Guidelines governing imaging for pediatric peripheral vascular disease for UnitedHealthcare Community Plan members in Ohio; applies to providers requesting imaging services and utilization reviewers assessing medical necessity.
No material clinical or coverage changes in this revision.
V1.0.2025version
Ohio onlyapplicability
Annualreview frequency
Requiredmanagement impact
<30 mL/minGFR threshold
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Not necessary
PET/CT stance
Coverage Criteria and General Rules
General coverage criteria
General coverage criteria — Advanced imaging is covered when supported by the clinical presentation, a recent pertinent clinical evaluation is available, and the results are expected to affect management. Appropriate modality selection per condition-specific guidance is required.
All of the following must be met for advanced imaging to be covered:
- A recent pertinent clinical evaluation (history, focused physical exam, and when applicable laboratory or prior imaging) since onset or change in symptoms has been performed or documented. (See Clinical Documentation and Age Considerations.)
- The requested imaging is expected to affect clinical management or treatment decisions.
- Less invasive or lower-cost diagnostic options (plain radiography, ultrasound, or Doppler as applicable) have been considered or performed unless guideline-supported scheduled surveillance or the clinical situation warrants immediate advanced imaging.
- The modality selected is appropriate for the clinical question per condition-specific sections (e.g., MRI preferred for soft-tissue/vascular malformations; CT/CTA or MRA when MRI is contraindicated or inconclusive).
Operational note: Repeat imaging is allowed only when there is evidence of progression, new symptoms, or documentation that the repeat study will alter management.
3D rendering and stereotactic localization rules
3D rendering and stereotactic localization — Use and billing rules for 3D rendering and stereotactic CT localization.
Do not bill CPT 76376 or 76377 with CAD, MRA, CTA, SPECT, PET, PET/CT, mammography (including MRI breast), CT colonography, cardiac MRI/CT, or coronary CTA.
CPT 76376 or 76377 may be considered for specific clinical scenarios (pre-operative planning for complex bony or facial fractures, congenital skull abnormalities in children, spinal/pelvic fractures, complex surgical planning, cerebral angiography, and select pelvic/abdominal cases) when conventional imaging is insufficient.
Report CPT 77011 for stereotactic CT localization when no radiologist interpretation is provided. Do not report both a diagnostic CT code (e.g., 70486) and 77011 for the same session. 3D rendering (CPT 76376/76377) should not be reported with 77011 or with the diagnostic CT when the stereotactic localization inherently generates a 3D dataset.
Image-guidance and ablation codes
Image-guidance for percutaneous procedures and ablation — Indications and billing guidance for imaging guidance codes.
CPT 77012 and 77021 report radiologic supervision/interpretation for CT- or MR-guided needle placement for percutaneous procedures and should only be billed with percutaneous surgical procedure codes (not open/excisional procedures).
CPT 77013 and 77022 include initial guidance, monitoring for repositioning, multiple ablations, and confirmation of adequate ablation for non-bone tumor ablations. CPT 77013 should be used only for non-bone ablation; CPT 20982 covers CT guidance for bone tumor ablations.
Only one unit of any single guidance code (eg, CPT 77012, 77013, 77021, 77022, 76942, 77002–77003) should be reported per individual encounter (date of service).
Whole-Body CT Imaging
Whole-Body CT Imaging — Coverage stance.
Whole-body CT (LifeScan) for screening asymptomatic individuals is not indicated and is not supported due to lack of demonstrated benefit versus radiation risk.
Whole-body low-dose CT is supported for oncologic staging in multiple myeloma per the Oncology Imaging Guidelines.
Whole-Body MR Imaging
Whole-Body MR Imaging — Coverage stance.
WBMRI is generally not supported as a standard evaluation except for select cancer predisposition syndromes and specific autoimmune conditions where guideline sections permit its use.
There are no established CPT/HCPCS codes for WBMRI; CPT 76498 is the appropriate code when WBMRI is approved.
WBMRI may be considered for interval cancer screening in individuals with defined cancer predisposition syndromes or for select disease-specific indications noted in condition-specific guidelines.
PET-MRI
PET-MRI — Coverage stance.
PET-MRI is generally not supported for most oncologic and neurologic conditions due to lack of standardization. It may be appropriate when:
- The individual meets condition-specific PET-MRI criteria; OR
- The individual meets guideline criteria for PET-CT, PET-CT is not available at the treating institution, and the provider requests PET-MRI in lieu of PET-CT.
Operational note: When allowed, PET-MRI should be reported as PET whole-body (CPT 78813) plus MRI unlisted (CPT 76498); diagnostic MRI codes may be indicated concurrently when clinically appropriate.
Quantitative MR Analysis
Quantitative MR Analysis — Coverage stance.
Quantitative multiparametric MRI analysis (Category III CPT codes such as 0648T/0649T, 0697T/0698T, 0865T/0866T) is considered investigational/experimental for routine clinical use due to limited demonstrated clinical utility.
These procedures are primarily used in clinical trials and lack widely recommended indications in clinical practice.
Appropriate Use / Medical Necessity
Appropriate Use / Medical Necessity — Peripheral vascular imaging general rules.
All of the following must be met for peripheral vascular advanced imaging to be medically necessary:
- A pertinent clinical evaluation (history, examination, and when applicable labs/prior imaging) since onset or change in symptoms has been performed or documented.
- The individual has active clinical signs or symptoms referable to the peripheral vascular system, unless guideline-supported surveillance is specified in a condition-specific section.
- The requested imaging is expected to influence management or treatment decisions.
Operational note: Advanced imaging for asymptomatic screening of peripheral vascular disorders is not supported unless explicitly stated in a condition-specific guideline.
Repeat Imaging Criteria
Repeat Imaging Criteria — When repeat imaging is supported.
Repeat imaging is covered when ALL of the following are met:
- There is objective evidence of disease progression or new/worsening clinical signs or symptoms; AND
- The repeat imaging will directly affect clinical management or treatment decisions; AND
- The timing of repeat imaging is consistent with accepted clinical practice or specified in a condition-specific guideline.
Modality Selection
Modality Selection — Considerations for choosing CT, MRI, US, or CTA/MRA.
MRI (without and with contrast as indicated) is preferred for soft-tissue characterization and evaluation of vascular malformations, vascular tumors, and AVMs when feasible.
CT/CTA or MRA may be indicated when MRI is contraindicated, inconclusive, or when rapid evaluation is required (eg, suspected acute compression, hemorrhage, or pulmonary embolism).
Ultrasound with Doppler is the preferred initial study for superficial lesions and for many venous evaluations.
CT should not routinely replace MRI solely to avoid sedation in children unless specifically recommended in a condition-specific guideline.
Lymphatic and Vascular Malformations, AVMs, and Vascular Tumors — Imaging indications.
Lymphatic malformations:
- Ultrasound is indicated as the initial examination for superficial lesions.
- MRI (without or without and with contrast) of the affected body part is indicated for deep tissue involvement, lesions too large for complete ultrasound assessment, inconclusive ultrasound findings, preoperative planning, and post-treatment evaluation.
- CT with contrast is indicated for acute enlargement or compression concerns when MRI is contraindicated.
Venous malformations:
Vasculitis and PET/CT
Vasculitis — Imaging considerations and limitations.
PET/CT is not medically necessary for management of pediatric vasculitis at this time.
Takayasu arteritis (large-vessel vasculitis):
- MRA or CTA is typically used for initial diagnosis and surveillance; PET may have similar accuracy to MRA for initial diagnosis in limited data but has not been shown to improve outcomes or assess treatment response.
- Imaging modality choice (CTA, MRA, or conventional angiography) should be guided by the clinical presentation, suspected vascular territory, and whether endovascular intervention or biopsy is anticipated.
- CTA or contrast-enhanced MRA of the chest (and abdomen as indicated) is recommended for initial evaluation and surveillance of thoracic aortic aneurysm, dissection, or genetically mediated aortopathy per condition-specific intervals.
- Imaging choice should account for need to avoid radiation (younger individuals) versus local availability and clinical urgency.
Aortic congenital vascular malformations:
- Ultrasound (including Doppler) is the initial study for many congenital vascular lesions in neonates and infants when accessible.
- Infants with suspected PHACE(S) should undergo targeted imaging to evaluate for associated cerebrovascular, cardiac, and aortic anomalies per the condition-specific guideline.
- MRI/MRA of the head and neck with and without contrast and echocardiography are commonly indicated for initial evaluation; CTA may be used if MRI/MRA are contraindicated or unavailable.
LUMBAR syndrome and extensive lower extremity involvement:
- Ultrasound with Doppler is an appropriate initial study for superficial vascular/lymphatic findings in the lower extremity.
Modality-Specific and Condition-Specific Indications
Ultrasound as initial exam: Ultrasound (including Doppler) is indicated as the initial examination for superficial soft-tissue and vascular lesions and procedural guidance when not obscured by bone.
Operator-dependent; limited by dense bone and large lesions; used to triage need for advanced imaging.
MRI preferred for soft-tissue extent: MRI (without contrast or without and with contrast) is indicated to characterize deep tissue involvement, evaluate extent and relationship to normal structures, for preoperative planning, and for post-treatment evaluation when ultrasound is inconclusive or lesion is large/deep.
Contrast use per specific indication; avoid repetitive GBCA exposures.
CT/CTA role: CT/CTA is indicated for trauma, complex fractures, pulmonary disease, evaluation of acute complications, or when MRI is contraindicated or inconclusive; CT can be used to characterize lesions but is generally less accurate than MRI for many vascular malformations.
MRI utilizing Xenon Xe 129 (listed as investigational/experimental)
3D rendering CPT CodesCPTCovered
76376
3D rendering; not requiring image post-processing on an independent workstation
76377
3D rendering; requiring image post-processing on an independent workstation
Breast biopsy (MRI-guided) CPT CodesCPTCovered
19085
Biopsy, breast, with placement of breast localization device(s) percutaneous; first lesion, including MR guidance
19086
Biopsy, breast, with placement of breast localization device(s) percutaneous; each additional lesion, including MR guidance
Imaging guidance procedure codesCPTCovered
75989
Imaging guidance for percutaneous drainage with placement of catheter
76942
Ultrasonic guidance for needle placement
77011
CT guidance for stereotactic localization
77012
CT guidance for needle placement
77013
CT guidance for, and monitoring of parenchymal tissue ablation
77021
MR guidance for needle placement
77022
MR guidance for, and monitoring of parenchymal tissue ablation
Bone ablation guidance referenceCPT
20982
CT guidance for bone tumor ablations (note referenced as appropriate for bone)
Prohibited combinationsmixed
No codes listed
Misc CPT guidance for breast biopsyCPT
77021
MR guidance for needle placement — not appropriate for breast biopsy
19085
Breast biopsy, first site (appropriate)
19086
Breast biopsy, additional concurrent biopsies (appropriate)
Guidance codes for percutaneous proceduresCPT
77013
CT guidance for ablation including initial guidance, monitoring repositioning, multiple ablations, and confirmation of coagulative necrosis (non-bone ablation only)
77022
MR guidance for ablation including initial guidance, monitoring repositioning, multiple ablations, and confirmation of coagulative necrosis
20982
CT guidance for bone tumor ablations
77012
Radiologic guidance code (other modality)
76942
Ultrasound guidance
77002
Fluoroscopic guidance
77003
Fluoroscopic guidance (additional)
Unlisted procedure codesCPT
76497
Unlisted CT procedure
76498
Unlisted MR procedure
78999
Unlisted diagnostic nuclear medicine procedure
Limited CT codeCPT
76380
Limited or follow-up CT scan
SPECT/CT and hybrid nuclear/CT codesCPT
78830
Hybrid nuclear/CT scan (single area, single day)
78831
Hybrid nuclear/CT scan (2 or more days)
78832
Hybrid nuclear/CT scan (2 areas with one day and 2-day study)
Imaging for suspected LUMBAR and lower body hemangiomasCPTCovered
76800
Ultrasound, spinal
76856
Ultrasound, pelvic; limited
72148
MRI Lumbar spine without contrast
72158
MRI Lumbar spine without and with contrast
72195
MRI Pelvis without contrast
72197
MRI Pelvis without and with contrast
74181
MRI Abdomen without contrast
74183
MRI Abdomen without and with contrast
74185
MRA Abdomen
72198
MRA Pelvis
1–10 of 15
1/2
Renal function threshold
Renal function thresholdGFR <30 mL/min — both iodinated CT contrast and gadolinium-based MRI contrast carry increased risk at this threshold (parity of contrast risks)
Clinical implicationExercise caution and justify contrast use when GFR <30 mL/min; consider alternative non-contrast imaging or modalities
Provider Requirements, Prior Authorization, and Documentation
Note
Application (For Ohio Only)
This Medical Policy only applies to the state of Ohio. Any requests for services that are stated as unproven or services subject to a coverage or quantity limit will be evaluated for medical necessity using Ohio Administrative Code 5160-1-01 and applicable federal/state/contractual requirements. Before using this policy, providers must check federal, state (OAC), and contractual requirements as they govern in the event of a conflict.
Policy applies to Ohio only; evaluate unproven services per OAC 5160-1-01
Check federal/state/contractual benefit terms; those terms govern
Prior Authorization
Prior Authorization and Repeat Imaging Guidance
Prior authorization requirements may apply for certain advanced imaging, PET/CT, PET-MRI substitution, and 3D rendering codes. Repeat advanced imaging generally requires documentation demonstrating disease progression, new disease, or that the repeat study will affect clinical management. Pre-procedural or pre-operative imaging may require prior authorization consistent with the procedure's authorization requirements; if the procedure is approved (or does not require authorization), appropriate preprocedural imaging may be approved.
Imaging Frequency and Surveillance Intervals
MRI (GBCAs) — repetitive exposure guidance
Gadolinium exposure considerationAssess necessity of repetitive gadolinium-based contrast exposures; limit repeated GBCA use and only use when additional information is expected
Regulatory contextFDA notes no current evidence that gadolinium retention is harmful but recommends limiting unnecessary GBCA exposures
Clinical practicePlan MRI sessions to minimize repeated anesthesia and avoid repeat GBCA when possible by obtaining all indicated body areas in a single session
Repeat imaging — general guidance
General repeat-imaging principleRepeat imaging is not generally necessary unless there is evidence of disease progression, recurrence, or documentation that repeat imaging will affect clinical management
Provider expectation
Contrast Use, Pregnancy, and Renal Function
Note
Note
Note
Note
Note
Note
Note
Note
Exclusions and Not Medically Necessary
PET/MRI is generally not supported. PET‑MRI may only be considered when the individual meets PET‑CT criteria, PET‑CT is not available at the treating institution, and the provider requests PET‑MRI in lieu of PET‑CT; when used, report PET whole‑body (CPT 78813) plus the unlisted MRI code (CPT 76498). Additionally, unbundling PET/CT into separate PET and diagnostic CT CPT codes is not supported. CPT 77021 is not an appropriate code for breast biopsy; MRI‑guided breast biopsy should be reported using CPT 19085 (first lesion) and 19086 (additional concurrent lesions).
PET/MRI is generally not supported. PET‑MRI may only be considered when the individual meets PET‑CT criteria, PET‑CT is not available at the treating institution, and the provider requests PET‑MRI in lieu of PET‑CT; when used, report PET whole‑body (CPT 78813) plus the unlisted MRI code (CPT 76498). Additionally, unbundling PET/CT into separate PET and diagnostic CT CPT codes is not supported. CPT 77021 is not an appropriate code for breast biopsy; MRI‑guided breast biopsy should be reported using CPT 19085 (first lesion) and 19086 (additional concurrent lesions).
PET/MRI is generally not supported. PET‑MRI may only be considered when the individual meets PET‑CT criteria, PET‑CT is not available at the treating institution, and the provider requests PET‑MRI in lieu of PET‑CT; when used, report PET whole‑body (CPT 78813) plus the unlisted MRI code (CPT 76498). Additionally, unbundling PET/CT into separate PET and diagnostic CT CPT codes is not supported. CPT 77021 is not an appropriate code for breast biopsy; MRI‑guided breast biopsy should be reported using CPT 19085 (first lesion) and 19086 (additional concurrent lesions).
PET/MRI is generally not supported. PET‑MRI may only be considered when the individual meets PET‑CT criteria, PET‑CT is not available at the treating institution, and the provider requests PET‑MRI in lieu of PET‑CT; when used, report PET whole‑body (CPT 78813) plus the unlisted MRI code (CPT 76498). Additionally, unbundling PET/CT into separate PET and diagnostic CT CPT codes is not supported. CPT 77021 is not an appropriate code for breast biopsy; MRI‑guided breast biopsy should be reported using CPT 19085 (first lesion) and 19086 (additional concurrent lesions).
Background and Rationale
These evidence‑based guidelines evaluate advanced imaging for cardiovascular and radiology indications in pediatric populations, including peripheral vascular disease. They are intended to guide appropriate imaging selection based on the literature, specialty society guidance, and expert input, while allowing clinicians to exercise independent medical judgment in individual cases.
Definitions — Guidelines
Guidelines definitionEvidence-based clinical guidelines — developed from national/international society guidance, peer-reviewed literature, and expert input
PurposeIntended to guide appropriate imaging selection without replacing clinical judgment
Infantile Hemangioma — incidence and natural history
Infantile Hemangioma (IH) epidemiology
Terms and Definitions
Investigational / Experimental — definition
Investigational/Experimental — definitionLacking sufficient supporting evidence, clinical utility, or collective opinion of support; considered experimental or investigational
Evidence standardsSupporting evidence includes peer-reviewed studies, randomized trials, cohort studies of sufficient power, or specialty society recommendations
Coverage implicationInvestigational procedures may be denied or considered not medically necessary
Standard or conventional imaging — definition
Standard or conventional imaging — definitionIncludes plain film, CT, MRI, and ultrasound; commonly used as initial and subsequent evaluations
Role
Prior Authorization Rules
Note
Note
Note
Note
Note
Note
Note
Document Version and Revision History
v1.0.2025policy_versionLatest
Initial publication of the Cardiovascular and Radiology Imaging Guidelines as version V1.0.2025 (applies to Pediatric Peripheral Vascular Disease imaging guidance).
- Ultrasound with Doppler is the initial study for superficial lesions.
- MRI (without or without and with contrast) is indicated for preoperative assessment to determine extent and relationship to normal structures.
- MRA or CTA has a limited role but may be used when MRI/CT are equivocal and results will impact acute management.
- CT with contrast is indicated when MRI is inconclusive or contraindicated. Consider CT chest with PE protocol/CTA chest when pulmonary embolism is suspected in syndromes with increased thrombotic risk (eg, Klippel-Trénaunay, CLOVES).
Arteriovenous malformations (AVMs) and fistulas:
- Ultrasound with Doppler is the initial examination for superficial lesions.
- MRI (without or without and with contrast) is indicated to evaluate extent and relationship to adjacent structures.
- MRA (contrast as requested) is indicated for evaluation and surveillance of known AVMs; both MRI and MRA may be indicated for preoperative planning.
- CT/CTA (with contrast) is indicated when MRI/MRA are inconclusive or contraindicated.
Vascular tumors:
- Ultrasound with Doppler is the initial examination for superficial vascular tumors and infantile hemangiomas when imaging is needed.
- MRI (without or without and with contrast) is indicated to evaluate extent, relationship to normal structures, and response to therapy.
- MRA may be used for evaluation and surveillance; CT/CTA may be used when MRI/MRA are inconclusive or contraindicated.
- Imaging is directed by organ system involvement (eg, chest imaging for pulmonary disease, brain imaging for CNS involvement) rather than routine whole-vessel imaging; select vascular imaging is used when clinical signs indicate large or medium vessel involvement or complications.
- MRI (with and without contrast as feasible) is indicated for detailed delineation of anatomy, relationship to airway and mediastinal structures, and preoperative planning.
- CTA/CT with contrast can be used when MRI is contraindicated, when faster acquisition is required, or to evaluate acute complications.
- MRI (without and with contrast) of the pelvis and lower extremity is indicated for evaluation of extensive disease, assessment of deeper structures, and preoperative planning.
Operational note: Infantile hemangiomas without high-risk features typically do not require routine imaging. When imaging is indicated for high-risk or complicated lesions, apply the general vascular tumor imaging rules (eg, start with ultrasound, proceed to MRI for extent/planning).
Limit radiation exposure and avoid double-contrast CT unless specifically indicated.
Nuclear medicine/PET: PET/CT is indicated primarily for oncologic, cardiac metabolic, or brain metabolic evaluations and is usually performed as combined PET/CT; PET/MRI is generally not supported except in select circumstances.
Unbundling PET/CT into separate PET and diagnostic CT codes is not supported.
Appropriate uses for CPT 76376/76377
Appropriate uses for CPT 76376/76377
3D rendering clinical scenarios: Use CPT 76376/76377 for 3D rendering in select scenarios such as congenital skull abnormalities (preoperative planning), complex fractures/dislocations (including spine/pelvis/acetabulum/intra-articular), complex facial fractures, cerebral angiography, and select pelvic/abdominal conditions when initial ultrasound is indeterminate or conventional imaging is insufficient.
Document concurrent physician supervision of the 3D reconstruction; 3D rendering codes should not be billed with specific prohibited modalities per coding guidance.
MRI-guided breast biopsy coding and indication
MRI-guided breast biopsy coding and indication
Breast biopsy coding: For MRI-guided percutaneous breast biopsy bill CPT 19085 for the first lesion (including MR guidance) and CPT 19086 for each additional concurrent lesion; CPT 77021 is not appropriate for breast biopsy coding.
Ensure documentation supports percutaneous approach and MR guidance.
Image guidance for tumor ablation
Image guidance for tumor ablation
Ablation guidance indications: Use CPT 77013 (CT) and CPT 77022 (MR) for guidance, monitoring repositioning, multiple ablations, and confirmation of coagulative necrosis during percutaneous tumor ablation; note that CPT 77013 is for non-bone ablation only and CPT 20982 is referenced for CT-guided bone tumor ablation.
Guidance codes apply only to percutaneous procedures and only one unit of a guidance code may be reported per encounter.
Select indications where whole-body modalities may be appropriate
Select indications where whole-body modalities may be appropriate
WBC modalities for cancer predisposition: Interval whole-body MRI may be recommended for cancer screening in individuals with select cancer predisposition syndromes.
Report WBMRI using CPT 76498; WBMRI otherwise not supported as routine cancer screening.
WBMRI for autoimmune disease: WBMRI can be approved in selected situations such as chronic recurrent multifocal osteomyelitis.
Refer to specific pediatric musculoskeletal imaging guidance.
PET-MRI substitution: PET-MRI may be used in lieu of PET-CT when the individual meets PET-CT criteria, PET-CT is unavailable at the treating institution, and the provider requests PET-MRI; when approved, report CPT 78813 + 76498.
Other reporting combinations are inappropriate.
Specific nuclear medicine indications
Specific nuclear medicine indications
Lymphoscintigraphy (CPT 78195) is indicated for evaluation of lower extremity lymphedema when a recent Doppler ultrasound is negative for valvular insufficiency.
Vascular flow and venous thrombosis nuclear studies listed as obsolete are not supported.
Radiopharmaceutical nuclear medicine studies (CPT 78800–78803) may be approved for evaluation of mycotic aneurysm, vascular graft infection, or infection of an indwelling central venous device.
Nuclear medicine is rarely used for routine PVD evaluation; use per specific indications.
CT/CTA indications
CT/CTA indications
CT/CTA primary indications: CT or CTA may be indicated for further evaluation of abnormalities suggested on prior US or MRI, and may be indicated without prior MR/US for lymphatic malformations, vascular abnormalities (vasculitis, thrombosis, aneurysm, dissection, varices), pre-operative planning, or assessment of post-operative complications.
CT should not be substituted for MRI solely to avoid sedation unless explicitly recommended in a condition section; limit radiation exposure when possible.
MRI indicated for lymphatic malformations when any listed condition applies
MRI indicated for lymphatic malformations when any listed condition applies
Lymphatic MRI indications: MRI (without contrast or without and with contrast) of the affected body part is indicated for lymphatic malformations that involve deep tissues, are too large to be completely imaged with ultrasound, have inconclusive ultrasound findings, require preoperative planning, or need post-treatment evaluation.
CT with contrast is limited to acute enlargement with compression concerns when MRI is contraindicated.
Imaging pathway for venous malformations
Imaging pathway for venous malformations
Venous imaging pathway: Begin with ultrasound with Doppler for superficial venous malformations; MRI without or without and with contrast is indicated for preoperative assessment and to evaluate extent and relationship to structures; MRA/CTA have limited roles and are reserved when MRI/CT are equivocal and results will impact acute management; CT with contrast is indicated when MRI is inconclusive or contraindicated.
When pulmonary embolism is suspected in syndromic patients (e.g., Klippel-Trénaunay, CLOVES), CT Chest with PE protocol (CPT 71260) or CTA Chest (CPT 71275) is indicated.
MRI indicated for capillary malformations when neurologic associations suspected
MRI indicated for capillary malformations when neurologic associations suspected
Capillary MRI indications: MRI (without contrast or without and with contrast) is indicated to evaluate occult underlying neurologic structures associated with encephalocele, spinal dysraphism, or Sturge–Weber syndrome when a capillary malformation raises concern.
Use ultrasound first for superficial assessment when appropriate.
AVM, fistula, and vascular tumor imaging recommendations
AVM, fistula, and vascular tumor imaging recommendations
AVM initial imaging: Ultrasound with Doppler is the initial exam for superficial AVMs/fistulas; MRI (without or without and with contrast) is indicated to define extent and relationship to normal structures; MRA (contrast as requested) is indicated for evaluation and surveillance of known AVMs.
CT/CTA may be used when MRI/MRA is inconclusive or contraindicated; both MRI and MRA may be necessary for preoperative planning but are unusual for routine surveillance.
Vascular tumor imaging: Ultrasound with Doppler as the initial exam; MRI with/without contrast indicated for evaluation, extent, relationship to structures, and response to therapy; MRA for evaluation and surveillance; CT/CTA when MRI/MRA inconclusive or contraindicated.
Routine use of both MRI and MRA for surveillance is usually unnecessary.
Evaluation and monitoring of Takayasu arteritis
Evaluation and monitoring of Takayasu arteritis — Covered when ANY of the following modalities are requested for the affected body area(s):
Modalities: MRA of the affected body area(s) (contrast as requested) OR CTA of the affected body area(s) (contrast as requested) OR ultrasound with Doppler of the affected body area(s).
Use the modality most appropriate for the clinical question and document indication.
Imaging intervals: Imaging every 3 months for treatment response during active systemic therapy; annual imaging for surveillance of known involved body areas.
Document treatment status and indication for interval imaging.
Evaluation and monitoring of polyarteritis nodosa
Evaluation and monitoring of polyarteritis nodosa — Covered when ANY of the following modalities are requested for the affected body area(s):
Modalities: MRA (contrast as requested) OR CTA (contrast as requested) OR ultrasound with Doppler of the affected body area(s).
Select modality per clinical question and document indication.
Imaging intervals: Imaging every 3 months during active systemic therapy for treatment response; annual imaging for surveillance of known involved body areas.
See modality-specific contrast guidance in general considerations.
Assessment of end-organ complications in small-vessel vasculitis
Assessment of end-organ complications in small-vessel vasculitis — Advanced imaging generally not indicated; imaging is indicated for evaluation of end-organ damage in specified conditions:
Indications: Ultrasound abdomen (CPT 76700) for HSP when evaluating gastrointestinal complications; CT sinuses (CPT 70486) and/or CT chest (CPT 71250/71260) for GPA/EGPA with new or worsening symptoms, to assess response when treatment change is considered, or annually to evaluate extent of disease.
Advanced imaging is not sensitive for primary assessment of small-vessel vasculitis; reserve imaging for end-organ evaluation or complications.
Imaging modalities indicated: Cardiac MRI (CPT 75557/75561) OR MRA Chest (CPT 71555) OR CT Chest with contrast (CPT 71260) OR CTA Chest (CPT 71275) may be indicated for evaluation of congenital aortic vascular malformations; choice depends on the clinical question and suspected associated findings.
Vascular rings may require concurrent esophagus/trachea imaging; select modality accordingly.
Visceral Artery Aneurysms indications
Visceral artery aneurysm imaging
Pediatric visceral artery imaging: Indications for pediatric visceral artery aneurysm imaging align with adult indications; use modality selection and protocols consistent with aortic and peripheral vascular guidelines.
No pediatric-specific deviations provided in this excerpt.
Infantile Hemangiomas — imaging indications and thresholds
Infantile hemangioma imaging guidance
When to image IH: Most infantile hemangiomas do not require imaging; use ultrasound with Doppler when diagnosis is uncertain or when high-risk clinical considerations exist; apply vascular tumor MRI/MRA/CT guidance when indicated.
Consult a hemangioma specialist when treatment is being considered.
High-risk indicators prompting cardiac evaluation: Multiple (>=5) or large cutaneous infantile hemangiomas may cause high-output cardiac failure; perform echocardiography (CPT 93303 with CPT 93320/93325) when clinically indicated (failure-to-thrive, hyperdynamic precordium, tachycardia, bounding pulses, palpable thrill or bruit).Multiple >=5 or large lesions
Anatomic-triggered imaging: Infantile hemangioma >=2.5 cm over the lumbar spine or sacrum in infants under 6 months indicates spinal ultrasound and/or MRI lumbar spine (CPT 72148/72158); lesions >=5 cm have increased risk of extracutaneous structural abnormalities and may prompt further imaging.>=2.5 cm over lumbar spine in infants <6 months; >=5 cm for increased extracutaneous risk
Choose ultrasound or MRI based on age and ultrasound adequacy.
Multiple (>=5) hemangiomas — screen for hepatic hemangiomas
Covered when ALL of the following are met
Multiple hemangiomas evaluation: Presence of multiple (5 or more) cutaneous infantile hemangiomas.>=5
Initial evaluation with abdominal ultrasound with Doppler (CPT 76700) to assess for hepatic hemangiomas; repeat Doppler ultrasound is indicated to monitor progression or response to treatment.
Suspected PHACE(S) — brain, vascular, cardiac, and orbital imaging
Covered when ANY of the following PHACE(S)-related indications are present
PHACE(S) imaging indications: Large (>=5 cm) infantile hemangioma of the face/scalp/neck OR facial/scalp/neck hemangioma <5 cm with at least one major PHACE(S) anomaly OR large (>=5 cm) upper chest/proximal upper extremity hemangioma with major anomalies OR large intraorbital hemangioma OR clinical suspicion based on other criteria.>=5 cm
Initial imaging includes MRI brain (70551/70553), MRI orbits (70540/70543), MRA head/neck (70544/70546/70547/70548/70549), MRA chest (71555), and screening transthoracic echocardiogram (CPT 93303 with CPT 93320/93325 as indicated); cardiac MRI indicated if echocardiogram abnormal.
Related warningGadolinium exposure in low GFR increases risk of Nephrogenic Systemic Fibrosis (NSF)
Guidance code units per encounter — general
Guidance code reporting ruleOnly one unit of any imaging guidance code should be reported per individual encounter (date of service).
Unit of service definitionUnit of service is the individual encounter, not number of lesions, aspirations, biopsies, injections, or localizations
Applies to modalitiesApplies to CT, MR, US, and fluoroscopic guidance codes (e.g., 77013, 77022, 77012, 77021, 76942, 77002-77003)
Guidance code units per encounter — examples
Example guidance codes (single unit per encounter)77013, 77022, 77012, 77021, 76942, 77002, 77003 — only one unit of these guidance codes may be reported per individual encounter (date of service).
Coding noteThe unit of service is the individual encounter, not the number of lesions or needle placements
Inappropriate practiceReporting multiple units of these guidance codes per encounter may prompt denial
Pediatric age threshold
Pediatric age cutoffIndividuals aged 18 years or younger (<= 18 years) are managed under the Pediatric Peripheral Vascular Disease imaging guidelines
ApplicationUse pediatric-specific modality and anesthesia considerations for patients <= 18 years
Surveillance implicationAge-based surveillance intervals are specified elsewhere for pediatric conditions
Multiple hemangiomas thresholdFive or more cutaneous infantile hemangiomas (>= 5) — triggers evaluation for hepatic hemangiomas via abdominal ultrasound with Doppler
Follow-upRepeat Doppler ultrasound abdomen indicated to monitor hepatic hemangiomas for progression or treatment response
Clinical riskMultiple (>=5) cutaneous IHs are associated with increased risk of hepatic involvement and high-output cardiac complications
Infantile hemangioma size risk thresholds
Size thresholds (disfigurement and spinal risk)>= 5 cm diameter — increased risk for disfigurement and extracutaneous structural abnormalities; >= 2.5 cm over the lumbar spine (in infants <6 months) — indication for spinal ultrasound and/or MRI lumbar spine
Action for >=2.5 cm lumbar lesionsPerform spinal ultrasound if <=6 months and/or MRI lumbar spine (CPT 72148/72158) as indicated
Clinical implicationLarge lesions (>=5 cm) warrant specialist evaluation and consideration of additional imaging and cardiac assessment if indicated
Cutaneous hemangiomas — liver ultrasound trigger
Trigger for liver ultrasoundPresence of five or more (>= 5) cutaneous infantile hemangiomas — indicates abdominal ultrasound with Doppler to screen for hepatic hemangiomas
Coding exampleUse CPT 76700 for ultrasound abdomen with Doppler when indicated
MonitoringRepeat Doppler ultrasound abdomen may be used to monitor progression or response to treatment
Size threshold for 'large' hemangioma
Definition of 'large' hemangioma>= 5 cm diameter — considered large; associated with increased risk and prompts evaluation for PHACE(S) or LUMBAR syndromes depending on location
Implication for PHACE(S)Facial/scalp/neck hemangiomas >=5 cm warrant brain/orbit MRI and MRA head/neck and echocardiographic screening
Implication for LUMBARLower-body hemangiomas >=5 cm prompt spinal ultrasound (<=6 months) and MRI spine/pelvis/abdomen as indicated
Repeat imaging: require evidence of progression/new disease or documentation that imaging will affect management
Pre-procedural imaging: may require prior authorization when the procedure requires it
Providers must review prior imaging and diagnostic tests before ordering advanced imaging
Prior Authorization
PET-MRI Reporting and Substitution Conditions
PET-MRI is not routinely supported. PET-MRI may be considered only when guideline criteria for PET-CT are met and PET-CT is not available at the treating institution; the provider must request PET-MRI in lieu of PET-CT. When allowed, report PET whole-body (CPT 78813) plus the MRI unlisted code (CPT 76498). Other reporting methods for PET-MRI are inappropriate. Note that PET/CT is not medically necessary for management of pediatric vasculitis at this time and requests for PET/CT for pediatric vasculitis may be denied.
PET-MRI allowed only if PET-CT criteria are met and PET-CT unavailable at treating institution
Report PET-MRI as CPT 78813 + CPT 76498 when authorized
PET/CT not medically necessary for pediatric vasculitis — likely denial risk
Prior Authorization
Prior Authorization for 3D Rendering (CPT 76376 / 76377)
3D rendering codes (CPT 76376, 76377) require documentation of concurrent physician supervision or participation in the post-processing and may require prior authorization even when the underlying imaging does not. Use CPT 76376 when no independent workstation is required; use CPT 76377 when an independent workstation is required. Do not use these codes for 2D reformatting.
Document physician supervision/participation in 3D reconstruction per ACR recommendations
Prior authorization may be required for 76376/76377 even if base study is not pre-authorized
Do not use 76376/76377 for 2D reformatting
Documentation Required
Clinical Documentation, Prior Imaging Review, and Outside Study Interpretation
Clinical documentation must substantiate medical necessity for advanced imaging or designated procedures. A pertinent clinical evaluation since onset or change in symptoms is required (recent history, physical exam, relevant labs, and prior imaging). Providers should obtain and review prior imaging and diagnostic test results before ordering further advanced imaging. When an outside exam is interpreted as a secondary interpretation, report CPT 76140 and document the reason for the secondary read.
Submit recent detailed history, physical exam, labs, and prior imaging when requesting advanced imaging
Review prior imaging before ordering additional studies
Use CPT 76140 for secondary interpretation of outside exams and document rationale
Billing Rule
Coding, Unlisted Procedures, Limited CT, and Guidance Code Unit Reporting
Coding and billing rules: use recommended modality-specific CPT codes for indicated studies; when no anatomic site–specific code exists, report unlisted CT/MR (CPT 76497/76498) and document that no Category I code applies and prefer reporting a relevant Category III code if available. Do not use CPT 76380 to report 'extra slices' with diagnostic CT codes. For percutaneous image-guidance codes (e.g., CPT 77013, 77022, 76942), only one unit should be reported per encounter; reporting multiple units for guidance codes per encounter is inappropriate.
When appropriate anatomic CPT exists, use it; otherwise use CPT 76497/76498 with documentation
Prefer Category III code over unlisted when available
Do not report CPT 76380 to cover extra slices alongside diagnostic CT codes
Report only one unit of percutaneous guidance codes per encounter
Note
Modality Selection, Sequencing, and Documentation of Indication/Interval
Modalities and sequencing: providers should escalate from less invasive/lower-cost modalities (e.g., ultrasound with Doppler) to advanced modalities when clinically appropriate. Ultrasound with Doppler is the initial exam for superficial lesions; MRI (with or without contrast) is indicated for deeper, large, or inconclusive ultrasound findings and for preoperative planning. CT/CTA may be used when MRI is contraindicated, inconclusive, or to further evaluate abnormalities suggested on ultrasound or MRI. Document the chosen modality, clinical indication, and the intended imaging interval/feedback loop (e.g., every 3 months for active treatment response; annually for surveillance where growth risks organ dysfunction).
Start with ultrasound for superficial lesions; escalate to MRI for deeper or complex lesions
CT/CTA used when MRI is contraindicated/inconclusive or to further characterize findings
Document indication and imaging interval (treatment response every 3 months during active therapy; annual surveillance when indicated)
Denial Risk
Overutilization Triggers and Screening-related Denial Risk
Overutilization triggers that may prompt review or denial include duplicate or questionably indicated exams, requests for CT with and without contrast (double-contrast) without clear indication, use of MRI solely to avoid radiation without clinical justification, and ordering advanced imaging for asymptomatic screening of peripheral vascular disease. Providers should ensure requested imaging will impact management and that less invasive options have been considered.
Common triggers: duplicate exams, unjustified CT with and without contrast, MRI requested to avoid radiation without indication
Advanced imaging for asymptomatic peripheral vascular screening is not supported and may be denied
Requests must show how imaging will change management
Documentation Required
Referenced and Specified CPT Codes for Indicated Imaging
Certain CPT-coded imaging studies are explicitly referenced for indicated assessments across the pediatric PVD guidance. Examples include, but are not limited to: Ultrasound with Doppler (various CPT codes), CT Chest protocols CPT 71250/71260/71275, MRI brain/orbits (CPT 70551/70553/70540/70543), MRA head/neck/chest (CPT 70544/70546/70547/70548/70549/71555), cardiac MRI (CPT 75557, 75561), PET whole-body (CPT 78813), unlisted MRI/CT (CPT 76498/76497), guidance codes (CPT 77013/77022), interpretation of outside studies (CPT 76140), and echocardiography (CPT 93303 with 93320/93325). Include the applicable CPT codes in documentation when requesting authorization.
Include modality-specific CPT codes (e.g., CPT 75557/75561, 71555, 71260, 71275, 78813, 76498, 76497, 76140, 77013, 77022, 93303) in requests
Document how the requested CPT-coded study maps to the clinical indication
Denial Risk
Imaging for Small-Vessel Vasculitis, PET/CT Denial Risk, and Interval Documentation
Imaging for small-vessel vasculitis should be reserved for evaluation of end-organ complications or complications of disease (e.g., abdominal ultrasound CPT 76700 for Henoch-Schönlein Purpura). PET/CT is not medically necessary for pediatric vasculitis management and is a denial risk. For conditions such as Takayasu arteritis or polyarteritis nodosa, document the chosen modality (MRA, CTA, or ultrasound), and adhere to indicated imaging intervals (every 3 months during active systemic therapy; annually for surveillance).
Reserve advanced imaging for small-vessel vasculitis primarily to assess end-organ complications (e.g., CPT 76700 for abdominal complications)
Do not approve PET/CT for routine pediatric vasculitis management
Document modality choice and interval when requesting serial monitoring
Ensure recent pertinent clinical evaluation and prior imaging review before ordering repeat advanced imaging
Prior authorization implicationRepeat studies lacking documented impact on management may not be approved or may require justification
Repeat imaging — requirement to affect management
Required evidence for repeatsRepeat imaging should only be performed when there is evidence of progression, new disease, or documentation that imaging will change management
Documentation neededClinical notes must show how repeat imaging results will influence treatment decisions or surveillance intervals
ExceptionsGuideline-supported scheduled follow-up and active treatment response imaging are exceptions (see condition-specific intervals)
Imaging interval — treatment response
Treatment response intervalImaging for treatment response may be approved every 3 months during active systemic therapy
ApplicabilityApplies to individuals with aggressive lesions receiving systemic therapy where imaging will inform treatment response
DocumentationOrder should document active systemic therapy and clinical question driving 3-month interval imaging
Surveillance imaging — annual interval
Surveillance intervalAnnual surveillance imaging may be approved for body areas at risk where growth could cause significant organ dysfunction or impairment
Use caseApplies to known vascular or lymphatic malformations under surveillance
DocumentationOrder should indicate surveillance intent and prior imaging or clinical rationale
MRA/CTA/Ultrasound with Doppler — active treatment interval
Active treatment monitoring — intervalMRA/CTA/Ultrasound with Doppler every 3 months during active systemic treatment for treatment response
Modalities specifiedMRA, CTA, and ultrasound with Doppler are acceptable modalities for serial monitoring during active treatment
DocumentationIndicate active systemic therapy and intended use (response assessment) when requesting imaging at 3-month intervals
Any indicated modality — annual surveillance
Surveillance modality frequencyAny indicated imaging modality for surveillance of known involved body areas may be performed annually
ContextAnnual surveillance intended to detect progressive vascular damage that may require intervention
DocumentationOrder should reference prior abnormal imaging or clinical rationale for annual surveillance
Echocardiogram / CTA or MRA — age-based surveillance
Echocardiogram / CTA or MRA — age-based intervalsSurveillance intervals for thoracic aortic disease vary by age: 0–2 years every 3 months; 3–12 years every 6 months; >=13 years annually if diameter <4.5 cm and growth <0.5 cm/year, otherwise every 6 months
Initial timingAt diagnosis and at 6 months after diagnosis (if older than 2 years) additional imaging is recommended
Modality pairingEchocardiogram with CTA or MRA of chest/abdomen/pelvis/head/neck as indicated for surveillance
Echocardiogram and CTA/MRA — 6-month follow-up
6-month post-diagnosis imagingPerform additional echocardiogram and CTA/MRA at 6 months after diagnosis if patient is older than 2 years
Follow-up planContinue surveillance per age-based schedule thereafter (see age-based intervals)
IndicationApplies when prior thoracic imaging was abnormal or genetic aortopathy suspected/confirmed
Note
PET/MRI is generally not supported. PET‑MRI may only be considered when the individual meets PET‑CT criteria, PET‑CT is not available at the treating institution, and the provider requests PET‑MRI in lieu of PET‑CT; when used, report PET whole‑body (CPT 78813) plus the unlisted MRI code (CPT 76498). Additionally, unbundling PET/CT into separate PET and diagnostic CT CPT codes is not supported. CPT 77021 is not an appropriate code for breast biopsy; MRI‑guided breast biopsy should be reported using CPT 19085 (first lesion) and 19086 (additional concurrent lesions).
PET/MRI is generally not supported. PET‑MRI may only be considered when the individual meets PET‑CT criteria, PET‑CT is not available at the treating institution, and the provider requests PET‑MRI in lieu of PET‑CT; when used, report PET whole‑body (CPT 78813) plus the unlisted MRI code (CPT 76498). Additionally, unbundling PET/CT into separate PET and diagnostic CT CPT codes is not supported. CPT 77021 is not an appropriate code for breast biopsy; MRI‑guided breast biopsy should be reported using CPT 19085 (first lesion) and 19086 (additional concurrent lesions).
Advanced imaging for asymptomatic screening of peripheral vascular disease is not supported. Specifically, nuclear medicine vascular flow imaging (CPT 78445) and venous thrombosis imaging codes (CPT 78456‑78458) are obsolete and are not supported for screening purposes.
PET/CT for the management of pediatric vasculitis is considered not medically necessary and is not recommended for routine management or assessment of treatment response in this population.
Occurs in approximately 5% of infants; rapid growth phase between 1–3 months with involution usually completed by age 4 in ~90%
Natural historyMost IHs do not require imaging; imaging reserved for diagnostic uncertainty, high-risk features, or syndromic concerns
Associated risksLarge or multiple lesions can cause high-output cardiac failure, airway obstruction, ulceration, disfigurement, or extracutaneous abnormalities
Infantile hemangiomas are the most common benign tumor of childhood, occurring in approximately 5% of infants and typically showing rapid growth between 1–3 months with involution completed in about 90% by age 4. Most IHs do not require imaging; ultrasound with Doppler is appropriate when the diagnosis is uncertain or when high‑risk features or syndromic concerns are present (for example, multiple lesions, large size or lumbar/sacral location).
Standard imaging is favored before advanced imaging unless condition-specific guidance indicates otherwise
Contrast considerationAppropriate use of contrast (without, with, without and with) is determined by condition-specific guidance
Concurrent supervision (3D rendering) — definitionActive physician participation in and monitoring of 3D reconstruction, including selection of anatomy, tissues, archived images, and adjustment of 3D product
DocumentationACR recommends documenting the physician's supervision/participation in 3D reconstruction
Prior authorization noteProviders may be required to obtain prior authorization on 3D rendering codes even if the base imaging does not require it
Guidance codes — definition and reporting rule
Guidance codes — definitionRadiologic guidance codes (e.g., 77013, 77022, 77012, 77021, 76942, 77002-77003) describe modality-specific imaging guidance for percutaneous procedures; only one unit may be reported per encounter
Appropriate useShould be billed only for percutaneous surgical procedures (not open/excisional/incisional surgeries)
Unit of serviceUnit equals individual encounter (date of service), not number of lesions
Unlisted CT/MR procedure — guidance
Unlisted CT/MR procedure (76497/76498) — guidanceUse when no anatomic site-specific CPT code exists; prefer a Category III code if available; document when used
Example usesNavigation/planning for neurosurgical procedures, custom joint arthroplasty planning, or studies requiring thinner cuts or different positions
Coding noteReport Category III code instead of unlisted if available
Imaging modality types — definition
Imaging modality types — defined examplesIncludes MRA, CTA, Ultrasound, Duplex, Nuclear Medicine, and PET with listed CPT procedure examples for each
Modality selectionChoice of modality depends on clinical question, body area, and contraindications (e.g., implants, need to avoid anesthesia)
Coding referenceSpecific CPT codes for MRA/CTA/US/Duplex/PET are listed in the guideline procedure code tables
Lymphatic malformation — definition
Lymphatic malformation — definitionDilated lymphatic channels filled with proteinaceous fluid that do not connect to normal lymphatic channels; typically soft, non-pulsatile masses
Clinical noteOften evaluated initially with ultrasound; MRI indicated for deep, large, inconclusive, or preoperative cases
Imaging implicationCT generally has limited role compared to MRI unless MRI contraindicated or inconclusive
Venous malformation — definition
Venous malformation — definitionSlow-flow lesions with dilated venous spaces, compressible and non-pulsatile; may change size with Valsalva and associate with syndromes
Clinical implicationUltrasound with Doppler is initial exam; MRI indicated for extent/preoperative planning
Associated syndromesMay appear in Klippel-Trenaunay, BRBN, Maffucci, Proteus, CLOVES, and others
Arteriovenous malformation (AVM) — definition
Arteriovenous malformation (AVM) — definitionNetwork of abnormal vascular channels between enlarged feeding arteries and draining veins lacking a normal capillary bed; often pulsatile with thrill or bruit
Clinical courseMay be aggressive, present at birth or grow near adolescence; ultrasound initial for superficial lesions, MRI/MRA for extent and surveillance
Imaging implicationCT/CTA used when MRI/MRA inconclusive or contraindicated
Vascular tumors — definition
Vascular tumors — definitionA heterogeneous group including infantile hemangiomas, epithelioid hemangioma, kaposiform hemangioendothelioma, Kaposi sarcoma, epithelioid hemangioendothelioma, and angiosarcoma
Imaging approachUltrasound with Doppler initial for superficial lesions; MRI for extent and response; MRA for surveillance when indicated
Clinical implicationSpecific tumor type and clinical features determine need and type of imaging
Takayasu arteritis — definition
Takayasu arteritis — definitionPredominant large vessel vasculitis occurring in children
Imaging implicationRequires MRA/CTA/Ultrasound with Doppler for evaluation and serial monitoring per guideline intervals
Clinical noteSurveillance intervals specified for active treatment and routine follow-up
Familial Aortopathies — definition
Familial aortopathies — definition and examplesInherited connective tissue disorders (e.g., Marfan, Ehlers-Danlos, Loeys-Dietz, FBN1/TGFBR1/TGFBR2/ACTA2/MYH11 mutations) increasing risk for aortic aneurysm/dissection
Imaging implicationRequire echocardiogram and may need CTA/MRA surveillance of head/neck/chest/abdomen/pelvis per age- and disease-based intervals
Screening noteScreening recommendations include family-history based echo and genetic considerations
PHACE(S) syndrome — definition
PHACE(S) syndrome — definition and componentsPosterior fossa malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta and Cardiac defects, Eye abnormalities; adding S denotes Sternal cleft or supraumbilical raphe
Clinical relevanceLarge facial/scalp/neck hemangiomas (>=5 cm) raise suspicion for PHACE(S) and prompt brain/orbit MRI, MRA head/neck/chest and echocardiographic screening
Imaging pathwayInitial imaging includes MRI brain/orbits and MRA head/neck/chest; echocardiogram (CPT 93303) with further cardiac MRI if abnormalities found
LUMBAR syndrome — definition
LUMBAR syndrome — definitionAssociation of Lower body infantile hemangiomas >=5 cm with Urogenital anomalies, Myelopathy (e.g., tethered cord), Bony deformities, Anorectal malformations, Arterial and Renal anomalies
Clinical triggerLarge (>=5 cm) lumbosacral/perineal or lower-extremity hemangiomas prompt spinal ultrasound and MRI as outlined in guideline
Imaging implicationSpine US (<=6 months), MRI lumbar spine (3–6 months) and MRI abdomen/pelvis/MRA as indicated by specialist recommendation or inadequate US