General Information
It is an expectation that all patients receive care/services from a licensed clinician.  All appropriate supporting documentation, including recent pertinent office visit notes, laboratory data, and results of any special testing must be provided.  If applicable: All prior relevant imaging results and the reason that alternative imaging cannot be performed must be included in the documentation submitted. 

Where a specific clinical indication is not directly addressed in this guideline, medical necessity determination will be made based on widely accepted standard of care criteria. These criteria are supported by evidence-based or peer-reviewed sources such as medical literature, societal guidelines and state/national recommendations.

Purpose
Indications for performing magnetic resonance angiography (MRA) or magnetic resonance venography (MRV) in the neck/cervical region.

Special Note
If there is a combination request* for an overlapping body part, either requested at the same time or sequentially (within the past 3 months) the results of the prior study should be:

• Inconclusive or show a need for additional or follow up imaging evaluation OR
• The office notes should clearly document an indication why overlapping imaging is needed and how it will change management for the patient.

(*Unless approvable in the Combination Section as noted in the guidelines)

Policy
INDICATIONS FOR NECK MRA
Cerebrovascular Disease

• Recent ischemic stroke or transient ischemic attack (see Background)^1,2,3
  • Note: For remote strokes with no prior vascular imaging, imaging can be considered based on location/type of stroke and documented potential to change management
• Known or suspected vertebrobasilar insufficiency (VBI) in patients with symptoms such as dizziness, vertigo, headaches, diplopia, blindness, vomiting, ataxia, weakness in both sides of the body, or abnormal speech^4,5,6
• Asymptomatic patients with an abnormal ultrasound of the neck or carotid duplex imaging (e.g., carotid stenosis ≥ 70%, technically limited study, aberrant direction of flow in the carotid or vertebral arteries)^7,8,9
• Symptomatic patients with an abnormal ultrasound of the neck or carotid duplex imaging (e.g., carotid stenosis ≥ 50%, technically limited study, aberrant direction of flow in the carotid or vertebral arteries)^7,10,11

Tumor/Pulsatile Mass

• Pulsatile mass on exam¹²
• Known carotid body tumors, or other masses such as a paraganglioma, arteriovenous fistula, pseudoaneurysm, atypical lymphovascular malformation^12,13,14

Note: Ultrasound (US) may be used to identify a mass overlying or next to an artery in initial work up of a pulsatile mass.

Other Extracranial Vascular Diseases

• Large vessel vasculitis
  • Giant cell with suspected extracranial involvement^15,16,17,18
  • Takayasu's Arteritis for evaluation at diagnosis and as clinically indicated for suspected extracranial involvement¹⁸
• Subclavian steal syndrome when ultrasound is positive or indeterminate OR for planning an intervention¹⁹
• Suspected carotid or vertebral artery dissection; secondary to trauma or spontaneous due to weakness of vessel wall^20,21
• Follow-up of known carotid or vertebral artery dissection within 3 – 6 months for evaluation of recanalization and/or to guide anticoagulation treatment^22,23
• Horner’s syndrome, non-central (miosis, ptosis, and anhidrosis)^24,25
• For evaluation of pulsatile tinnitus (subjective or objective) for suspected arterial vascular etiology^26,27
• For further evaluation of a congenital vascular malformation of the head and neck²⁸
• Known extracranial vascular disease that needs follow-up or further evaluation^29,30,31

Pre- or Post-Operative/Procedural Evaluation
Pre-operative/procedural evaluation

• Pre-operative evaluation for a planned surgery or procedure

Post-operative/procedural evaluation

• A follow-up study may be needed to help evaluate a patient’s progress after treatment, procedure, intervention, or surgery; or to evaluate postsurgical/posttreatment changes²⁸ Documentation requires a medical reason that clearly indicates why additional imaging is needed for the type and area(s) requested.

Further Evaluation of Indeterminant Findings
Unless follow up is otherwise specified within the guideline:

• For initial evaluation of an inconclusive finding on a prior imaging report that requires further clarification
• One follow-up exam of a prior indeterminate MR/CT finding to ensure no suspicious interval change has occurred. (No further surveillance unless specified as highly suspicious or change was found on last follow-up exam.)

Genetic Syndromes and Rare Diseases 

• For patients with fibromuscular dysplasia (FMD):³²
  • One-time vascular study from brain to pelvis
• Vascular Ehlers-Danlos syndrome:³³
  • At diagnosis and then every 18 months
  • More frequently if abnormalities are found
• Loeys-Dietz:^18,34
  • At diagnosis and then every two years
  • More frequently if abnormalities are found
• Takayasu's Arteritis:¹⁸
  • For evaluation at diagnosis then as clinically indicated
• Spontaneous coronary arteries dissection (SCAD)³⁵
  • One-time vascular study from brain to pelvis
• For other syndromes and rare diseases not otherwise addressed in the guideline, coverage is based on a case-by-case basis using societal guidance

Combination Studies
Brain/Neck/Chest/Abdomen/Pelvis MRA

• For patients with fibromuscular dysplasia (FMD), a one-time vascular study from brain to pelvis^36,37
• Vascular Ehlers-Danlos syndrome: At diagnosis and then every 18 months; more frequently if abnormalities are found^38,39
   
• Loeys-Dietz: at diagnosis and then every two years, more frequently if abnormalities are found⁴⁰
• For assessment in patients with spontaneous coronary artery dissection (SCAD), can be done at time of coronary angiography⁴¹

Brain MRA and Neck MRA 

• Recent ischemic stroke or transient ischemic attack (TIA) (see Background)^1,2,42
  • Note: For remote strokes with no prior vascular imaging, imaging can be considered based on location/type of stroke and documented potential to change management
• Known or suspected vertebrobasilar insufficiency (VBI) in patients with symptoms such as dizziness, vertigo, headaches, diplopia, blindness, vomiting, ataxia, weakness in both sides of the body, or abnormal speech^4,5
• Suspected carotid⁴³ or vertebral⁴⁴ artery dissection; secondary to trauma⁴⁵ or spontaneous due to weakness of vessel wall^3,20,21
• Follow-up of known carotid or vertebral artery dissection within 3 – 6 months for evaluation of recanalization and/or to guide anticoagulation treatment^3,22,23
• Horner’s syndrome, non-central (miosis, ptosis, and anhidrosis)²⁴
• Large vessel vasculitis (Giant cell or Takayasu arteritis) with suspected intracranial and extracranial involvement
• Asymptomatic patients with an abnormal ultrasound of the neck or carotid duplex imaging (e.g., internal carotid stenosis > 70%, technically limited study, aberrant direction of flow in the carotid or vertebral arteries) and patient is surgery or angioplasty candidate^7,8,9
• Symptomatic patients with an abnormal ultrasound of the neck or carotid duplex imaging (e.g., carotid stenosis ≥ 50%, technically limited study, aberrant direction of flow in the carotid or vertebral arteries) and patient is surgery or angioplasty candidate^7,8,10
• For evaluation of pulsatile tinnitus (subjective or objective) for suspected arterial vascular etiology²⁶

Brain MRI/Brain MRA/Neck MRA

• Recent ischemic stroke or transient ischemic attack (See Background)
• Suspected carotid or vertebral artery dissection with focal or lateralizing neurological deficits
• Pulsatile tinnitus with concern for a suspected arterial vascular and/or intracranial etiology (should include IACs)^46,47
• Giant cell arteritis with suspected intracranial and extracranial involvement
• Approved indications as noted above and being performed in a child under 8 years of age who will need anesthesia for the procedure and there is a suspicion of concurrent vascular and intracranial pathology⁴⁸

Note: CTA and MRA are generally comparable noninvasive imaging alternatives each with their own advantages and disadvantages. Brain MRI can be combined with Brain CTA/Neck CTA.

Neck/Chest/Abdomen/Pelvis MRA

• Takayasu's Arteritis: For evaluation at diagnosis then as clinically indicated¹⁸

Rationale
MRA and CTA are generally comparable noninvasive imaging alternatives, each with their own advantages and disadvantages. MRA is an excellent screening test since it does not utilize ionizing radiation²⁸

MRA vs CTA for Carotid Artery Evaluation^49,50
Duplex US, CTA and contrast-MRA are common choices for carotid artery evaluation. Limitations of MRA include difficulty in patients with claustrophobia and the risk of 
nephrogenic systemic sclerosis with gadolinium contrast agents in specific patients. Advantages of CTA over MRA include superior spatial resolution, rapid image acquisition, 
decreased susceptibility to motion artifacts and artifacts from calcification as well as being better able to evaluate slow flow and tandem lesions. However, it can also overestimate high-grade stenosis. Limitations of CTA include radiation exposure to the patient, necessity of IV contrast, and risk of contrast allergy and contrast nephropathy.

MRA and Dissection
Craniocervical dissections can be spontaneous or traumatic. Patients with blunt head or neck trauma who meet Denver Screening criteria should be assessed for cerebrovascular injury (although about 20% will not meet criteria). The criteria include focal or lateralizing neurological deficits (not explained by head CT), infarct on head CT, face, basilar skull, or cervical spine fractures, cervical hematomas that are not expanding, glasgow coma score less than 8 without CT findings, massive epistaxis, cervical bruit or thrill.^20,51,52 Spontaneous dissection presents with headache, neck pain with neurological signs or symptoms.

There is often minor trauma or precipitating factor (e.g., exercise, neck manipulation). Dissection is thought to occur due to weakness of the vessel wall, and there may be an 
underlying connective tissue disorder. Dissection of the extracranial vessels can extend intracranially and/or lead to thrombus, which can migrate into the intracranial circulation causing ischemia. Therefore, MRA of the head and neck is warranted.^21,53,54

MRA and Recent Stroke or Transient Ischemic Attack

• When revascularization therapy is not indicated or available in patients with an ischemic stroke or TIA, the focus of the work-up is on secondary prevention. Both stroke and TIA should have an evaluation for high-risk modifiable factors such as carotid stenosis atrial fibrillation as the cause of ischemic symptoms.⁵⁵ Diagnostic recommendations include neuroimaging evaluation as soon as possible, preferably with magnetic resonance imaging, including DWI; noninvasive imaging of the extracranial vessels should be performed, and noninvasive imaging of intracranial vessels is reasonable.⁵⁶
• Patients with a history of stroke and recent work-up with new signs or symptoms indicating progression or complications of the initial CVA should have repeat brain imaging as an initial study. Patients with remote or silent strokes discovered on imaging should be evaluated for high-risk modifiable risk factors based on the location and type of the presumed etiology of the brain injury

Acronyms
CTA: Computed tomography angiography
CVA: Cerebrovascular accident
FMD: Fibromuscular dysplasia
IAC: Internal auditory canal
IV: Intravenous
MRA: Magnetic resonance angiography
MRV: Magnetic resonance venography
SCAD: Spontaneous coronary artery dissection
TIA: Transient ischemic attack
US: Ultrasound
VBI: Vertebrobasilar Insufficiency

Contraindications and Preferred Studies

• Contraindications and reasons why a CT/CTA cannot be performed may include: impaired renal function, significant allergy to IV contrast, pregnancy (depending on trimester).
• Contraindications and reasons why an MRI/MRA cannot be performed may include: impaired renal function, claustrophobia, non-MRI compatible devices (such as non-compatible defibrillator or pacemaker), metallic fragments in a high-risk location, patient exceeds weight limit/dimensions of MRI machine.

References 

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Coding section

Procedure and diagnosis codes on Medical Policy documents are included only as a general reference tool for each policy. They may not be all-inclusive. 

This medical policy was developed through consideration of peer-reviewed medical literature generally recognized by the relevant medical community, U.S. FDA approval status, nationally accepted standards of medical practice and accepted standards of medical practice in this community, Blue Cross Blue Shield Association technology assessment program (TEC) and other nonaffiliated technology evaluation centers, reference to federal regulations, other plan medical policies, and accredited national guidelines.

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