Classifying these Anomalies: 4 Questions
Question 1: Is this a high-flow lesion?
The goal is to separate the low-flow vascular malformations (lymphatic and venous malformations) from the high flow lesions, which include the proliferative vascular anomalies and any arterial-containing vascular malformation. To determine if a lesion has high flow, look for the presence of numerous flow voids and flow-related enhancement on GRE sequences. On dynamic contrast-enhanced sequences (time-resolved imaging), high-flow lesions enhance avidly within six seconds of adjacent supplying arterial enhancement; more slowly enhancing lesions are deemed low-flow lesions. Comparison with the contralateral side may be helpful to assess for preferential flow.
If the answer to this question is yes, go to question 2.
Otherwise, skip to question 3.
Question 2: Is there a soft tissue mass?
This question applies to the high-flow vascular anomalies, and is used to distinguish the arteriovenous malformations and fistulas from the proliferative vascular anomalies. AVM’s and AVF’s typically present with flow voids without a discrete mass, whereas the presence of a mass and characteristic growth pattern leads one towards the proliferative anomalies.
If the answer to this question is yes, skip to question 4.
If not, you’re left with an AVM or AVF.
Question 3: Is there septal or more diffuse enhancement?
This question applies to the low-flow vascular anomalies, and is used to distinguish lymphatic from venous malformations, as described above. Remember, lymphatic and venous malformations can coexist (venolymphatic or lymphaticovenous malformations).
Question 4: Could this be something more ominous?
This question applies to the high-flow proliferative vascular anomalies, and should always be considered when the clinical course does not follow that expected of an infantile or congenital hemangioma or when imaging findings are atypical (Fig. 10). Biopsy would then be recommended. Potential etiologies include Kaposiform hemangioendothelioma (KHE), tufted angioma (TA), fibromatoses (Fig. 10), and malignant lesions such as the congenital infantile fibrosarcoma.
Summary
The evaluation and treatment of vascular anomalies remains difficult, even under the best of circumstances. At New York University, we’ve found that the best approach to tackling these lesions involves a multidisciplinary vascular anomalies team comprised of pediatricians, surgeons, dermatologists, and radiologists. Together, a consensus is achieved regarding the diagnosis, and the need for treatment.
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