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Horizon BCBSNJ
Uniform Medical Policy ManualSection:Radiology
Policy Number:169
Effective Date: 03/10/2020
Original Policy Date:02/23/2016
Last Review Date:02/11/2020
Date Published to Web: 04/13/2016
Subject:
Pediatric Peripheral Vascular Disorder Imaging Policy

Description:
_______________________________________________________________________________________

IMPORTANT NOTE:

The purpose of this policy is to provide general information applicable to the administration of health benefits that Horizon Blue Cross Blue Shield of New Jersey and Horizon Healthcare of New Jersey, Inc. (collectively “Horizon BCBSNJ”) insures or administers. If the member’s contract benefits differ from the medical policy, the contract prevails. Although a service, supply or procedure may be medically necessary, it may be subject to limitations and/or exclusions under a member’s benefit plan. If a service, supply or procedure is not covered and the member proceeds to obtain the service, supply or procedure, the member may be responsible for the cost. Decisions regarding treatment and treatment plans are the responsibility of the physician. This policy is not intended to direct the course of clinical care a physician provides to a member, and it does not replace a physician’s independent professional clinical judgment or duty to exercise special knowledge and skill in the treatment of Horizon BCBSNJ members. Horizon BCBSNJ is not responsible for, does not provide, and does not hold itself out as a provider of medical care. The physician remains responsible for the quality and type of health care services provided to a Horizon BCBSNJ member.

Horizon BCBSNJ medical policies do not constitute medical advice, authorization, certification, approval, explanation of benefits, offer of coverage, contract or guarantee of payment.

__________________________________________________________________________________________________________________________

TABLE OF CONTENTS
Pediatric PVD Imaging Policy
Procedure Codes Associated with PVD Imaging
PEDPVD-1: General Guidelines
PEDPVD-2: Vascular Anomalies
PEDPVD-3: Vasculitis
PEDPVD-4: Disorders of the Aorta and Visceral Arteries

Procedure Codes Associated with PVD Imaging
MRA
CPT®
Upper Extremity MRA
73225
Lower Extremity MRA
73725
CTA
CPT®
CTA Abdominal Aorta with Bilateral Iliofemoral Runoff
75635
Upper Extremity CTA
73206
Lower Extremity CTA
73706
Nuclear Medicine
CPT®
PET Imaging; limited area (this code not used in pediatrics)
78811
PET Imaging: skull base to mid-thigh (this code not used in pediatrics)
78812
PET Imaging: whole body (this code not used in pediatrics)
78813
PET with concurrently acquired CT; limited area (this code rarely used in pediatrics)
78814
PET with concurrently acquired CT; skull base to mid-thigh
78815
PET with concurrently acquired CT; whole body
78816
Ultrasound
CPT®
Duplex scan of extracranial arteries; complete bilateral study
93880
Duplex scan of extracranial arteries; unilateral or limited study
93882
Non-invasive physiologic studies of extracranial arteries, complete bilateral study
93875
Limited bilateral noninvasive physiologic studies of upper or lower extremity arteries
93922
Complete bilateral noninvasive physiologic studies of upper or lower extremity arteries
93923
Duplex scan of upper extremity arteries or arterial bypass grafts; complete bilateral
93930
Duplex scan of upper extremity arteries or arterial bypass grafts; unilateral or limited
93931
Non-invasive physiologic studies of extremity veins, complete bilateral study
93965
Duplex scan of extremity veins including responses to compression and other maneuvers; complete bilateral study
93970
Duplex scan of extremity veins including responses to compression and other maneuvers; unilateral or limited study
93971
Duplex scan of hemodialysis access (including arterial inflow, body of access, and venous outflow)
93990


Policy:
NOTE: For Medicare Advantage, Medicaid and FIDE-SNP, please refer to the Coverage Sections below for coverage guidance.)

PEDPVD-1: General Guidelines

PEDPVD-1.1: Age Considerations
PEDPVD-1.2: Imaging Appropriate Clinical Evaluation
PEDPVD-1.3: Modality General Considerations

This General Policy section provides an overview of the basic criteria for which Pediatric Peripheral Vascular Disease (PVD) 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.

PEDPVD-1.1: Age Considerations

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

Many conditions affecting the peripheral vascular 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, 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 vascular disease imaging guidelines, and members who are ≥ 18 years old should be imaged according to the Adult peripheral vascular disease imaging guidelines, except where directed otherwise by a specific guideline section.

PEDPVD-1.2: Imaging Appropriate Clinical Evaluation

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

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

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

Unless otherwise stated in a specific guideline section, repeat imaging studies of the peripheral vascular 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.

PEDPVD-1.3: Modality General Considerations

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

MRI

    ® MRI is generally performed without and with contrast unless the member has a documented contraindication to gadolinium or 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 imaging sessions should be planned with a goal of minimizing anesthesia exposure 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.
    ® The presence of surgical hardware or implanted devices may preclude MRI.
    ® The selection of best examination may require coordination between the provider and the imaging service.

CT
    ® CT or CTA may be appropriate for further evaluation of abnormalities suggested on prior US or MRI Procedures.
    ® CT may be appropriate without prior MR or US, especially in the following (non-exhaustive list of) settings:
      ¡ Lymphatic malformations
      ¡ Vascular abnormalities including vasculitis, thrombosis, narrowing, aneurysm, dissection, and varices.
      ¡ For preoperative planning or assessment of post-operative complications.
    ® In some cases, especially in follow-up of a known finding, it may be appropriate to limit the exam to the region of concern to reduce radiation exposure.
    ® CT should not be used to replace MRI in an attempt to avoid sedation unless listed as a recommended study in a specific guideline section.
    ® The selection of best examination may require coordination between the provider and the imaging service.

Ultrasound
    ® Ultrasound can be helpful in evaluating arterial, venous, and lymphatic malformations.
    ® Ultrasound can be limited by the imaging window and the member body type.
    ® CPT® codes vary by body area and presence or absence of Doppler imaging and are included in the table at the beginning of this guideline.

Nuclear Medicine
    ® Nuclear medicine studies are rarely used in the evaluation of peripheral vascular disorders, but are indicated in the following circumstances:
      ¡ Lymphoscintigraphy (CPT® 78195) is indicated for evaluation of lower extremity lymphedema when a recent Doppler ultrasound is negative for valvular insufficiency.
      ¡ Vascular flow imaging (CPT® 78445) is an obsolete study that has been replaced by MRA, CTA, or Duplex ultrasonography, and is not supported for any indication at this time.
      ¡ Venous thrombosis imaging (CPT® 78456, CPT® 78457, and CPT® 75458) are obsolete studies that have been replaced by MRA, CTA, or Duplex ultrasonography, and are not supported for any indication at this time.
      ¡ Radiopharmaceutical nuclear medicine studies (CPT® 78800, CPT® 78801, CPT® 78802, or CPT® 78803 can be approved for evaluation of the following:
        § Mycotic aneurysms
        § Vascular graft infection
        § Infection of central venous catheter or other indwelling device
The guidelines listed in this section for certain specific indications are not intended to be all-inclusive; clinical judgment remains paramount and variance from these guidelines may be appropriate and warranted for specific clinical situations.

References
1. Muratore F, Pipitone N, Salvarani C, Schmidt WA. Imaging of vasculitis: State of the art. Best Practice & Research Clinical Rheumatology. 2016;30(4):688-706. doi:10.1016/j.berh.2016.09.010.
2. American College of Radiology. Practice parameter for performing and interpreting magnetic resonance imaging (MRI): Amended 2018 (Resolution 44). ACR.org. https://www.acr.org/-/media/ACR/Files/Practice-Parameters/mr-perf-interpret.pdf?la=en. Published October 1, 2018. Accessed July 18, 2019.
3. Faerber EN, Abramson SJ, Benator RM, et. al. Practice Parameters by Modality: ACR–ASER–SCBT-MR–SPR Practice parameter for the performance of pediatric computed tomography (CT). American College of Radiology | American College of Radiology. https://www.acr.org/Clinical-Resources/Practice-Parameters-and-Technical-Standards/Practice-Parameters-by-Modality. Published 2017. Accessed July 18, 2019.
4. Ing C, Dimaggio C, Whitehouse A, et al. Long-term Differences in Language and Cognitive Function After Childhood Exposure to Anesthesia. Pediatrics. 2012;130(3):e476-e485. doi:10.1542/peds.2011-3822.
5. Monteleone M, Khandji A, Cappell J, Lai WW, Biagas K, Schleien C. Anesthesia in Children. Journal of Neurosurgical Anesthesiology. 2014;26(4):396-398. doi:10.1097/ana.0000000000000124..
6. Dimaggio C, Sun LS, Li G. Early Childhood Exposure to Anesthesia and Risk of Developmental and Behavioral Disorders in a Sibling Birth Cohort. Anesthesia & Analgesia. 2011;113(5):1143-1151. doi:10.1213/ane.0b013e3182147f42.
7. Macdonald A, Burrell S. Infrequently Performed Studies in Nuclear Medicine: Part 2. Journal of Nuclear Medicine Technology. 2009;37(1):1-13. doi:10.2967/jnmt.108.057851.
8. Mcneill GC, Witte MH, Witte CL, et al. Whole-body lymphangioscintigraphy: preferred method for initial assessment of the peripheral lymphatic system. Radiology. 1989;172(2):495-502. doi:10.1148/radiology.172.2.2748831.
9. Palestro CJ, Brown ML, Forstrom LA, et. al. SNMMI Procedure Standard for 111In-Leukocyte Scintigraphy for Suspected Infection/Inflammation 3.0. SNMMI. http://www.snmmi.org/ClinicalPractice/content.aspx?ItemNumber=6414. Published June 2, 2004. Accessed July 18, 2019.
10. De Vries EFJ, Roca M, Jamar F, Israel O, Signore A. Guidelines for the labelling of leucocytes with 99mTc-HMPAO. European Journal of Nuclear Medicine and Molecular Imaging. 2010;37(4):842-848. doi:10.1007/s00259-010-1394-4.
11. Fraum TJ, Ludwig DR, Bashir MR, Fowler KJ. Gadolinium-based contrast agents: A comprehensive risk assessment. Journal of Magnetic Resonance Imaging. 2017;46(2):338-353. doi:10.1002/jmri.25625.
12. Center for Drug Evaluation and Research. Medical Imaging Drugs Advisory Committee. U.S. Food and Drug Administration. https://www.fda.gov/advisory-committees/human-drug-advisory-committees/medical-imaging-drugs-advisory-committee. Published September 8, 2017. Accessed July 18, 2019.
13.Center for Drug Evaluation and Research. New warnings for gadolinium-based contrast agents (GBCAs) for MRI. U.S. Food and Drug Administration. https://www.fda.gov/drugs/drug-safety-and-availability/fda-drug-safety-communication-fda-warns-gadolinium-based-contrast-agents-gbcas-are-retained-body. Published May 16, 2018. Accessed July 18, 2019.

PEDPVD-2: Vascular Anomalies
PEDPVD-2.1: General Information
PEDPVD-2.2: Lymphatic Malformations
PEDPVD-2.3: Venous Malformations
PEDPVD-2.4: Capillary Malformations
PEDPVD-2.5: Arteriovenous Malformations (AVMs) and Fistulas
PEDPVD-2.6: Vascular Tumors

PEDPVD-2.1: General Information

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

Vascular and lymphatic malformations encompass a broad variety of conditions and have very heterogeneous natural history and treatment approaches. Lesions can be divided into low flow lesions (lymphatic, capillary and venous malformations), and high flow lesions (arteriovenous malformations and fistulas).

Members with aggressive lesions being treated with systemic therapy can have imaging (see specific sections for details regarding modality and contrast level) approved for treatment response every 3 months during active treatment.

Annual surveillance imaging of known vascular or lymphatic malformations can be approved for body areas where growth could cause significant organ dysfunction or functional impairment.

PEDPVD-2.2: Lymphatic Malformations

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

Lymphatic malformations are composed of dilated lymphatic channels filled with proteinaceous fluid and do not connect to normal lymphatic channels. They are typically soft, non-pulsatile masses with normal overlying skin.

Ultrasound is indicated as an initial examination for superficial lesions.

    ® Large lesion characterization may be limited by ultrasound imaging window.
    ® Ultrasound is also limited in evaluating malformation relationship to airway or bony structures.

MRI without contrast or without and with contrast of the affected body part is indicated for:
    ® Lymphatic malformations involving deep tissues
    ® Malformations too large to be completely imaged with ultrasound
    ® Inconclusive ultrasound findings
    ® Preoperative planning

CT is of limited value in evaluating lymphatic malformations
    ® CT with contrast of the affected body part can be approved for lesions with acute enlargement and concerns for compression when MRI is contraindicated.

PEDPVD-2.3: Venous Malformations

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

Venous malformations are slow-flow lesions characterized by dilated venous spaces and a normal arterial component. They are soft, compressible, non-pulsatile lesions that are usually blue to deep purple in color. Lesions can range from very small to large infiltrating ones. Some may change size with Valsalva.

Venous malformations are usually isolated, but they may be seen in multiple syndromes including Klippel-Trenaunay (KT) syndrome, Blue Rubber Bleb Nevus syndrome (BRBN), Maffucci syndrome, Proteus syndrome, Bannayan-Riley-Ruvalcaba syndrome, Parkes-Weber syndrome and congenital lipomatous overgrowth, vascular malformations, epidermal nevi and scoliosis/skeletal/spinal anomalies (CLOVES) syndrome.

Ultrasound with Doppler is indicated as an initial examination for superficial lesions.

    ® Large lesion characterization may be limited by ultrasound imaging window.
    ® Ultrasound is also limited in evaluating malformation relationship to airway or bony structures.

MRI without contrast or without and with contrast of the affected body part can be approved for venous malformations for preoperative assessment to evaluate the extent of malformation and their relationship to normal structures.

MRA or CTA has a limited role in evaluating most venous malformations, but may be approved (contrast as requested of the affected body part) if MRI or CT are equivocal and the results will impact acute management decisions.

CT can also be used to characterize venous malformations and their relationship to normal structures but is generally not as accurate as MRI.

    ® CT with contrast of the affected body part can be approved when MRI is inconclusive or contraindicated

PEDPVD-2.4: Capillary Malformations

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

Capillary malformations also known as port wine stains are characterized by a collection of small vascular channels in the dermis and generally do not require advanced imaging because the diagnosis is made clinically. However, MR imaging (without contrast or without and with contrast) may be approved to evaluate occult underlying neurologic structures, since these malformations are associated with encephalocele, spinal dysraphism, or Sturge-Weber syndrome

PEDPVD-2.5: Arteriovenous Malformations (AVMs) and Fistulas

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

Arteriovenous malformations are characterized by a network of multiple abnormal vascular channels interposed between enlarged feeding arteries and draining veins. The arteriovenous fistula has a single communication interposed between a feeding artery and a draining vein. The normal capillary bed is absent in both lesions. Both lesions may have an aggressive clinical course and are characterized by a reddish pulsatile mass which has a thrill or bruit. Though often recognized at birth, these lesions may grow and present near adolescence.

Ultrasound with Doppler is indicated as an initial examination for superficial lesions.

    ® Large lesion characterization may be limited by ultrasound imaging window.
    ® Ultrasound is also limited in evaluating AVM relationship to airway or bony structures.

MRI without contrast or without and with contrast of the affected body part is also indicated for evaluation of AVMs, and is useful in evaluating the extent of AVMs and their relationship to normal structures.

MRA (contrast as requested) of the affected body part can be approved for evaluation and surveillance of known AVMs.

It is unusual for both MRI and MRA to be necessary for routine treatment response or surveillance imaging of AVMs, but both may be approved for preoperative planning.

CT and CTA can also be used to characterize AVMs and their relationship to normal structures, but is generally not better than MRI and has associated radiation risks.

    ® CT with contrast and/or CTA (contrast as requested) of the affected body part can be approved when MRI and/or MRA is inconclusive or contraindicated.

PEDPVD-2.6: Vascular Tumors

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

Vascular tumors include a variety of benign, borderline, and malignant tumors, which have variable clinical courses, including but not limited to Epithelioid hemangioma, Kaposiform hemangioendothelioma, Kaposi sarcoma, Epithelioid hemangioendothelioma, and Angiosarcoma of soft tissue.

Ultrasound with Doppler is indicated as an initial examination for vascular tumors.

    ® Large lesion characterization may be limited by ultrasound imaging window.
    ® Ultrasound is also limited in evaluating malformation relationship to airway or bony structures.

MRI without contrast or without and with contrast of the affected body part is also indicated for evaluation of vascular tumors, and is useful in evaluating the extent of arteriovenous malformations and their relationship to normal structures, as well as response to therapy.

MRA (contrast as requested) of the affected body part can be approved for evaluation and surveillance of known vascular tumors.

It is unusual for both MRI and MRA to be necessary for routine treatment response or surveillance imaging of vascular tumors, but both may be approved for preoperative planning.

CT and CTA can also be used to characterize vascular tumors and their relationship to normal structures, but is generally not better than MRI and has associated radiation risks.

    ® CT with contrast and/or CTA (contrast as requested) of the affected body part can be approved when MRI and/or MRA is inconclusive or contraindicated.

References
1. Pizzo PA, Poplack DG, Krishnamurthy R, Daldrup-Link HE, Jones JY, et. al. Imaging studies in the diagnosis and management of pediatric malignancies. In: Principles and Practice of Pediatric Oncology. Vol 7. Philadelphia: Wolters Kluwer; 2016:185-234.
2. Kliegman R, Nelson WE, Martin KL. Chapter 650: Vascular Disorders. In: Nelson Textbook of Pediatrics: 20th ed. Philadelphia, PA, PA: Elsevier, Saunders; 2016:3122-3128.
3. Blei F, Guarini A. Current workup and therapy of infantile hemangiomas. Clinics in Dermatology. 2014;32(4):459-470. doi:10.1016/j.clindermatol.2014.02.001.
4. Cahill AM, Nijs ELF. Pediatric Vascular Malformations: Pathophysiology, Diagnosis, and the Role of Interventional Radiology. CardioVascular and Interventional Radiology. 2011;34(4):691-704. doi:10.1007/s00270-011-0123-0.
5. Bagrodia N, Defnet AM, Kandel JJ. Management of lymphatic malformations in children. Current Opinion in Pediatrics. 2015;27(3):356-363. doi:10.1097/mop.0000000000000209.
6. Wassef M, Blei F, Adams D, et al. Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies. Pediatrics. 2015;136(1):e203-e214. doi:10.1542/peds.2014-3673.
7. Kutz AM, Aranibar L, Lobos N, Wortsman X. Color Doppler Ultrasound Follow-Up of Infantile Hemangiomas and Peripheral Vascularity in Patients Treated with Propranolol. Pediatric Dermatology. 2015;32(4):468-475. doi:10.1111/pde.12596.
8. Adams DM, Trenor CC, Hammill AM, et al. Efficacy and Safety of Sirolimus in the Treatment of Complicated Vascular Anomalies. Pediatrics. 2016;137(2). doi:10.1542/peds.2015-3257.
9. Snyder E, Puttgen K, Mitchell S, Ahlawat S, Tekes A. Magnetic Resonance Imaging of the Soft Tissue Vascular Anomalies in Torso and Extremities in Children: An Update With 2014 International Society for the Study of Vascular Anomalies Classification. Journal of Computer Assisted Tomography. 2017;42(2):167-177. doi:10.1097/rct.0000000000000675.
10. Merrow AC, Gupta A, Patel MN, Adams DM. 2014 Revised Classification of Vascular Lesions from the International Society for the Study of Vascular Anomalies: Radiologic-Pathologic Update. RadioGraphics. 2016;36(5):1494-1516. doi:10.1148/rg.2016150197.
11. Johnson CM, Navarro OM. Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 1: classification, sonographic approach and vascular tumors. Pediatric Radiology. 2017;47(9):1184-1195. doi:10.1007/s00247-017-3885-y.
12. Johnson CM, Navarro OM. Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 2: vascular malformations. Pediatric Radiology. 2017;47(9):1196-1208. doi:10.1007/s00247-017-3906-x.
13. Sadick M, Müller-Wille R, Wildgruber M, Wohlgemuth W. Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies. RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren. 2018;190(09):825-835. doi:10.1055/a-0620-8925.
14. Olivieri B, White CL, Restrepo R, Mckeon B, Karakas SP, Lee EY. Low-Flow Vascular Malformation Pitfalls: From Clinical Examination to Practical Imaging Evaluation—Part 2, Venous Malformation Mimickers. American Journal of Roentgenology. 2016;206(5):952-962. doi:10.2214/ajr.15.15794.
15. White CL, Olivieri B, Restrepo R, Mckeon B, Karakas SP, Lee EY. Low-Flow Vascular Malformation Pitfalls: From Clinical Examination to Practical Imaging Evaluation—Part 1, Lymphatic Malformation Mimickers. American Journal of Roentgenology. 2016;206(5):940-951. doi:10.2214/ajr.15.15793.

PEDPVD-3: Vasculitis
PEDPVD-3.1: General Information
PEDPVD-3.2: Large Vessel Vasculitis
PEDPVD-3.3: Medium Vessel Vasculitis
PEDPVD-3.4: Small Vessel Vasculitis
PEDPVD-3.1: General Information

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

Systemic vasculitis is much less common in children than in adults, although the diagnostic pathways and treatment options are similar.

PET/CT is considered investigational for management of pediatric vasculitis at this time.

    ® There are limited data suggesting PET may have similar accuracy to MRA in the initial diagnosis of Takayasu arteritis but is not helpful in assessing treatment response and has not been shown to improve member outcomes to date.

PEDPVD-3.2: Large Vessel Vasculitis

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

Takayasu arteritis is the predominant large vessel vasculitis occurring in children.

Any of the following modalities may be indicated for evaluation of Takayasu arteritis:

    ® MRA of the affected body area(s) (contrast as requested)
    ® CTA of the affected body area(s) (contrast as requested)
    ® Ultrasound with Doppler of the affected body area(s)

Imaging is indicated at the following intervals:
    ® Every 3 months for treatment response during active treatment in members being treated with systemic therapy.
      ¡ See specific sections for details regarding modality and contrast level.
    ® Annually for surveillance of known involved body areas to detect progressive vascular damage that may require intervention.

PEDPVD-3.3: Medium Vessel Vasculitis

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

Polyarteritis nodosa and Kawasaki Disease are the primary medium vessel vasculitides occurring in children.

Imaging guidelines for Kawasaki Disease are in the pediatric cardiac imaging guideline, see Pediatric Cardiac Imaging Policy (Policy # 161 in the Radiology Section); PEDCD-6: Kawasaki Disease

For evaluation of polyarteritis nodosa:

    ® Any of the following modalities may be indicated:
      ¡ MRA of the affected body area(s) (contrast as requested)
      ¡ CTA of the affected body area(s) (contrast as requested)
      ¡ Ultrasound with Doppler of the affected body area(s)
    ® Imaging is indicated at the following intervals:
      ¡ Every 3 months during active treatment with systemic therapy for treatment response.
        § For details regarding modality and contrast level see PEDPVD-1.3: Modality General Considerations
      ¡ Annually for surveillance of known involved body areas to detect progressive vascular damage that may require intervention.
PEDPVD-3.4: Small Vessel Vasculitis

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

Advanced imaging is not sensitive enough to detect changes in small vessels, and is not indicated for primary assessment of any small vessel vasculitis.

End-organ damage occurs with several of the small vessel vasculitides. Advanced imaging is indicated for the following:

    ® Granulomatosis with polyangiitis (GPA, formerly known as Wegener’s granulomatosis):
      ¡ CT Sinuses (CPT®70486) and/or CT Chest without contrast (CPT®71250) or with contrast (CPT®71260) is indicated for the following:
        § New or worsening clinical symptoms affecting the body area requested
        § To assess response to medical therapy when a change in treatment regimen is being considered
        § Annually-to evaluate the extent of disease
    ® Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss Syndrome):
      ¡ CT Chest without contrast (CPT®71250) or with contrast (CPT®71260) is indicated in the following circumstances:
        § New or worsening clinical symptoms affecting the body area requested
        § To assess response to medical therapy when a change in treatment regimen is being considered
        § Annually-to evaluate the extent of disease
    ® Immune complex associated small-vessel vasculitis [immunoglobulin A–associated vasculitis (IgAV)]:
      ¡ Doppler ultrasound of the affected body part (most commonly abdomen) is indicated in the following circumstances:
        § New or worsening clinical symptoms affecting the body area requested
        § To assess response to medical therapy when a change in treatment regimen is being considered
        § Annually-to evaluate the extent of disease
References
1. Muratore F, Pipitone N, Salvarani C, Schmidt WA. Imaging of vasculitis: State of the art. Best Practice &amp; Research Clinical Rheumatology. 2016;30(4):688-706. doi:10.1016/j.berh.2016.09.010.
2. Lensen KDF, Comans EFI, Voskuyl AE, et al. Large-Vessel Vasculitis: Interobserver Agreement and Diagnostic Accuracy of18F-FDG-PET/CT. BioMed Research International. 2015;2015:1-8. doi:10.1155/2015/914692. .
3. Soussan M, Nicolas P, Schramm C, et al. Management of Large-Vessel Vasculitis With FDG-PET. Medicine. 2015;94(14). doi:10.1097/md.0000000000000622. .
4. Besson FL, Parienti J-J, Bienvenu B, et al. Diagnostic performance of 18F-fluorodeoxyglucose positron emission tomography in giant cell arteritis: a systematic review and meta-analysis. European Journal of Nuclear Medicine and Molecular Imaging. 2011;38(9):1764-1772. doi:10.1007/s00259-011-1830-0.
5. Ardoin SP and Fels E. Vasculitis syndromes. Nelson Textbook of Pediatrics, chapter 167. eds Kliegman RM, Stanton BF, St. Geme JW III, Schor NF. 20th edition 2016, pp 1215-1224.
6. Soliman M, Laxer R, Manson D, Yeung R, Doria AS. Imaging of systemic vasculitis in childhood. Pediatric Radiology. 2015;45(8):1110-1125. doi:10.1007/s00247-015-3339-3.
7. Sharma AM, Singh S, Lewis JE. Diagnostic Approach in Patients With Suspected Vasculitis. Techniques in Vascular and Interventional Radiology. 2014;17(4):226-233. doi:10.1053/j.tvir.2014.11.002.
8. Ammirati E, Moroni F, Pedrotti P, et al. Non-Invasive Imaging of Vascular Inflammation. Frontiers in Immunology. 2014;5:1-15. doi:10.3389/fimmu.2014.00399.
9. Granata C, Damasio MB, Zaottini F, et al. Imaging of Childhood Vasculitis. Radiologic Clinics of North America. 2017;55(5):1131-1143. doi:10.1016/j.rcl.2017.05.001.
10. Broncano J, Vargas D, Bhalla S, Cummings KW, Raptis CA, Luna A. CT and MR Imaging of Cardiothoracic Vasculitis. RadioGraphics. 2018;38(4):997-1021. doi:10.1148/rg.2018170136.

PEDPVD-4: Disorders of the Aorta and Visceral Arteries
PEDPVD-4.1: Thoracic Aortic Disease
PEDPVD-4.2: Aortic Congenital Vascular Malformations
PEDPVD-4.3: Visceral Artery Aneurysms
PEDPVD-4.1: Thoracic Aortic Disease

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

MRA Chest (CPT® 71555) or CTA Chest (CPT® 71275) can be used in members with Takayasu arteritis, William syndrome, and Ehlers Danlos syndrome for both:

    ® Screening
    ® Follow-up of thoracic aortic abnormalities

MRAs (preferred) or CTAs from the head through the pelvis may be performed in members diagnosed with Loeys-Dietz syndrome for:
    ® Screening-one time
    ® Follow-up imaging of discovered aneurysms-no more frequently than annually as requested by a specialist

For other conditions please see discussions indicated elsewhere in the guidelines
    ® Marfan Syndrome- See Adult Chest Imaging Policy (Policy #150 in the Radiology Section); CH-29.3 Screening Guidelines for Familial Syndromes
    ® Coarctation of the Aorta- See Pediatric Cardiac Imaging Policy (Policy # 161 in the Radiology Section); PEDCD-2.3 Congenital Heart Disease
    ® Congenital rubella syndrome- See Pediatric Cardiac Imaging Policy (Policy # 161 in the Radiology Section); PEDCD-2.3 Congenital Heart Disease
    ® Kawasaki Syndrome- See Pediatric Cardiac Imaging Policy (Policy # 161 in the Radiology Section); PEDCD-6 Kawasaki Disease
    ® Neurofibromatosis- See Pediatric Cardiac Imaging Policy (Policy # 161 in the Radiology Section); PEDCD-1.2 Pediatric Cardiac Imaging Appropriate Clinical Evaluation

PEDPVD-4.2: Aortic Congenital Vascular Malformations

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

Cardiac MRI without contrast (CPT® 75557) or without and with contrast (CPT® 75561), MRA Chest (CPT® 71555), CT Chest with contrast (CPT® 71260), or CTA Chest (CPT® 71275) may be indicated for evaluation.

Vascular rings may impact both the esophagus and trachea. See Pediatric Neck Imaging Policy (Policy # 165 in the Radiology Section); PEDNECK-7: Esophagus and/or Pediatric Neck Imaging Policy (Policy # 165 in the Radiology Section); PEDNECK-8: Trachea for additional guidelines.

PEDPVD-4.3: Visceral Artery Aneurysms

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

Visceral artery imaging indications in pediatric members are identical to those for adult members. See Adult Peripheral Vascular Disease Imaging Policy (Policy #158 in the Radiology Section); PVD-6: Aortic Disorders and Renal Vascular Disorders and Visceral Artery Aneurysms for imaging guidelines.

References
1. Hanneman K, Newman B, Chan F. Congenital Variants and Anomalies of the Aortic Arch. RadioGraphics. 2017;37(1):32-51. doi:10.1148/rg.2017160033.
2. Erbel R, Aboyans V, Boileau C, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases. European Heart Journal. 2014;35(41):2873-2926. doi:10.1093/eurheartj/ehu281.
3. Coley BD, Chan FD. Acquired diseases of the great vessels. In: Caffey's Pediatric Diagnostic Imaging. Vol 1. 12th ed. Elsevier/Saunders; 2013:835.
4. Coley BD, Chan FD. Congenital diseases of the thoracic great arteries. In: Caffey's Pediatric Diagnostic Imaging. Vol 1. 12th ed. Elsevier/Saunders; 2013:772.
5. Loughborough WW, Minhas KS, Rodrigues JCL, et al. Cardiovascular Manifestations and Complications of Loeys-Dietz Syndrome: CT and MR Imaging Findings. RadioGraphics. 2018;38(1):275-286. doi:10.1148/rg.2018170120.
6. Huang Y, Qu G. Faculty of 1000 evaluation for 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology Society of Thoracic Surgeons, and Society for Vascular Medicine.F1000 - Post-publication peer review of the biomedical literature. 2010. doi:10.3410/f.4998963.4932064.

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 Vascular Disorder Imaging Policy
Peripheral Vascular Disorder Imaging Policy, Pediatric
Computed Tomography, Peripheral Vascular Disorders, Pediatric
CT, Peripheral Vascular Disorders, Pediatric
Computed Tomography Angiography, Peripheral Vascular Disorder, Pediatric
CTA, Peripheral Vascular Disorder, Pediatric
Magnetic Resonance Imaging, Peripheral Vascular Disorders, Pediatric
MRI, Peripheral Vascular Disorders, Pediatric
Positron Emission Tomography, Peripheral Vascular Disorders, Pediatric
PET, Peripheral Vascular Disorders, Pediatric
Ultrasound, Peripheral Vascular Disorders, Pediatric
Nuclear Medicine Studies, Peripheral Vascular Disorders, Pediatric
Doppler Studies, Peripheral Vascular Disorder, Pediatric
Duplex Scan, Peripheral Vascular Disorder, 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*

    HCPCS

    * CPT only copyright 2020 American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.

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    Medical policies can be highly technical and are designed for use by the Horizon BCBSNJ professional staff in making coverage determinations. Members referring to this policy should discuss it with their treating physician, and should refer to their specific benefit plan for the terms, conditions, limitations and exclusions of their coverage.

    The Horizon BCBSNJ Medical Policy Manual is proprietary. It is to be used only as authorized by Horizon BCBSNJ and its affiliates. The contents of this Medical Policy are not to be copied, reproduced or circulated to other parties without the express written consent of Horizon BCBSNJ. The contents of this Medical Policy may be updated or changed without notice, unless otherwise required by law and/or regulation. However, benefit determinations are made in the context of medical policies existing at the time of the decision and are not subject to later revision as the result of a change in medical policy

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