Lymphangiographic Classification of Primary Lymphedema
| Congenital primary lymphedema | Acquired primary lymphedema |
|---|---|
| Aplasia or hypoplasia of lymphatics | Intraluminal or intramural lymphangio-obstructive edema |
| Abnormalities of abdominal or thoracic lymph trunks | Distal |
| Valvular incompetence (associated with megalymphatics and often | Proximal obliteration, often with distal dilatation |
| chylous reflux) | Combined |
| Obstruction of lymph nodes by hilar fibrosis |
Modified from Browse et al. (23).