Clinical Appropriateness Guidelines: Advanced Imaging

Appropriate Use Criteria: Pediatric Head & Neck Effective Date: March 1, 2016

Proprietary

Date of Origin: 10/29/2014 Last revised: 08/27/2015 Last reviewed: 08/27/2015

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Copyright © 2016. AIM Specialty Health. All Rights Reserved www.aimspecialtyhealth.com Table of Contents

Administrative Guideline Disclaimer...... 3 Use of AIM’s Diagnostic Imaging Guidelines...... 4 Multiple Simultaneous Imaging Requests...... 5 General Imaging Considerations...... 6

Head & Neck Imaging MRI Head/Brain – ...... 8 CTA of the Head: Cerebrovascular – Pediatrics...... 17 MRI Head/Brain – Pediatrics...... 20 MRA of the Head: Cerebrovascular – Pediatrics...... 28 Functional MRI (fMRI) Brain – Pediatrics...... 31 PET Brain Imaging – Pediatrics...... 32 CT Orbit, Sella Turcica, Posterior Fossa, Temporal Bone, including Mastoids – Pediatrics...... 33 MRI Orbit, Face & Neck (Soft Tissues) – Pediatrics...... 36 CT Paranasal Sinus & Maxillofacial Area – Pediatrics...... 40 MRI Temporomandibular Joint (TMJ) – Pediatrics...... 43 CT Neck for Soft Tissue Evaluation – Pediatrics...... 45 CTA of the Neck – Pediatrics...... 49 MRA of the Neck – Pediatrics...... 51

Table of Contents | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 2 Administrative Guideline: Disclaimer

BY ACCEPTING THESE DOCUMENTS, I ACKNOWLEDGE ACCEPTANCE OF THE FOLLOWING TERMS AND CONDITIONS FOR ACCESS AND USE OF THE CLINICAL GUIDELINES:

AIM Specialty Health (AIM) has developed proprietary clinical appropriateness guidelines (together with any updates, referred to collectively as the “Guidelines”). The Guidelines are designed to evaluate and direct the appropriate utilization of high technology diagnostic imaging services. They are based on data from the peer-reviewed scientific literature, from criteria developed by specialty societies and from guidelines adopted by other health care organizations. Access to these Guidelines is being provided for informational purposes only. AIM is under no obligation to update its Guidelines. Therefore, these Guidelines may be out of date.

The Guidelines are protected by copyright of AIM as permitted by and to the full extent of the law. These rights are not released, transferred, or assigned as a result of allowing access. You agree that you do not have any ownership rights to the Guidelines and you are expressly prohibited from selling, assigning, leasing, licensing, reproducing or distributing the Guidelines, unless authorized in writing by AIM.

The Guidelines do not constitute medical advice and/or medical care, and do not guarantee results or outcomes. The Guidelines are not a substitute for the experience and judgment of a or other health care professionals. Any clinician seeking to apply or consult the Guidelines is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patient’s care or treatment. The Guidelines do not address coverage, benefit or other plan specific issues.

AIM reviews and revises its Guidelines as necessary to reflect current evidence based . However, AIM makes no guarantee that its Guidelines at all times reflect the most up-to-date information.

Administrative Guideline | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 3 Administrative Guideline: Use, Development and Review of AIM Guidelines

Use of AIM’s Diagnostic Imaging Guidelines: AIM’s proprietary clinical appropriateness guidelines are designed to evaluate and direct the appropriate utilization of elective, high technology advanced imaging services. In the process, multiple functions are accomplished: ●● To promote the most efficient and cost-effective use of evidence-based advanced imaging services ●● To assist the practitioner as an educational tool ●● To encourage standardization of medical practice patterns and reduce variation in clinical evaluation ●● To curtail the performance of inappropriate, elective advanced imaging studies ●● To reduce the performance of duplicate advanced imaging studies ●● To advocate biosafety issues, including unnecessary radiation exposure (for CT and plain film radiography) and MRI safety concerns ●● To enhance quality of healthcare for elective advanced imaging studies, using evidence-based medicine and outcomes research from numerous resources

AIM Guideline Development Process and Resources: AIM reviews its proprietary clinical appropriateness guidelines on an ongoing basis, throughout the year based on the results of the research and development process and feedback from and other providers. New Guidelines are also developed as needed. Development of appropriate use criteria within AIM guidelines is based on objective medical evidence including assessment of potential benefits and harms. The resources considered during AIM guideline development can include but are not limited to: ●● Professional Society Guidelines ●● Professional Society Appropriate Use Criteria ●● Agency for Healthcare Research and Quality (AHRQ) Comparative Effectiveness Guidelines ●● Recommendations from the United States Preventive Services Task Force ●● National Guideline Clearinghouse ●● Centers for Medicare and Medicaid Services (CMS) ●● Initiatives sponsored by Specialty Licensing Boards, including but not limited to Choosing Wisely recommendations ●● National Guideline Clearinghouse ●● The latest scientific and clinical peer-reviewed literature

Guideline Review: AIM’s proprietary guidelines for appropriate use of advanced imaging are reviewed routinely by: ●● An External Expert Panel, consisting of physicians from multiple specialties and practice settings across the United States ●● Health Plan Medical Directors ●● Other clinical reviewers, under the governance of our clients’ state regulatory agencies ●● Subject matter specialty physician experts and primary care physicians

Administrative Guideline | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 4 Administrative Guideline: General Imaging Considerations for All Modalities, Body Parts, and CPT Codes

Standard Anatomic Coverage for Multiple Simultaneous Imaging Requests The major area of concern is contiguous body parts where clinical signs and symptoms may be coming from abnormalities involving either region or different modalities can be considered to evaluate the same anatomy for the same clinical problem. These are areas where ordering multiple tests before the results of any of the tests are known lead to inappropriate imaging. General Considerations for Multiple Simultaneous Imaging Requests Rapid breakthroughs in technology, with attendant rise of new imaging tests available to improve patient management, have created a dilemma for clinicians. Many factors in choosing the right test now come into play. One must consider basic data in the decision-making process. Considerations include the possible effect on patient management, the pretest probability that the patient is affected by a particular disease, the prevalence of the disease in the population, and the accuracy (sensitivity/ specificity) of the test. When a screening approach is adopted, rather than targeting the particular test or anatomic site with the highest pretest probability of success, the possibility of one or more of the tests being superfluous and not contributing meaningfully to patient management increases to an unacceptable level. For this reason, simultaneous ordering of multiple examinations may subject these examinations to more intensive levels of review than would be the case if these same tests were ordered sequentially. Depending on the clinical situation, one or more of the requested studies might not meet medical necessity criteria until the results of the lead study are known. Common Indications for Multiple Simultaneous Imaging Requests ●● The initial diagnosis/staging or follow-up of patients ●● Follow-up of patients who have had operative procedures on multiple anatomic sites ●● Patients in whom the suspected anatomic abnormality might extend into multiple regions, such as diverticulitis or suspected syringomyelia Common Inappropriate Multiple Simultaneous Imaging Requests ●● Brain MRA ordered routinely with brain MRI without vascular indications ●● Brain CT ordered simultaneously with sinus CT for headache ●● Multiple levels of spine MRI’s or CT’s for diffuse back pain or radicular symptoms ●● Cervical spine and shoulder MRI’s ordered simultaneously for shoulder pain ●● Pelvic or hip MRI’s ordered simultaneously with lumbar spine MRI for hip pain ●● Pelvic CT ordered routinely with abdominal CT for suspected upper quadrant disease processes ●● CT Angiography (CTA) utilizes the data obtained from standard CT imaging. Request for a CT exam, in addition to CT Angiography of the same anatomic area AND during the same imaging session, is inappropriate.

Administrative Guideline | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 5 Imaging Considerations for all Exams

●● Duplicative testing or repeat imaging of the same anatomic area with same or similar technology may be subject to high-level review and may not be medically necessary unless there is a persistent diagnostic problem or there has been a change in clinical status (e.g., deterioration) or there is a medical intervention which warrants interval reassessment. ●● Request for re-imaging due to technically limited exams is the responsibility of the imaging providers. ●● In general, follow-up exams should be performed only when there is a clinical change, with new signs or symptoms. ●● AIM’s clinical guidelines do not supersede the enrollee’s health plan medical policy specific to a given exam for a given anatomic structure. Imaging Considerations Specific to CT and CTA

●● Advantages of CTA over MRA include higher sensitivity for detection of mural calcification; usually shorter scan time, which results in less motion, pulsation and turbulent flow artifact; avoidance of MRA in-plane flow as a cause of apparent exaggerated stenosis; more facile detection of surgical clips and stents. ●● Disadvantages of CTA include radiation exposure and use of intravascular iodinated contrast material. ●● Multi-detector row CT is preferred but not required in the performance of CTA, when compared with single detector CT. ●● CTA studies are typically performed through acquisition of thin CT sections, during intravenous bolus infusion of iodinated contrast material. ●● Contrast-enhancement for CT/CTA may be contraindicated in certain circumstances, such as a documented to intravenous contrast material and renal insufficiency. Special consideration should also be given to patients with multiple myeloma. ●● CT Angiography (CTA) utilizes imaging data from standard CT acquisitions. Request for a CT exam in addition to CT Angiography of the same anatomic area during the same imaging session is inappropriate. Imaging Considerations Specific to MRI and MRA Patient Compatibility Issues: ●● Artifact due to patient motion may have a particularly significant impact on exam quality. ●● Metallic implants present in spine and brain. ●● Eye and brain for metallic foreign bodies. ●● Breath hold requirements: ○○ Some imaging sequences require breath holding and this may be difficult or impossible for some patients. ●● Claustrophobic patients: ○○ Patients with claustrophobia may need to be premedicated in order to tolerate the imaging procedure. Rarely patients with severe claustrophobia will not be suitable candidates for imaging.

Biosafety Issues: ●● Ordering and imaging providers are responsible for considering biosafety issues prior to MRI/MRA examination, to ensure patient safety. Among the generally recognized contraindications to MRI/MRA exam performance are permanent pacemakers (some newer models are MRI/MRA compatible and others may be safe depending on sequences used; contact imaging facility for substantiation), implantable cardioverter-defibrillators (ICD), intracranial aneurysm surgical clips that are not compatible with MR imaging, as well as other devices considered unsafe in MRI scanners (including certain implanted materials in the patient as well as external equipment, such as portable oxygen tanks). ●● Contrast utilization is at the discretion of the ordering and imaging providers.

Administrative Guideline | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 6 Ordering Issues: ●● The CPT code assignment for an MRI procedure is based on the anatomic area imaged. Requests for multiple MRI exams of the same anatomic area to address patient positional changes, additional sequences or equipment are not allowed. These variations or extra sequences are included within the original imaging request. ●● There are rare circumstances when both CT and MRI exams should be ordered for the same clinical presentation. The specific rationale for each study must be delineated at the time of request. ●● There are uncommon circumstances when both MRA and CTA should be ordered for the same clinical presentation. The specific rationale must be delineated at the time of request. ●● Advantages of MRA, compared with CTA include avoidance of radiation exposure as well as intravascular administration of iodinated contrast material. ●● Disadvantages of MRA, compared with CTA, include lower sensitivity for detection of mural calcification; usually longer scanning time, with potential for greater motion, pulsation and turbulent flow artifact; in-plane flow causing apparent exaggerated stenosis; greater difficulty in identifying surgical clips and stents. Reference/Literature Review

1. Bossuyt PM, Irwig L, Craig J, Glasziou P. Comparative accuracy: assessing new tests against existing diagnostic pathways. BMJ. 2006;332(7549):1089-1092. 2. Centers for Medicare and Medicaid Services. National Coverage Determination for Magnetic Resonance Imaging (NCD 220.2). Medicare Coverage Database. Effective July 7, 2011; Implementation September 26, 2011. http://www.cms.gov/ medicare-coverage-database/details/ncd-details.aspx?NCDId=177. Accessed October 27, 2011. 3. Dodd JD. Evidence-based practice in : steps three and four–appraise and apply diagnostic radiology literature. Radiology. 2007;242(2):342-354. 4. Dubilet PM, Cain KC. The superiority of sequential over simultaneous testing. Med Decis Making. 1985;5(4):447-451. 5. Fryback DG, Thornbury JR. The efficacy of diagnostic imaging. Med Decis Making. 1991;11(2):88-94. 6. Hollingworth W, Jarvik JG. Technology assessment in radiology: putting the evidence in evidence-based radiology. Radiology. 2007;244(1):31-38. 7. Kuhns LR, Thornberry JR, Freyback DG. Decision Making in Imaging. Chicago: Year Book Medical Publishers;1989. 8. Ng CS, Palmer CR. Analysis of diagnostic confidence and diagnostic accuracy: a unified framework. Br J Radiol. 2007;80(951):152-160. 9. Owens DK, Qaseem A, Chou R, Shekelle P; Clinical Guidelines Committee of the American College of Physicians. High-value, cost-conscious health care: concepts for clinicians to evaluate the benefits, harms, and costs of medical interventions. Ann Intern Med. 2011;154(3):174-180.

Administrative Guideline | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 7 Computed Tomography (CT) Head - Pediatrics

CPT Codes 70450 ����������������� CT of head, without contrast 70460 ����������������� CT of head, with contrast 70470 ����������������� CT of head, without contrast, followed by re-imaging with contrast Standard Anatomic Coverage ●● From the skull base to vertex, covering the entire calvarium and intracranial contents ●● Scan coverage may vary, depending on the specific clinical indication Technology Considerations ●● MRI of the head is preferable to CT in most clinical scenarios, due to its superior contrast resolution and lack of beam-hardening artifact adjacent to the petrous bone (which may limit visualization in portions of the posterior fossa and brainstem on CT) ●● Exceptions to the use of brain MRI as the neuroimaging study of choice and clinical situations where CT head is preferred: osseous assessment of the calvarium, skull base and maxillofacial bones, including detection of calvarial and facial bone fractures; calcified lesions; initial evaluation of recent craniocerebral trauma; and acute intracranial hemorrhage (parenchymal, subarachnoid, subdural, epidural) ●● MRI is more sensitive for detection of shearing trauma to the brain and diffuse axonal injury. It is also the preferred technique for assessment of subacute and chronic intracranial hemorrhage ●● CT of the head is an alternative exam in patients for whom MRI is contraindicated or who cannot tolerate MRI ●● Imaging studies of the head and neck are inherently bilateral, thus duplicate requests for these studies are inappropriate ●● CT is not typically indicated for sudden hearing loss in the absence of other neurological findings1,2 Common Diagnostic Indications The following diagnostic indications for head CT are accompanied by pre-test considerations as well as clinical supporting data and prerequisite information

Abnormality detected on other imaging study which requires additional clarification to direct treatment

♦ List may not be exclusive | CT Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 8 Common Diagnostic Indications Abnormality on neurological exam when suggestive of intracranial (All of the following) ●● Findings must be considered within the context of a differential diagnosis ●● At least one temporal characteristic: ○○ New onset ○○ Persistent ○○ Progressive ●● At least one abnormality on neurological exam (see Table 1 below) Note: The following neurologic symptoms have individual guidelines. For the evaluation of any of these neurologic symptoms, please see their individual guideline: Confusion / altered mental status (see Mental status changes), Headache (atraumatic), seizures (see Seizure disorder), Syncope (includes loss of consciousness), Tinnitus, Vertigo and dizziness (includes lightheadedness) Table 1: Neurologic abnormalities include: Cognitive Sensory Motor ●● Memory loss ●● Visual deficits – see visual ●● Weakness ●● Personality change disturbance guideline ○○ Hemiparesis or hemiplegia ●● Spatial neglect ●● Facial numbness ○○ Monoparesis ●● Agnosia ●● Paresthesia documented on ○○ Facial weakness ●● Apraxia neurological exam ●● Neurologic gait abnormality (including ataxia, ●● Language deficits ●● Impairments of taste and smell spastic, circumduction/magnetic) (including aphasia (including anosmia, paraosmi and ●● Ataxia or impaired voluntary and paraphasia) dysgeusia) coordination(including dysarthria,dysphagia dysmetria, dysdiadokinesia) ●● Abnormal reflexes (hyperreflexia (when unilateral or asymmetric), extensor plantar response (Babinski )and Hoffmann’s sign)

Ataxia, congenital or hereditary (any one♦ of the following) ●● Ataxia-telangiectasia ●● Congenital anomaly (particularly one of the posterior fossa) ●● Fragile X syndrome

Cerebrovascular accident (CVA or stroke) or transient ischemic attack (TIA) ●● Sudden onset of new clinical finding on exam that suggests CVA or TIA Note: Includes acute vascular injury. Among patients being evaluated for CVA and possible thrombolytic , unenhanced CT is generally preferred as the initial modality (within the first 24 hours of symptom onset) to detect a possible hemorrhagic stroke or mass lesion

Chiari malformation (Arnold-Chiari malformation)

♦ List may not be exclusive | CT Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 9 Common Diagnostic Indications

Congenital anomaly, not otherwise listed ●● Diagnosis or management (any one♦ of the following) ○○ Agenesis of the corpus callosum ○○ Blake pouch cyst ○○ Cephalocele ○○ Dandy walker spectrum ○○ De Morsier syndrome (septo-optic dysplasia) ○○ Encephalocele ○○ Focal cortical dysplasia ○○ Grey matter heterotopia ○○ Hemimegalencephaly ○○ Holoprosencephaly ○○ Joubert syndrome ○○ Nasal glioma ○○ Rhombencephalosynapsis ○○ Schizencephaly

Contraindication to MRI ●● Patient meets criteria for MRI exam (any one of the following) ○○ Patient has a contraindication to MRI (examples include metallic foreign bodies, permanent pacemaker, implantable cardioverter-defibrillators, and intracranial aneurysm surgical clips that are not compatible with MR imaging) ○○ Patient is claustrophobic and unable to tolerate MRI

Craniosynostosis Note: This includes brachycaphaly (coronal synostosis), plagiocephaly (unilateral coronal or lambdoidal synostosis), scaphocephaly/dolichocephaly (sagittal synostosis), syndromic craniosynostosis (Apert syndrome or Crouzon syndrome), and trigonocephaly (metopic synostosis)

Developmental delay3-8 (any one of the following) ●● Cerebral palsy ●● Global developmental delay (any two of the following) ○○ Activities of daily living ○○ Cognition ○○ Motor skills (gross/fine) ○○ Social/personal ○○ Speech/language Note: Global developmental delay is defined as significant delay or loss of milestones in at least two of the domain’s listed above

Headache, new onset9-10 (any one of the following) ●● Family or personal medical history of disorders that may predispose one to central nervous system (CNS) lesions and clinical/laboratory findings that suggest CNS involvement (Including but not limited to vascular malformations, aneurysms, brain neoplasms, infectious/inflammatory conditions such as sarcoidosis or personal history of and tuberculosis) ●● Headache that awakens the patient repeatedly from sleep or develops upon awakening ●● Neurological abnormalities such as nystagmus, papilledema, gait or motor disturbances ●● Patient is developmentally delayed ●● Sudden onset and severe headache (includes thunderclap headache or worst headache of life) Note: New onset headaches are headaches that have occurred for the first time within 30 days of the imaging request. If associated with trauma or infection, see separate indication for Trauma or Infectious/Inflammatory

♦ List may not be exclusive | CT Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 10 Common Diagnostic Indications

Headache, persistent or recurrent (any one of the following) ●● Abnormal neurological exam11-15 (any one of the following) ○○ Abnormal reflexes (hyperreflexia/hyporeflexia) ○○ Altered mental status (confused or disoriented) ○○ Cranial nerve deficit ○○ Gait /motor dysfunction ○○ Nystagmus ○○ Seizure ○○ Sensory deficit (numbness, paresthesia) ○○ Sign of increased intracranial pressure (increased head circumference, vomiting, papilledema, headache worse with valsalva) ○○ Vomiting ●● Change in quality (pattern or intensity) of a previously stable headache10,11 ●● Headache persisting for a period of up to 6 months duration and not responsive to medical treatment, when no prior imaging has been done to evaluate the headache Note: Persistent headaches are those lasting more than 30 days; recurrent headaches are those where the resolution and recurrence are separated by at least a 30 day period. If separated by less than 30 days, these headaches would be considered persistent

Hearing loss Note: This includes conductive, sensorineural16-18, and mixed hearing loss. CT is generally preferred for conductive and mixed. MRI is generally preferred for sensorineural

Hemorrhage or hematoma ●● For non-traumatic, non-CVA and non-tumor-related intracranial bleed (includes hemorrhage secondary to anticoagulation or blood dyscrasia) Note: CT is the preferred technique for evaluation of acute intracranial hemorrhage; MRI is generally preferred for evaluation of subacute and chronic hemorrhage

Horner’s syndrome, when MRI is contraindicated

Hydrocephalus (ventriculomegaly) ●● Patient is younger than five (5) months of age following an abnormal or non-diagnostic ultrasound Note: MRI is generally preferred for initial evaluation of patients with hydrocephalus. For patients with an indwelling shunt, CT is usually adequate in the diagnostic follow-up of hydrocephalus

Increased intracranial pressure (any one♦ of the following) ●● Bulging fontanelle ●● Headache worse with valsalva ●● Increased head circumference ●● Papilledema ●● Vomiting

Infectious or inflammatory process (any one♦ of the following) ●● Cerebral or cerebellar abscess ●● Encephalitis (including limbic encephalitis) ●● Meningitis ●● Neurocysticercosis ●● Opportunistic infection, particularly with immunosuppressed or other immunodeficient conditions ●● Subdural empyema

♦ List may not be exclusive | CT Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 11 Common Diagnostic Indications

Lumbar puncture, prior to procedure38 ●● With signs of increased intracranial pressure (any one of the following): ○○ Papilledema ○○ Absent venous pulsations on fundoscopic exam ○○ Altered mental status ○○ Abnormal neurological exam ○○ Evidence for meningeal irritation (any one♦ of the following): ■■ Neck stiffness ■■ Kernig’s signs ■■ Brudzinski’s sign

Macrocephaly ●● Patient’s head circumference is greater than two (2) standard deviations from the mean for his/her age and gender (any one of the following) ○○ Patient is younger than five (5) months of age following non-diagnostic ultrasound ○○ Patient is five (5) months of age or older

Mental status change ●● Abnormality on a neurologic exam

Microcephaly19 ●● Patient’s head circumference is less than two (2) standard deviations from the mean for his/her age and gender

Multiple sclerosis or other white-matter diseases, when MRI is contraindicated or not tolerated (any one of the following) ●● Diagnosis ●● Evaluation of changes in neurologic signs and symptoms ●● For multiple sclerosis (any one of the following) ○○ Assess asymptomatic disease progression ○○ Evaluate response to treatment ○○ Track disease progression to establish a prognosis

Neurocutaneous disorder (phakomatosis) (any one♦ of the following) ●● Neurofibromatosis ●● Sturge-Weber syndrome ●● Tuberous sclerosis ●● Von Hippel-Lindau disease (VHL)

Neuroendocrine abnormality ●● When clinical findings are suggestive of a pituitary lesion Note: MRI is generally preferred over CT for evaluation of pituitary lesions

Papilledema

Post-operative or post-procedure evaluation Note: For post-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pre-operative evaluation or pre-procedure evaluation Note: For pre-operative evaluation of conditions not specifically referenced elsewhere in this guideline

♦ List may not be exclusive | CT Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 12 Common Diagnostic Indications Pseudotumor cerebri Seizures and epilepsy Complex febrile seizure ●● Patient is between 6 months and five years of age and febrile (any one of the following) ○○ More than one seizure during a febrile period ○○ Prolonged seizure lasting more than 15 minutes Note: Imaging is not generally indicated for simple febrile seizures24-28 Neonatal/Infantile seizure29 ●● Patient is age two years or younger (any one of the following) ○○ Afebrile ○○ If initial CT or MRI is non-diagnostic, periodic follow-up could be considered at 6-month intervals up to 30 months27 Childhood/Adolescent seizure ●● Patient is older than two years of age (any one of the following) ○○ Focal neurologic finding of cognitive impairment, motor impairment, or persistent neurologic deficit after the seizure (postictal) 28 ○○ Idiopathic epilepsy with atypical clinical course26-30 ○○ Partial seizures26,28 ○○ Seizures increasing in frequency and severity despite optimal medical management ○○ When there is an electroencephalogram (EEG) abnormality that is inconsistent with idiopathic epilepsy (Also referred to as benign epilepsy) Note: Seizure, defined as a single episode of excessive neuronal activity that causes changes in attention and behavior.31 Epilepsy, defined as the presence of recurrent seizures. Idiopathic epilepsy includes benign childhood epilepsy with centrotemporal spikes (rolandic) (BECTS), childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and simple febrile seizures) Syncope or near syncope (any one of the following) ●● Abnormality on neurological examination ●● Persistent neurological symptom ●● Seizure activity was witnessed or highly suspected at the time of the syncope Note: Syncope is defined as complete loss of consciousness. Near syncope is defined as partial loss of consciousness. Trauma, head (any one of the following) ●● Evaluation and/or management unless the patient is low risk (see table below for what constitutes low risk24,32-36 ●● Non-accidental injury (NAI)37 Note: Guideline does not apply to patients with bleeding diathesis or intracranial shunts. Pediatric patient is considered to be Low Risk when all of the following clinical features are present: Infants (younger than 2 years of age) Children (2 years of age and older) ●● Normal mental status ●● Normal mental status ●● Normal behavior ●● Normal behavior ●● No loss of consciousness ●● No loss of consciousness ●● No scalp hematoma other than frontal ●● No vomiting ●● No history of high risk motor vehicle collision ●● No severe headache (MVC) (High risk MVCs are those with patient ●● No signs of (hemotympanum, rhinorrhea, otorrhea, ejection, passenger death, or rollover, or “raccoon” eyes, post auricular hematoma) pedestrian or unhelmeted bicyclist struck by a ●● No history of high risk motor vehicle collision (MVC) (High risk MVCs are motor vehicle) those with patient ejection, passenger death, or rollover, or pedestrian or ●● No history of high risk non-MVC trauma (High unhelmeted bicyclist struck by a motor vehicle) risk non-MVC trauma are falls greater than 3 ●● No history of high risk non-MVC trauma (High risk non-MVC trauma feet or head struck by a high-impact object [e.g. are falls greater than 5 feet or head struck by a high-impact object [e.g. baseball]) baseball])

♦ List may not be exclusive | CT Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 13 Common Diagnostic Indications Tumor, benign or malignant (any one♦ of the following) ●● Evaluation of metastatic disease ●● Evaluation of primary intracranial tumors ●● Surveillance of unexplained mass/lesion identified on prior imaging without pathologic tissue confirmation (examples include suspected arachnoid cyst or epidermoid cyst)

Vascular abnormality ●● Structural abnormality (any one♦ of the following) ○○ Aneurysm ○○ Arteriovenous malformation (AVM) ○○ Cavernous malformation ○○ Cerebral vein thrombosis ○○ Dural arteriovenous fistula (DAVF) ○○ Dural venous sinus thrombosis ○○ Venous angioma Note: Either CTA or MRA may be preferred

Ventricular shunt assessment

Vertigo and dizziness

Visual disturbance ●● When unexplained by ophthalmologic exam and patient history Note: Includes visual field loss, diplopia, and other alterations in vision CT is generally not indicated in the following clinical situations The indications listed in this section generally do not require advanced imaging using CT. If there are circumstances that require CT imaging, a peer-to-peer discussion may be required. Migraine Note: Imaging is not generally indicated for typical presentations. For atypical cases see headache, new onset or headache, persistent or recurrent References 1. American Academy of Otolaryngology — Head and Neck Foundation. Choosing Wisely: Five Things Physicians and Patients Should Question. ABIM Foundation; February 21, 2013. www.choosingwisely.org 2. Stac hler RJ, Chandrasekhar SS, Archer SM, et al. Clinical practice guideline: Sudden hearing loss. Otolaryngol Head Neck Surg. 2012;146(3 Suppl):S1-S35. 3. Accardo J, Kammann H, Hoon AH Jr. Neuroimaging in cerebral palsy. J Pediatr. 2004;145(2 Suppl):S19-S27. 4. Momen AA, Jelodar G, Dehdashti H. Brain magnetic resonance imaging findings in developmentally delayed children. Int J Pediatr. 2011;2011:386984. 5. Shevell M, Ashwal S, Donley D, et al. Practice parameter: evaluation of the child with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of and The Practice Committee of the Child Neurology Society. Neurology. 2003;60(3):367-380. 6. Truiwit CL, Barkovich AJ, Koch TK, Ferriero DM. Cerebral palsy: MR finding in 40 patients. Am J Neuroradiol. 1992;13:67-78. 7. Candy EJ, Hoon AH, Capute AJ, Bryan RN. MRI in motor delay: important adjunct to classification of cerebral palsy. Pediatr Neurol. 1993;9:421-429. 8. Ashwal S, Russman BS, Blasco P, et al. Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2004;62(6):851-863. 9. Medina LS, D’Souza B, Vasconcellos E. Adults and children with headache: evidence-based diagnostic evaluation.

♦ List may not be exclusive | CT Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 14 Neuroimag Clin N Am. 2003;13:225-235. 10. Alexiou GA, Argyropoulou MI. Neuroimaging in childhood headache: a systematic review. Pediatr Radiol. 2013;43(7):777-784. 11. Frishberg BM, Rosenberg JH, Matchar DB, et al; U.S. Headache Consortium. Evidence-Based Guidelines in the Primary Care Setting: Neuroimaging in Patients with Non-acute Headache. American Academy of Neurology; April 2000. 12. Alehan FK. Value of neuroimaging in the evaluation of neurologically normal children with recurrent headache. J Child Neurol. 2002 Nov;17(11):807-809. 13. Dooley JM, Camfield PR, O’Neill M, Vohra A. The value of CT scans for children with headaches. Can J Neurol Sci. 1990;17:309-310. 14. Medina LS, Pinter JD, Zurakowski D, Davis R, Kuban K, Barnes PD. Children with headache: clinical predictors of the surgical space-occupying lesions and the role of neuroimaging. Radiology. 1997;202:819-824. 15. Strain JD. ACR Appropriateness Criteria on headache-child. J Am Coll Radiol. 2007;4(1):18-23. 16. Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 1: Practical aspects for neuroradiologists. AJNR Am J Neuroradiol. 2012 Feb;33(2):211-217. 17. Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 2: syndromic and acquired causes. AJNR Am J Neuroradiol. 2012 Mar;33(3):399-406. 18. Morzaria S, Westerberg BD, Kozak FK. Evidence-based algorithm for the evaluation of a child with bilateral sensorineural hearing loss. J Otolaryngol. 2005 Oct;34(5):297-303. 19. Ashwal S, Michelson D, Plawner L, Dobyns WB, Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Practice parameter: Evaluation of the child with microcephaly (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2009;73(11):887-897. 20. Baguley D, McFerran D, Hall D. Tinnitus. Lancet. 2013 Nov 9;382(9904):1600-1607. 21. Sajisevi M, Weissman JL, Kaylie DM. What is the role of imaging in tinnitus? Laryngoscope. 2014 Mar;124(3):583-4. 22. Vattoth S, Shah R, Curé JK. A compartment-based approach for the imaging evaluation of tinnitus. AJNR Am J Neuroradiol. 2010 Feb;31(2):211-218. 23. Weissman JL, Hirsch BE. Imaging of tinnitus: a review. Radiology. 2000;216:342-349 24. American Academy of Pediatrics. Choosing Wisely: Five Things Physicians and Patients Should Question. ABIM Foundation; February 21, 2013. www.choosingwisely.org 25. American Academy of Pediatrics.Subcommittee on Febrile Seizures. Febrile seizures: Guideline for the neurodiagnostic evaluation of the child with a simple febrile seizure. Pediatrics. 2011 Feb;127(2):389-394. 26. National Health Services, National Institute for Clinical Excellence. The epilepsies: The diagnosis and management of the epilepsies in adults and children in primary and secondary care. October 2004. http://www.nice.org.uk/guidance/ cg137. Accessed September 10, 2014. 27. Gaillard WD, Chiron C, Cross JH, et al; ILAE, Committee for Neuroimaging, Subcommittee for Pediatric. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia. 2009 Sep;50(9):2147-2153. 28. Hirtz D, Ashwal S, Berg A, et al. Practice parameter: evaluating a first nonfebrile seizure in children: report of the quality standards subcommittee of the American Academy of Neurology, The Child Neurology Society, and The American Epilepsy Society. Neurology. 2000 Sep 12;55(5):616-623. 29. Hsieh DT, Chang T, Tsuchida TN, et al. New-onset afebrile seizures in infants: role of neuroimaging. Neurology. 2010;74(2):150-156. 30. Berg AT, Mathern GW, Bronen RA, et al. Frequency, prognosis and surgical treatment of structural abnormalities seen with magnetic resonance imaging in childhood epilepsy. Brain. 2009;132(Pt 10):2785-2797. 31. Fisher RS, van Emde Boas W, Blume W, et al. Epileptic seizures and epilepsy: definitions proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE). Epilepsia. 2005;46(4):470-472. 32. Duhaime AC, Alario AJ, Lewander WJ, et al. Head injury in very young children: mechanisms, injury types, and ophthalmologic findings in 100 hospitalized patients younger than 2 years of age. Pediatrics.1992; 90:179-185. 33. Easter JS, Bakes K, Dhaliwal J, Miller M, Caruso E, Haukoos JS. Comparison of PECARN, CATCH, and CHALICE rules for children with minor head injury: a prospective cohort study. Ann Emerg Med. 2014 Aug;64(2):145-152, 152.e1-5. 34. Schonfeld D, Bressan S, Da Dalt L, Henien MN, Winnett JA, Nigrovic LE. Pediatric Emergency Care Applied Research Network head injury clinical prediction rules are reliable in practice. Arch Dis Child. 2014;99(5):427-431. 35. American Association of Neurological Surgeons. Choosing Wisely: Five Things Physicians and Patients Should

♦ List may not be exclusive | CT Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 15 Question. ABIM Foundation; 2014. Available at www.choosingwisely.org. 36. Kuppermann N, Holmes, JF, Dayan PS, et al; Pediatric Emergency Care Applied Research Network (PECARN). Identification of children at very low-risk of clinically-important brain injuries after head trauma:A prospective cohort study. Lancet. 2009;374(9696):1160-1170. 37. Meyer JS, Gunderman R, Coley BD, et al. ACR Appropriateness Criteria® on suspected physical abuse-child. J Am Coll Radiol. 2011;8(2):87-94. 38. Edlow JA, Panagos PD, Godwin SA, Thomas TL, Decker WW. Clinical policy: Critical issues in the evaluation and management of adult patients presenting to the emergency department with acute headache. J Emerg Nurs. 2009;35(3):e43-e71.

♦ List may not be exclusive | CT Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 16 CT Angiography (CTA) Head: Cerebrovascular – Pediatrics

CPT Codes 70496 ����������������� Computed tomographic angiography, head, with contrast material(s), including noncontrast images, if performed, and image postprocessing Angiography includes imaging of all blood vessels, including arteries and veins. The code above includes CT Venography. Standard Anatomic Coverage ●● CTA may be performed to assess the major intracranial arteries of the anterior and posterior circulations (including the Circle of Willis) as well as the venous structures (major veins and dural venous sinuses) ●● For specific clinical indications, exams may be tailored to the region of interest Technology Considerations ●● CTA of the head is an alternative exam in patients who cannot undergo MRA ●● During diagnostic interpretation, it is extremely useful to have images displayed on a workstation capable of multiplanar reformations and three-dimensional reconstructions Common Diagnostic Indications The following diagnostic indications for head CTA are accompanied by pre-test considerations as well as clinical supporting data and prerequisite information

Abnormality detected on other imaging study which requires additional clarification to direct treatment

Aneurysm (any one♦ of the following) ●● Family history of intracranial aneurysm ●● Follow-up of known or suspected intracranial aneurysm ●● Hereditary disorder, such as autosomal dominant polycystic kidney disease (10%-20% occurrence of aneurysm), Ehlers Danlos syndrome type IV, and Neurofibromatosis type 1

Arteriovenous malformation (AVM)

Congenital anomaly of the cerebral circulation ●● Diagnosis prior to an interventional procedure Note: This includes persistent fetal carotid vertebrobasilar anastomosis (e.g. hypoglossal artery, otic artery, proatlantal artery, or trigeminal artery). Most congenital variations are incidental findings with no clinical significance1

Dural arteriovenous fistula (DAVF)

Dissection

Endovascular neurointerventional procedure ●● For post-treatment evaluation (any one of the following) ○○ Arteriovenous malformation (AVM) ○○ Dural arteriovenous fistula (DAVF) ○○ Intracranial aneurysm

♦ List may not be exclusive | CTA of the Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 17 Common Diagnostic Indications Headache (any one of the following) ●● Exertional headache ●● Positional headache ●● Sudden onset of worst headache of life

Intracranial hemorrhage ●● For identification of the source of hemorrhage Note: CT is the preferred technique for evaluation of acute intracranial hemorrhage. MRI is generally preferred for evaluation of subacute and chronic hemorrhage

Intramural hematoma

Post-operative or post-procedure evaluation Note: For post-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pre-operative evaluation or pre-procedure evaluation Note: For pre-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Recent cerebrovascular accident (CVA) ●● Demonstrated on head CT or MRI

Stenosis or occlusion of the carotid or cerebral arteries (Any one of the following) ●● Known steno-occlusive disease, particularly (any one♦ of the following): ○○ Idiopathic progressive arteriopathy of childhood ○○ Moyamoya disease ○○ Sickle cell disease ●● Patient is symptomatic (any one♦ of the following) ○○ Confusion ○○ Difficulty speaking or understanding speech ○○ Dizziness ○○ Gait disturbance ○○ Loss of balance or coordination ○○ Loss of consciousness ○○ Numbness, weakness or paralysis of the face, arm or leg, on one side of the body ○○ Sudden severe headache, that is unexplained ○○ Visual disturbance, particularly in one eye Note: Stenosis or occlusion in the pediatric population is usually a result of vascular abnormalities from a systemic or congenital disease and rarely from atherosclerotic disease

♦ List may not be exclusive | CTA of the Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 18 Common Diagnostic Indications

Stenosis or occlusion of the vertebral or basilar arteries ●● Signs or symptoms of vertebrobasilar insufficiency (VBI) or vertebral basilar ischemia (any one♦ of the following) ○○ Acute sensorineural hearing loss ○○ Ataxia ○○ Diplopia ○○ Dysarthria ○○ Dysphagia ○○ Facial numbness or paresthesia ○○ Limb or trunk sensory deficit ○○ Loss of taste sensation ○○ Motor paresis ○○ Nystagmus ○○ Syncope ○○ Vertigo ○○ Visual field defect Note: Symptoms of VBI are usually temporary, due to diminished blood flow in the posterior circulation of the brain. Stenosis or occlusion in the pediatric population is usually a result of vascular abnormalities from a systemic or congenital disease and rarely from atherosclerotic disease

Thromboembolic disease of major intracranial arterial or venous systems Note: Includes dural venous sinus thrombosis

Tinnitus ●● Pulsatile tinnitus for vascular etiology

Traumatic vascular injury

Vascular supply to tumor

Vasculitis

Venous thrombosis or compression References 1. Uchino A, Sawada A, Takase Y, Kudo S.MR angiography of anomalous branches of the internal carotid artery. AJR Am J Roentgenol. 2003 Nov;181(5):1409-1414.

♦ List may not be exclusive | CTA of the Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 19 Magnetic Resonance Imaging (MRI) Head/Brain – Pediatrics

CPT Codes 70551 ����������������� MRI Head, without contrast 70552 ����������������� MRI Head, with contrast 70553 ����������������� MRI Head, without contrast, followed by re-imaging with contrast Standard Anatomic Coverage ●● From skull base to vertex, covering the entire calvarium and intracranial contents, including the internal auditory canals ●● Scan coverage may vary, depending on the specific clinical indication Technology Considerations Modality overview ●● MRI of the head is preferable to CT in most clinical scenarios, due to its superior contrast resolution and lack of beam-hardening artifact adjacent to the petrous bone (which may limit visualization in portions of the posterior fossa and brainstem on CT). Exceptions to the use of brain MRI as the neuroimaging procedure of choice and situations with preferred head imaging using CT include: osseous assessment of the calvarium, skull base and maxillofacial bones, including detection of calvarial and facial bone fractures; calcified lesions; initial evaluation of recent craniocerebral trauma; and acute intracranial hemorrhage (parenchymal; subarachnoid; subdural; epidural) ●● MRI is more sensitive for detection of shearing trauma to the brain and diffuse axonal injury. It is also the preferred technique for assessment of subacute and chronic intracranial hemorrhage ●● CT of the head is an alternative exam in patients who cannot undergo MRI ●● Images of the pituitary gland, maxillary sinuses or internal auditory canals (IACs) are included within the single assigned CPT code for MRI imaging of the head and are not separately billable as multiple concurrent head MRI exams ●● Imaging studies of the head and neck are inherently bilateral, thus duplicate requests for these studies are inappropriate Indications for contrast The majority of indications for brain MRI do not require contrast (CPT 70551). MRI brain with and without contrast (CPT 70553) is appropriate when there is concern for neoplasm, infection/inflammation and vascular malformations, and may be indicated for the following pathways (note some clinical scenarios within these indications may not require contrast): ●● Abnormality detected on other imaging study which ●● Neurocutaneous disorder requires additional clarification to direct treatment ●● Neuroendocrine abnormality ●● Ataxia ●● Neurological findings/deficits ●● Headache ●● Post-operative or post-procedure evaluation ●● Hearing loss ●● Pseudotumor cerebri ●● Hydrocephalus ●● Seizures and epilepsy ●● Infectious or inflammatory process ●● Tumor ●● Multiple sclerosis or other demyelinating white matter ●● Vascular abnormality disease ●● Visual disturbance Imaging with contrast is rarely appropriate for the evaluation of other indications for brain MRI in this guideline

♦ List may not be exclusive | MRI of the Head/Brain | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 20 Common Diagnostic Indications The following diagnostic indications for head MRI are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Abnormality detected on other imaging study which requires additional clarification to direct treatment

Abnormality on neurological exam when suggestive of intracranial pathology (All of the following) ●● Findings must be considered within the context of a differential diagnosis ●● At least one temporal characteristic: ○○ New onset ○○ Persistent ○○ Progressive ●● At least one abnormality on neurological exam (see Table 1 below) Note: The following neurologic symptoms have individual guidelines. For the evaluation of any of these neurologic symptoms, please see their individual guideline: Confusion / altered mental status (see Mental status changes), Headache (atraumatic), seizures (see Seizure disorder), Syncope (includes loss of consciousness), Tinnitus, Vertigo and dizziness (includes lightheadedness) Table 1: Neurologic abnormalities include: Cognitive Sensory Motor ●● Memory loss ●● Visual deficits – see visual ●● Weakness ●● Personality change disturbance guideline ○○ Hemiparesis or hemiplegia ●● Spatial neglect ●● Facial numbness ○○ Monoparesis ●● Agnosia ●● Paresthesia documented on ○○ Facial weakness ●● Apraxia neurological exam ●● Neurologic gait abnormality (including ataxia, ●● Language deficits ●● Impairments of taste and smell spastic, circumduction/magnetic) (including aphasia (including anosmia, paraosmi and ●● Ataxia or impaired voluntary and paraphasia) dysgeusia) coordination(including dysarthria,dysphagia dysmetria, dysdiadokinesia) ●● Abnormal reflexes (hyperreflexia (when unilateral or asymmetric), extensor plantar response (Babinski )and Hoffmann’s sign)

Ataxia, congenital or hereditary (any one♦ of the following) ●● Ataxia-telangiectasia ●● Congenital anomaly (particularly one of the posterior fossa) ●● Fragile X syndrome

Cerebrovascular accident (CVA or stroke) or transient ischemic attack (TIA) ●● Sudden onset of new clinical finding on exam that suggests CVA or TIA Note: Includes acute vascular injury. Among patients being evaluated for CVA and possible thrombolytic therapy, unenhanced CT is generally preferred as the initial modality (within the first 24 hours of symptom onset) to detect a possible hemorrhagic stroke or mass lesion

Chiari malformation (Arnold-Chiari malformation)

♦ List may not be exclusive | MRI of the Head/Brain | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 21 Common Diagnostic Indications

Congenital anomaly, not otherwise listed ●● Diagnosis or management (any one♦ of the following) ○○ Agenesis of the corpus callosum ○○ Blake pouch cyst ○○ Cephalocele ○○ Dandy walker ○○ De Morsier syndrome ○○ Encephalocele ○○ Focal cortical dysplasia ○○ Grey matter heterotopia ○○ Hemimegalencephaly ○○ Holoprosencephaly ○○ Joubert syndrome ○○ Nasal glioma ○○ Rhombencephalosynapsis ○○ Schizencephaly

Developmental delay4-6 (any one of the following) ●● Cerebral palsy4, 8-10 ●● Global developmental delay (any two of the following) ○○ Activities of daily living ○○ Cognition7 ○○ Motor skills (gross/fine) ○○ Social/personal ○○ Speech/language Note: Global developmental delay is defined as significant delay or loss of milestones in at least two of the domain’s listed above

Diffuse axonal injury

Encephalopathy

Headache, new onset12,13,19 (any one of the following) ●● Family or personal medical history of disorders that may predispose one to central nervous system (CNS) lesions and clinical/laboratory findings that suggest CNS involvement (Including but not limited to vascular malformations, aneurysms, brain neoplasms, infectious/inflammatory conditions such as sarcoidosis or personal history of meningitis and tuberculosis) ●● Headache that awakens the patient repeatedly from sleep or develops upon awakening ●● Neurological abnormalities such as nystagmus, papilledema, gait or motor disturbances ●● Patient is developmentally delayed ●● Sudden onset and severe headache (includes thunderclap headache or worst headache of life) Note: New onset headaches are headaches that have occurred for the first time within 30 days of the imaging request. If associated with trauma or infection, see separate indication for Trauma or Infectious/Inflammatory

♦ List may not be exclusive | MRI of the Head/Brain | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 22 Common Diagnostic Indications

Headache, persistent or recurrent (any one of the following) ●● Abnormal neurological exam14-17 (any one of the following) ○○ Abnormal reflexes (hyperreflexia/hyporeflexia) ○○ Altered mental status (confused or disoriented) ○○ Cranial nerve deficit ○○ Gait /motor dysfunction ○○ Nystagmus ○○ Seizure ○○ Sensory deficit (numbness, paresthesia) ○○ Sign of increased intracranial pressure (increased head circumference, vomiting, papilledema, headache worse with valsalva) ○○ Vomiting ●● Change in quality (pattern or intensity) of a previously stable headache13,14 ●● Headache persisting for a period of up to 6 months duration and not responsive to medical treatment, when no prior imaging has been done to evaluate the headache Note: Persistent headaches are those lasting more than 30 days; recurrent headaches are those where the resolution and recurrence are separated by at least a 30 day period. If separated by less than 30 days, these headaches would be considered persistent

Hearing loss Note: This includes conductive, sensorineural, and mixed hearing loss. CT is generally preferred for conductive and mixed. MRI is generally preferred for sensorineural25-27

Hemorrhage or hematoma ●● For non-traumatic, non-CVA and non-tumor-related intracranial bleed (includes hemorrhage secondary to anticoagulation or blood dyscrasia) Note: CT is the preferred technique for evaluation of acute intracranial hemorrhage; MRI is generally preferred for evaluation of subacute and chronic hemorrhage

Horner’s syndrome

Hydrocephalus (ventriculomegaly) ●● Patient is younger than five (5) months of age following an abnormal or non-diagnostic ultrasound Note: MRI is generally preferred for initial evaluation of patients with hydrocephalus. For patients with an indwelling shunt, CT is usually adequate in the diagnostic follow-up of hydrocephalus

Increased intracranial pressure (any one♦ of the following) ●● Bulging fontanelle ●● Headache worse with valsalva ●● Increased head circumference ●● Papilledema ●● Vomiting

Infectious or inflammatory process (any one♦ of the following) ●● Cerebral or cerebellar abscess ●● Encephalitis (including limbic encephalitis) ●● Meningitis ●● Neurocysticercosis ●● Opportunistic infection, particularly with immunosuppressed or other immunodeficient conditions ●● Subdural empyema

♦ List may not be exclusive | MRI of the Head/Brain | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 23 Common Diagnostic Indications

Macrocephaly ●● Patient’s head circumference is greater than two (2) standard deviations from the mean for his/her age and gender (any one of the following) ○○ Patient is younger than five (5) months of age following non-diagnostic ultrasound ○○ Patient is five (5) months of age or older

Mental status change ●● Abnormality on a neurologic exam

Microcephaly3 ●● Patient’s head circumference is less than two (2) standard deviations from the mean for his/her age and gender

Multiple sclerosis or other white-matter diseases (any one of the following) ●● Diagnosis ●● Evaluation of changes in neurologic signs and symptoms ●● For multiple sclerosis (any one of the following) ○○ Assess asymptomatic disease progression ○○ Evaluate response to treatment ○○ Track disease progression to establish a prognosis

Neurocutaneous disorder (phakomatosis) (any one♦ of the following) ●● Neurofibromatosis ●● Sturge-Weber syndrome ●● Tuberous sclerosis ●● Von Hippel-Lindau disease (VHL)

Neuroendocrine abnormality ●● When clinical findings are suggestive of a pituitary lesion

Papilledema

Post-operative or post-procedure evaluation Note: For post-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pre-operative evaluation or pre-procedure evaluation Note: For pre-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pseudotumor cerebri

♦ List may not be exclusive | MRI of the Head/Brain | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 24 Common Diagnostic Indications Seizures and epilepsy Complex febrile seizure19 ●● Patient is between 6 months and five years of age and febrile (any one of the following) ○○ More than one seizure during a febrile period ○○ Prolonged seizure lasting more than 15 minutes Note: Imaging is not generally indicated for simple febrile seizures1,2,21,23,24 Neonatal/Infantile seizure20 ●● Patient is age two years or younger (any one of the following) ○○ Afebrile ○○ If initial CT or MRI is non-diagnostic, periodic follow-up could be considered at 6-month intervals up to 30 months21 Childhood/Adolescent seizure ●● Patient is older than two years of age (any one of the following) ○○ Focal neurologic finding of cognitive impairment, motor impairment, or persistent neurologic deficit after the seizure (postictal)23 ○○ Idiopathic epilepsy with atypical clinical course20-24 ○○ Partial seizures23,24 ○○ Seizures increasing in frequency and severity despite optimal medical management ○○ When there is an electroencephalogram (EEG) abnormality that is inconsistent with idiopathic epilepsy (Also referred to as benign epilepsy) Note: Seizure, defined as a single episode of excessive neuronal activity that causes changes in attention and behavior18. Epilepsy, defined as the presence of recurrent seizures11. Idiopathic epilepsy includes benign childhood epilepsy with centrotemporal spikes (rolandic) (BECTS), childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and simple febrile seizures)

Syncope or near syncope (any one of the following) ●● Abnormality on neurological examination ●● Persistent neurological symptom ●● Seizure activity was witnessed or highly suspected at the time of the syncope Note: Syncope is defined as complete loss of consciousness. Near syncope is defined as partial loss of consciousness.

Trauma Note: Includes non-accidental trauma.32 Acute bleeding is better imaged with CT

Tumor, benign or malignant (any one♦ of the following) ●● Evaluation of metastatic disease ●● Evaluation of primary intracranial tumors ●● Surveillance of unexplained mass/lesion identified on prior imaging without pathologic tissue confirmation (Examples include suspected arachnoid cyst or epidermoid cyst)

♦ List may not be exclusive | MRI of the Head/Brain | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 25 Common Diagnostic Indications Vascular abnormality ●● Structural abnormality (any one♦ of the following) ○○ Aneurysm ○○ Arteriovenous malformation (AVM) ○○ Cavernous malformation ○○ Cerebral vein thrombosis ○○ Dural arteriovenous fistula (DAVF) ○○ Dural venous sinus thrombosis ○○ Venous angioma Note: CTA or MRA may be preferred

Ventricular shunt assessment

Vertigo and dizziness

Visual disturbance ●● When unexplained by ophthalmologic exam and patient history Note: Includes visual field loss, diplopia, and other alterations in vision MRI is generally not indicated in the following clinical situations The indications listed in this section generally do not require advanced imaging using MRI. If there are circumstances that require MRI imaging, a peer-to-peer discussion may be required. Migraine Note: Imaging is not generally indicated for typical presentations. For atypical cases see headache, new onset or headache, persistent or recurrent References 1. American Academy of Pediatrics. Five Things Physicians and Patients Should Question. ABIM Foundation; February 21, 2013. www.choosingwisely.org 2. American Academy of Pediatrics.Subcommittee on Febrile Seizures. Febrile seizures: Guideline for the neurodiagnostic evaluation of the child with a simple febrile seizure. Pediatrics. 2011 Feb;127(2):389-394. 3. Ashwal S, Michelson D, Plawner L, et al. Practice parameter: Evaluation of the child with microcephaly (an evidence- based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2009;73(11):887-897. 4. Accardo J, Kammann H, Hoon AH Jr. Neuroimaging in cerebral palsy. J Pediatr. 2004;145(2 Suppl):S19-S27. 5. Momen AA, Jelodar G, Dehdashti H. Brain magnetic resonance imaging findings in developmentally delayed children. Int J Pediatr. 2011;2011:386984. 6. Shevell M, Ashwal S, Donley D, et al. Practice parameter: evaluation of the child with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society. Neurology. 2003 Feb 11;60(3):367-380. 7. Curry CJ, Stevenson RE, Aughton D, et al. Evaluation of mental retardation: recommendations of a Consensus Conference: American College of . Am J Med Genet. 1997;72(4):468-477. 8. Truiwit CL, Barkovich AJ, Koch TK, Ferriero DM. Cerebral palsy: MR finding in 40 patients. Am J Neuroradiol. 1992;13:67-78. 9. Candy EJ, Hoon AH, Capute AJ, Bryan RN. MRI in motor delay: important adjunct to classification of cerebral palsy. Pediatr Neurol. 1993;9:421-9. 10. Ashwal S, Russman BS, Blasco P, et al. Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2004;62(6):851-863. 11. Rastogi S, Lee C, Salamon N. Neuroimaging in pediatric epilepsy: a multimodality approach. Radiographics. 2008;28(4):1079-1095

♦ List may not be exclusive | MRI of the Head/Brain | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 26 12. Medina LS, D’Souza B, Vasconcellos E. Adults and children with headache: evidence-based diagnostic evaluation. Neuroimag Clin N Am. 2003;13:225-235. 13. Alexiou GA, Argyropoulou MI. Neuroimaging in childhood headache: a systematic review. Pediatr Radiol. 2013 Jul;43(7):777-784. 14. Frishberg BM, Rosenberg JH, Matchar DB, et al; U.S. Headache Consortium. Evidence-Based Guidelines in the Primary Care Setting: Neuroimaging in Patients with Non-acute Headache. American Academy of Neurology; April 2000. 15. Alehan FK. Value of neuroimaging in the evaluation of neurologically normal children with recurrent headache. J Child Neurol. 2002;17(11):807-809. 16. Strain JD. ACR Appropriateness Criteria on headache-child. J Am Coll Radiol. 2007;4(1):18-23. 17. Medina LS, Pinter JD, Zurakowski D, Davis R, Kuban K, Barnes PD. Children with headache: clinical predictors of the surgical space-occupying lesions and the role of neuroimaging. Radiology. 1997;202:819-824. 18. Fisher RS, van Emde Boas W, Blume W, et al. Epileptic seizures and epilepsy: definitions proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE). Epilepsia.2005;46(4):470-472. 19. Committee on Quality Improvement, Subcommittee on Febrile Seizures. Practice Parameter: Long-term Treatment of the Child With Simple Febrile Seizures. Pediatrics. 1999;103;1307. 20. Hsieh DT, Chang T, Tsuchida TN, et al. New-onset afebrile seizures in infants: role of neuroimaging. Neurology. 2010;74(2):150-156. 21. Gaillard WD, Chiron C, Cross JH, et al.; ILAE, Committee for Neuroimaging, Subcommittee for Pediatric. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia. 2009;50(9):2147-2153. 22. Berg AT, Mathern GW, Bronen RA, et al. Frequency, prognosis and surgical treatment of structural abnormalities seen with magnetic resonance imaging in childhood epilepsy. Brain. 2009;132(Pt 10):2785-2597. 23. National Health Services, National Institute for Clinical Excellence. The epilepsies: The diagnosis and management of the epilepsies in adults and children in primary and secondary care. October 2004. http://www.nice.org.uk/guidance/ cg137. Accessed September 10, 2014. 24. Hirtz D, Ashwal S, Berg A, et al. Practice parameter:evaluating a first nonfebrile seizure in children: report of the quality standards subcommittee of the American Academy of Neurology, the Child Neurology Society, and the American Epilepsy Society. Neurology. 2000; 55:616-623. 25. Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 1: Practical aspects for neuroradiologists. AJNR Am J Neuroradiol. 2012 Feb;33(2):211-217. 26. Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 2: syndromic and acquired causes. AJNR Am J Neuroradiol. 2012 Mar;33(3):399-406. 27. Morzaria S, Westerberg BD, Kozak FK. Evidence-based algorithm for the evaluation of a child with bilateral sensorineural hearing loss. J Otolaryngol. 2005 Oct;34(5):297-303. 28. Baguley D, McFerran D, Hall D. Tinnitus. Lancet. 2013 Nov 9;382(9904):1600-1607. 29. Sajisevi M, Weissman JL, Kaylie DM. What is the role of imaging in tinnitus? Laryngoscope. 2014 Mar;124(3):583-584. 30. Vattoth S, Shah R, Curé JK. A compartment-based approach for the imaging evaluation of tinnitus. AJNR Am J Neuroradiol. 2010 Feb;31(2):211-218. 31. Weissman JL, Hirsch BE. Imaging of tinnitus: a review. Radiology. 2000;216:342-349 32. Meyer JS, Gunderman R, Coley BD, et al. ACR Appropriateness Criteria® on suspected physical abuse-child. J Am Coll Radiol. 2011;8(2):87-94.

♦ List may not be exclusive | MRI of the Head/Brain | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 27 MR Angiography (MRA) Head: Cerebrovascular – Pediatrics

CPT Codes 70544 ����������������� Magnetic resonance angiography, head, without contrast 70545 ����������������� Magnetic resonance angiography, head, with contrast 70546 ����������������� Magnetic resonance angiography, head, without contrast, followed by re-imaging with contrast Angiography includes imaging of all blood vessels, including arteries and veins. The codes above include MR Venography. Standard Anatomic Coverage ●● MRA may be performed to assess the major intracranial arteries of the anterior and posterior circulations (including the Circle of Willis) as well as the venous structures (major cerebral veins and dural venous sinuses) ●● For specific clinical indications, exams may be tailored to the region of interest Technology Considerations ●● MRA refers to a group of diverse MR pulse sequences. These include time-of-flight (TOF) imaging, phase contrast (PC) techniques and three-dimensional (3-D), T1-weighted gradient echo acquisitions obtained during intravenous bolus infusion of a paramagnetic contrast agent (gadolinium chelate) ●● A workstation is necessary for most MRA studies, to acquire multiplanar reformations, shaded surface displays, volume renderings and maximum intensity projection (MIP) images. Post-processing of MRA data with a MIP reconstruction algorithm allows for 3-dimensional images to be rotated and viewed in different planes, improving visualization of superimposed vessels ●● CTA of the head is an alternative exam in patients who cannot undergo MRA ●● An MRA of the head includes imaging of the entire arteriovenous system of the brain. Separate requests for concurrent imaging of the arteries and the veins in the head are inappropriate Common Diagnostic Indications The following diagnostic indications for head MRI are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Abnormality detected on other imaging study which requires additional clarification to direct treatment

Aneurysm (any one♦ of the following) ●● Family history of intracranial aneurysm ●● Follow-up of known or suspected intracranial aneurysm ●● Hereditary disorder, such as autosomal dominant polycystic kidney disease (10%-20% occurrence of aneurysm), Ehlers Danlos syndrome type IV, and Neurofibromatosis type I

Arteriovenous malformation (AVM)

Congenital anomaly of the cerebral circulation ●● Diagnosis prior to an interventional procedure Note: This includes persistent fetal carotid vertebrobasilar anastomosis (e.g. hypoglossal artery, otic artery, proatlantal artery, or trigeminal artery). Most congenital variations are incidental findings with no clinical significance1

Dural arteriovenous fistula (DAVF)

Dissection

♦ List may not be exclusive | MRA of the Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 28 Common Diagnostic Indications Endovascular neurointerventional procedure ●● For post-treatment evaluation (any one of the following) ○○ Arteriovenous malformation (AVM) ○○ Dural arteriovenous fistula (DAVF) ○○ Intracranial aneurysm

Headache (any one of the following) ●● Exertional headache ●● Positional headache ●● Sudden onset of worst headache of life

Intracranial hemorrhage ●● For identification of the source of hemorrhage Note: CT is the preferred technique for evaluation of acute intracranial hemorrhage. MRI is generally preferred for evaluation of subacute and chronic hemorrhage

Intramural hematoma

Multiple sclerosis Note: Evaluation of venous structures to assess for venous stenosis as a cause of multiple sclerosis (referred to as chronic cerebrospinal venous insufficiency [CCSVI]) is considered not medically appropriate. MRV in preparation for either a neurosurgical or percutaneous procedure to treat multiple sclerosis is therefore inappropriate.

Post-operative or post-procedure evaluation Note: For post-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pre-operative evaluation or pre-procedure evaluation Note: For pre-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Recent cerebrovascular accident (CVA) ●● Demonstrated on head CT or MRI

Stenosis or occlusion of the carotid or cerebral arteries (Any one of the following) ●● Known steno-occlusive disease, particularly (any one♦ of the following): ○○ Idiopathic progressive arteriopathy of childhood ○○ Moyamoya disease ○○ Sickle cell disease ●● Patient is symptomatic (any one♦ of the following) ○○ Confusion ○○ Difficulty speaking or understanding speech ○○ Dizziness ○○ Gait disturbance ○○ Loss of balance or coordination ○○ Loss of consciousness ○○ Numbness, weakness or paralysis of the face, arm or leg, on one side of the body ○○ Sudden severe headache, that is unexplained ○○ Visual disturbance, particularly in one eye Note: Stenosis or occlusion in the pediatric population is usually a result of vascular abnormalities from a systemic or congenital disease and rarely from atherosclerotic disease

♦ List may not be exclusive | MRA of the Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 29 Common Diagnostic Indications

Stenosis or occlusion of the vertebral or basilar arteries ●● Sign or symptom of vertebrobasilar insufficiency (VBI) or vertebral basilar ischemia (any one♦ of the following) ○○ Acute sensorineural hearing loss ○○ Ataxia ○○ Diplopia ○○ Dysarthria ○○ Dysphagia ○○ Facial numbness or paresthesia ○○ Limb or trunk sensory deficit ○○ Loss of taste sensation ○○ Motor paresis ○○ Nystagmus ○○ Syncope ○○ Vertigo ○○ Visual field defect Note: Symptoms of VBI are usually temporary, due to diminished blood flow in the posterior circulation of the brain. Stenosis or occlusion in the pediatric population is usually a result of vascular abnormalities from a systemic or congenital disease and rarely from atherosclerotic disease

Thromboembolic disease of major intracranial arterial or venous systems Note: Includes dural venous sinus thrombosis

Tinnitus ●● Pulsatile tinnitus for vascular etiology

Traumatic vascular injury

Vascular supply to tumor

Vasculitis

Venous thrombosis or compression References 1. Uchino A, Sawada A, Takase Y, Kudo S.MR angiography of anomalous branches of the internal carotid artery. AJR Am J Roentgenol. 2003 Nov;181(5):1409-1414.

♦ List may not be exclusive | MRA of the Head | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 30 Functional Magnetic Resonance Imaging (fMRI) Brain – Pediatrics

CPT Codes 70554 ����������������� Magnetic resonance imaging, brain, functional MRI; including test selection and administration of repetitive body part movement and/or visual stimulation, not requiring physician or psychologist administration 70555 ����������������� Magnetic resonance imaging, brain, functional MRI; including test selection and administration of repetitive body part movement and/or visual stimulation, requiring physician or psychologist administration of entire neurofunctional testing Standard Anatomic Coverage ●● From the skull base to vertex, covering the intracranial contents ●● Scan coverage may vary, depending on the specific clinical indication Technology Considerations ●● Functional MRI of the brain may be used to localize eloquent areas in the brain, prior to resection of neoplasm or medically intractable epileptogenic foci ●● Studies have shown excellent agreement in language localization, when comparing functional brain MRI with the Wada test and direct electrical stimulation ●● Advantages of functional brain MRI over a Wada test include the non-invasive technique (not requiring catheter placement and contrast injection), lack of ionizing radiation, shorter time-requirement, lower cost and quicker post- procedural recovery. Additionally, the Wada test is considered limited in right hemisphere dominance ●● Advantages of functional brain MRI over intraoperative electrocortical stimulation include its non-invasive technique and more extensive anatomic brain mapping. Direct electrical stimulation is an invasive procedure, which usually evaluates only one hemisphere (limiting assessment for partial or bilateral language dominance) and usually identifies only eloquent brain regions on the surface of the brain ●● Functional MRI may successfully map primary brain activities related to motor, sensory and language functions. Examples of tasks which may be used include sentence completion (to map language) and bilateral hand squeeze task (for sensory motor mapping) Common Diagnostic Indications The following diagnostic indications for functional MRI (fMRI) of the brain are accompanied by pre-test considerations and supporting clinical data

Brain tumor ●● For pre-operative neurosurgical planning, as a replacement for a Wada test or direct electrical stimulation mapping

Seizures/epilepsy refractory to medical treatment ●● For pre-operative neurosurgical planning, as a replacement for a Wada test or direct electrical stimulation mapping

♦ List may not be exclusive | Functional MRI of the Brain | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 31 Positron Emission Tomography (PET) Brain Imaging – Pediatrics

CPT Codes 78608 ����������������� PET brain, metabolic evaluation Commonly Used Radiopharmaceuticals ●● 2-(fluorine-18) fluoro-2-deoxy-d-glucose (FDG) Scan coverage may vary, depending on the specific clinical indication Technology Considerations ●● Coding conventions call for this code to be used for oncologic scanning of brain tumors Common Diagnostic Indications The following diagnostic indications for brain PET are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Brain tumor (Any one of the following) ●● Initial treatment strategy (diagnosis, staging) ●● Subsequent treatment strategy (follow up to differentiate post-treatment (radiation, surgery, chemotherapy) scarring from residual/recurrent disease)

Seizures/epilepsy refractory to medical treatment1 (All of the following) ●● Patient has failed conventional medical therapy ●● Pre-surgical evaluation to locate the foci of intractable seizure activity References 1. Rastogi S, Lee C, Salamon N. Neuroimaging in pediatric epilepsy: a multimodality approach. Radiographics. 2008;28(4):1079-1095.

♦ List may not be exclusive | PET Brain Imaging | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 32 Computed Tomography (CT) Orbit, Sella Turcica, Posterior Fossa, Temporal Bone, including Mastoids – Pediatrics

CPT Codes 70480 ����������������� CT of orbit, sella or posterior fossa and outer, middle or inner ear, without contrast 70481 ����������������� CT of orbit, sella or posterior fossa and outer, middle or inner ear, with contrast 70482 ����������������� CT of orbit, sella or posterior fossa and outer, middle or inner ear, without contrast, followed by re-imaging with contrast Standard Anatomic Coverage ●● The anatomic coverage and protocol specifications will vary, depending on the clinical indication. Anatomic evaluation includes the internal auditory canals (IACs), posterior fossa, sella turcica, orbits and temporal bone, with the mastoid air cells ●● Targeted evaluation, such as CT of the temporal bones, involves collimated views through the region of interest, often in two imaging planes: axial images (petrous bones through mastoid tips) and coronal views (temporomandibular joints through temporal bones) Technology Considerations ●● CT is often the preferred study for suspected fracture or follow-up of a known fracture, foreign body detection, assessment of calcified lesions and temporal bone evaluation ●● With capability for high-resolution osseous imaging, CT can provide detailed anatomic depiction of the temporal bone anatomy, including the middle and inner ear structures ●● MRI (unless contraindicated) is usually preferred over CT for evaluation of the sella turcica, internal auditory canal regions and visual pathways, as well as for most soft tissue tumor evaluation ●● Bony changes from a sellar, parasellar or orbital mass or infectious process are usually well demonstrated by CT ●● Ordering a CT of the head (CPT codes 70450-70470) in addition to a CT of the orbits is not necessary in most cases ●● Imaging studies of the head and neck are inherently bilateral, thus duplicate requests for these studies are inappropriate Common Diagnostic Indications The following diagnostic indications for CT of the orbit, sella, posterior fossa and temporal bone are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Abnormality detected on other imaging study which requires additional clarification to direct treatment

Cholesteatoma Note: CT is the preferred modality of evaluation for cholesteatoma. This includes both acquired and congenital types of cholesteatoma. Acquired (secondary) cholesteatoma: more common form (98%), presenting as a mass comprised of keratin debris and lined by squamous epithelium. Predisposing conditions are recurrent otitis media or trauma. Congenital (primary) cholesteatoma (epidermoid): uncommon lesion (2%), arising from aberrant embryonic ectodermal rests in middle ear, mastoids or petrous bone.

Cochlear implant ●● Pre-operative or post-operative evaluation

Congenital anomaly of the orbit, temporal bone, sella turcica, or posterior fossa

♦ List may not be exclusive | CT of Orbit, Sella, Fossa | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 33 Common Diagnostic Indications

Foreign body ●● Following non-diagnostic radiograph (any one of the following): ○○ Evaluation for metallic foreign bodies in the orbits ○○ High clinical suspicion for a foreign body Note: Conventional radiographs detect the majority of radiopaque foreign bodies

Hearing loss Note: This includes conductive, sensorineural1-3, and mixed hearing loss. CT is generally preferred for conductive and mixed. MRI is generally preferred for sensorineural

Infectious or inflammatory process (any one♦ of the following) ●● Abscess ●● Cellulitis (including orbital cellulitis) ●● Malignant otitis externa ●● Osteomyelitis ●● Otomastoiditis

Orbital/ocular evaluation ●● Evaluation of the symptoms and objective findings (any one♦ of the following): ○○ Exophthalmos (abnormal protrusion of the eyeball) ○○ Extraocular myopathy ○○ Nystagmus (rapid, involuntary, oscillating ocular movements) ○○ Optic neuritis ○○ Papilledema ○○ Proptosis (forward displacement of the eyeball) ○○ Strabismus (inability of one eye to accomplish binocular vision with the other, due to extra-ocular muscle imbalance) ○○ Thyroid ophthalmopathy ○○ Visual field defect ○○ Visual loss unexplained by ophthalmic evaluation

Orbital pseudotumor

Osseous lesion evaluation Note: Such as fibrous dysplasia, Paget’s disease, and otosclerosis

Pain, localized facial ●● When persistent and unexplained

Post-operative or post-procedure evaluation Note: For post-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pre-operative evaluation or pre-procedure evaluation Note: For pre-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Tinnitus, pulsatile

Skull base evaluation ●● For suspected or known tumors

♦ List may not be exclusive | CT of Orbit, Sella, Fossa | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 34 Common Diagnostic Indications

Trauma (any one♦ of the following) ●● Soft tissue injury ●● Fracture

Tumor, benign or malignant (any one♦ of the following) ●● Evaluation of metastatic disease ●● Evaluation of primary intracranial tumors

Vertigo and dizziness References 1. Huang BY. Zdanski C, Castillow M. Pediatric sensorineural hearing loss, part 1: Practical aspects for neuroradiologists. AJNR Am J Neuroradiol. 2012;33(2):211-217 2. Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 2: syndromic and acquired causes. AJNR Am J Neuroradiol. 2012;33(3):399-406. 3. Morzaria S, Wasterberg BD, Kozak FK. Evidence-based algorithm for the evaluation of a child with bilateral sensorineural hearing loss. J Otolaryngol. 2005;34(5):297-303.

♦ List may not be exclusive | CT of Orbit, Sella, Fossa | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 35 Magnetic Resonance Imaging (MRI) Orbit, Face & Neck (Soft Tissues) – Pediatrics

CPT Codes 70540 ����������������� MRI orbit, face and neck, without contrast 70542 ����������������� MRI orbit, face and neck, with contrast 70543 ����������������� MRI orbit, face and neck, without contrast, followed by re-imaging with contrast Standard Anatomic Coverage ●● Scan coverage is dependent on the specific anatomic area of clinical interest. Exams usually include multi-planar imaging, using different pulse sequences Technology Considerations ●● MRI is usually preferred over CT for evaluation of the sella turcica and visual pathways, unless contraindicated ●● CT is generally the modality of choice for traumatic injury, calcified lesions, localized infection (for example, orbital extension of an adjacent complicated sinusitis), and foreign body evaluation, after initial radiographic evaluation for a radiopaque foreign body ●● CT of the neck is an alternative exam in patients who cannot undergo MRI ●● Imaging studies of the head and neck are inherently bilateral, thus duplicate requests for these studies are inappropriate ●● MRI of the orbit, face and neck is not allowed for imaging the IACs. See MRI of the brain (CPT codes 70551 – 70553) Common Diagnostic Indications The following diagnostic indications for MRI of the orbit, face and neck (soft tissues) are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Abnormality detected on other imaging study which requires additional clarification to direct treatment

Congenital anomaly, not otherwise listed

Glottic lesion ●● Assessment following endoscopic detection

Hoarseness, dysphonia, or vocal cord weakness - suspected to result from recurrent laryngeal nerve pathology1,2,15,16 ●● When hoarseness persists more than one month ●● When laryngoscopy is non-diagnostic or shows vocal cord paralysis Note: Dysphonia is defined as a disorder characterized by altered vocal quality, pitch, loudness, or vocal effort that impairs communication or otherwise reduces quality of life

Horner’s syndrome

Infectious or inflammatory process (Any one♦ of the following) ●● Abscess ●● Cellulitis (including orbital cellulitis) ●● Osteomyelitis

♦ List may not be exclusive | MRI of Orbit, Face, Neck | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 36 Common Diagnostic Indications Lymphadenopathy ●● Following non-diagnostic ultrasound (any one of the following) ○○ Failed to resolve after a six (6) week course of therapy3,4 ○○ Red flag sign6 (any one of the following): ■■ Constitutional symptom ■■ Firm/immobile and larger than 3 cm in diameter3 ■■ Painless ■■ Persistent enlargement on exam for more than 2 weeks ■■ Supraclavicular or posterior triangle ■■ Ulceration ○○ Sonographic features suggestive of nodal malignancy3,5 Note: In the presence of red flag signs, biopsy may be more appropriate than advanced imaging

Neck mass, malignant (biopsy proven) ●● Management and surveillance (any one♦ of the following) ○○ Lymphoma, Hodgkin’s or non-Hodgkin’s ○○ Neuroblastoma ○○ Rhabdomyosarcoma

Neck mass, diagnosis and evaluation ●● Following non-diagnostic ultrasound3,4,6,7 (any one of the following) ○○ Diagnosis when ultrasound demonstrates a solid neck mass other than lymph node ○○ Evaluation of complication (including hemorrhage, infection, mass effect of the airway) Note: For lymph nodes, see Lymphadenopathy

Neck mass, cystic (All of the following) ●● Following non-diagnostic ultrasound ●● Pre-operative evaluation to define the extent of disease Note: This includes brachial cleft cyst, capillary hemangioma, thyroglossal duct cyst, and venolymphatic malformation.

Nasal indications, not otherwise listed (any one♦ of the following) ●● Anosmia ●● Nasal airway obstruction or polyposis refractory to medical therapy ●● Recurrent epistaxis

Obstructive thyroid nodule or thyromegaly (goiter) (any one♦ of the following) ●● Associated with mass effect on the upper airway or esophagus ●● Following non-diagnostic thyroid ultrasound or thyroid scintigraphy ●● Pre-operative evaluation

♦ List may not be exclusive | MRI of Orbit, Face, Neck | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 37 Common Diagnostic Indications Orbital indications, not otherwise listed (any one♦ of the following) ●● Absence of a red reflex on exam (concern for retinoblastoma or rhabdomyosarcoma) ●● Extraocular myopathy ●● Extraocular weakness or nonconjugate eye movements ●● Nystagmus ●● Orbital pseudotumor ●● Optic neuritis ●● Papilledema (swelling and elevation of optic disc) ●● Proptosis ●● Strabismus ●● Thyroid ophthalmopathy ●● Visual loss unexplained by ophthalmic evaluation

Stridor ●● Following non-diagnostic soft tissue neck radiograph and ENT evaluation Note: This applies to subacute and chronic presentations of stridor. Soft tissue radiographs are generally sufficient to diagnose the common causes such as croup and foreign bodies (latter is often a medical emergency). In the pediatric population, common causes of stridor include vascular ring, laryngotracheomalacia, subglottic stenosis

Torticollis (twisted or rotated neck) (any one of the following) ●● Childhood (acquired) torticollis with clinical findings that suggest another cause (such as infection, neoplasm, trauma) ●● Congenital muscular torticollis (fibromatosis colli)8-13 in newborns age 8 months or less11,12 (All of the following) ○○ Following non-diagnostic cervical spine radiograph ○○ Following non-diagnostic ultrasound ○○ Unresponsive to four (4) weeks of conservative therapy Note: Childhood torticollis is typically associated with myositis of the sternocleidomastoid/trapezius14, and advanced imaging is generally not indicated unless there are additional signs/symptoms to suggest another cause. For congenital muscular torticollis, typical cases do not generally require imaging and atypical cases can be confirmed by ultrasound

Trauma to the orbit and face Note: CT preferable for bony assessment

Trauma to the soft tissues of the neck

Tumor, benign or malignant ●● Evaluation of anatomic structures when not otherwise specified in this guideline (any one♦ of the following) ○○ Facial structures ○○ Larynx and subglottic regions ○○ Nasopharynx, oropharynx and hypopharynx ○○ Neck soft tissues, surrounding the airway and glands ○○ Optic nerve ○○ Orbit ○○ Salivary glands ○○ Sella turcica (pituitary tumors including macroadenoma and microadenoma) ○○ Sinuses ○○ Thyroid and parathyroid glands

♦ List may not be exclusive | MRI of Orbit, Face, Neck | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 38 Common Diagnostic Indications

Upper airway obstruction Note: This includes tracheal stenosis

Wegener’s granulomatosis, suspected or known Note: Presentation includes recurrent epistaxis and stridor References 1. American Academy of Otolaryngology — Head and Neck Surgery Foundation. Choosing Wisely: Five Things Physicians and Patients Should Question. ABIM Foundation; February 21, 2013. www.choosingwisely.org.Accessed September 10, 2014. 2. Schwartz SR, Cohen SM, Dailey SH, et al. Clinical practice guideline: hoarseness (dysphonia). Otolaryngol Head Neck Surg. 2009;141(3 Suppl 2):S1-S31. 3. Meier JD, Grimmer JF. Evaluation and management of neck masses in children. Am Fam Physician. 2014;89(5):353- 358. 4. Rozovsky K, Hiller N, Koplewitz BZ, Simanovsky N. Does CT have an additional diagnostic value over ultrasound in the evaluation of acute inflammatory neck masses in children? Eur Radiol. 2010;20(2):484-490. 5. Ludwig BJ, Wang J, Nadgir RN, Saito N, Castro-Aragon I, Sakai O. Imaging of cervical lymphadenopathy in children and young adults. AJR Am J Roentgenol. 2012;199(5):1105-1113. 6. Rosenberg HK. Sonography of pediatric neck masses. Ultrasound Q. 2009;25(3):111-127. 7. Lloyd C, McHugh K. The role of radiology in head and neck tumours in children. Cancer Imaging. 2010;10:49-61. 8. Snyder EM, Coley BD. Limited value of plain radiographs in infant torticollis. Pediatrics. 2006;118(6):e1779-1784. 9. Wei JL, Schwartz KM, Weaver AL, Orvidas LJ. Pseudotumor of infancy and congenital muscular torticollis: 170 cases. Laryngoscope. 2001;111(4 Pt 1):688-695. 10. Ballock RT, Song KM. The prevalence of nonmuscular causes of torticollis in children. J Pediatr Orthop. 1996;16(4):500- 504. 11. Haque S, Bilal Shafi BB, Kaleem M. Imaging of torticollis in children. Radiographics. 2012;32(2):557-571. 12. Morrison DL, MacEwen GD. Congenital muscular torticollis: observations regarding clinical findings, associated conditions, and results of treatment. J Pediatr Orthop. 1982;2(5):500-505. 13. Do TT. Congenital muscular torticollis: current concepts and review of treatment. Curr Opin Pediatr. 2006;18(1):26-29. 14. Pharisa C, Lutz N, Roback MG, Gehri M. Neck complaints in the pediatric emergency department: a consecutive case series of 170 children. Pediatr Emerg Care. 2009 Dec;25(12):823-826. 15. Sadoughi B, Fried MP, Sulica L, Blitzer A. Hoarseness evaluation: a transatlantic survey of laryngeal experts. Laryngoscope. 2014;124(1):221-226. 16. Paul BC, Branski RC, Amin MR, et al. Diagnosis and management of new-onset hoarseness: a survey of the American Broncho-Esophagological Association. Ann Otol Rhinol Laryngol. 2012;121(10):629-634.

♦ List may not be exclusive | MRI of Orbit, Face, Neck | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 39 Computed Tomography (CT) Paranasal Sinus & Maxillofacial Area – Pediatrics CPT Codes 70486 ����������������� CT of maxillofacial area, without contrast 70487 ����������������� CT of maxillofacial area, with contrast 70488 ����������������� CT of maxillofacial area, without contrast, followed by re-imaging with contrast Standard Anatomic Coverage ●● Includes the sinuses, facial structures and maxillary regions. Individual scan coverage depends on the specific clinical request, but generally includes images through the entire frontal, ethmoid, maxillary and sphenoid sinuses. Coverage may be extended to include the mandible and temporomandibular joint (TMJ) in select cases and depending on the clinical indication. CT sections may be obtained in one or two (usually coronal and axial) planes Technology Considerations ●● The prevalence of sinus inflammatory disease is high. The disease is estimated to affect approximately 33 million US citizens ●● This guideline includes reference to rhinosinusitis in the evaluation of sinus inflammatory disease, since sinusitis usually involves the nasal passage as well as the paranasal sinuses ●● Clinicians should distinguish presumed acute bacterial rhinosinusitis from acute rhinosinusitis due to viral upper respiratory infections and non-infectious conditions ●● Acute sinusitis is considered a self-limiting disease, since most patients improve within 2 weeks, despite the etiology and treatment option used ●● Sinus CT is not usually performed at the time of initial clinical presentation with acute uncomplicated sinusitis ●● Sinus CT is often reserved for difficult cases or delineation of anatomy prior to planned sinus surgery, as follows: ○○ Limited (coronal) Sinus CT – typically used for recurrent or refractory sinus inflammatory disease, or if the diagnosis is in doubt ○○ Full Sinus CT – generally performed for surgical planning to interrogate for osteomeatal obstruction, fungal sinusitis, facial or orbital cellulitis complicating sinusitis and suspected malignancy ●● CT of the paranasal sinuses is appropriately coded to CPT 70486. There are no required number of slices or phases for contrast-enhanced exams that constitute a paranasal sinus and maxillofacial CT study. This code may be used to describe limited or complete imaging of the sinuses ●● CT of the maxillofacial area is a bilateral study. Separate requests to image the right and left facial area are not allowed ●● For temporomandibular joints, CT may be used after MRI if diagnosis still in doubt or when MRI cannot be done Common Diagnostic Indications The following diagnostic indications for sinus CT are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Abnormality detected on other imaging study which requires additional clarification to direct treatment

Anosmia

Congenital anomaly

Foreign body in the maxillofacial region

Fungal or other complex sinus infection

♦ List may not be exclusive | CT Paranasal Sinus & Maxillofacial Area | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 40 Common Diagnostic Indications Mucocele of paranasal sinuses

Nasal airway obstruction refractory to medical therapy

Osteomyelitis of the facial bones

Polyposis

Post-operative or post-procedure evaluation Note: For post-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pre-operative or pre-procedure evaluation Note: For pre-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Recurrent epistaxis

Sinusitis, acute and subacute (any one of the following) ●● Screening of immunocompromised patient or a patient who is likely to become immunocompromised by therapy (for example, prior to chemotherapy or transplant1) ●● Management of complications of acute sinusitis1,2 (any one of the following) ○○ Abscess, intracranial or orbital ○○ Encephalitis or cerebritis ○○ Meningitis ○○ Sinus thrombosis ○○ Invasive fungal sinusitis in immunocompromised patients Note: Any episode of sinusitis with duration of less than 30 (acute) or 31-90 (subacute) days1. Sinusitis is generally rare in patients under 3 years of age

Sinusitis, chronic or recurrent (any one of the following) ●● Corroborate diagnosis of chronic sinusitis prior to a prolonged course of ●● Diagnose underlying medical condition (any one of the following) ○○ Chronic or asthma ○○ Ciliary motility disorder ○○ Craniofacial abnormality ○○ Cystic fibrosis ○○ Immunodeficiency ●● Diagnose unilateral sinusitis ●● Post-operative management of complications ●● Pre-operative evaluation to determine whether the patient is a surgical candidate ●● Pre-operative image guidance study Note: Any episode of sinusitis that persists beyond ninety (90) days1. Indication includes recurrent acute bacterial sinusitis (RABS), defined as episodes of sinusitis lasting less than 30 days with at least 10 symptom-free days in-between.At least three (3) such episodes within six (6) months or four (4) episodes within a year are required to qualify1.

♦ List may not be exclusive | CT Paranasal Sinus & Maxillofacial Area | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 41 Common Diagnostic Indications Temporomandibular disease (TMD), diagnosis3 (any one of the following) ●● Panorex has abnormal findings requiring further characterization (any one of the following): ○○ Arthritis (infectious or inflammatory – particularly rheumatoid) ○○ Bone infarct ○○ Bone neoplasm ○○ Osteolysis ○○ Trauma ●● Panorex is inconclusive or not available ●● Panorex is normal with high clinical suspicion for TMJ pathology when the results will change management (for example, headache of oromaxillofacial origin5) Note: Temporomandibular disease (TMD) is a collective term, which includes disorders of both the masticatory muscles and the temporomandibular joint3,4. CT is not generally indicated when a muscular etiology for TMD is suspected. Most temporomandibular joint (TMJ) pathology can be evaluated with a panorex radiograph3.

Trauma to the facial bones ●● Significant injury

Tumor or mass lesion in the sinonasal region

Wegener’s granulomatosis, suspected or known Note: Presentation includes recurrent epistaxis and stridor References 1. Wald ER, Applegate KE, Bordley C, et al; American Academy of Pediatrics. Clinical practice guideline for the diagnosis and management of acute bacterial sinusitis in children aged 1 to 18 years. Pediatrics. 2013;132(1):e262-e280. 2. Setzen G, Ferguson BJ, Han JK, et al. Clinical consensus statement: appropriate use of computed tomography for paranasal sinus disease. Otolaryngol Head Neck Surg. 2012;147(5):808-816. 3. American Academy of Orofacial Pain. Orofacial Pain: Guidelines for Assessment, Diagnosis, and Management. De Leeuw R, Klasser GD, eds. Chicago: Quintessence Publishing Co., Inc.; 2013. 4. American Association of Oral and Maxillofacial Surgeons (AAOMS). Clinical Paper: Temporomandibular Disorders. Rosemont, IL: AAOMS; 2013. 5. Scrivani SJ, Keith DA, Kaban LB. Temporomandibular disorders. N Engl J Med. 2008;359(25):2693-2705.

♦ List may not be exclusive | CT Paranasal Sinus & Maxillofacial Area | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 42 Magnetic Resonance Imaging (MRI) Temporomandibular Joint (TMJ) – Pediatrics

CPT Codes 70336 ����������������� MRI of Temporomandibular Joint(s) Standard Anatomic Coverage ●● Bilateral study, including open and closed mouth views, often performed with surface coils ●● Images may be obtained in axial, (oblique) sagittal and (oblique) coronal planes Technology Considerations ●● Conventional radiographs and/or Panorex films should be used for initial evaluation of bony abnormalities ●● Some of the common causes for temporomandibular joint dysfunction include direct trauma, habitual misuse of the TMJs and various arthritides, including degenerative joint disease ●● For a known or suspected fracture of the mandibular condyles and TMJ regions, further evaluation following initial radiographs is usually undertaken with CT ●● MRI may be used to evaluate for internal derangements and articular disc dysfunction in the TMJs ●● Dynamic ultrasound is an alternative technique for detecting disc displacement in the TMJs ●● MRI of the TMJ is inherently a bilateral procedure. Separate entries for the right and left TMJs are not allowed ●● CT may be used after MRI if diagnosis still in doubt, when MRI cannot be done, or as a primary modality when evaluation of the osseous components of the TMJ is the primary goal (for instance trauma) Common Diagnostic Indications The following diagnostic indications for temporomandibular joints (TMJ) MRI are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Abnormality detected on other imaging study which requires additional clarification to direct treatment

Arthropathy of the temporomandibular joints ●● Following non-diagnostic radiograph, or Panorex view of the TMJ (any one♦ of the following) ○○ Infectious arthritis ○○ Inflammatory arthritis (rheumatoid arthritis is the most common) ○○ Post-traumatic arthritis

Frozen jaw

Juvenile Idiopathic Arthritis (JIA)1 (All of the following) ●● In patients with established diagnosis of JIA when radiographs fail to provide sufficient information for management ●● Results will be used to determine whether the patient receives intraarticular therapy or other substantive change in management

Post-operative or post-procedure evaluation Note: For post-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pre-operative or pre-procedure evaluation Note: For pre-operative evaluation of conditions not specifically referenced elsewhere in this guideline

♦ List may not be exclusive | MRI Temporomandibular Joint | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 43 Common Diagnostic Indications Temporomandibular joint dysfunction (All of the following) ●● Following non-diagnostic radiographs or Panorex ●● Failed conservative therapy that includes (any one of the following) ○○ NSAIDs or acetaminophen ○○ Oral appliance (such as a bite block) ○○ Short-term trial of soft diet with proper chewing techniques ●● Persistent symptoms that include (any one♦ of the following) ○○ Clicking sensation, particularly during jaw movement ○○ Facial asymmetry and/or deformity (stable or changing) ○○ Locking ○○ Other functional impairments with mastication ○○ Persistent orofacial pain ○○ Unstable occlusion, with or without other symptoms

Trauma to the temporomandibular joints ●● Following non-diagnostic radiograph, Panorex view, or CT of the TMJ Note: For assessment of meniscal position and integrity References 1. Magni-Manzoni S, Malattia C, Lanni S, Ravelli A. Advances and challenges in imaging in juvenile idiopathic arthritis. Nat Rev Rheumatol. 2012 Mar 27;8(6):329-36.

♦ List may not be exclusive | MRI Temporomandibular Joint | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 44 Computed Tomography (CT) Neck for Soft Tissue Evaluation – Pediatrics

CPT Codes 70490 ����������������� CT Soft Tissues of Neck, without contrast 70491 ����������������� CT Soft Tissues of Neck, with contrast 70492 ����������������� CT Soft Tissues of Neck without contrast, followed by re-imaging with contrast Standard Anatomic Coverage ●● Axial images from the skull base to the clavicles Technology Imaging Considerations ●● CT is generally the modality of choice for the following indications: detection of sialolithiasis (salivary gland calculi); following trauma to the soft tissues of the neck; and during foreign body evaluation, after initial radiographic assessment for a radiopaque foreign body Common Diagnostic Indications The following diagnostic indications for neck CT are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Abnormality detected on other imaging study which requires additional clarification to direct treatment

Foreign body in the upper aerodigestive tract or surrounding neck tissues ●● Following non-diagnostic neck radiograph for soft tissue evaluation

Glottic lesion ●● Further assessment following endoscopic detection

Hoarseness, dysphonia, or vocal cord weakness - suspected to result from recurrent laryngeal nerve pathology1,2,16,17 ●● When hoarseness persists more than one month ●● When laryngoscopy is non-diagnostic or shows vocal cord paralysis Note: Dysphonia is defined as a disorder characterized by altered vocal quality, pitch, loudness, or vocal effort that impairs communication or otherwise reduces quality of life

Horner’s syndrome

Infectious or inflammatory process (any one♦ of the following) ●● Abscess, particularly peritonsillar and retropharyngeal ●● Cellulitis ●● Deep neck space infection ●● Lemierre’s syndrome ●● Osteomyelitis

Laryngeal edema

♦ List may not be exclusive | CT Neck (Soft Tissue) | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 45 Common Diagnostic Indications Lymphadenopathy ●● Following non-diagnostic ultrasound (any one of the following) ○○ Failed to resolve after a six (6) week course of therapy3,4 ○○ Red flag sign6 (any one of the following) ■■ Constitutional symptom ■■ Firm/immobile and larger than 3 cm in diameter3 ■■ Painless ■■ Persistent enlargement on exam for more than 2 weeks ■■ Supraclavicular or posterior triangle ■■ Ulceration ○○ Sonographic features suggestive of nodal malignancy3,5 Note: In the presence of red flag signs, biopsy may be more appropriate than advanced imaging

Neck mass, malignant (biopsy proven) ●● Management and surveillance (any one♦ of the following) ○○ Lymphoma, Hodgkin’s or non-Hodgkin’s ○○ Neuroblastoma ○○ Rhabdomyosarcoma

Neck mass, diagnosis and evaluation ●● Following non-diagnostic ultrasound3,4,6,7 (any one of the following) ○○ Diagnosis when ultrasound demonstrates a solid neck mass other than lymph node ○○ Evaluation of complication (including hemorrhage, infection, mass effect of the airway) Note: For lymph nodes, see Lymphadenopathy

Neck mass, cystic (All of the following) ●● Following non-diagnostic ultrasound ●● Pre-operative evaluation to define the extent of disease Note: This includes brachial cleft cyst, capillary hemangioma, thyroglossal duct cyst, and venolymphatic malformation.

Obstructive thyroid nodule or thyromegaly (goiter) (any one of the following) ●● Associated with mass effect on the upper airway or esophagus ●● Following thyroid ultrasound or thyroid scintigraphy ●● Pre-operative evaluation

Parathyroid adenoma8 (any one of the following) ●● Following non-diagnostic parathyroid ultrasound ●● Following non-diagnostic parathyroid scintigraphy ●● Patient with a failed parathyroidectomy ●● Pre-operative evaluation in a patient with aberrant anatomy Note: MRI may be appropriate for patients with recurrent hyperparathyroidism when there is a need to locate residual abnormal parathyroid tissue

♦ List may not be exclusive | CT Neck (Soft Tissue) | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 46 Common Diagnostic Indications Retropharyngeal neck mass Note: Includes retropharyngeal abscess, hematoma, and suppurative lymphadenopathy

Salivary/parotid gland ductal calculi (sialolithiasis)

Stridor ●● For subacute and chronic stridor, advanced imaging may follow neck (soft tissue) radiographs Note: This applies to subacute and chronic presentations of stridor. Soft tissue radiographs are generally sufficient to diagnose the common causes such as croup and foreign bodies (latter is often a medical emergency). In the pediatric population, common causes of stridor include vascular ring, laryngotracheomalacia, subglottic stenosis

Torticollis (twisted or rotated neck) (any one of the following) ●● Childhood (acquired) torticollis with clinical findings that suggest another cause (such as infection, neoplasm, trauma) ●● Congenital muscular torticollis (fibromatosis colli)9-14 in newborns age 8 months or less12-13 (All of the following) ○○ Following non-diagnostic cervical spine radiograph ○○ Following non-diagnostic ultrasound ○○ Unresponsive to four (4) weeks of conservative therapy Note: Childhood torticollis is typically associated with myositis of the sternocleidomastoid/trapezius15, and advanced imaging is generally not indicated unless there are additional signs/symptoms to suggest another cause. For congenital muscular torticollis, typical cases do not generally require imaging and atypical cases can be confirmed by ultrasound

Trauma to the soft tissues of the neck

Tumor, benign or malignant ●● For diagnosis, staging, evaluation of response to treatment, and pre-operative assessment

Upper airway obstruction Note: This includes tracheal stenosis

Vocal cord paralysis ●● Unexplained, following endoscopic diagnosis Note: May be unilateral or bilateral. CT may aid in localizing the side and level of vocal cord paralysis References 1. American Academy of Otolaryngology — Head and Neck Surgery Foundation. Choosing Wisely: Five Things Physicians and Patients Should Question. ABIM Foundation; February 21, 2013. Available at www.choosingwisely.org. 2. Schwartz SR, Cohen SM, Dailey SH, et al. Clinical practice guideline: hoarseness (dysphonia). Otolaryngol Head Neck Surg. 2009;141(3 Suppl 2):S1-S31. 3. Meier JD, Grimmer JF. Evaluation and management of neck masses in children. Am Fam Physician. 2014;89(5):353- 358. 4. Rozovsky K, Hiller N, Koplewitz BZ, Simanovsky N. Does CT have an additional diagnostic value over ultrasound in the evaluation of acute inflammatory neck masses in children? Eur Radiol. 2010;20(2):484-490. 5. Ludwig BJ, Wang J, Nadgir RN, Saito N, Castro-Aragon I, Sakai O. Imaging of cervical lymphadenopathy in children and young adults. AJR Am J Roentgenol. 2012;199(5):1105-1113. 6. Rosenberg HK. Sonography of pediatric neck masses. Ultrasound Q. 2009;25(3):111-127. 7. Lloyd C, McHugh K. The role of radiology in head and neck tumours in children. Cancer Imaging. 2010;10:49-61. 8. Johnson NA, Tublin ME, Ogilvie JB. Parathyroid imaging: technique and role in the pre-operative evaluation of primary hyperparathyroidism. AJR Am J Roentgenol. 2007 Jun;188(6):1706-1715.

♦ List may not be exclusive | CT Neck (Soft Tissue) | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 47 9. Snyder EM, Coley BD. Limited value of plain radiographs in infant torticollis. Pediatrics. 2006;118(6):e1779-1784. 10. Wei JL, Schwartz KM, Weaver AL, Orvidas LJ Pseudotumor of infancy and congenital muscular torticollis: 170 cases. Laryngoscope. 2001;111(4 Pt 1):688-695. 11. Ballock RT, Song KM. The prevalence of nonmuscular causes of torticollis in children. J Pediatr Orthop. 1996;16(4):500- 504. 12. Haque S, Bilal Shafi BB, Kaleem M. Imaging of torticollis in children. Radiographics. 2012;32(2):557-571. 13. Morrison DL, MacEwen GD. Congenital muscular torticollis: observations regarding clinical findings, associated conditions, and results of treatment. J Pediatr Orthop. 1982;2(5):500-505. 14. Do TT. Congenital muscular torticollis: current concepts and review of treatment. Curr Opin Pediatr. 2006;18(1):26-29. 15. Pharisa C, Lutz N, Roback MG, Gehri M. Neck complaints in the pediatric emergency department: a consecutive case series of 170 children. Pediatr Emerg Care. 2009 Dec;25(12):823-826. 16. Sadoughi B, Fried MP, Sulica L, Blitzer A. Hoarseness evaluation: a transatlantic survey of laryngeal experts. Laryngoscope. 2014;124(1):221-226. 17. Paul BC, Branski RC, Amin MR, et al. Diagnosis and management of new-onset hoarseness: a survey of the American Broncho-Esophagological Association. Ann Otol Rhinol Laryngol. 2012;121(10):629-634.

♦ List may not be exclusive | CT Neck (Soft Tissue) | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 48 CT Angiography (CTA) Neck – Pediatrics

CPT Codes 70498 ����������������� CTA of neck, with contrast material(s), including noncontrast images, if performed, and image post- processing Angiography includes imaging of all blood vessels, including arteries and veins. The code above includes CT Venography. Standard Anatomic Coverage ●● CTA of the neck involves image acquisition from the aortic arch to the skull base, to visualize major vessels which include the extracranial carotid arteries and vertebral arteries. The major venous structures may also be interrogated with CT angiographic technique Technology Considerations ●● Duplex Doppler examination of the extracranial carotid arteries is often performed prior to CTA ●● CTA of the neck is an alternative exam in patients who cannot undergo MRA Common Diagnostic Indications The following diagnostic indications for neck CTA are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Abnormality detected on carotid Doppler ultrasound or other imaging study which requires additional clarification to direct treatment

Aneurysm

Arteriovenous malformation

Congenital anomaly of the carotid and vertebrobasilar circulations

Dissection

Horner’s syndrome

Intramural hematoma

Multiple sclerosis Note: Evaluation of venous structures to assess for venous stenosis as a cause of multiple sclerosis (referred to as chronic cerebrospinal venous insufficiency [CCSVI]) is considered not medically appropriate. MRV in preparation for either a neurosurgical or percutaneous procedure to treat multiple sclerosis is therefore inappropriate.

Post-operative or post-procedure evaluation Note: For post-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pre-operative or pre-procedure evaluation Note: For pre-operative evaluation of conditions not specifically referenced elsewhere in this guideline

♦ List may not be exclusive | CTA Neck | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 49 Common Diagnostic Indications Stenosis or occlusion of the extracranial carotid arteries (Any one of the following) ●● Known steno-occlusive disease, particularly (any one♦ of the following): ○○ Idiopathic progressive arteriopathy of childhood ○○ Moyamoya disease ○○ Sickle cell disease ●● Patient is symptomatic (any one♦ of the following) ○○ Confusion ○○ Difficulty speaking or understanding speech ○○ Dizziness ○○ Gait disturbance ○○ Loss of balance or coordination ○○ Loss of consciousness ○○ Numbness, weakness or paralysis of the face, arm or leg on one side of the body ○○ Sudden severe headache that is unexplained ○○ Visual disturbance, particularly in one eye ●● Following work-up with duplex Doppler examination of the carotid arteries, unless diagnosis is substantiated by clinical exam findings Note: Stenosis or occlusion in the pediatric population is usually a result of vascular abnormalities from a systemic or congenital disease and rarely from atherosclerotic disease

Stenosis or occlusion of the vertebral arteries ●● Signs or symptoms of vertebrobasilar insufficiency (VBI) or vertebral basilar ischemia (any one♦ of the following) ○○ Acute sensorineural hearing loss ○○ Ataxia ○○ Diplopia ○○ Dysarthria ○○ Dysphagia ○○ Facial numbness or paresthesia ○○ Limb or trunk sensory deficit ○○ Loss of taste sensation ○○ Motor paresis ○○ Nystagmus ○○ Syncope ○○ Vertigo ○○ Visual field defect Note: Symptoms of VBI are usually temporary, due to diminished blood flow in the posterior circulation of the brain. Stenosis or occlusion in the pediatric population is usually a result of vascular abnormalities from a systemic or congenital disease and rarely from atherosclerotic disease

Thromboembolic disease of major extracranial arterial and/or venous systems Note: This includes venous thrombosis/occlusion

Traumatic vascular injury to the extracranial carotid and vertebral arteries

Vasculopathy Note: This includes Vasculitis and fibromuscular dysplasia (FMD)Venous thrombosis or compression

Venous thrombosis or compression

♦ List may not be exclusive | CTA Neck | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 50 MR Angiography (MRA) Neck – Pediatrics

CPT Codes 70547 ����������������� MRA of Neck without contrast 70548 ����������������� MRA of Neck with contrast 70549 ����������������� MRA of Neck without contrast, followed by re-imaging with contrast Angiography includes imaging of all blood vessels, including arteries and veins. The codes above include MR Venography. Standard Anatomic Coverage ●● Acquisitions from the aortic arch to the skull base to visualize the major vessels including the extracranial carotid arteries and vertebral arteries. The major venous structures may also be interrogated with MR angiographic techniques Technology Considerations ●● Duplex Doppler examination of the extracranial carotid arteries is often performed prior to MRA ●● CTA of the neck is an alternative exam in patients who cannot undergo MRA ●● MRA of the neck is inherently bilateral, thus duplicate requests to image the right and left side of the neck are inappropriate Common Diagnostic Indications The following diagnostic indications for Neck MRA are accompanied by pre-test considerations as well as supporting clinical data and prerequisite information

Abnormality detected on carotid Doppler ultrasound or other imaging study which requires additional clarification to direct treatment

Aneurysm

Arteriovenous malformation

Congenital anomaly of the carotid or vertebrobasilar circulation

Dissection

Horner’s syndrome

Intramural hematoma

Multiple sclerosis Note: Evaluation of venous structures to assess for venous stenosis as a cause of multiple sclerosis (referred to as chronic cerebrospinal venous insufficiency [CCSVI]) is considered not medically appropriate. MRV in preparation for either a neurosurgical or percutaneous procedure to treat multiple sclerosis is therefore inappropriate.

Post-operative or post-procedure evaluation Note: For post-operative evaluation of conditions not specifically referenced elsewhere in this guideline

Pre-operative or pre-procedure evaluation Note: For pre-operative evaluation of conditions not specifically referenced elsewhere in this guideline

♦ List may not be exclusive | MRA Neck | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 51 Common Diagnostic Indications Stenosis or occlusion of the extracranial carotid arteries (Any one of the following) ●● Known steno-occlusive disease, particularly (any one♦ of the following): ○○ Idiopathic progressive arteriopathy of childhood ○○ Moyamoya disease ○○ Sickle cell disease ●● Patient is symptomatic (any one♦ of the following) ○○ Confusion ○○ Difficulty speaking or understanding speech ○○ Dizziness ○○ Gait disturbance ○○ Loss of balance or coordination ○○ Loss of consciousness ○○ Numbness, weakness or paralysis of the face, arm or leg on one side of the body ○○ Sudden severe headache that is unexplained ○○ Visual disturbance, particularly in one eye ●● Following work-up with duplex Doppler examination of the carotid arteries, unless diagnosis is substantiated by clinical exam findings Note: Stenosis or occlusion in the pediatric population is usually a result of vascular abnormalities from a systemic or congenital disease and rarely from atherosclerotic disease

Stenosis or occlusion of the vertebral arteries ●● Signs or symptoms of vertebrobasilar insufficiency (VBI) or vertebral basilar ischemia (any one♦ of the following) ○○ Acute sensorineural hearing loss ○○ Ataxia ○○ Diplopia ○○ Dysarthria ○○ Dysphagia ○○ Facial numbness or paresthesia ○○ Limb or trunk sensory deficit ○○ Loss of taste sensation ○○ Motor paresis ○○ Nystagmus ○○ Syncope ○○ Vertigo ○○ Visual field defect Note: Symptoms of VBI are usually temporary, due to diminished blood flow in the posterior circulation of the brain. Stenosis or occlusion in the pediatric population is usually a result of vascular abnormalities from a systemic or congenital disease and rarely from atherosclerotic disease

Thromboembolic disease of major extracranial arterial and/or venous systems Note: This includes venous thrombosis/occlusion

Traumatic vascular injury to the extracranial carotid or vertebral artery

Vasculopathy Note: This includes vasculitis and fibromuscular dysplasia (FMD) venous thrombosis or compression

Venous thrombosis or compression

♦ List may not be exclusive | MRA Neck | Copyright © 2016. AIM Specialty Health. All Rights Reserved. 52