PANCE Blueprint Cardiology (13%)

Arteriovenous malformation

Patient will present as → a 63-year-old illiterate female with a history of right motor partial seizures and generalization since the age of 23 years arrives at the emergency room due to acute right hemiparesis. Neurological examination reveals a right hemiparesis, right tactile and pain hypoesthesia, poor fluency, temporal and spatial disorientation, and a Mini-Mental State Examination score of 5 points (one for immediate memory, two for naming, one for repetition, and one for commands). MRI is performed, demonstrating a massive AVM in the left hemisphere of the frontotemporoparietal region (9.2 × 6.0 cm) with parenchymal compression and microangiopathy.

In about half of all brain AVMs waht is the first sign?
In about half of all brain AVMs, hemorrhage is the first sign.
What is the gold standard for the diagnosis, treatment planning, and follow-up after treatment of brain AVMs?
Angiography is the gold standard for the diagnosis, treatment planning, and follow-up after treatment of brain AVMs

Arteriovenous malformation is an abnormal connection between arteries and veins, bypassing the capillary system.

  • Arteriovenous malformations (AVMs) are the most dangerous of the congenital vascular malformations, with the potential to cause intracranial hemorrhage and epilepsy in many cases
  • Brain AVMs occur in about 0.1 percent of the population, one-tenth the incidence of intracranial aneurysms
  • Supratentorial lesions account for 90 percent of brain AVMs
  • Brain AVMs underlie 1 to 2 percent of all strokes, 3 percent of strokes in young adults, and 9 percent of subarachnoid hemorrhages

Brain AVMs are usually present between the ages of 10 and 40 years.  In about half of all brain AVMs, intracranial hemorrhage is the first sign (41 to 79 percent)

In people without hemorrhage, signs and symptoms of a brain AVM may include:

  • Seizure: 11-13 percent of patients with AVM present with seizure
  • Headache: 0.2 percent of patients with headache and normal neurologic examinations were found to have an AVM
  • Focal neurologic deficit

Risk factors include being male and having a family history of AVM

Angiography is the gold standard for the diagnosis, treatment planning, and follow-up after treatment of brain AVMs

  • Brain AVMs are typically first identified on cross-sectional imaging (CT or MRI). MRI is more sensitive, particularly in the setting of an acute intracerebral hemorrhage
  • A combination of MRI and angiography is often used to assess the likely success and risks of surgical, endovascular, or radiosurgical therapy
001-Arteriovenous-malformation-TOF-MIP-6

MRA of a large arteriovenous malformation on the left

Surgical excision is the mainstay of treatment; radiosurgery is a useful option in lesions deemed at high risk for surgical therapy, and endovascular embolization can be a useful adjunct to these techniques

  • Treatment for brain AVMs can be symptomatic, and patients should be followed by a neurologist for any seizures, headaches, or focal neurologic deficits

osmosis Osmosis
Picmonic
Subarachnoid hemorrhage (SAH)

IM_MED_SubararachnoidHemorrhage_v1.6_

Subarachnoid hemorrhage (SAH) is a bleed in the subarachnoid space, which occurs most commonly due to rupture of a berry aneurysm. It can also occur from trauma and AVM. Patients often complain of the ‘worst headache of my life.

Play Video + Quiz

Question 1
In the evaluation of suspected arteriovenous malformations, which imaging modality is considered the definitive standard for diagnosis?
A
Contrast-enhanced Computed Tomography (CT)
Hint:
While useful for initial assessment, CT with contrast does not offer the same level of vascular detail as DSA for diagnosing AVMs.
B
Magnetic Resonance Imaging (MRI)
Hint:
MRI is beneficial for visualizing AVMs and assessing their impact on surrounding brain tissue but lacks the vascular detail provided by DSA.
C
Digital Subtraction Angiography (DSA)
D
Carotid Doppler ultrasound
Hint:
This imaging modality is used for assessing blood flow in the carotid arteries, not for detailed visualization of AVMs.
E
Functional MRI (fMRI)
Hint:
fMRI is primarily used to map brain activity and is not a standard diagnostic tool for AVMs. It does not provide the detailed vascular imaging necessary for diagnosing AVMs.
Question 1 Explanation: 
Digital Subtraction Angiography (DSA) is considered the gold standard for diagnosing arteriovenous malformations. It provides detailed visualization of the vascular structure of AVMs, crucial for accurate diagnosis and treatment planning.
Question 2
What is the most common presenting symptom/sign in a patient with arteriovenous malformation?
A
Headache
Hint:
0.2 percent of patients with a headache and normal neurologic examinations were found to have an AVM
B
Seizure
Hint:
11-13 percent of patients with AVM
C
Intracranial hemorrhage
D
Focal neurologic deficit
Hint:
This presentation is fairly unusual for cerebral AVM. While a vascular steal syndrome has been hypothesized to cause this presentation, in most cases this is related to mass effect, hemorrhage, or seizure.
E
Visual disturbances
Hint:
Visual symptoms may occur depending on the AVM's location, especially if it affects the occipital lobe or optic pathways. However, they are less common as an initial symptom compared to headaches.
Question 2 Explanation: 
In about half of all brain AVMs, intracranial hemorrhage is the first sign (41 to 79 percent). 11-13 percent of patients with AVM present with seizure, 0.2 percent of patients with a headache and normal neurologic examinations were found to have an AVM and focal neurologic deficits are fairly unusual for cerebral AVM.
Question 3
A 40-year-old man is diagnosed with an asymptomatic cerebral arteriovenous malformation discovered incidentally during evaluation for migraines. The AVM is small and located in a non-eloquent area of the brain. What is the most appropriate management for this patient?
A
Conservative management with regular follow-up
B
Immediate surgical resection
Hint:
Indicated for symptomatic, accessible AVMs or those that have bled.
C
Stereotactic radiosurgery
Hint:
Considered for small, surgically inaccessible AVMs, more so if symptomatic.
D
Anticoagulation therapy
Hint:
Not a treatment for AVM and could increase the risk of hemorrhage.
E
High-dose corticosteroids
Hint:
Used to reduce edema in certain neurological conditions, not for managing AVMs.
Question 3 Explanation: 
In the case of an asymptomatic, small arteriovenous malformation located in a non-eloquent area of the brain, conservative management with regular follow-up is often the most appropriate approach. The risks of intervention may outweigh the benefits in such cases, especially when the AVM is not causing symptoms.
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References: Merck Manual · UpToDate

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