Phlebolymphedema: Is It A New Concept?

Venous and lymphatic systems are inseparable "dual" outflow systems with mutually complimentary function so that such mutual interdependence between these two systems causes a new condition to affect both systems simultaneously when one of the two systems should fail to provide sufficient compensation to the other system, known as "phlebolymphedema" (PLE): combined condition of chronic venous insufficiency (CVI)/chronic venous hypertension and chronic lymphatic insufficiency (CLI)/chronic lymphedema. PLE is, therefore, an unavoidable outcome of the joint failure of this "inseparable" venous-lymphatic circulation system, presenting as a combined condition of venolymphatic edema caused by CVI and CLI due to various etiopathogenesis. PLE can be managed more effectively when open and/or endovascular therapy is added to basic compression therapy to control the CVI and CLI together. Primary PLE is caused mostly by vascular malformation components of Klippel-Trenaunay syndrome as the combined condition of CVI attributed to marginal vein (MV)/venous malformation and CLI attributed to primary lymphedema/lymphatic malformation. CVI attributed to reflux of MV can be treated with MV resection, whereas CVI attributed to deepvein dysplasia can usually be treated with conventional compression therapy alone. Secondary PLE is usually the outcome of deep-vein thrombosis (DVT)/postthrombotic syndrome (PTS). CVI attributed to PTS can be further improved with correction of the venous outflow obstruction with angioplasty and stenting, especially when the DVT sequalae is involved at multiple levels of the iliacfemoral-popliteal vein system.