PEDPN-1.1: Age Considerations

PEDPN-1.2: Appropriate Clinical Evaluation

PEDPN-1.3: Modality General Considerations

This General Policy section provides an overview of the basic criteria for which Peripheral Nerve Disorder (PND) imaging may be medically necessary. Details regarding specific conditions or clinical presentations and the associated criteria for which imaging is medically necessary are described in subsequent sections.

PEDPN-1.1: Age Considerations

For this condition imaging is medically necessary based on the following criteria:

Many conditions affecting the peripheral nervous system in the pediatric population are different diagnoses than those occurring in the adult population. For those diseases which occur in both pediatric and adult populations, minor differences may exist in management due to member age, comorbidities, and differences in disease natural history between children and adults.

† Members who are <18 years old should be imaged according to the Pediatric Peripheral Nerve Disorders Imaging Guidelines, and members who are ≥18 years old should be imaged according to the Adult Peripheral Nerve Disorders Imaging Guidelines, except where directed otherwise by a specific guideline section.

PEDPN-1.2: Appropriate Clinical Evaluation

For this condition imaging is medically necessary based on the following criteria:

† A recent (within 60 days) evaluation including a detailed history, physical examination with a thorough neurologic examination, and appropriate laboratory studies should be performed prior to considering advanced imaging (CT, MRI, Nuclear Medicine), unless the member is undergoing guideline-supported scheduled follow-up imaging evaluation.

† Unless otherwise stated in a specific guideline section, the use of advanced imaging to screen asymptomatic members for disorders involving the peripheral nervous system is not supported. Advanced imaging of the peripheral nervous system should only be approved in members who have documented active clinical signs or symptoms of disease involving the peripheral nervous system.

† Unless otherwise stated in a specific guideline section, repeat imaging studies of the peripheral nervous system are not necessary unless there is evidence for progression of disease, new onset of disease, and/or documentation of how repeat imaging will affect member management or treatment decisions.

PEDPN-1.3: Modality General Considerations

For this condition imaging is medically necessary based on the following criteria:

† MRI

® MRI without and with contrast is the preferred modality for pediatric peripheral nerve imaging unless otherwise stated in a specific guideline section.
® Due to the length of time required for MRI acquisition and the need to minimize member movement, anesthesia is usually required for almost all infants (except neonates) and young children (age <7 years) as well as older children with delays in development or maturity. This anesthesia may be administered via oral or intravenous routes. In this member population, MRI sessions should be planned with a goal of minimizing anesthesia exposure by adhering to the following considerations:
¡ MRI procedures can be performed without and/or with contrast use as supported by these condition based guidelines. If intravenous access will already be present for anesthesia administration and there is no contraindication for using contrast, imaging without and with contrast may be appropriate if requested. By doing so, the requesting provider may avoid repetitive anesthesia administration to perform an MRI with contrast if the initial study without contrast is inconclusive.
  Recent evidence based literature demonstrates the potential for gadolinium deposition in various organs including the brain after the use of MRI contrast.
  The U.S. Food and Drug Administration (FDA) has noted that there is currently no evidence to suggest that gadolinium retention in the brain is harmful and restricting gadolinium-based contrast agents (GBCAs) use is not warranted at this time. It has been recommended that GBCA use should be limited to circumstances in which additional information provided by the contrast agent is necessary and the necessity of repetitive MRIs with GBCAs should be assessed.
¡ If multiple body areas are supported by Horizon BCBSNJ guidelines for the clinical condition being evaluated, MRI of all necessary body areas should be obtained concurrently in the same anesthesia session.

® CT is rarely used in the evaluation of pediatric peripheral nerve disorders. See specific guideline sections for indications.

® Ultrasound is rarely used in the evaluation of pediatric peripheral nerve disorders. See specific guideline sections for indications.

® Nuclear medicine studies are not generally indicated in the evaluation of peripheral nerve disorders. See PEDPN-2: Neurofibromatosis for specific imaging guidelines regarding PET/CT in evaluation of peripheral nerve tumors.

References

1. Bowen BC. Magnetic resonance imaging of the peripheral nervous system. Imaging of the Nervous System. eds. Latchaw RE, Kucharczyk J, Moseley ME, et al. Philadelphia, Elsevier. 2005;1479-1497.
2. Ing C, DiMaggio C, Whitehouse A, et al. Long-term differences in language and cognitive function after childhood exposure to anesthesia. Pediatrics. 2012 Sep;130(3):e476-e485.
3. Monteleone M, Khandji A, Cappell J, et al. Anesthesia in children: perspectives from nonsurgical pediatric specialists. J Neurosurg Anesthesiol. 2014 Oct;26(4):396-398.
4. DiMaggio C, Sun LS, and Li G. Early childhood exposure to anesthesia and risk of developmental and behavioral disorders in a sibling birth cohort. Anesth Analg. 2011 Nov;113(5):1143-1151.
5. Fraum TJ, Ludwig DR, Bashir MR, et al. Gadolinium-based contrast agents: a comprehensive risk assessment. J Magn Reson Imaging. 2017;46(2):338–353.
6. FDA Medical Imaging Drug Advisory Committee meeting 9/8/17. https://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs/MedicalImagingDrugsAdvisoryCommittee/UCM574746.pdf.
7. Raybaud C and Barkovich AJ. The Phakomatoses. Pediatric neuroimaging. Chapter 6. Philadelphia, Wolters Kluwer, 5th edition. 2012;569-636.
8. Soderlund KA, Smith AB, Rushing EJ, et al. Radiologic-pathologic correlation of pediatric and adolescent spinal neoplasms: Part 2. Intradural extramedullary spinal neoplasms. AJR Am J Roentgenol. 2012;198 (1):44-51.
9. Blumfield E, Swenson DW, Iyer RS, Stanescu AL. Gadolinium-based contrast agents — review of recent literature on magnetic resonance imaging signal intensity changes and tissue deposits, with emphasis on pediatric patients. Pediatric Radiology. 2019;49(4):448-457. doi:10.1007/s00247-018-4304-8.

PEDPN-2: Neurofibromatosis

PEDPN-2: Neurofibromatosis – General Information

PEDPN-2.1: Neurofibromatosis 1

PEDPN-2.2: Neurofibromatosis 2

PEDPN-2: Neurofibromatosis – General Information
For this condition imaging is medically necessary based on the following criteria:

This guideline section includes imaging indications for members with neurofibromatosis and known benign lesions. For cancer screening guidelines, See Pediatric Oncology Imaging Policy (Policy #166 in the Radiology Section); PEDONC-2.3: Neurofibromatosis 1 and 2 (NF1 and NF2) Imaging Guidelines. For guidelines related to known malignancies in members with NF1, see the appropriate imaging guideline for the specific cancer type.

PEDPN-2.1: Neurofibromatosis 1

For this condition imaging is medically necessary based on the following criteria:

† Most cutaneous neurofibromas and deep plexiform neurofibromas do not cause symptoms, and routine surveillance imaging of these lesions has not been shown to improve outcomes.

® The decision to obtain testing such as imaging studies depends upon the history and physical findings. Clinical evaluation appears to be more useful to detect complications than are screening investigations in asymptomatic members.
® The Genetics Committee of the American Academy of Pediatrics have published diagnostic and health supervision guidelines for children with NF1. Surveillance includes:
¡ Annual physical examination
¡ Annual ophthalmologic examination in children
¡ Regular developmental assessment of children
¡ MRI for follow-up of clinically suspected tumors

® Every 3 months for treatment response in members receiving active treatment
® New or worsening clinical symptoms suggesting progression
® Preoperative planning

† PET imaging is not supported for plexiform neurofibroma surveillance in asymptomatic members at this time as the positive predictive value is only 60 to 65% even in symptomatic members.

† MRI without and with contrast is appropriate for any clinical symptoms suggestive of change in a known plexiform neurofibroma in a member with NF1.

† Although PET imaging has a positive predictive value of only 61 to 63% in NF1 members with suspected transformation to MPNST, the negative predictive value is high (96 to 99%).

® PET imaging is indicated for evaluating NF1 members with clinical symptoms concerning for malignant transformation of a known plexiform neurofibromas when all of the following conditions exist:
¡ Recent MRI is inconclusive regarding transformation or progression.
¡ Negative PET will result in a decision to avoid biopsy in a difficult or morbid location.
® Inconclusive PET findings should lead to biopsy of the concerning lesion.
¡ Repeat PET studies are not indicated due to the poor positive predictive value in this setting.
® CT or three-dimensional CT reconstructions may be necessary when surgical treatment of bony lesions is being planned

For this condition imaging is medically necessary based on the following criteria:

† MRI Brain without and with contrast (CPT^® 70553) is indicated for members with known vestibular schwannomas in the following circumstances:

® Annual imaging for progression in unresected tumors
® New or worsening clinical symptoms, including hearing loss
® Preoperative planning

† Members with NF2 and known ependymoma should be imaged according to guidelines in Pediatric Oncology Imaging Policy (Policy #166 in the Radiology Section); PEDONC-4.8: Ependymoma Imaging Guidelines.

References

1. Friedman JM. Neurofibromatosis 1. GeneReviews™ [Internet] Adam MP, Ardinger HH, Pagon RA, et al. eds. Last Revision: May 17, 2018.
2. Williams VC, Lucas J, Babcock MA, et al. Neurofibromatosis Type I revisited. Pediatrics. 2009 Jan;123(1):124-133.
3. Karajannis MA and Ferner RE. Neurofibromatosis-related tumors: emerging biology and therapies. Curr Opin Pediatr. 2015 Feb;27(1):26-33.
4. Meany H, Dombi E, Reynolds J, et al. 18-Fluorodeoxyglocose-Positron Emission Tomography (FDG-PET) evaluation of nodular lesions in patients with Neurofibromatosis Type I and Plexiform Neurofibromas (PN) or Malignant Peripheral Nerve Sheath Tumors (MPNST). Pediatr Blood Cancer. 2013 Jan;60(1): 59-64..
5. Corbemale P, Valeyrie-Allanore L, Giammarile F, et al. Utility of 18F-FDG PET with a semi-quantitative index in the detection of sarcomatous transformation in patients with Neurofibromatosis Type 1, PLOS One. 2014 Feb;e85954.
6. Chirindel A, Chaudhry M, Blakeley JO, et al. 18F-FDG PET/CT qualitative and quantitative evaluation in neurofibromatosis Type 1 patients for detection of malignant transformation: comparison of early to delayed imaging with and without liver activity normalization. J Nucl Med. 2015 Mar;56(3):379-385.
7. Evans DG. Neurofibromatosis 2. GeneReviews™[Internet] Adam MP, Ardinger HH, Pagon RA, et al. eds. Last Update: March 15, 2018.
8. Blakeley JO, Evans DG, Adler J, et al. Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis Type 2. Am J Med Genetic A. 2012 Dec;158(1):24-41.
9. Hersh JH. American Academy of Pediatrics Committee on Genetics. Health supervision for children with neurofibromatosis. Pediatrics. 2008;121:633.
10. Hirbe AC, and Gutmann DH. Neurofibromatosis Type 1: a multidisciplinary approach to care. Lancet Neurol. 2014;13:834–843..
11. Evans DGR, Salvador H, Chang VY, et al. Cancer and Central Nervous System Tumor Surveillance in Pediatric Neurofibromatosis 1. Clinical Cancer Research. 2017;23(12):e46-53.
12. Evans DGR, Salvador H, Chang VY, et al. Cancer and Central Nervous System Tumor Surveillance in Pediatric Neurofibromatosis 2 and Related Disorders. Clinical Cancer Research. 2017;23(12):e54-61.
13. Kleinerman RA. Radiation-sensitive genetically susceptible pediatric sub-populations. Pediatric Radiology. 2008;39(S1):27-31. doi:10.1007/s00247-008-1015-6.
14. Vezina G, Barkovich AJ. Neurocutaneous disorders. In: Barkovich AJ, Raybaud C, eds. Pediatric Neuroimaging. 6th ed. Wolters Kluwer Philadelphia. 2019; 633-702.

PEDPN-3: Brachial Plexus

For this condition imaging is medically necessary based on the following criteria:

Disorders of the brachial plexus can generally be identified and distinguished from lesions in other locations by clinical, electromyography and nerve conduction (EMG/NCV) examination. If the diagnosis remains unclear, advanced imaging can be helpful as a preoperative study to evaluate the anatomy of brachial plexus lesions which should have already been defined by clinical examination.

† MRI is the preferred modality for imaging the brachial plexus. The goal of imaging is to visualize the entire course of the neural network from the preganglionic to the postganglionic segments.

® CT is not often useful and should not be used as a substitute for MRI.
® Unilateral brachial plexus studies should be ordered as MRI Upper Extremity Other Than Joint without contrast (CPT^® 73218) or without and with contrast (CPT^® 73220).
® Bilateral brachial plexus studies should be ordered as MRI Chest without contrast (CPT^® 71550) or without and with contrast (CPT^® 71552). For upper trunk lesions, MRI Neck without contrast (CPT^® 70540) is indicated.
® It is rare for more than one CPT^® code to be necessary to adequately image the brachial plexus area of interest. These requests should be forwarded for Medical Director Review.
® MRI Shoulder without contrast (CPT^® 73221) or without and with contrast (CPT^® 73223) is indicated in infants with brachial plexopathy due to birth trauma if requested for preoperative planning. These members often have glenohumeral dysplasia and require shoulder surgery.
® Ultrasound also may be indicated in infants with brachial plexus injury to show the glenoid dysplasia and associated shoulder subluxation
® If there is clinical suspicion for cervical nerve root avulsion, MRI Cervical Spine without contrast (CPT^®72141) is indicated.
® In members with a known malignancy or post-treatment syndrome, PET/CT skull base to mid-thigh (CPT^® 78815) may be approved if there is a contraindication to MRI.

1. Wippold FJ, Cornelius RS, Aiken AH, et al. Plexopathy. ACR Appropriateness Criteria^®. American College of Radiology. 2016:1-10.
2. Menashe SJ, Tse R, Nixon JN, et al. Brachial plexus birth palsy: multimodality imaging of spine and shoulder abnormalities in children. AJR Am J Roentgenol. 2015 Feb;204(2):W199-W206.
3. Somashekar DK, DiPietro MA, Joseph JR, et al. Ultility of ultrasound in noninvasive preoperative workup of neonatal brachial plexus palsy. Pediatr Radiol. 2016;46:695-703.
4. Volpe JJ. Injuries of extracranial, cranial, intracranial, spinal cord and peripheral nervous system structures. In: Volpe’s Neurology of the Newborn, 6th ed. Elsevier. Philadelphia 2018; 1093-1123.

PEDPN-4: Gaucher Disease

For this condition imaging is medically necessary based on the following criteria:

Gaucher disease is group of autosomal recessive inborn errors of metabolism characterized by lack of the enzyme acid ß -glucuronidase with destructive ceramide storage in various tissues. Gaucher disease is a treatable disorder (enzyme replacement) in which the liver, spleen, and bone marrow/bones are the most affected organs.

† Three types of Gaucher disease are recognized:

® Type I (non-neuropathic form or adult form): progressive hepatomegaly, splenomegaly, anemia and thrombocytopenia, and marked skeletal involvement; lungs and kidneys may also be involved, but central nervous system is spared
® Type II (acute neuropathic form or infantile form): severe progressive neurological involvement with death by 1 to 2 years of age; hepatomegaly, splenomegaly, is also present (usually evident by 6 months of age)
® Type III: type I with neurological involvement

® Repeat imaging is indicated every 12 months, to assess treatment response for members on enzyme replacement therapy or to assess disease progression for members in surveillance.

® Repeat imaging is indicated every 12 months, to assess treatment response for members on enzyme replacement therapy or to assess disease progression for members in surveillance.

® For members with documented pulmonary involvement, repeat imaging is indicated every 12 months, to assess treatment response for members on enzyme replacement therapy or to assess disease progression for members in surveillance.

References

1. Simpson WL, Hermann G, and Balwani M. Imaging of Gaucher disease. World J Radiol. 2014 Sep;6:657-668.
2. Anderson H, Kaplan P, Kacena K, et al. Eight-year clinical outcomes of long-term enzyme replacement therapy for 884 children with Gaucher disease Type I. Pediatrics. 2008;122(6):1182-1190.

Medicaid Coverage:

For members enrolled in Medicaid and NJ FamilyCare plans, Horizon BCBSNJ applies the above medical policy.

FIDE SNP:

For members enrolled in a Fully Integrated Dual Eligible Special Needs Plan (FIDE-SNP): (1) to the extent the service is covered under the Medicare portion of the member’s benefit package, the above Medicare Coverage statement applies; and (2) to the extent the service is not covered under the Medicare portion of the member’s benefit package, the above Medicaid Coverage statement applies.

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Horizon BCBSNJ Medical Policy Development Process:

This Horizon BCBSNJ Medical Policy (the “Medical Policy”) has been developed by Horizon BCBSNJ’s Medical Policy Committee (the “Committee”) consistent with generally accepted standards of medical practice, and reflects Horizon BCBSNJ’s view of the subject health care services, supplies or procedures, and in what circumstances they are deemed to be medically necessary or experimental/ investigational in nature. This Medical Policy also considers whether and to what degree the subject health care services, supplies or procedures are clinically appropriate, in terms of type, frequency, extent, site and duration and if they are considered effective for the illnesses, injuries or diseases discussed. Where relevant, this Medical Policy considers whether the subject health care services, supplies or procedures are being requested primarily for the convenience of the covered person or the health care provider. It may also consider whether the services, supplies or procedures are more costly than an alternative service or sequence of services, supplies or procedures that are at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of the relevant illness, injury or disease. In reaching its conclusion regarding what it considers to be the generally accepted standards of medical practice, the Committee reviews and considers the following: all credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, physician and health care provider specialty society recommendations, the views of physicians and health care providers practicing in relevant clinical areas (including, but not limited to, the prevailing opinion within the appropriate specialty) and any other relevant factor as determined by applicable State and Federal laws and regulations.

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Index:
Pediatric Peripheral Nerve Disorder Imaging Policy
Peripheral Nerve Disorder Imaging Policy, Pediatric
Computed Tomography, Peripheral Nerve Disorders, Pediatric
CT, Peripheral Nerve Disorders, Pediatric
Magnetic Resonance Imaging, Peripheral Nerve Disorders, Pediatric
MRI, Peripheral Nerve Disorders, Pediatric
Positron Emission Tomography, Peripheral Nerve Disorders, Pediatric
PET, Peripheral Nerve Disorders, Pediatric
Ultrasound, Peripheral Nerve Disorders, Pediatric
Nuclear Medicine Studies, Peripheral Nerve Disorders, Pediatric

References:


Codes:
(The list of codes is not intended to be all-inclusive and is included below for informational purposes only. Inclusion or exclusion of a procedure, diagnosis, drug or device code(s) does not constitute or imply authorization, certification, approval, offer of coverage or guarantee of payment.)

CPT*