The lymphedema
General
The lymphatic system is vital. It is connected to the blood system and performs organic functions as important as the cardiovascular system.
​
The lymphatic system includes:
​
-
on the one hand a network, whose one-way vessels collect and transport lymph to the lymph nodes, which are small purification stations. Contrary to the blood circulation which benefits from the cardiac pump, the progression of the lymph through this network is autonomous and ensured by the contractility of its vessels. The peripheral lymph is translucent and yellowish while the lymph of digestive origin, called “chyle”, richer in fat, is whitish. After passing through the network of vessels and lymph nodes, the lymph, cleaned of waste and impurities, continues its flow and finally flows into the bloodstream in the subclavian veins under the clavicles.
-
on the other hand, various lymphoid organs, such as the lymph nodes, spleen, thymus, tonsils and lymphoid tissues associated with the mucous membranes. These organs are mainly responsible for the immune defense.
The lymphatic system plays an essential role as it takes over an immunological and hormonal function. By transporting fluid, proteins, lipids and eliminating cell and metabolic waste products, it ensures the body's immune defense. It is also involved in healing processes.
​
Very little considered for centuries, although the body contains as much lymph as blood, the lymphatic system has been receiving increasing attention from scientists in recent years. An implication of this system is evoked today in diseases that affect a large proportion of the world's population such as cancer, obesity, essential arterial hypertension, atherosclerosis, glaucoma and also neurodegenerative diseases.
​
What is a lymphedema (LE)?
​
A lymphedema is an accumulation of lymph that most often sits in the subcutaneous tissues causing local swelling. All anatomical regions with lymphatic vessels can be affected.
​
A LE develops in an area of the body when the capacity of the local lymphatic system no longer allows sufficient drainage of lymph. The volume of undrained lymph accumulates in mainly subcutaneous tissue. This results in a swelling which is known as LE. Most often observed in the lower limbs, other regions like the upper limbs, face, trunk and even the genitals can also be affected.
​
There are more than 250 million cases of LE worldwide, of which more than 120 million are caused by a parasitic disease, filariasis, which is present in 49 countries. And these figures are very probably underestimated!
​
LE is still a little-known disease today. Few specialists qualified to treat it properly and its impact on quality of life is often overlooked by medical staff.
​
The increase in volume of the limb worries patients, modifies their body image and generates at least some discomfort, but often a handicap. The psychological impact is not to be underestimated and is aggravated by the chronicity of the disease.
​
What causes lymphedema?
​
Several causes can be responsible for LE and depending on the causes, two forms of LE are described: primary and secondary Lymphedema.
​
In industrialized countries, 90% of LE are of secondary origin, two thirds of which are due to cancer. Primary LE are part of rare diseases and can be associated with other malformations in different syndromes.
​
A primary LE is a genetic disease that causes a disorder in the development of vessels and/or lymph nodes. These malformations cause a local decrease in lymphatic transport capacity, which results in an accumulation of lymph. The result is LE. It mainly affects the lower limbs but can affect all anatomical regions.
​
Prevalence (number of cases in a population at a given time) is estimated at 1 in 6,000 people. Primary LE occurs worldwide. In the large majority of cases (more than 90%), primary LE affects an individual without any family member being affected (sporadic case). The other cases are familial forms in the context of Nonne-Milroy disease (congenital primary LE) and Meige disease (non-congenital primary LE). Primary LE can also be part of more complex clinical pictures such as Klippel-Trenaunay-Weber, Parkes-Weber, Klinefelter, Turner and Noonan syndromes among others.
​
Women are more often suffer from primary LE than men (2/3 of cases).
​
A secondary LE results from obstruction or damage to vessels and/or lymph nodes primarily as a result of surgery, radiation therapy, accident, infection or lymph node removal. Secondary LE related to cancer treatment are the majority and mainly concern breast, gynecological, genitourinary, ear, nose and throat cancers, melanomas and sarcomas. Secondary non-cancer-related LE occur most often in the context of other diseases such as chronic venous insufficiency (phlebo-lymphedema), lipoedema (lipo-lymphedema), obesity, diabetes or in the context of infection or trauma.
Diagnosis
The diagnosis of LE is essentially based on a thorough anamnesis and clinical examination of the patient. In addition, we currently have various imaging techniques available to visualize lymphatic vessels and assess their function:
Indocyanine Green Lymphofluoroscopy
​
Lymphofluoroscopy is a recent (2009) minimally invasive dynamic imaging technique that allows observation of the superficial lymphatic vascular system.
The method consists of injecting a diluted dye under the skin and observing its migration using an infrared camera. The dye used, indocyanine green, has the particularity of being highly fluorescent when irradiated with infrared radiation.
The operator can then use the camera to follow the progress of the dye in the superficial lymphatic vessels in real time on a screen.
​
Lymphofluoroscopy is a very valuable means of mapping the superficial lymphatic vessels through which approximately 80% of the limb lymph circulates. It also makes it possible to evaluate their function with great sensitivity. It is possible, thanks to this examination, to detect disorders of lymphatic circulation before the clinical appearance of LE and thus to allow a preventive approach to the pathology.
In addition, the information it provides can serve as a guide for the practitioner when applying manual lymphatic drainage and therapeutic compression, as well as in cases where surgical treatment of lymphatic disorders may be considered.
​
lymphatic vessels
Lymphoscintigraphy
For patients in whom lymphofluoroscopy has not provided all the necessary information, lymphoscintigraphy may be offered. The particular interest of this technique lies in the qualitative but also quantitative analysis of lymph migration and its ability to assess deep lymphatic vessels not accessible by lymphofluoroscopy.
Lympho-IRM
​
This examination is usually reserved for patients for whom an LE surgical treatment option may be considered.
Treatment
Currently, there is no curative treatment for LE.
​
Proposed treatments aim to reduce and stabilize LE in order to slow its evolution and prevent complications over time.
​
Conservative treatment through a combination of skin care, manual lymphatic drainage, pressure bandages and exercise remain the basic treatment today.
​
After maximum reduction of LE, the wearing of a custom-made, flat-knit elastic support (stocking, toe cap, cuff, glove) allows the volume to be stabilized. If necessary, night-time compression can be offered. Involvement of the patient in his or her management is essential and necessary to maintain long-term results. Patients are therefore encouraged to engage in appropriate physical activity, skin care, self-bandaging and, where possible, self-drainage. The goal is to make the patient a co-care giver and to make him or her as autonomous as possible in the management of the disease.
​
If various alternative physical treatments exist on the market such as pressotherapy, endermology (LPG), lymphtouch, etc., it must be stressed that they do not replace the basic conservative treatment described above, but may in some appropriate cases complement it.
​
In addition to conservative treatment, in certain specific situations, an option for surgical treatment of LE is available. Various microsurgical techniques are currently practiced, mainly lympho-venous shunts, lymph node transfer and fibro-lipo-lymph-aspiration. These techniques, combined with conservative treatment, can help to improve the symptoms of LE, slow its evolution and reduce the frequency of conservative maintenance treatments.
​
Microsurgical preparation of lympho-venous shunts is a procedure in which several lymphatic vessels in the region where the lymph pathways are blocked are connected to a small vein, allowing lymph to drain directly into the bloodstream, bypassing the obstruction that causes LE.
​
Microsurgical lymph node transfer involves removing lymph nodes from a healthy region and transferring them to the LE region. The transplanted lymph nodes are vascularized through a small local artery and vein and are expected to compensate for local lymphatic insufficiency.
​
​The two techniques described above can, in some cases, be combined and performed at the same time during the operation.
​
Fibro-Lipo-Lymph-Aspiration (FLLA) is a technique reserved for cases of LE where lymphatic stasis in the affected limb causes fatty transformation of the congested tissue. During this procedure, excess fat and fibrous tissue is aspirated through small skin incisions using flexible cannulas.
​
The option of surgical treatment of LE is discussed between the patient, the angiologist and the surgeon during a joint consultation.
​
Conservative treatment should always precede surgical treatment.
​
Regardless of the microsurgical procedure performed, a compressive treatment remains essential for the vast majority of patients.
​
The Gold Standard for the treatment of LE remains at present the conservative treatment.
​
The management of LE should ideally take place at the earliest stages of the disease, when lymphatic insufficiency is detected by an imaging technique in people at risk of LE or when the LE is still at a reversible stage.
​
This is why it is absolutely essential to emphasize the prevention of LE and not wait until it is chronic and irreversible before reacting.
​
Nevertheless, the treatments available today, at all stages of the disease, allow, with patient compliance, a significant reduction in LE.
Decongestion phase
Stabilization phase
Following surgery, I am at risk of lymphedema
or
I have lymphedema, what should I do?
Contact your doctor.
​
A specialized consultation with an angiologist is essential to assess your situation and, if necessary, confirm the diagnosis of LE. Your treatment will be discussed.
​
If treatment is appropriate, your care will be organized. You will be referred to a practitioner for conservative treatment. A regular follow-up, at least annually, with your angiologist is recommended.