Patient Evaluation Differential Diagnosis Co-morbidity

Lymphedema: Patient Evaluation, Differential Diagnosis, & Co-morbidity Paula JB Stewart MD, LANA-CLT November 5, 2005 WHEN LIFE SURROUNDS US WITH PROBLEMS, REMEMBER NO QUESTION CAN BE ASKED IF THE ANSWER IS NOT ALREADY IN THE UNIVERSE. maya angelou Lymphedema  Definition: the accumulation of protein rich fluid in the interstitium which causes chronic inflammation and reactive fibrosis of the affected tissues. Lymphedema  Results  from:   Mechanical insufficiency due to injury to the lymph vessels or impairment of lymph flow due to paralysis, blockage or inadequacy of lymphatics Dynamic insufficiency in which the lymph flow exceeds the transport capacity of the intact lymphatic system Combined insufficiency Anatomy of the Lymphatics most distal, blind pouches which absorb interstitial fluid.  Precollectors: move lymph fluid from the capillaries to the collectors.  Collectors: are a series of valved chambers which propel lymph fluid towards the trunk through contraction of the muscle in the vessel walls.These are called lymphangions.  Capillaries: Anatomy of the Lymphatics  Lymph Nodes: the lymph fluid passes through the nodes, found in the articulations of the large joints , the mesentery and the neck (600-700). The lymph nodes filter waste and regulate protein content in the lymph fluid. Anatomy of the Lymphatics  Ducts: eventually the lymph fluid is transported into larger deeper vessels called trunks which empty into either the thoracic duct or the right lymphatic duct. The thoracic duct drains the left arm and both legs and three quarters of the trunk, the right lymphatic duct drains the right arm and one quarter of the trunk. Primary Lymphedema  Definition: dysplasia of the lymphatic system resulting in impaired lymph transport and accumulation of protein rich fluid in the interstitium with subsequent swelling of the tissues.  Accounts for 10% of all lymphedema Primary Lymphedema  Pathology:     Hypoplasia of lymph collectors (most common) Hyperplasia of lymph collectors Aplasia of single lymph collectors Lymph node hypoplasia combined with fibrosis Primary Lymphedema  Types:  Type I Nonne-Milroy- hereditary congenital elephantiasis due to aplasia of the lymphatics Type II non-congenital familial lymphedema • Lymphedema preacox occurs before 35 • Lymphedema tarda occurs at a later date  Primary Lymphedema  Primary  lymphedema accompanied by congenital syndromes: Klippel-Trenaunay-Syndrome: • • • • • hemangioma (95%) hypertrophy of bones and soft tissues (93%) varices (76%) lymphangioma (8%) pain (32%) • Gloviczki 1991 Primary Lymphedema  Syndromes:  Turner‟s Syndrome: absence of an X chromosome in women • drawfism • dysplasia of mesodermal tissue (kidneys and ovaries) • hypoplasia of the pelvis and patella • facial anomalies • lymphedema due to hypoplastic lymph vessels Primary Lymphedema  Syndromes:  Noonan‟s Syndrome (no chromosomal anomaly) • • • • • dwarfism skeletal anomalies late puberty congenital heart disease intestinal lymphangiectasis Secondary Lymphedema  Acquired  Lymphedema: 90% of lymphedema in the US Causes • • • • • Surgery Trauma Infection Malignant lymphedema Filarial disease:>100 million cases worldwide Lymphedema: signs and symptoms           Swelling Discomfort or pain Tingling or numbness Pressure or tightness Heaviness in the limb Increased infections Fibrotic skin changes Lymphorrhea Decreased mobility Impaired wound healing Lymphedema: causes in the cancer patient  Surgery: with removal of the tumor there is almost always a lymph node dissection for staging purposes. This disruption of the lymphatic system results in approximately a 20% incidence of lymphedema in breast cancer patients. The sentinel lymph node biopsy is growing in acceptance for staging the breast cancer patient. A negative biopsy spares the patient a dissection and thus reduces the risk of lymphedema to 3-4% without radiation, 7-11% with radiation. Lymphedema: causes in the cancer patient Radiation: causes fibrosis of the tissues irradiated, which reduces the circulation of lymph fluid in the affected area. It also reduces the regeneration of new vessels and scars down lymph nodes, impairing their response to infection. In breast cancer patients the risk of lymphedema increase to greater than 30% for those treated with lymph node dissection and radiation. Lymphedema: causes in the cancer patient Malignant tumor growth: can block a lymph node or vessel, preventing the flow of lymph fluid. Mixed Edemas  Phlebo-lymphedema  treatment: similar to lymphedema. Responds well to MLD and CDT. Graduated compression, weight loss, limb elevation are all important. Mixed Edemas  Lipedema  definition: bilateral lower extremity symmetrical fatty deposits, with sparing of the foot and ankle and absent Stemmer‟s sign. The legs are often painful to palpation and easy bruising is often noted. Mixed Edemas Lipo-lymphedema definition:accumulation of edema fluid in the presence of lipedema diagnosis: lymphoscintigraphy and indirect lymphography treatment: Mild cases treated with class II garments. More severe cases respond to wraps, MLD and Class III compression garments. The Dangerous Look Alikes Lymphedema  Deep Venous Thrombosis (DVT)  Congestive Heart Failure (CHF)  Cellulitis   Malignant  All can cause edema and can be associated with lymphedema MLD contraindicated in each with exceptions Malignant Lymphedema  Definition: impaired lymph flow due to malignant tumor infiltration and blockage or compression of lymphatic vessels and lymph nodes. Diagnosis: physical exam and history which typically reveal rapid onset, neuropathic pain, weakness, skin discoloration or unusual lesions, proximal or genital location, lymphadenopathy, tense edema.  Malignant Lymphedema Ultrasound, CT, MRI, Lymphoscintigraphy.  Treatment: palliative or curative treatment of tumor with radiation, surgery or chemo. If terminal then no contraindication to MLD and compression bandages or garments. If not terminal, then treat with bandages and garments alone. (NB: this requires excellent communication with Oncologist)  Diagnosis: Deep Venous Thrombosis formation of a thrombus or clot in the deep venous system due to hypercoagulability or venous stasis.  Diagnosis: Ultrasound or CT. Venography not recommended.  Treatment: anticoagulation or IVC filter. During initial bedrest keep affected limb elevated.  Definition: Cellulitis an acute streptococcal infection of the skin and the subcutaneous fatty tissues. (Weissleder)  Diagnosis: Examination and history  Treatment: antibiotics. Elevation initially, then light compression. Once inflammation has resolved initiate MLD if necessary.  Definition: Other Look Alikes *Post-phlebitic syndrome *Anasarca *Myxedema *Inflammatory arthritis *Complex regional pain syndrome *Idiopathic cyclic edema *Artificial lymphedema Post-Phlebitic Syndrome fluid accumulation in the interstitium that arises from chronic structural damage to the deep veins that resulted from an episode of thrombophlebitis.  Diagnosis: Doppler studies, CT, MRI  Treatment: Compressive garments, moderate cases can benefit from CDT and surgery may be necessary for severe cases.  Definition: Anasarca generalized edema that results from severe (<2.5dl) hypoalbuminemia due to reduced plasma oncotic pressure.  Diagnosis: Serum albumin and prealbumin, clinical history and exam.  Treatment: Increase protein intake, gentle diuresis and light compression and elevation of affected limbs.  Definition: Myxedema  Definition: Deposition of mucinous substances in the skin which results from thyroid dysfunction.   Diagnosis: clinical manifestations of thyroid disease such as, dry skin, brittle nails, thinning hair, decreased sweat and orange colored skin in conjunction with abnormal thyroid tests. Treatment: correct thyroid abnormality Inflammatory Arthritis inflammatory disease of the joints with systemic manifestations. Includes Rheumatoid, Reiter‟s, Psoriatic, Ankylosing spondylitis, Ulcerative colitis, Regional enteritis, gout, and bacterial joint infections.  Diagnosis: physical exam, synovial fluid exam, blood work  Definition: Inflammatory Arthritis  Treatment: appropriate medications, splints, therapy, adaptive equipment. Complex Regional Pain Syndrome a chronic painful condition associated with neurovascular disturbance and dystrophic changes of the skin and bones. Three phases.  Diagnosis: physical exam, bone scan, xray  Treatment: Early on, steroids and aggressive desensitization. Later stellate ganglia blocks.  Definition: Idiopathic Cyclic Edema Periodic rapid weight gain and generalized edema in women of reproductive age.  Diagnosis: History, exam  Treatment: salt restriction, mild diuretics, weight loss if overweight, consider OCs  Definition: Artificial Lymphedema Self-induced lymphedema most often by strangulation of the limb or tapping  Diagnosis: indirect lymphangiography and lymphoscintigraphy can rule out true lymphedema  Treatment: CDT, and appropriate psychological support.  Definition: Localized Edema limb = lymphedema or venous obstruction or dependent edema of paralyzed limb or DVT  Facial = allergic reaction or superior vena cava syndrome  Bilateral lower extremity edema = inferior vena cava syndrome or ascites or abdominal mass  Unilateral Patient Evaluation  History taking tools  Measurement  Physical examination Patient Evaluation  History   taking Onset of swelling and time course Known cause? • Cancer, Trauma, Family history, Infections    Pain Infections Wounds Patient Evaluation  History  taking Co-morbidities • • • • • • • Congestive Heart Failure Renal Failure Diabetes Deep Venous Thrombosis Thyroid Disease On steroids Arthritis Patient Evaluation  History   taking Family History Social History • Habits  Tobacco, Etoh, Exercise • Occupation • Social support • Home setting Patient Evaluation  Volume  Measurement Techniques   Circumferential measurement every 2-4 cm on a board. Use 4cm to calculate volume. Water displacement technique: technique messy and difficult. Perometry is becoming the new gold standard in measurement. Does not differentiate fat and edema. Misses the proximal limb. Patient Evaluation  Calculating      the Volume of a cylinder: Formula: v = (pi)r x r(h) V = volume Pi = 3.14159 R = radius H = height • Circumference = 2(pi)r or r = c/2(pi). If H=4, then V = pi(c/2(pi) x c/2(pi))4. with cancelling Volume = c xc/pi Patient Evaluation  Other    Things to Measure: ABI if Cardiovascular disease Tonometry for skin pliability Labs for thyroid disease, Cancer tumor markers, BNP, Electrolytes, Hormonal status Patient Physical Examination  Vital Signs, Weight, Height, and BMI  Inspect affected limb(s): asymmetry, skin color, lesions: verrucal hyperplasia, transudative weeping, papillomas, scars, burns, cuts, bites, onchymycosis, wounds.  Check trunk in arm LE, genitals in leg LE  Palpate: masses, lymphadenopathy, skin temperature, turgor, texture, tenderness, cords, pitting, Stemmer‟s sign Patient Physical Examination  Stemmer‟s Sign: grasp the 2nd toe or 2nd finger of affected limb below the PIP. Pinch the skin and attempt to lift upward. If the skin does not lift, it is a positive Stemmer‟s Sign and indicates LE. A negative sign does not mean that there is not LE. Lymphedema: diagnosis Stages of lymphedema: Stage I: Reversible with elevation. Pitting edema. Stage II: Spontaneously irreversible. Some fibrosis with variable pitting. Stage III: Lymphostatic elephantiasis with thick fibrotic skin and little pitting. Lymphedema: diagnosis Classification of edema: 1+ Edema barely detectable 2+ A slight indentation after skin is depressed 3+ A deeper indentation, which returns to normal within 5-30 seconds 4+ Limb is 1.5-2 times normal circumference Diagnostic Tools exam and history most important! If diagnosis uncertain then consider:  Lymphoscintigraphy: a nuclear medicine procedure to evaluate the superficial and deep lymphatics  Indirect lymphography: a radiographic evaluation of the superficial lymphatics  (not widely available)  Physical Lymphedema Treatment  Five steps in treatment Program known as Comprehensive Lymphedema Management (CLM)      Skin care Manual Lymphatic Drainage Complex wrapping with low stretch bandages Exercise program with compression Fit with compressive garment Lymphedema  “In the past physicians played down the importance of lymphedema and pointed out ..that it is rare, that there is no effective treatment, that the patient must learn to „live with it‟, or even that it will get better some day or will go away. (They) have failed to instruct patients on how to avoid lymphedema after surgery or radiation therapy and continue to grossly understate the incidence of this serious and lifelong illness.” • Dr. Robert Lerner Conclusions  Lymphedema is a serious and chronic condition with no cure at this time.  Correct diagnosis is essential in providing effective treatment  With diligent treatment and ongoing management lymphedema can be controlled, reducing complications. Primary Lymphedema TAKE A MONTH AND SHOW SOME KINDNESS FOR THE FOLKS WHO THOUGHT THAT BLINDNESS WAS AN ILLNESS THAT AFFECTED THE EYES ALONE. maya