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Clinically Suspected Vascular Malformation of the Extremities

Variant: 1   Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
Procedure Appropriateness Category Relative Radiation Level
US duplex Doppler extremity area of interest Usually Appropriate O
MRA extremity area of interest without and with IV contrast Usually Appropriate O
MRI extremity area of interest without and with IV contrast Usually Appropriate O
CTA extremity area of interest with IV contrast Usually Appropriate Varies
US extremity area of interest with IV contrast May Be Appropriate O
MRA extremity area of interest without IV contrast May Be Appropriate O
MRI extremity area of interest without IV contrast May Be Appropriate O
CT extremity area of interest with IV contrast May Be Appropriate Varies
CT extremity area of interest without IV contrast May Be Appropriate Varies
Arteriography extremity area of interest Usually Not Appropriate Varies
Radiography extremity area of interest Usually Not Appropriate Varies
CT extremity area of interest without and with IV contrast Usually Not Appropriate Varies

Variant: 2   Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
Procedure Appropriateness Category Relative Radiation Level
US duplex Doppler extremity area of interest Usually Appropriate O
MRA extremity area of interest without and with IV contrast Usually Appropriate O
MRI extremity area of interest without and with IV contrast Usually Appropriate O
CTA extremity area of interest with IV contrast Usually Appropriate Varies
US extremity area of interest with IV contrast May Be Appropriate O
Arteriography extremity area of interest May Be Appropriate Varies
MRA extremity area of interest without IV contrast May Be Appropriate (Disagreement) O
MRI extremity area of interest without IV contrast May Be Appropriate O
CT extremity area of interest with IV contrast May Be Appropriate Varies
CT extremity area of interest without and with IV contrast May Be Appropriate (Disagreement) Varies
Radiography extremity area of interest Usually Not Appropriate Varies
CT extremity area of interest without IV contrast Usually Not Appropriate Varies

Panel Members
Piotr Obara, MDa; Justin McCool, MDb; Sanjeeva P. Kalva, c; Bill S. Majdalany, MDd; Jeremy D. Collins, MDe; Jens Eldrup-Jorgensen, MDf; Suvranu Ganguli, MDg; Andrew J. Gunn, MDh; A. Tuba Karagulle Kendi, MDi; Minhaj S. Khaja, MD, MBAj; Patrick D. Sutphin, MD, PhDk; Kanupriya Vijay, MD, MBBSl; Karin E. Dill, MDm.
Summary of Literature Review
Introduction/Background
Special Imaging Considerations
Initial Imaging Definition

Initial imaging is defined as imaging at the beginning of the care episode for the medical condition defined by the variant. More than one procedure can be considered usually appropriate in the initial imaging evaluation when:

  • There are procedures that are equivalent alternatives (i.e., only one procedure will be ordered to provide the clinical information to effectively manage the patient’s care)

OR

  • There are complementary procedures (i.e., more than one procedure is ordered as a set or simultaneously wherein each procedure provides unique clinical information to effectively manage the patient’s care).
Discussion of Procedures by Variant
Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
A. Radiography Extremity
Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
B. US Duplex Doppler Extremity
Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
C. US Extremity Area of Interest with IV Contrast
Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
D. CT Extremity
Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
E. CTA Extremity
Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
F. MRI Extremity
Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
G. MRA Extremity
Variant 1: Upper or lower extremity. Suspected vascular malformation presenting with pain or findings of physical deformity including soft-tissue mass, diffuse or focal enlargement, discoloration, or ulceration. Initial imaging.
H. Arteriography Extremity
Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
A. Radiography Extremity
Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
B. US Duplex Doppler Extremity
Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
C. US Extremity Area of Interest with IV Contrast
Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
D. CT Extremity
Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
E. CTA Extremity
Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
F. MRI Extremity
Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
G. MRA Extremity
Variant 2: Upper or lower extremity. Vascular murmur (bruit or thrill). Initial imaging.
H. Arteriography Extremity
Summary of Highlights
Supporting Documents

The evidence table, literature search, and appendix for this topic are available at https://acsearch.acr.org/list. The appendix includes the strength of evidence assessment and the final rating round tabulations for each recommendation.

For additional information on the Appropriateness Criteria methodology and other supporting documents, please go to the ACR website at https://www.acr.org/Clinical-Resources/Clinical-Tools-and-Reference/Appropriateness-Criteria.

Gender Equality and Inclusivity Clause
The ACR acknowledges the limitations in applying inclusive language when citing research studies that predates the use of the current understanding of language inclusive of diversity in sex, intersex, gender, and gender-diverse people. The data variables regarding sex and gender used in the cited literature will not be changed. However, this guideline will use the terminology and definitions as proposed by the National Institutes of Health.
Appropriateness Category Names and Definitions

Appropriateness Category Name

Appropriateness Rating

Appropriateness Category Definition

Usually Appropriate

7, 8, or 9

The imaging procedure or treatment is indicated in the specified clinical scenarios at a favorable risk-benefit ratio for patients.

May Be Appropriate

4, 5, or 6

The imaging procedure or treatment may be indicated in the specified clinical scenarios as an alternative to imaging procedures or treatments with a more favorable risk-benefit ratio, or the risk-benefit ratio for patients is equivocal.

May Be Appropriate (Disagreement)

5

The individual ratings are too dispersed from the panel median. The different label provides transparency regarding the panel’s recommendation. “May be appropriate” is the rating category and a rating of 5 is assigned.

Usually Not Appropriate

1, 2, or 3

The imaging procedure or treatment is unlikely to be indicated in the specified clinical scenarios, or the risk-benefit ratio for patients is likely to be unfavorable.
Relative Radiation Level Information

Potential adverse health effects associated with radiation exposure are an important factor to consider when selecting the appropriate imaging procedure. Because there is a wide range of radiation exposures associated with different diagnostic procedures, a relative radiation level (RRL) indication has been included for each imaging examination. The RRLs are based on effective dose, which is a radiation dose quantity that is used to estimate population total radiation risk associated with an imaging procedure. Patients in the pediatric age group are at inherently higher risk from exposure, because of both organ sensitivity and longer life expectancy (relevant to the long latency that appears to accompany radiation exposure). For these reasons, the RRL dose estimate ranges for pediatric examinations are lower as compared with those specified for adults (see Table below). Additional information regarding radiation dose assessment for imaging examinations can be found in the ACR Appropriateness Criteria® Radiation Dose Assessment Introduction document.

Relative Radiation Level Designations

Relative Radiation Level*

Adult Effective Dose Estimate Range

Pediatric Effective Dose Estimate Range

O

0 mSv

 0 mSv

☢

<0.1 mSv

<0.03 mSv

☢☢

0.1-1 mSv

0.03-0.3 mSv

☢☢☢

1-10 mSv

0.3-3 mSv

☢☢☢☢

10-30 mSv

3-10 mSv

☢☢☢☢☢

30-100 mSv

10-30 mSv

*RRL assignments for some of the examinations cannot be made, because the actual patient doses in these procedures vary as a function of a number of factors (e.g., region of the body exposed to ionizing radiation, the imaging guidance that is used). The RRLs for these examinations are designated as “Varies.”
References
1. Nassiri N, Cirillo-Penn NC, Thomas J. Evaluation and management of congenital peripheral arteriovenous malformations. J Vasc Surg. 62(6):1667-76, 2015 Dec.
2. Kramer U, Ernemann U, Fenchel M, et al. Pretreatment evaluation of peripheral vascular malformations using low-dose contrast-enhanced time-resolved 3D MR angiography: initial results in 22 patients. AJR Am J Roentgenol. 196(3):702-11, 2011 Mar.
3. McCafferty I.. Management of Low-Flow Vascular Malformations: Clinical Presentation, Classification, Patient Selection, Imaging and Treatment. [Review]. Cardiovasc Intervent Radiol. 38(5):1082-104, 2015 Oct.
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17. Wiesinger I, Jung W, Zausig N, et al. Evaluation of dynamic effects of therapy-induced changes in microcirculation after percutaneous treatment of vascular malformations using contrast-enhanced ultrasound (CEUS) and time intensity curve (TIC) analyses. Clin Hemorheol Microcirc. 69(1-2):45-57, 2018.
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Disclaimer

The ACR Committee on Appropriateness Criteria and its expert panels have developed criteria for determining appropriate imaging examinations for diagnosis and treatment of specified medical condition(s). These criteria are intended to guide radiologists, radiation oncologists and referring physicians in making decisions regarding radiologic imaging and treatment. Generally, the complexity and severity of a patient’s clinical condition should dictate the selection of appropriate imaging procedures or treatments. Only those examinations generally used for evaluation of the patient’s condition are ranked.  Other imaging studies necessary to evaluate other co-existent diseases or other medical consequences of this condition are not considered in this document. The availability of equipment or personnel may influence the selection of appropriate imaging procedures or treatments. Imaging techniques classified as investigational by the FDA have not been considered in developing these criteria; however, study of new equipment and applications should be encouraged. The ultimate decision regarding the appropriateness of any specific radiologic examination or treatment must be made by the referring physician and radiologist in light of all the circumstances presented in an individual examination.