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Lymphedema: an overview Margarita Correa MD, FAAPMR Physical Medicine and Rehabilitation Physical Medicine Institute 2020 Oakley Seaver Dr, Ste 1 Clermont, FL

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Blood and Lymphatic Circulation <ul><li>It belongs to the circulatory system: </li></ul><ul><li>one way for the blood to leave the heart , the arterial system, and 2 ways to return (the venous and lymphatic pathways) </li></ul><ul><li>Lymph is a fluid which originates in the connective tissue spaces of the body </li></ul><ul><li>Once it has entered the first lymph capillaries (initial lymph capillaries) this fluid is called lymph </li></ul>

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Microcirculation <ul><li>In contrast to the blood circulation, this system has slow rhythm , low velocity and low pressure </li></ul><ul><li>Part of the constituents of the blood will filter out the capillaries </li></ul><ul><li>This filtration will be further reabsorbed in the lymphatic capillaries </li></ul>

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Lymphatic System <ul><li>In fact if the lymphatic system did not recover the protein rich fluid the body will probably develop major systemic edema, protein loss & autointoxication and die in 24 - 48 hr </li></ul>

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Lymphatic System <ul><li>The lymphatics gradually increase in size: lymph capillaries  precollectors  lymphatic collectors  ducts and trunks  major venous circulation just before reaching the heart behind the clavicles ( angulus venosus ) </li></ul>

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Composition of the Lymph <ul><li>Lymph is usually clear , transparent or yellowish alkaline fluid, slightly less viscuos than blood </li></ul><ul><li>When the lymph is filled with the absorbed fat of the digestion in the intestines, becomes whitish and viscous and is called “ chyle ” – thoracic duct </li></ul>

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Composition of the Lymph <ul><li>Water </li></ul><ul><li>Proteins </li></ul><ul><li>Lipids </li></ul><ul><li>Carbohydrates </li></ul><ul><li>Enzymes </li></ul><ul><li>Urea </li></ul><ul><li>Minerals </li></ul><ul><li>Hormones </li></ul><ul><li>Some dissolved gases (i.e. CO2 ) </li></ul><ul><li>Cells (lymphocytes, macrophages, erythrocytes) </li></ul><ul><li>Toxins </li></ul><ul><li>Bacteria </li></ul><ul><li>Body waste, bits of cell debris, etc. </li></ul>

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Composition of Lymph <ul><li>75-100 gm proteins/day escape from the blood circulation; this is about 50% of the protein circulating in the blood plasma per day </li></ul><ul><li>These proteins are transported in the lymphatic vessels </li></ul><ul><li>Lymph production 2.1 – 3.0 lt/day </li></ul>

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Lymphatic Capillaries <ul><li>The lymphatic circulation is a one way structure beginning with the lymph capillaries </li></ul><ul><li>Lymph capillaries have no valves in their central endothelial channels -> aspirating force , pinocytosis & micropinocytosis </li></ul>

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Lymphatic Capillaries <ul><li>Anchoring filaments help the lymph capillaries to open widely if there’s significant fluid pressure in the connective tissue </li></ul><ul><li>This helps when the tissue is moved manually by Lymph Drainage Therapy or similar lymphatic therapies </li></ul>

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Lymphatic Capillaries <ul><li>These edges overlap allowing the entry of protein </li></ul>

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Pre-collectors <ul><li>Have one-way valves spaced about every 2-3 mm </li></ul><ul><li>These valves help to prevent backflow and to move lymph in one direction to the big collectors </li></ul><ul><li>These valves are essentially invaginations of the lymph vessel walls; usually consist of two leaflets (“bicuspid” valves) </li></ul>

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Lymph Collectors <ul><li>The lymph collectors are the main transporting vessels of the lymphatic system </li></ul><ul><li>They are large vessels with valves and muscular units , which carry the lymph to the surrounding lymph nodes </li></ul><ul><li>Consist of three (3) layers corresponding more or less to the intima , media , and adventitia layers of arteries and veins </li></ul>

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Lymph Collectors <ul><li>The bicuspid valve is found at each end of the lymphangions or at specific inter-valvular units of the lymph collectors </li></ul><ul><li>Lymphangions give the lymphatic vessels the characteristic appearance of a “ pearl necklace” </li></ul><ul><li>Lymphangions are considered the functional unit of the lymphatic system </li></ul>

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Lymph Trunks and Ducts <ul><li>Lymph trunks and ducts are the biggest lymph collectors of the body and carry lymph to the terminal pathways in the deep venous system at the base of the neck </li></ul><ul><li>In particular, the thoracic duct is the largest lymphatic vessel in the body </li></ul>

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Lymph Nodes <ul><li>Functions: </li></ul><ul><li>Biological filtration - specific immune cells destroys foreign or unwanted substances which can be then handled by the liver and eliminated by the urinary tract, digestive system, skin and lungs </li></ul><ul><li>Immunological - provide an environment for capturing and destroying microbes through phagocytosis and other process </li></ul><ul><li>Lymphocyte production and maturation </li></ul>

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Lymph Circulation <ul><li>Superficial circulation , just under the dermo-epidermal junction . It accounts for about 70% of upper limb lymph flow. Not directly stimulated by exercise. </li></ul><ul><li>Deep circulation of the muscles, below the fascia </li></ul><ul><li>Very deep circulation of the viscera, which is stimulated by muscle contraction </li></ul>

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Physiology <ul><li>80-90% of the fluid filtered into the interstitium from capillaries is reabsorbed into the venous side </li></ul><ul><li>The remaining 10-20% of the fluid and protein is removed from the interstitium by the lymphatic vessels </li></ul>

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Physiology <ul><li>Any condition that increases the capillary pressure, interstitial fluid proteins or capillary permeability or decreases plasma colloid osmotic pressure alters the equilibrium ( Starling equilibrium ) causes fluid movement out of arterial capillaries into the interstitium. </li></ul>

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Pathophysiology <ul><li>Occlusion or damage to either the venous side of the capillaries or to the lymphatic system may decrease reabsorption or lymphatic vessel drainage of fluid and protein causing lymphedema </li></ul>

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Primary Lymphedema

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Primary Lymphedema <ul><li>Lymphatic vessels or nodes are either absent ( aplastic ), underdeveloped ( hypoplastic ) or too large and incompetent ( hyperplastic ) </li></ul><ul><li>70-90% woman </li></ul><ul><li>The earlier it appears the worse the prognosis </li></ul><ul><li>Non-pitting edema </li></ul>

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Kinmonth’s Age Classification <ul><li>Lymphedema Congenitum and Hereditary </li></ul><ul><li>Lymphedema Type 1: Milroy’s Syndrome </li></ul><ul><li>Hereditary lymphedema represent only 1-2 % </li></ul><ul><li>of the primary lymphedema </li></ul><ul><li>Lymphedema present at birth (congenital) </li></ul><ul><li>accounts for 15 % of primary lymphedema </li></ul><ul><li> </li></ul>

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Lymphedema Type 1: Milroy’s Syndrome <ul><li>Autosomal dominant is more common that the recessive form </li></ul><ul><li>Usually bilateral in the lower limbs </li></ul>

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Lymphedema Praecox and Hereditary <ul><li>Lymphedema Type 2: Meige’s Syndrome </li></ul><ul><li>75-80% of primary lymphedema </li></ul><ul><li>Onset at puberty </li></ul><ul><li>Usually result of lymphatic hypoplasia </li></ul><ul><li>Also affects the distal lymphatics of one or both legs </li></ul>

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Primary Lymphedema <ul><li>Lymphedema Tardum </li></ul><ul><li>Similar to lymphedema praecox, the only difference is that it appears after age 35 </li></ul>

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Secondary Lymphedema <ul><li>Surgery, biopsy, dissection, radiation </li></ul><ul><li>Numerous surgical interventions, especially for cancer involve the removal of lymph nodes or their destruction by radiation therapy causing fibrosis </li></ul><ul><li>Lymph vessels can regenerate after being cut but it can be inhibited by the formation of scar tissue </li></ul>

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Secondary Lymphedema <ul><li>Metastatic carcinomas </li></ul><ul><li>can cause obstruction of the lymphatic system </li></ul><ul><li>Trauma and burns </li></ul><ul><li>physically destroys lymph vessels or nodes and provoke local or regional lymphedema, at least temporarily </li></ul>

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Secondary Lymphedema <ul><li>Filariasis </li></ul><ul><li>Endemic lymphedema, elephantiasis, involves the infection by parasitic nematodes (worms) of the order Filarioidea </li></ul>

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Secondary Lymphedema <ul><li>Lipolymphedema </li></ul><ul><li>Lipedema begins as a pathological accumulation of fat in which lymphatic circulation is normal, later lipedema can develop into a lymphostatic edema called lipo-lymphedema </li></ul><ul><li>The mechanism is compression of the lymph vessels by the adipose layer </li></ul>

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Lipolymphedema - Lipedema

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Lipolymphedema - Lipedema

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Conditions Related to Lymphedema <ul><li>Cardiofaciocutaneous syndrome </li></ul><ul><li>Cholestasis-oedema syndrome, Norwegian type </li></ul><ul><li>Cumming syndrome </li></ul><ul><li>Hennekam syndrome </li></ul><ul><li>Klippel-Trenaunay syndrome </li></ul><ul><li>Lymphoedema-distichiasis syndrome </li></ul><ul><li>Noonan’s syndrome </li></ul><ul><li>Rheumatoid disease </li></ul><ul><li>Turner’s syndrome </li></ul>

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Breast CA Lymphedema Incidence <ul><li>Lumpectomy with sentinel node technique -> 2% risk for developing lymphedema </li></ul><ul><li>Sentinel node (inject the surrounding tumor tissue with radioisotope and visualize the lymph nodes uptake at the axillary region – during surgery inject blue dye and remove these nodes) </li></ul>

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Clinical Diagnosis of Lymphedema <ul><li>Lymphedema is considered chronic after 3 months </li></ul><ul><li>If the patient persists with a difference of 2 to 3 cm in circumference or 10% volume difference between the affected and unaffected limb for more than 6 weeks after surgery </li></ul>

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Lymphedema First Signs <ul><ul><li>Puffiness </li></ul></ul><ul><ul><li>Stiffness </li></ul></ul><ul><ul><li>Fatigue, malaise, discomfort </li></ul></ul><ul><ul><li>Tightness </li></ul></ul><ul><ul><li>Skin tension </li></ul></ul><ul><ul><li>Heaviness </li></ul></ul><ul><ul><li>Heat </li></ul></ul><ul><ul><li>Pain, affecting the skin or the articulation </li></ul></ul><ul><ul><li>Numbness and paresthesias </li></ul></ul>

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Lymphedema <ul><li>Other Signs Associated with Swelling </li></ul><ul><li>Fibrosis: thickening, and dryness of the skin (hyperkeratosis), papillomas </li></ul><ul><li>Decreased ROM, stiffness in the affected limb or contractures of the joints </li></ul>

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Stemmer’s sign <ul><ul><li>It reflects the degree of distal fibrosis , usually making it an especially accurate indicator for primary lymphedema of the lower extremity </li></ul></ul><ul><ul><li>The sign is positive in 82% to 92% of primary lower limb lymphedemas and in 56% of cases of secondary lower limb lymphedema </li></ul></ul>

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Lymphedema Diagnosis <ul><li>Special Diagnostic Tools: Imaging </li></ul><ul><ul><li>These are generally used for difficult diagnoses to help assess whether the patient is suffering from a pure lymphedema or a mixed form : </li></ul></ul><ul><ul><li>veno or phlebo-lymphedema </li></ul></ul><ul><ul><li>lipolymphedema </li></ul></ul><ul><ul><li>lymphedema secondary to an arterial condition </li></ul></ul><ul><ul><li>angiodysplasia, etc </li></ul></ul>

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Lymphedema Diagnosis <ul><li>Lymphoscintigraphy </li></ul><ul><ul><li>Is the gold standard for diagnosis and follow up of lymphedema . </li></ul></ul><ul><ul><li>Tc99 colloid is injected between digits and the radiotracer distributes along the lymphatic system </li></ul></ul><ul><ul><li>Yields more functional than anatomical information </li></ul></ul>

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Lymphoscintigraphy

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Diagnosis <ul><li>Computed tomography (CT) </li></ul><ul><ul><li>Provides more anatomic than functional information </li></ul></ul><ul><ul><li>Specific images in lymphedema: </li></ul></ul><ul><ul><ul><li>Enlargement of the skin </li></ul></ul></ul><ul><ul><ul><li>Subcutaneous tissue is heterogenous </li></ul></ul></ul><ul><ul><ul><li>Lymphatic lakes </li></ul></ul></ul><ul><ul><ul><li>Muscle compartment </li></ul></ul></ul>

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Diagnosis <ul><li>Magnetic Resonance Imaging (MRI) </li></ul><ul><ul><li>Diffuse dermal and subcutaneous </li></ul></ul><ul><ul><li>edema </li></ul></ul><ul><ul><li>Thickness between fat lobules is </li></ul></ul><ul><ul><ul><li>increased </li></ul></ul></ul><ul><ul><li>The fat cells is isolated by fibrosis </li></ul></ul><ul><ul><ul><li>(intralobular fibrosis) </li></ul></ul></ul><ul><ul><li>Specific “ honeycomb ” aspect of the </li></ul></ul><ul><ul><ul><li>subcutaneous compartment </li></ul></ul></ul><ul><ul><li>Increased size of the subcutaneous </li></ul></ul><ul><ul><ul><li>fat compartment </li></ul></ul></ul>

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Diagnosis <ul><li>Consider Arterial and Venous Duplex if a deep venous thrombosis (DVT) or arterial insufficiency is suspected </li></ul>

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Differential Diagnosis of Chronic Leg Edema <ul><li>elevated pulmonary artery pressure (often due to obstructive sleep apnea) </li></ul><ul><li>congestive heart failure (CHF) </li></ul><ul><li>venous insufficiency </li></ul><ul><li>use of non-steroidal anti-inflammatory drugs (NSAID's) </li></ul><ul><li>proteinuria (>1 g daily) </li></ul><ul><li>idiopathic causes </li></ul>

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Lymphedema Stages <ul><li>Stage 0 – Latent </li></ul><ul><li>Mild lymphedema – Stage 1 – still pitting </li></ul><ul><li>< 3 cm larger than the opposite extremity </li></ul><ul><li>Moderate lymphedema – Stage 2 – non pitting </li></ul><ul><ul><li>3 - 5 cm difference </li></ul></ul><ul><li>Severe lymphedema – Stage 3 - lymphostatic elephantiasis </li></ul><ul><ul><li>> 5 cm difference </li></ul></ul>

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Stage 0 <ul><li>Stage 0: Subclinical or Latent Stage </li></ul><ul><li>This stage cannot be detected clinically </li></ul><ul><li>Microscopic examination usually reveals abnormally tortuous and dilated lymphatic vessels and widening of subcutaneous spaces caused by increased pressure in lymphatic capillaries </li></ul>

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Stage 1 Lymphedema <ul><li>Stage 1 (Reversible) </li></ul><ul><li>The swelling usually resolves spontaneously </li></ul><ul><li>Pitting, soft edema </li></ul><ul><li>High protein edema </li></ul><ul><li>Focal fibrosclerotic tissue alterations </li></ul>

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Stage 1 Lymphedema

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Stage 2 Lymphedema <ul><li>Stage 2 (Spontaneously irreversible) </li></ul><ul><li>The skin does not move much and is chronically inflamed </li></ul><ul><li>Fibroblasts predominate in the stagnant, protein rich liquid </li></ul><ul><li>Skin becomes thickened developing hyperkeratosis </li></ul><ul><li>This condition will not reverse on its own, but can usually be reduced through CDT </li></ul>

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Stage 2 Lymphedema

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Stage 3 Lymphedema <ul><li>Stage 3 (Elephantiasis) </li></ul><ul><li>Non pitting </li></ul><ul><li>Very hard edema, with no skin movement </li></ul><ul><li>Significant fibrosis and hardening of the skin; proliferation of adipose tissue </li></ul><ul><li>Lymphostatic verucosis (warts), papilloma formation, skin fissuring </li></ul><ul><li>Significant loss of joint mobility and movement </li></ul>

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Elephantiasis

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Complications <ul><li>Lymphangitis </li></ul><ul><li>patient with erythema, increased swelling, calor, fever, chills; treat as an EMERGENCY !! </li></ul><ul><li>Rx: oral vs. I.V. antibiotic, if skin laceration add topical antibiotic </li></ul><ul><li>Lymphangiosarcoma – </li></ul><ul><li>Stewart-Treves Syndrome </li></ul>

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Complications <ul><li>Infection and inflammation are believed to damage the lymphatic system and trigger lymphedema </li></ul><ul><li>Infection is often difficult to treat because of lymphostasis and may cause fibrosis, which further compromises the lymphatic draining system </li></ul>

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Absolute Contraindications for Decongestive Therapy <ul><li>Hemorrhage (bleeding) </li></ul><ul><li>Acute enuresis – renal failure </li></ul><ul><li>* Carcinomatous invasion to the </li></ul><ul><li>extremity </li></ul><ul><li>* No large scientific studies have shown any increase in metastasis in cancer patients treated with lymphatic drainage (palliative) </li></ul><ul><li>Sensory impairment i.e. Brachial or </li></ul><ul><li>lumbosacral plexus injury </li></ul>

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Absolute Contraindications <ul><li>Acute infection, fever, early inflammatory disease - caution with chronic tuberculosis or toxoplasmosis which can be reactivated and become acute </li></ul><ul><li>Serious circulatory problems - venous thrombosis (risk of embolism) and peripheral arterial insufficiency </li></ul><ul><li>Major cardiac conditions - unstable angina pectoris, acute myocardial infarction and uncompensated congestive heart failure </li></ul>

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Contraindications <ul><li>Major Cardiac conditions </li></ul><ul><li>It’s not recommended to work on cardiac edemas if the cardiac problem is not under control due to additional liquid coming from the tissue drainage increasing the cardiac pre-load </li></ul><ul><li>This arises a degree of uncertainty in respect to patients with compensated congestive heart failure and a severe left ventricular dysfunction (LVEF <30%) Work close with physician ! </li></ul>

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