Lymphedema an overview

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

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

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

Lymphatic System 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

Lymphatic System 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 )

Composition of the Lymph Lymph is usually clear , transparent or yellowish alkaline fluid, slightly less viscuos than blood 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

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

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

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

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

Lymphatic Capillaries These edges overlap allowing the entry of protein

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

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

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

Lymph Trunks and Ducts 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 In particular, the thoracic duct is the largest lymphatic vessel in the body

Lymph Nodes Functions: 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 Immunological - provide an environment for capturing and destroying microbes through phagocytosis and other process Lymphocyte production and maturation

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

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

Physiology 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.

Pathophysiology 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

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

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

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

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

Primary Lymphedema Lymphedema Tardum Similar to lymphedema praecox, the only difference is that it appears after age 35

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

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

Secondary Lymphedema Filariasis Endemic lymphedema, elephantiasis, involves the infection by parasitic nematodes (worms) of the order Filarioidea

Secondary Lymphedema Lipolymphedema 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 The mechanism is compression of the lymph vessels by the adipose layer

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

Breast CA Lymphedema Incidence Lumpectomy with sentinel node technique -> 2% risk for developing lymphedema 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)

Clinical Diagnosis of Lymphedema Lymphedema is considered chronic after 3 months 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

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

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

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

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

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

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

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

Diagnosis Consider Arterial and Venous Duplex if a deep venous thrombosis (DVT) or arterial insufficiency is suspected

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

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

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

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

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

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

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

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

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

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

Contraindications Major Cardiac conditions 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 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 !

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Lymphedema an overview