Table 2. Classification systems with outcomes (8,14,18,19,26,36-38).
Grading scheme | Modality | Category | Anatomic features and hallmarks | Treatment | Implications for treatment |
---|---|---|---|---|---|
Goyal et al. | MRI based | Grade 1 | Well defined, ≤5 cm diameter | Sclerotherapy (as monotherapy) | 71% excellent results, 29% good results, 0% poor results |
Grade 2A | Well defined, >5 cm | 22% excellent results, 44% good results, 33% poor results | |||
Grade 2B | Ill defined, ≤5 cm diameter | 26% excellent results, 15% good results, 60% poor results | |||
Grade 3 | Ill defined, >5 cm | 0% excellent results, 43% good results, 57% poor results | |||
Fayad et al. | MRI based | Extent | Focal, multifocal, or diffuse | Sclerotherapy | Multifocal treatments and multiple sessions are required |
Tissue layer involvement | Skin/subcutaneous/muscle/tendon/bony cortex/marrow | Risk of skin or nerve injury is considered; muscular involvement; risk of contracture; bone or marrow involvement; risk of fracture |
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Connection | Deep venous system | Increased risk of deep venous thrombosis | |||
Dubois | DPP based | Cavitary | Cavities with late venous drainage without abnormal veins | Sclerotherapy | Improved results |
Spongy | Small, ‘honeycomb’ cavities and venous drainage | Difficult to treat, particularly when the VM is intramuscular | |||
Dysmorphic | Rapid opacification of dysmorphic veins | Improved results, however recurrence is likely | |||
Dubois/Puig | DPP based | Type I | Isolated, well circumscribed without visible venous drainage | Sclerotherapy | Highest rate of cure or satisfactory result, 92.3% complication-free predictive value |
Type II | Malformation draining into normal veins | High rate of cure or satisfactory result, 93.8% complication-free predictive value | |||
Type III | Malformation draining into ectatic dysplastic veins | 50% exclusion rate, higher risk of complications | |||
Type IV | Venous ectasia | 60% exclusion rate & higher risk of complications | |||
Berenguer et al. | Direct contrast venogram | Lobulated | Rounded clusters of vascular spaces | Sclerotherapy (multiple therapy sessions, 1–10) | 27% in all three categories rated outcome as near normal, 38% reported marked improvement, 13% reported slight improvement, and 4% reported no change |
Varicose | Irregular dilated channels | No significant difference in outcome could be shown between categories | |||
Combined | Combination of the above | ||||
Boston Children’s | Based on imaging and clinical features | Spongiform | Large sponge-like singular lesion | – | Most common type |
Phlebectatic | Large diameter combined vascular malformation | Increased risk of deep vein thrombosis and pulmonary embolism | |||
Aneurysmatic | Involves large veins such as inferior vena cava | Increased risk of deep vein thrombosis and pulmonary embolism | |||
Reticular | Small veins, spider-like appearance, combined vascular syndromes | 50% have spontaneous Tie-2 mutations (unlike the autosomal dominant inheritance pattern found in familial mucocutaneous VMs) |
VM, venous malformation.