildung der Hirngefaesse, zerebrovaskulaere arteriovenoese Missbildung, Zerebrale arteriovenöse Fehlbildungen
Korean 대뇌 혈관의 동정맥 기형
Hungarian Cerebrovascularis arteriovenosus malformatio
Norwegian Cerebrale arteriovenøse misdannelser, Arteriovenøse misdannelser, cerebrale
Dutch Arterioveneuze malformatie van cerebrale vaten, cerebrovasculaire arterioveneuze misvorming, Arterioveneuze malformatie, intracraniële