The circulatory system is the unsung hero of our body, and it’s not just a bunch of blood vessels. The lymphatic system is often overlooked but is vital to our health. It is central to our immune defence and tissue fluid balance. Sometimes this system can malfunction and cause swelling that doesn’t go away in the legs or arms, a feeling of heaviness, and a condition that seriously decreases quality of life. In medicine, lymphoedema, in its advanced stages often referred to as “elephantiasis”, is a process that becomes chronic if not properly managed.
What is the lymphatic system and what causes it to malfunction?
The lymphatic system is the body’s “wastewater treatment plant". It gathers the fluids, proteins and cellular debris that leak out of the blood vessels into the tissues and returns them to the blood stream. This fluid is carried by lymphatic vessels that run alongside the veins (the venous system).
What causes lymphoedema?
Lymphoedema is the accumulation of a protein-rich fluid in the interstitial spaces as a consequence of a failure of lymph fluid transport. This condition is studied in two large groups:
- Primary Lymphoedema: This is caused by congenital absence or poor development of lymphatic pathways. It can occur at a young age or in adolescence.
- Secondary Lymphoedema: The most common form of the disease It occurs due to damage to healthy lymphatic pathways caused by trauma, cancer surgery (removal of lymph nodes), radiotherapy, infections or chronic vascular diseases.
A hidden link between lymphoedema and vascular diseases
Venous disease is often misdiagnosed or co-diagnosed with lymphoedema. Especially in patients with chronic venous insufficiency (CVI), high pressure in the veins can also put a strain on the lymphatic system over time.
- • Venous lymphoedema: Fluid retention in the legs caused by the deterioration of venous valves (venous reflux). This overloads the capacity of the lymphatic vessels and creates a condition which we call "phlebedema. "If varicose veins are left untreated, this can permanently damage the lymphatic vessels and cause secondary lymphoedema.
- • Post-operative risk: Lymph vessels may be affected in operations such as varicose vein surgery (stripping) or endovenous laser (EVLA). This can cause temporary or permanent lymphoedema, but it is rare.
Lymphoedema symptoms: Is it just swelling?
Lymphoedema may look like a simple swelling on the outside but is actually a complex and progressive condition characterised by the build-up of protein-rich fluid in the tissues due to an insufficiency of the lymphatic system. The body's response to this state is not only felt but also seen in profound alterations to the skin structure. The symptoms are similar to venous insufficiency (varicose veins), but there are a few differentiating differences:
Swelling:
Distribution and distinguishing feature One of the most important visual signs differentiating lymphoedema from other types of oedema is the swelling involving the dorsum of the foot and the fingers/toes. Venous oedema due to venous insufficiency tends to be confined to the ankle, while lymphoedema causes the fingers to swell, making the hand or foot look “puffy”.
Physical sensitivity and sensation
Other subjective complaints reported by patients are swelling and:
- Heaviness and fullness: An indescribable heaviness in the limb affected (arm or leg).
- Tightness and aching: discomfort due to the skin stretching.
- Reduced Mobility: Swelling in the joints can lead to less flexibility and trouble with daily activities.
Changes in the structure of the skin
When the fluid accumulates and becomes chronic, irreversible damage to the skin begins:
- • Fibrosis (hardening of the skin): Initially the oedema is soft and pitting (leaves an imprint when pressed); later it becomes firm because of deposition of protein, and the skin becomes woody.
- • Orange Peel Appearance: When skin pores become deeper, the skin may have the texture of an orange peel.
- • Wart-like bumps: In the later stages, you may see small, wart-like bumps and blisters that leak lymphatic fluid on the skin.
- • Colour Changes: Redness, inflammation (stasis dermatitis) or browning (pigmentation) may occur on the skin.
Tendency to infection
Lymph fluid is loaded with protein, providing the perfect environment for the bacteria to thrive. This makes lymphoedema patients very susceptible to recurrent skin infections, such as erysipelas, and lymphatic inflammations (lymphangitis). The attack of the infection causes further damage of the lymph vessels and progression of the disease.
Far advanced: Elephantiasis
If the growth is not controlled, the limb increases to an excessive size, the skin thickens all over and a condition known as "elephantiasis" results. At this stage it is not a cosmetic issue but a medical problem that greatly restricts the patient's mobility and quality of life.
Diagnosis and differentiation:
The physical examination and venous Doppler ultrasound by a specialist physician are of critical importance to differentiate whether the swelling in the legs is due to the lymphatic system or a venous (vein) problem. Very rarely, lymphoedema may also be a complication post-varicose vein surgeries.
Lymphoedema; who is at risk?
Lymphoedema is a chronic condition caused by a disruption of the lymphatic system, leading to fluid accumulation between tissues. Certain groups are at higher risk for this disease.
- • Those with chronic vascular disease: As the lymphatic system runs in parallel with the venous system, especially in patients with chronic venous insufficiency (CVI), high pressure in the veins can over time fatigue the lymphatic pathways, leading to the development of “venous lymphoedema".
- • Patients who have had vascular surgery or intervention: In open surgical (stripping) procedures for varicose vein treatment the risk of developing lymphoedema or lymphatic cysts is between 0.02% and 1.82%. In closed methods such as endovenous laser (EVLA) or radiofrequency, the risk is between 0% and 9%.
- • Cancer patients: Surgery for cancer that involves the removal of lymph nodes and radiotherapy (radiation therapy) permanently damage the lymphatic pathways and greatly increase the risk of developing secondary lymphoedema.
- • Those with genetic predisposition: Individuals with a family history of "primary lymphoedema", which arises from congenital deficiencies or malformations of the lymphatic pathways, have a higher risk.
- • Overweight and obese people: Extra body weight puts physical strain on lymphatic pathways, so lymph fluid can’t drain, causing swelling.
- • Pregnant women: Pregnancy hormones and the pressure of a growing uterus can slow lymph flow, increasing the risk.
- • Those with repeated infections and trauma: Infections such as erysipelas, which damage the lymphatic pathways, or serious blows to the leg area can make individuals susceptible to lymphoedema.
Treatment Methods for Lymphoedema
Lymphoedema (lymph oedema) is a chronic progressive disease that arises from the failure of the lymphatic system to adequately drain fluid, proteins and cellular debris from the interstitial space. The treatment process is founded on the principle of draining this protein-rich fluid that accumulates in the tissues (decongestion) and maintaining the achieved improvement for a lifetime (maintenance).
The principal methods of treatment of lymphoedema are:
Manual lymphatic drainage (MLD)
This technique is one of the most important elements of lymphoedema treatment. It is a special massage technique carried out by specialised physiotherapists.
- • Mode of action: Compared to classical massage, it is applied in the direction of lymph flow with very light pressure.
- • Function: It bypasses blocked or damaged lymphatic pathways to transfer fluid to functioning healthy lymph nodes and vessels. This method can be especially effective in relieving treatment-resistant oedema associated with vascular diseases such as varicose veins.
Compression therapy
After the lymph fluid is drained, external pressure is needed to prevent the fluid from re-accumulating in the tissues.
- • Multilayer bandaging: Usually used in the first (drainage) phase of treatment with low-elasticity bandages to reduce leg volume.
- • Special lymphoedema stockings: These are used during the maintenance phase, when the condition has stabilised. For lymphoedema, “extra firm” and high-pressure flat-knit special compression garments, like Class IV (>40 mmHg), are generally favoured.
- • Medical adaptive compression systems (MAC): It is a modern alternative thanks to its Velcro structure, with which the patient can adjust the pressure themselves and easily put it on and take it off. The margin of error is less than a band-aid and increases the patient’s adherence to treatment.
Intermittent pneumatic compression (IPC)
These systems are usually called “lymph drainage devices” and are regarded as auxiliary devices that can be used in a clinical setting and at home.
- • Application: The cuffs (with air chambers) are worn on the leg and inflated in a certain cycle by means of a pump.
- • Advantage: It forces the fluid upwards, controlling the oedema and helping to reduce the painful symptoms. It is used as a complementary method to other compression methods when they are not enough.
Hygiene and Skin Care
In lymphedematous tissue a protein-rich fluid accumulates, and this provides a good medium for infections.
- • Infection control: Small cuts or breaks in the skin can easily result in serious infections such as cellulitis or lymphangitis.
- • Moisturising: Daily care with pH-neutral moisturisers is vital for the success of the treatment to prevent the skin drying out and cracking.
Exercise and lifestyle modifications
- • Activation of muscle pump: Exercise that is regular causes the muscles in the leg to contract and squeeze the lymph vessels, helping to move the fluid upwards.
- • Leg elevation: Elevating the legs above heart level during rest (at least 30 minutes, 3 times a day) reduces the effect of gravity and alleviates swelling.
- • Weight control: Obesity physically restricts lymphatic drainage, so maintaining an ideal weight is advised.
Prof. Dr. Cenk Eray Yıldız’s approach
Prof. Dr Cenk Eray Yıldız, a cardiovascular surgery specialist with over 25 years of surgical experience, adopts a personalised multidisciplinary approach in the treatment of difficult diseases such as lymphoedema and lipedema.
In the treatment of lymphoedema, Prof. Dr Yıldız emphasises the points as follows:
- • Correct diagnosis: The venous Doppler ultrasound and clinical examination help to establish if the cause of the oedema is lymphatic or venous (vascular).
- • Early intervention: To manage the disease before it reaches the “elephantiasis” stage (Stage 3).
- • Complementary solutions: not only physical therapy, but also a concomitant treatment of any underlying vascular blockage or insufficiency (using minimally invasive methods such as laser or radiofrequency) if existent.
Lymphatic oedema treatment: Recovery and considerations
Lymphoedema is not a disease that disappears completely but a chronic condition that must be managed with discipline throughout life. Thus the “recovery process” is the transition from the phase of intensive decongestion (the reduction of swelling) to the phase of maintenance (preservation) of this condition and the rules that must be followed for a lifetime.
Following treatment of lymphoedema, the following process and considerations should be borne in mind:
Phase of treatment and expectation of recovery
- • Two-phase process The treatment is usually planned in two phases: The first phase (decongestion phase) aims at draining the fluid accumulated in the tissues. The second phase aims at maintaining the achieved improvement.
- • The role of compression: Once the swelling is controlled, special high-pressure (generally Class IV, >40 mmHg) lymphoedema stockings or medical adaptive compression systems (MAC) are used to prevent fluid accumulation again.
Hygiene and skin care
Lymphedematous tissue is very prone to infections. The success of the treatment depends on the maintenance of skin health:
- • Daily cleansing and moisturising: Compression therapy can dehydrate the skin; therefore, it is important to gently cleanse and moisturise the skin daily with pH-neutral moisturisers. This prevents cracks in the skin and the consequent entry of bacteria.
- • Infection prevention: Minor cuts, insect bites and injuries to the edges of the nails can lead to serious infections such as lymphangitis (inflammation of the lymphatic vessels) or cellulitis. If there is an injury, the area should be disinfected immediately and monitored.
Physical activity and movement
- • Stimulation of the muscle pump: Exercise can activate the muscle pump mechanism, resulting in a 70% increase in lymph flow and improvement of symptoms.
- • Leg elevation: Elevation of the legs above the level of the heart during rest (at least 30 minutes, 3 times a day) reduces the effect of gravity and prevents fluid accumulation.
Walking: Normal walks after treatment can enhance blood circulation and lower the risk of clots.
Lifestyle and supportive treatment
- • Weight management: Extra weight puts additional pressure on the lymphatic system, causing a difficulty in circulation. An ideal weight is the direct matter of the success of the treatment.
- • Device support: Intermittent pneumatic compression (IPC) devices for home use can help reduce painful symptoms and keep oedema under control, in addition to manual lymph drainage.
- • Clothing: Avoid very tight clothing that can restrict blood flow and knickers that compress the legs/groin.
When Should I See a Doctor?
- • Regular check-ups: Periodical doctor check-ups and Doppler ultrasound checks should be performed if necessary in an effort to evaluate the effectiveness of the treatment.
- • Emergencies: If you see sudden and severe swelling, sharp pain, redness or fever (signs of infection) in your leg, see a specialist straight away.
F.A.Q:
Does lymphoedema improve on its own?
No, lymphoedema is a long-term and progressive condition. If not treated medically, the swelling worsens and the skin structure breaks down.
Is a compression stocking enough for lymphoedema?
In general, no. Varicose vein stockings are circular knit; in lymphoedema, special flat-knit compression garments that do not embed into the tissue are required.
Is lymphoedema confined to the legs?
No, it can be seen in the arms, particularly after breast cancer surgery, and sometimes in the face and genital areas.
Why should one exercise during treatment?
Every time the muscles of your legs (especially the calf muscles) contract, they squeeze fluid toward the heart by applying pressure on the lymphatic vessels.
Can lymphoedema be treated with surgery?
Sometimes surgery is tried, for example, lymphovenous anastomosis (linking a lymph vessel to a vein) or lymph node transplantation, but the main treatment is physical therapy (CDT).
What do I do when I board the plane?
Lymphoedema can be triggered by changes in pressure. On long flights it’s a good idea to wear your compression garment and drink plenty of water.
Does pregnancy make lymphoedema worse?
Yes, hormonal changes and increased intra-abdominal pressure may impede lymphatic flow.
Does a hot bath or sauna help lymphoedema?
No, Heat makes blood vessels dilate (vasodilation) and allows more fluid to escape into the tissue, which causes more swelling.
Is there infection in lymphoedema?
Yes, if there is a stagnation of lymph fluid, the risk of infection increases. This condition is called cellulitis or lymphangitis.
What is the effect of salt intake on lymphoedema?
Too much salt may cause water retention in the body, and this can increase complaints of oedema.
What is the length of time for manual lymphatic drainage (massage)?
Sessions typically last 45-60 minutes and are recommended 3-5 times a week initially.
Can lymphoedema be treated with medication?
No drug directly opens the lymphatic pathways, but venoactive drugs can reduce fluid leakage by strengthening the blood vessel walls.
How to select a pair of shoes?
Choose shoes which do not constrict the leg and which are comfortable and orthopaedic; it is forbidden to walk barefoot (because of the risk of infection).
What is the difference between lymphoedema and lipedema?
Lipedema is an asymmetrical accumulation of fat that spares the dorsum of the foot. However, over the years lipedema may develop into lymphoedema (lipo-lymphoedema).
Do you use compression bandages at night?
The bandages are usually left on for 24 hours (including the night) during the first phase of treatment.
How do I make an appointment with Prof. Dr Cenk Eray Yıldız?
You can book an appointment at his clinic in Bakırköy, Istanbul, or through his official website.
