What Is Lymphedema? Causes, Symptoms And Treatment

What Is Lymphedema? Causes, Symptoms And Treatment
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What Is Lymphedema?

It is the accumulation of protein-rich fluid in the interstitial space due to impaired lymphatic flow. It is a chronic and progressive condition. Skin and subcutaneous tissue are affected. Fibrosclerosis develops in the skin.

What Is The Basic Function Of The Lymphatic System?

  • Lymphatic system provides tissue-fluid regulation
  • To drain protein-rich fluid, macromolecules and cells from the interstitium into the venous circulation
  • Interstitial lymph vessels absorb fats and fat-soluble vitamins (ADEK) and drain into the venous system
  • The immune system has functions
  • It protects the body against diseases by producing and transporting lymphocytes.

Which Structures Does The Lymphatic System Consist Of?

It consists of lymph vessels (collectors), lymph nodes, lymphatic organs.

If we talk about the anatomy of the lymphatic system

Lymph drainage from both lower limbs, the gastrointestinal tract and the left upper body (upper limbs, chest wall, back, shoulder and breast) is connected to the venous circulation by the thoracic duct. The thoracic duct opens into the venous angle between the left subclavian and left internal jugular veins and drains ¾ of the body lymph.

The lymph drainage of the right upper body drains into the right lymphatic duct and the right venous angle. In the extremities there is a superficial lymphatic system draining the skin and subcutaneous tissue and a deep system draining subfascial structures such as muscle and bone. These two systems in the upper extremities merge in the axilla, and the two systems in the lower extremities merge in the pelvis.

What Does Lymph Fluid Contain?

Protein, water, cells (RBC; WBC; lymphocytes, etc.), residual products and other foreign substances, fatty acids, lipids, coagulation factors, electrolytes, urea, amino acids, creatinine, carbohydrates

How Is Lymphatic Transport Provided?

The intrinsic propulsive force of the lymphatic vessel (lymphangion): 6 contractions per minute. Increases 10 times with exercise. It is provided by muscle contraction, passive movement of body parts, arterial pulsation, breathing (diaphragm movements, pressure changes in the chest and abdomen), tissue mobilization (massage, MLD).

What Are Lymphatic Organs?

  • Primary lymphatic organs: bone marrow, thymus
  • Secondary lymphatic organs: lymph nodes, spleen, diffuse lymphatic tissue (tonsils, GALT, BALT, Peyer’s patches)

What Are The Properties And Functions Of Lymph Nodes?

  • Lymph fluid concentrates in the lymph nodes (4-8 litres/day)
  • Lymph node count 450-600
  • Concentrates the protein in the lymph fluid
  • Filters pathogens
  • Provides lymphocyte maturation and replication

Can You Tell Us A Little About The Physiology Of The Iymphatic System?

Despite the high pressure in the vascular flow in the systemic circulation, the lymph flow takes place in the low pressure system. The cycle of interstitial fluid begins in the lymphatic capillaries and precollectors adjacent to the interstitial space.

This is supported by local arterial pulsation, skeletal muscle contraction and one-way valves preventing backflow. These initial lymphatics merge with the veins. They are equipped with a layer of smooth muscle in increasing proportions in the vessels they join. This smooth muscle layer supports the peristaltic movement of the lymph fluid towards the systemic circulation by its contraction.

How Does Iymphedema Occur?

Lymphoedema occurs when the lymphatic load exceeds the carrying capacity of the lymphatic system. In contrast to generalised oedematous conditions, capillary filtration rate is normal in patients with lymphoedema.

It is a difficult condition to treat if not diagnosed in the early period.

How Lymphedema Classification Is Made:

We divide lymphedema into two groups: Primary and Secondary.

Primary lymphedema: This is caused by congenital and/or hereditary conditions (pathological development of lymphatic vessels). It is classified according to the age of onset: Congenital lymphedema (the onset of swelling is between birth and 2 years of age), Precocious lymphedema (during puberty or during pregnancy before the age of 35), Tarda lymphedema (after the age of 35). The incidence is 8% in newly diagnosed patients presenting to lymphedema clinics and 28% in lymphedema caused by non-cancer diseases. With the exception of tarda lymphedema, these conditions predominantly affect the lower extremities. Primary upper extremity lymphedema is rare. The average age of onset is 38, and the female to male ratio is 1.2. Most cases are not associated with a genetic condition.

In the suspicion of primary lymphedema, physical findings or congenital anomalies that may be associated with hereditary diseases should be evaluated. These include short stature (Turner syndrome), hemangioma/Port wine stains (Klippel-Trenaunay-Weber syndrome), and Shield chest (Turner syndrome, Noonan syndrome).

Secondary lymphedema: This is lymphedema that occurs as a result of other diseases or treatments. Etiology and risk factors in developed countries, the most common cause is cancer or cancer treatment. The most common cause worldwide is filariasis. Other risk factors include: advanced age, obesity, inflammatory arthritis, infection, chronic venous insufficiency, trauma, and iatrogenic causes. Cancer-related lymphedema can be of two types:

Infiltration of lymphatic vessels: by infiltration of tumor cells into lymphatic channels. The most common cancer associated with lymphedema is breast cancer. In a systematic review, the rate in breast cancer patients is 17%. The risk is highest in the first 2 years after diagnosis. Risk factors include axillary node dissection, the number of axillary nodes removed, mastectomy rather than local excision, and a high body mass index.

What Other Cancers Are Associated With An Increased Risk Of Lymphoedema?

  • Sarcoma 30 per cent
  • Lower limb melanoma 28
  • Gynaecological cancer 20
  • Genitourinary cancer 10 per cent
  • Head and neck cancer 3 per cent

Surgical removal of lymph nodes and radiation therapy increase the risk.

What Are The Factors Affecting The Development Of Lymphedema After Breast Cancer?

  • Type of axillary surgery
  • Infection or injury in the arm after surgery
  • Other surgeries to be performed on the dominant arm
  • Increased body weight or body mass index (BMI above 25)
  • Radiation and chemotherapy received by the patient
  • The efficacy of genetic predisposition is controversial

Can You Talk About The Association Of Inflammatory Arthritis And Iymphoedema?

  • Uni-bilateral lower or upper extremity lymphoedema is a rare extraarticular manifestation of inflammatory joint disease.
  • It is most commonly observed in rheumatoid arthritis and psoriatic arthritis (due to psoriasis).
  • Although the mechanism is not clear in rheumatoid arthritis, it is thought to occur as a result of inflammatory synovitis causing fibrosis in the lymph ducts.
  • Lymphoedema resolves with the treatment of arthritis in some patients.
  • In psoriatic arthritis, a similar picture develops as a result of diffuse tenosynovitis.

Is Obesity Important In The Development Of Lymphedema?

  • It is an independent risk factor for lymphoedema in cancer patients.
  • Massive localised lymphedema may occur in morbidly obese patients.

What Is Considered In Clinical Evaluation In Lendoedema?

  • Initiation
  • Favoured side
  • Presence of swelling
  • Presence of skin manifestations
  • Whether it causes discomfort
  • Whether there is joint movement limitation

How Is Staging Performed In lymphoedema?

  • Latent phase (0):
    • It is the period of subclinical lymphoedema.
    • There are focal fibrosclerotic tissue changes.
    • There are no clinical signs of lymphoedema. Patients may feel a feeling of heaviness in their extremities. Most are asymptomatic.
    • Functional isotope scintigraphy is used in diagnosis.
  • Reversible (I):
    • Stage of reversible lymphoedema
    • High protein lymphoedema
    • Pitting oedema: A very soft, gode-leaving oedema.
    • Elevation reduces swelling, possibly ‘congestion pain’
    • Fibrosclerotic tissue changes are absent/focal
    • Basic diagnostic procedures are performed
  • Irreversible (II):
    • Irreversible lymphoedema stage
    • There is diffuse fibrosclerosis, connective and adipose tissue proliferation.
    • There is a hard brown swelling that does not decrease with elevation.
    • Stemmer’s sign is positive.
    • Basic diagnostic procedures are performed
  • Elephantiasis (III):
    • Diffuse severe fibrosclerosis, connective and adipose tissue proliferation
    • Like stage II, disability
    • Lymphostatic elephantiasis.
    • Basic diagnostic procedures
  • Steward Treves syndrome (lymphangiosarcoma): Stage 4

Diagnosis of Lymphoedema :

  • Anamnesis & physical examination
  • Stemmer’s sign: predictive
  • Starting age
  • Areas covered
  • Associated symptoms: pain, etc.
  • Medications: Non-steroidal painkillers may cause oedema. Diuretics are contraindicated in lymphoedema.
  • Progression of symptoms is questioned
  • History of previous diseases, diseases that may be associated with lymphedema, travel, infection, surgery or radiotherapy is taken.
  • Family history is questioned

What Is Questioned In The Anamnesis?

  • Your swelling
  • Localisation
  • Feeling of heaviness, fullness, tension, stiffness, restlessness, etc.
  • Paresthesia, paresis
  • Cramps
  • Acute & insidious
  • Intermittent & fixed
  • Increasing & decreasing factors
  • Pain, numbness, weakness “red flags

How Is Physical Examination Performed?

INSPECTION

  • Limb asymmetry
  • Swelling area
  • Skin changes: colour, discharge, cyst, fistula, pitting, skin fold thickness, skin integrity
  • Skin lesion: ulceration, rash, trauma/insect bites, scratches/scrapes, fungal infections, papillomatosis, hyperketarosis

PALPATION

  • Stemmer’s sign (1976- Robert Stemmer)
    • When the toe or 2nd finger is pulled from the metacarpal root, the skin lifts. If there’s oedema, we can’t lift it.
    • May be positive at any stage
    • False positivity is rare
  • Heat: chronic inflammation, infection
  • Nabazans
  • Pitting
  • Fibrosis
  • Sensitivity

PHYSICAL EXAMINATION

  • General systemic examination: Examination of the head, neck, heart, lungs, abdomen, vascular system, skin, soft tissues, lymph nodes
  • Posture
  • Range of motion (especially in the affected limb)
  • Other edematous areas
  • Neurological examination

What Are The General Features Of Primary Lymphoedema?

  • Short length
  • Port wine stains or haemangiomas
  • Chest deformity
  • Positive Stemmer sign
  • Clinical measurements
  • Measurement of limb volume: in diagnosis and treatment monitoring
    • Water displacement method
    • Circumference measurements:
      • MKF joint, wrists, 10 cm distal to lateral epicondyle, 15 cm proximal to lateral epicondyle
      • Significant difference of more than 2 cm in patients with arm oedema
    • Optoelectronic volumetry

How To Recognise Early Swelling Due To lymphatic Impairment?

There is no standard diagnosis method.

Limb volume and circumferential measurements are used to classify the severity of lymphoedema. USG, perometry, bioimpedance, preoperative and postoperative photography, subjective description of the patient, mathematical volume assessments related to circumferential measurements of the extremity are among other methods used.

Where are the circumferential measurements taken?

  • Metacarpophalangeal/ Metatarsophalangeal joints
  • Wrist / 2 cm above the medial malleolus
  • Lateral epicondyle 10 cm below /Patella 10 cm below the lower corner
  • 10/12 cm above the lateral epicondyle/10 cm above the upper corner of the patella
  • Drawing from the styloid process of the ulna to the axilla at 4 cm intervals and volumetric evaluation on computer

A difference of >2 cm or more is significant.

Imaging modalities are helpful if the diagnosis cannot be made by history, physical examination and imaging modalities. Ultrasonography is recommended in all patients with new-onset extremity swelling. Ultrasonography is widely used to evaluate tissue thickness and content and to assess skin integrity.

Infrared rays are used in perometry evaluation, and limb volume is determined rapidly. However, it is an expensive method. Bioimpedance measures tissue resistance to electric current to determine extracellular fluid volume. It is not routinely applied, it is helpful in the diagnosis of early lymphedema.

Other diagnostic methods include lymphoscintigraphy, quantitative lymphoscintigraphy, microlenangiography, indirect lymphography, MRI and CT, PET, Doppler USG, dynamic contrast-enhanced MRI.

Which Diseases Should We Think Of In The Differential Diagnosis Of Iymphedema?

Chronic venous insufficiency, acute deep vein thrombosis, postthrombotic syndrome, limb hypertrophy, lipoedema, myxoedema, tumour should be considered.

Are There Any Important Points You Would Like To Say About lymphoedema?

Lymphoedema is a chronic and progressive process. Today, there is no cure with the current treatments, that is, unfortunately, we cannot come to a point where we can say that you don’t need to do anything anymore. However, it is possible to stop the progression of the disease and reduce the severity of the condition. Prevention and awareness training is mandatory. Early diagnosis is necessary so that we can take the condition under control at the earliest stage. Patient compliance and loyalty to treatment are very important in treatment.

Is It Possible To Prevent lymphoedema? What Can Be Done?

Yes, the measures that can be taken in this regard are as follows:

  • Primary prevention
    • Reducing the size of the lymph node dissection area
    • Advanced radiotherapy techniques
    • Appropriate upper extremity exercises and prophylactic physiotherapy
    • Prevention of infections
  • Secondary prevention
    • Meticulous skin hygiene & nail care
    • Protection of the skin part involved: sunscreen, gloves, etc.
    • Creams that moisturise the skin should be used, topical antibiotics should be used in the formation of a small abrasion that may develop such as insect bites, animal scratches, needle sticks.
    • If an insect bites: wash with soap, apply cream, wrap in gauze, do not glue
    • Oil-free, odourless creams: Ph 5.5
    • Do not leave the limb under the influence of gravity for a long time: sitting, standing, sitting with crossed legs, etc.
    • Tight clothing should not be worn on the extremity with lymphoedema: it creates a tourniquet effect and prevents lymph flow
    • Vaccination, acupuncture, intravenous intervention, etc. should be avoided on the extremity with lymphoedema
    • Local application of heat to the extremity should be avoided: blood flow and lymph fluid production are increased. Sauna, steam baths, living in hot climates
    • Excessive cold should not be applied
    • Maintain ideal body weight
    • At high altitude, on aeroplane journeys (especially if they last longer than 4.5 hours), it may be helpful to use compression garments, exercise and massage.

Can You Talk About lymphoedema Treatment?

Treatment is difficult, but even more difficult if it is not diagnosed at an early stage. The aim is to prevent the occurrence of lymphoedema and to control the swelling of the extremities if it has already occurred. The causative disease is usually not correctable. Measurement of limb circumference or volume measurements are the most common methods of monitoring the degree of lymphedema during treatment. Early diagnosis and treatment of lymphoedema in breast cancer allows the use of conservative treatments. Untreated lymphedema progresses over time and restricts daily activities.

Can You Tell Us About The Methods Used In The Treatment Of lymphoedema?

There are conservative treatment and surgical treatment methods. Patient motivation and compliance are very important in treatment.

What Are Conservative Treatment Methods?

  • Education
  • MLLB/compression garments
  • Manual lymphatic drainage (MLD)
  • Skin care
  • Exercise
  • Elevation
  • Treatment of concomitant diseases
  • Pain-disability treatment
  • Psychosocial support
  • PICP: pneumatic intermittent compression pumps
  • Other medicines

What Is Complete Decongestive Therapy?

It is a two-phase treatment. It can be used in adults and children. Trained physician/physiotherapist/nurse is important for success. With this treatment, limb volume decreases by 50-70%. It consists of careful skin and nail care, manual lymph drainage, compression bandage, therapeutic exercises and patient education.

Complete decongestive therapy

  • Phase I Decompression phase
    • Intensive phase (treatment phase)
    • Treatment 5 days a week for 2-4 weeks
    • Manual lymph drainage
    • Compression bandage (21-24 hours)
    • Therapeutic exercises
    • Skin-nail care
    • Education
    • Follow-up with weekly volume and circumference measurements
  • Phase II Protection phase
    • Ensuring the continuation of the benefit provided in the first phase
    • Compression garment (during waking hours throughout the day)
    • Compression bandage (night)
    • Exercises
    • Skin and nail care
    • Self-Manual lymph drainage (if necessary)
    • Follow-up visits: Follow-up with volume and circumference measurements: 6 months or less

Manual lymph drainage -MLD

  • The aim is to promote the elimination of lymph fluid from congested areas by increasing the activity of normal lymphatics and bypassing ineffective or obstructed lymph vessels.
  • A gentle massage technique following the lymph pathways
  • It is not sufficient alone. It must be applied together with compression bandaging
  • Increases lymph flow
  • Increases malabsorption of protein-rich fluid
  • Provides relaxation
  • Analgesic effect
  • Provides fluid mobilisation from the affected area to the unaffected area
  • Manual mechanical traction and compression are applied in a certain order without slipping on the skin
  • 45-60 min/day
  • It is usually performed with the patient lying down (unless there is head and neck oedema)
  • Start with deep diaphragmatic breathing
  • Firstly, unaffected lymph nodes and body parts are treated
  • Movements are slow and rhythmic
  • Gentle pressure is applied. Otherwise, if the pressure is hard, it increases blood flow, the skin turns red and more fluid passes into the tissues
  • It is finished with deep diaphragmatic breathing.
  • Usually once a day, sometimes twice a day, sometimes three times a week
  • Duration of treatment is three weeks or more
  • Repeat intervals from 3 months to 1 year
  • The basic techniques include fixed circular, pumping technique, scooping technique (on the extremity) and rotatory technique (on the trunk).

The order to be followed in arm oedema in MLD is as follows:

  • Cervical paraspinal lymph vessels and lymph nodes
  • Superficial and deep lymphatic structures of the abdomen
  • Contralateral axillary lymph nodes
  • Axillo-axillary lymphatic vessels
  • Ipsilateral inguinal lymph nodes
  • Axilloinguinal of the lymph vessels
  • Intercostal lymph vessels
  • Parasternal lymph nodes
  • Ipsilateral breast tissue

ToWhom Is Manual lymph Drainage Not Applicable?

Absolute contraindications to MLD:

  • Active neoplasia in the affected limb (information that massage spreads metastases is controversial)
  • Acute cellulitis/erysipelas
  • Heart failure
  • Kidney failure
  • Acute deep vein thrombosis
  • Hepatic cirrhosis
  • Untreated thyroid dysfunction

MLD Relative contraindications

  • Uncontrolled HT
  • DM: pain sensation may be impaired due to vasculopathy or neuropathy. In this case, inappropriate clothing can lead to injury or infection
  • Asthma: parasympathetic activation may stimulate an asthma attack. MLD is started with 20 min, if there is no negative response to treatment, the normal duration is reached by increasing 5-10 min.
  • Paralysis: flaccid limbs are sufficient when compression stockings and garments are used….

Compression bandage-MLLB (Multi-layer inelastic lymphedema bandaging)

  • Low resting pressure: applies a constant pressure to the limb at rest
  • High working (exercise) pressure: when the muscles contract and expand, as during exercise, they exert pressure against the resistance of the bandage and the pressure in the limb increases intermittently, the pressure increase stimulates lymphatic pumping and lymph reabsorption

What Are The Effects Of Compression Bandage-MLLB?

  • Increased tissue pressure reduces filtration
  • Prevents re-accumulation of fluid
  • Compression and movement allows lymphostatic fibrosis to dissolve
  • Increases muscle and joint pump (venous and lymphatic return)

Compression Bandage (MLLB) Is Not Applied To Whom (MLLB Contraindications)

  • Severe arterial insufficiency
  • Uncontrolled heart failure
  • Severe peripheral neuropathy

What Are The Recommended Materials For Upper Extremity Compression Bandage - MLLB?

  • Cotton tubular bandage
  • Finger bandage (4-5 cm)
  • Soft synthetic wool or soft foam roll (10 cm)
  • Inelastic bandages: short stretch bandages (one 6 cm, one 8 cm, two or three 10-12 cm)
  • Foam backing

For Which Patients Are Compression Garments Suitable?

  • Have the ability to wear the garment
  • With tough, supple skin
  • No or very little deformity of the extremities
  • No or very little pitting oedema
  • Cohesive and motivated
  • Can be restricted by compression garment for swelling
  • Able to tolerate and use clothing
  • Suitable for people who can control and maintain skin integrity.

In compression garments, distal pressure is higher than proximal pressure. Their function is mobilisation of oedema fluid and prevention of fluid accumulation. They do not reduce oedema, they ensure the preservation of the existing volume. Compression garments should be renewed every 3-6 months when they lose elasticity. The patient should have two garments, while washing one, the other should be worn. Clothes and bandages should be washed with warm water. The patient should put the bandages on the towel and dry, not hang, but can hang the garment.

What Does Skin Nail Care Include?

  • Reduce the risk of infection
  • The patient should be warned about the risk of infection
  • Keep the skin clean and maintain its integrity
  • Protect the skin from injuries (cuts, scratches, burns, insect/fly bites, etc.)
  • Intervene immediately in case of hurt/injury

What Does Self-Care Training Include?

  • Skin nail care
  • Prevention of infections
  • Therapeutic exercises
  • Self-bandaging
  • Self-manual lymphatic drainage (MLD) if necessary
  • Putting on and taking off compression garments
  • Follow-up visits (at least every 6 months)

If We List The Patient Education In Titles, What Is Taught To The Patient?

The patient should be taught skin nail care, self-bandaging, putting on and taking off compression garments, self MLD (if necessary), SLD (simple lymphatic drainage self massage).

Information should be given about the measures to be taken to prevent infections and what to do and what not to do when there is evidence of infection. The importance of follow-up visits should be emphasised.

Can You Tell Us About Drug Therapy And Other Treatments For Iymphoedema?

  • Chronic pharmacological treatments are not recommended for patients with lymphedema. Antibiotics are used in the presence of infection. Drugs are useless, especially diuretics should not be given unless there is an additional pathological condition. Immunotherapy, mesotherapy, vitamin E and selenium supplements, gene therapy, hyperbaric oxygen therapy/ozone therapy, laser therapy, kinesiotaping (taping) are among the methods applied in treatment.
  • Pneumatic pumps: Intermittent pneumatic compression pumps (IPCP) inflate intermittently from distal to proximal with the help of a plastic sleeve motor placed on the arm. IPCP may have one or multiple chambers. Lymph drainage is recommended beforehand: If there is oedema in the root, it must be done beforehand. IPCP plays a role in reducing oedema by reducing capillary filtrate and thus reducing lymph formation rather than accelerating lymph return. The extremity should be in elevation during the application. Untreated non-pitting lymphedema (late stage lymphedema: fibrosis, advanced stage), deep vein thrombosis/pulmonary embolism/thrombophlebitis/acute skin infection (eg: cellulitis, erysipelas), uncontrolled/severe cardiac failure/pulmonary oedema/ischemic vascular disease, active metastatic disease affecting the oedematous area, oedema at the root of the affected limb or truncal oedema and severe peripheral neuropathy.
  • Which methods are used in surgical treatment? Surgical reduction, procedures bypassing lymphatic obstructions, liposuction treatments can be applied in surgical treatment. Surgical treatment has been developing in recent years.

In patients who develop lymphedema after breast cancer, if there is massive lymphedema resistant to general treatments or if there is lymphedema starting years after primary surgery without obvious trauma, the presence of other accompanying diseases should be investigated.
Recurrence of breast cancer in the axillary region or development of lymphangiosarcoma should be excluded by MRI or CT

Which Complications Associated With Lymphedema Can Be Seen?

Lymphedema may result in deterioration in the quality of life of patients, more frequent infections, deterioration in skin structures (hyperkeratosis, papillomatous lesions, colour changes, etc.), psychological problems, deterioration in posture as a result of weight gain, functional inadequacy, loss of joint range of motion, very rarely cancer development.

  • Firstly, it is started with plaster clothing.
  • Each finger is wrapped in 4-5 layers
  • Spiral or herringbone shape with plaster cotton or sponge
  • Pressure is controlled when winding
  • Pressure should be more distal and less proximal
  • Circulation is controlled
  • Babylastic gloves (baby gloves) are more comfortable to wear and do not damage clothing

Laplace's Law

Wider, oedematous extremities require higher levels of compression, and the desired pressure can be achieved by increasing the bandage layers and increasing the tension during application.

What Would You Like To Say About Compression Garments?

  • They are used to ensure the maintenance of long-term lymphoedema treatment
  • They are also used for pophylaxis or as part of initial treatment
  • Patients clothes
    • As long as they’re awake
    • Only during exercise
    • They use it for 24 hours

2 should be taken (during washing)

Ready-made garment is preferred in mild to moderate lymphoedema (stage 1-2). It is more affordable and has no waiting time. It does not include the fingers. It may create unbalanced pressure because it does not fully adapt to the arm shape.

 

Personalised garment is preferred in case of severe lymphedema (stage 3). It can be gloved or one piece according to the needs of the patient. Pressure distribution is balanced. It is more expensive and takes time to be produced and delivered.

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