WHAT IS PHLEBOLYMPHEDEMA - Primary Phlebolymphedema - Secondary Phlebolymphedema - Pediatric and Primary Lymphedema - Secondary Lymphedema - Lymphostatic Elephantiasis - Rare Disease

Phlebolymphedema is a unique condition of combined insufficiency of both the venous and lymphatic systems. There is a very close relationship between the venous and lymphatic systems; therefore a venous obstruction may develop a phlebo-lymphedema. Here we find a failure in the dual outflow system, and although being totally different hemodynamic mechanisms, they are mutually dependant and complementary circulation systems. Due to their intimately integrated function, failure in one system means then additional burden and overload of the other.

There are various causes: Primary Phlebolymphedema and Secondary Phlebolymphedema

Primary Phlebolymphedema is caused by a congenital defect, involving an outset of diffused trancular lymphatic malformation, affecting both the venous and lymphatic systems. Primary Phlebolymphedema is still a relatively unknown condition and in the majority of cases represents the clinical manifestation of Klippel –Trenaunay Syndrome. The presence of an arteriovenous malformation makes the overall condition of Primary Phlebolymphedema much harder to manage. It is also thought that Long-term strain on the lymphatic system can be a cause of phlebo-lymphedema

Secondary Phlebolymphedema is an acquired condition that starts off with chronic Venus insufficiency due to various causes, and which later leads to chronic lymphatic insufficiency. In the presence of venous hypertension, which is characteristic of most venous disorders, the increase in lymphatic flow becomes much greater than the lymph transport capacity. Treatment for chronic Secondary Phlebolymphedema consists of treating the venous abnormality and watching regression of the lymphatic problem.

The diagnosis of Primary and Secondary Phlebolymphedema is based on a detailed history and physical examination, and related tests.

The majority of patients with phlebolymphedema will require a course of Complete Decongestive Therapy (CDT). CDT consists of an acute phase to reduce the lymphedema, followed by a lifelong maintenance phase to prevent a recurrence. Treatments consist of Manual Lymph Drainage (MLD), skincare, exercise, and Compression Bandaging with short-stretch compression bandages. It is recommended that treatment be performed by a certified lymphedema physiotherapist. Read more about what is the best treatment option for lymphedema HERE.

Patients should be specially careful when choosing a lymphedema Multidisciplinary Expert Centre of Reference/Excellence, as not all centers and counties offer or provide the same treatment options. The best choice is the Expert Reference Centre of Excellence that applies Complete Decongestive Therapy (CDT), which is recognized as the "Gold Standard" treatment for Lymphedema. Read more about the components that form part of the Conservative Treatment in the German Scientific Protocol HERE.

Information source and references:

• Phlebolymphedema–ACommon Underdiagnosed and Undertreated Problem in the Wound CareClinic
• Phlebolymphemeda:usually unrecognized, often poorly treated
• Phlebolymphedema:is it a new concept?
• Phlebolymphedemais the ultimate comorbidity/outcome of lymphedema
• Phlebolymphedema:Disregarded cause of immunocompromised district

SCIENTIFIC SOCIETIES

• GERMAN SCIENTIFIC SOCIETY GUIDELINE: Diagnostics and Therapy of Lymphoedema.
• THE INTERNATIONAL SOCIETY OF LYMPHOLOGY: Consensus Document.

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LYMPHEDEMA INCIDENCE 
AND PREVALENCE
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