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Lymphland International Lymphedema Online
Lipedema, Lymphedema, and Lipo-Lymphedema What is Lipedema? Lipedema, which is also known as painful fat syndrome, is abnormal symmetrical swelling due to accumulations of fat and fluid located in the tissues just under the skin of the hips and legs, down to the ankles but not extending to the feet. Also there are fatty bulges on the outer surfaces of the thighs. [1] This inherited condition occurs almost exclusively in women and usually develops during puberty. Additional causes of this consition include a hormone component, microcirculation disorders, and inflammation. Lipedema and Lymphedema Confused While lipedema is not a disorder of the lymphatic system, lipedema and lymphedema are often confused because both conditions involve enlargement of the legs. Although distinctly different conditions, lipedema can cause lower extremity lymphedema to be present as a comorbidity. There are several features about lipedema that distinguish it from bilateral lower extremity lymphedema. Lipedema is symmetrical because the excess fat involves both legs equally. Lymphedema is not symmetrical and often occurs in only one leg. Lipedema swelling creates a characteristic ring at the base of the ankle. The swelling stops here and does not affect the feet. Lymphedema swelling, due to primary lymphedema, often begins at the foot and progresses up the leg. Secondary lymphedema most commonly begins near the trunk and progresses down the leg and includes the foot. Lipedema does not include swelling of the foot. Lymphedema swelling affects the leg and the foot. Lipedema causes severe pain paticularl when the patient is touched. Lymphedema does cause pain; however, it is rarely this severe. Lipedema often causes bruising and subcutaneous bleeding. Lymphedema symptoms do not include bruising and subcutaneous bleeding. Lipedema does not have Stemmer’s sign as a diagnostic indicator Lymphedema has Stemmer’s sign as a diagnostic indicator. The Symptoms of Lipedema In the early stages of lipedema, the upper part of the body may be slim. Despite a slender upper body, fat accumulates from the tops of the hips to the ankles. As weight is gained, it accumulates in the areas of the hips and legs. When weight is lost, the fat decrease occurs in areas other than those affected by lipedema. Weight loss does not occur in the area between the waist and ankles. Fat extends down the legs and creates a ring of fatty tissue that overlaps the tops of the feet. Swelling develops in the legs during the second half of the day; however, this swelling decreases during sleep. Pain is present, particularly along the shin. In the early stages nodules develop. In the later stages lobules develop. Diagnostic Errors Diet is not the answer to the problem! Often patients are told that they are fat and weight loss is recommended. However the fatty tissue accompanying lipedema cannot be significantly decreased by diet. Obesity, caused by overeating, responds to a proper dietary regime: lipedema does not. This is not primary lymphedema. Another common diagnostic error is that the condition is primary lymphedema. A misdiagnosis of primary lymphedema results in frustration for the person seeks out lymphedema therapy with poor results. Differential diagnostic keys include: In lymphedma, Stemmer’s sign is positive. In lymphdema, the swelling affects both the feet and legs (but not the hips). In lymphedema, pain is not usually present along the shinbone. Complications of Lipedema Obesity due to gaining excess weight is a common complication of lipedema. As more weight is gained, additional stresses are placed on all of the body systems. As lipedema progresses into the later stages, secondary lymphedema affecting the legs often develops as a complication. Swelling of the feet, and a positive Stemmer’s sign, are indications that lymphedema is present. Lipo-lymphedema is the combination of lipedema, obesity, and lymphedema. Lipo-lymphedema can also develop in combination with chronic venous insufficiency, idiopathic edema, or other vascular disorders. The Treatment of Lipedema At this time there is no effective treatment for lipedema. Evaluation by an endocrinologist, and the treatment of endocrine imbalances, may help. When lymphedema is also present, treating that condition can also help; however, the lymphedema treatment must be modified to accommodate the pain and other needs due to the lipedema. Weight control, without drastic dieting, can help to prevent other conditions associated with obesity. Water exercises are helpful. The support of the water eases the strain on the joints. It is also relaxing and makes movements easier. What about Liposuction? Liposuction is a surgical procedure to remove excessive fat from under the skin. Liposuction has been studied as a potential treatment of lipedema; however, it is not accepted for this purpose because of the risk that such surgery can make the condition worse. [2] Although often perceived as being minor cosmetic surgery, this is not true of liposuction. Instead it is an invasive procedure that cannot be performed without causing additional damage to the lymphatic system that can make the condition worse. When lymphedema is already present, there is also the increased risk of infection or poor healing after this surgery. What about Gastric Bypass? Gastric bypass surgery is performed to bring about the loss of body weight; however, in those with lipedema this weight will not be lost in the areas affected by that condition. When gross obesity is present, such surgery may be necessary; however, it is unrealistic to expect a reduction in the fatty areas of the hips and legs. Treating Lipedema and Lymphedema When lipedema and lymphedema are present concurrently, the lymphedema therapist must balance the needs of both conditions. Manual lymph drainage with only light strokes is used initially, because of the pain associated with lipedema. These gentle strokes open the superficial lymphatics (located just under the skin) and decrease the pain by clearing blocked draining areas and clogged lymph nodes. After several sessions, when the pain is less, the therapist can start working on the affected limbs. Bandaging and compression garments are not applied until the patient is able to tolerate this compression without pain. Some researchers believe that once the patient can tolerate compression, this may help to reduce the amount of fat that is present. http://www.lymphnotes.com/article.php/id/164/ --- What is Venous Oedema? Chronic venous insufficiency is a common chronic condition, particularly in those of advancing years. It is characterised by lower leg oedema, heaviness, tightness, aching and discomfort, all of which are relieved, to some extent, by elevation. This oedema is called venous oedema. One way valves situated every few millimetres in the veins assist the heart to pump blood back to the heart. With increasing age and other factors such as being overweight, chronic heart failure, hypertension, pregnancy, deep vein thrombosis or an excessive amount of standing, an additional strain is placed on the leg veins by the resultant high gravitational forces causing them to dilate. This, in turn, makes the valves less effective in the process of returning blood to the heart by allowing backflow of blood to occur and collection or "pooling" of blood in the calves. The valves become non-functional and the veins misshapen. These are termed varicose veins. They cause stagnation of blood in the calves and an increased amount of fluid to pass from the blood capillaries into the tissues and the onset of oedema. As the condition progresses, the lymphatic drainage from the legs becomes overloaded and the condition of the skin deteriorates. It becomes pigmented, dry, fragile, easily breaking down and becoming infected, leading to ulceration. What is the Treatment of Venous Oedema? Vascular surgery does not always provide long term relief for CVI. However, compression therapy has been shown to be effective in improving venous and lymphatic drainage from lower limbs, as well as keeping oedema under control. Mild to moderate oedema is treated by fitting low to medium compression calf stockings. Moderate to severe oedema require multi-layer bandaging for a few days to reduce the oedema before fitting stockings. --- The term lipedema was first used by Allen and Hines (1940) to describe a symmetrical "swelling" of both legs, extending from the hips to the ankles, caused by deposits of subcutaneous adipose (fatty) tissue. The underlying etiology of these fat deposits remains unknown. While lipedema is not a disorder of the lymphatic system per se, it is frequently confused with bilateral lower extremity lymphedema. It occurs almost exclusively in women and may have an associated family history (20 per cent of cases) and is usually accompanied by hormonal disorders as well (Strossenreuther, 1999). If present in a man, it is accompanied by massive hormonal disorder. Fat in the lower extremities extends to the malleoli (ankle bones), often with flaps of tissue hanging over the foot. The feet are not affected; occasionally, lipedema is found in the arms. Typically, there are also fatty bulges in the medial proximal thigh and the medial distal thigh, just above the knee. Clinically, the affected individuals complain of pitting edema as the day progresses, which is relieved by prolonged elevation of the leg(s) overnight (Rank and Wong, 1966; Rudkin and Miller, 1994; Casley-Smith, 1997). In Stage I, the skin is still soft and regular, but nodular changes can be felt upon palpation (see photo #1). There are no color changes in the skin and the subcutaneous tissues have a spongy feel, like a soft rubber doll. In Stage II, the subcutaneous tissue becomes more nodular and tough. Large fatty lobules begin to form on the medial distal and proximal thigh and medial and lateral ankles just above the malleoli (see photo # 2). Pitting edema is common, increasing as the day progresses. The individual may report hypersensitivity over the anterior tibial (shin) area. Skin color changes occur in the lower leg, indicative of secondary lymphedema, which often occurs in later stage lipedema. Pathophysiology of Lipedema (Strossenreuther, 1999). There are many histological and physiological changes that occur in lipedema. There is a decrease in the elasticity of the skin and underlying connective tissue. The basement membrane of blood vessels is thickened and there are disturbances in vasomotion. There is decreased vascular resistance, increased skin perfusion, and increased capillary filtration. There is increased venous/blood capillary pressure causing increased ultrafiltration. These vascular changes combined with the decreased efficiency of the calf muscle pump, result in both the dependent pitting edema seen in Stage I, as well as the the secondary lymphedema that often complicates lipedema in its later stages. Histological changes seen in lipedema include a thinning of the epidermal layer, thickening of the subcutaneous tissue layer, fibrosis of arterioles, tearing of elastic fibers, dilated venules and capillaries, and hypertrophy and hyperplasia of fat cells. Clinical studies show that there is enlargement of the pre-lymphatic channels (Stoberl et al., 1986) as well as defects in capillary perfusion (Weinert and Leeman, 1991). Some authors have reported no alteration in lymphatic transport (Brautigam et al., 1998) while others (Bilancini et al., 1995) have reported decreased lymph outflow in those individuals with lipedema. Foldi and Foldi (1993) reported an increase in fat cell growth during lymphostasis. Medical Management Diagnosis. The diagnosis of lipedema is difficult if the clinician is unfamiliar with this condition. Often, these people are told that they are "fat" and should just lose weight to resolve the problem. For reasons still unknown, the fatty tissue accompanying this condition cannot be significantly decreased by diet. It is not uncommon for a diagnosis of primary lymphedema to be made. This results in frustration for the person who then seeks out lymphedema therapy with poor results. There are several significant clinical differences between lipedema and bilateral primary lymphedema. The feet are not involved in lipedema; while they are edematous with a positive Stemmer's sign in lymphedema, Stemmer's sign is negative in lipedema (see fig. 12-17). The "swelling" in lipedema is symmetrical, while in primary lymphedema usually one limb is more involved than the other. The subcutaneous tissues feel rubbery in lipedema. In advanced Stage II lymphedema, there is significant subcutaneous fibrosis, which feels firmer than lipedema. While there have been reported incidences of cellulitis in Stage II lipedema (usually with a component of lymphedema as well), the frequency of cellulitis in Stage II lymphedema is much higher. The time of onset of the "swelling" in lipedema is usually around puberty and 90 per cent of these cases have accompanying diagnoses of hormonal disturbance (thyroid, pituitary, or ovarian). This is usually not the case with primary lymphedema. A lymphoscintigram may be helpful to differentiate between lymphedema and lipedema, however, there can be conflicting results as lymphedema often occurs to some degree in the later stages of lipedema, probably due to impairment of lymph flow caused by the pressure of fatty tissue. In fact, there are clinical cases of bilateral lower extremity lymphedema in the morbidly obese individual; the onset of the lymphedema occurs after body weight exceeds 350-400 pounds. It is plausible to suspect that the pressure of a large apron of abdominal fat can effectively block lymph flow through the inguinal area causing the lymphedema but there is a difference between these cases and lipedema because obesity does not cause lipedema. Lipedema is caused by a hormonal imbalance resulting in excessive deposition of adipose tissue, most often in the lower extremities (see Figs. 12-21; 12-22) although it can occur in the upper extremities as well. Treatment and Prognosis. There is no effective medical treatment for lipedema and the prognosis is guarded; however, significant functional improvements are possible with good program compliance and therapy intervention. Medical management involves treating the hormonal disturbance as effectively as possible and providing nutritional guidance to avoid additional weight gain. Many of these individuals have endured years of ridicule because of their physical appearance and become recluses in their homes, further limiting their activity level. As lipedema progresses and the hypersensitivity increases, they feel less inclined to walk or exercise because of the pain. They inevitably gain more weight due to the inactivity and depression, often finding food their only comfort. The primary goal of therapy intervention in the person with lipedema is symptomatic relief and realistic improvement of trunk and lower extremity function. Application of the combined lymphedema treatments has shown some success in relieving the pain and hypersensitivity in the lower legs and improving general mobility. Usually, a lower level of compression is needed to support a lipedematous limb, compared to a lymphedematous limb of the same size and girth. This guideline applies to the compression garments as well. These individuals often require more padding under the compression bandages, particularly in the anterior tibial area. They do not tolerate the heavier, denser compression fabrics and usually require a lower grade compression garment than someone with uncomplicated lymphedema. The therapist must remember, however, that later stage lipedema is often accompanied by lymphedema as well, and the treatment and management must take that factor into consideration when recommending exercise and garments. The main goals of intervention are to decrease pain and hypersensitivity, to decrease the lymphedematous component of the disease, and to assist the individual in maintaining and/or reducing adipose tissue through exercise and nutritional guidance. The compression garments can help to decrease the adipose tissue with exercise and weight loss. The most difficult task is fitting the compression garments. They must be custom made due to the large size of the individual and are often uncomfortable at the waist, particularly when sitting. Making the radical change in daily activity level is most challenging for these individuals. Providing continued support and encouragement is important. Networking is helpful and is facilitated by offering a support group, even when held on an irregular, informal basis. An hour-long educational meeting, even if only offered three or four times per year can provide a neutral meeting place for people to begin networking. Nothing can compare to the encouragement and hope that an individual with lipedema/lymphedema can derive from seeing and talking with someone else living with the same problem and hearing how others cope on a day-to-day basis. Therapists can learn some of the best guidance on exercise and coping with garments in a group like this. http://www.lymphedema-therapy.com/Lipedema.htm Lipedema - NLN Question Corner How is lipedema differant than lymphedema? http://www.lymphnet.org/question04-03.html Lipoedema - Topic Discussion http://www.uklymph.com/forums/viewtopic.php?p=871&sid=4bb84ad883d284b7d4608f7b049fe77b is www.patriciacornute.com --- Lymphedema is manageable, not curable Many people may know someone who has an enlarged or swollen arm or leg, but few have heard of the chronic disorder of lymphedema. Lymphedema is swelling of a limb or other body part due to damage to the lymphatic system. It can occur in arms after breast cancer surgery or in legs after any treatment that alters the flow of lymphatic fluid from the legs. The lymphatic system runs throughout the body. It helps carry lymph from the body tissue back into circulation. Lymph is a clear, thick, syrup-like fluid that moves through the lymph system toward the blood system. In surgery to remove cancer, lymph nodes, which are attached to lymphatic vessels, often are removed. "The lymph node acts as a filter," said Stuart Tipping, M.D., an oncologist at Marshfield Clinic-Marshfield Center. "It helps fight infection and often filters out cancer cells that can then grow in the lymph node." People who are at highest risk for lymphedema are those who have had damage from surgery, radiation, infection or trauma to one of the major lymph node areas. A person also can be born with absent or dysfunctional lymph vessels. Lymphedema can occur in arms, legs, the pelvis, face and breast tissue. Lymphedema may occur immediately after the surgery or event, or as many as 15 years later. That's why patients must be careful when carrying items, wearing jewelry or doing certain activities. Patients should also not allow blood to be drawn, blood pressure checked or IVs started in the affected limb and they should protect themselves from bites, scratches or constriction. Extra precautions also may be needed for air travel. The diameter of the affected limb can vary by a few inches over time. Humid weather generally increases the size. Patients should call their surgeon or primary healthcare provider if they have any changes in their affected limb, such as swelling or infection. The first sign of a problem may be jewelry that feels tighter than normal. And the earlier lymphedema is recognized, the more likely treatment will be successful in controlling it, said Julie Will, an occupational therapist trained in caring for patients with lymphedema. Once the limb swells, it is possible to decrease the amount of fluid through various non-invasive techniques. However, if it is allowed to remain swollen over a longer period, the treatment may take longer, but it can still be beneficial. Untreated lymphedema can lead to skin problems and increased risk of infection. Some patients have been hospitalized for days to treat infections that were not treated early. Patients also can lose their mobility if the affected limb is a leg, and, in some cases, their ability to care for themselves or use their limb effectively. Options for treatment have grown over the past decade. Steps include elevating the affected limb at heart level or above when possible. This lets gravity help drain the lymph system. Exercise, including aerobic exercise and a program of specific movements, helps increase the flow of the fluid. Sometimes a compression garment or wrap can help reduce the size of the limb, and manual massage and lymph node clearing techniques help force the fluid out of the limb. Patients also must reduce their salt intake because salt causes the body to retain fluid. Patients with lymphedema are referred to the Occupational and Physical Therapy Center at Marshfield Clinic-Marshfield Center. Vivian Gamble, like Will, an occupational therapist with certification and additional training in comprehensive lymphedema management, teaches patients how to better manage lymphedema themselves. They give each patient a thorough evaluation prior to beginning therapy. Each therapy session includes clearing the lymph fluid from unaffected lymph vessels near the affected limb. The therapist performs a manual lymphatic therapy a specific massage technique to that drains and moves the lymphatic fluid. It is useful to reroute lymph fluid around damaged or blocked areas. "It's a very specific massage," Will said. "We don't use a lot of pressure because the part of the lymph system we work with is very close to the skin's surface and deep pressure would only block the flow of fluid." After manual lymphatic therapy, a multi-layered low or non-elastic compression wrap is applied to the limb. This is not the same as an ace wrap, which is highly elastic. "The purpose of this wrap is to provide firm external support to the tissue and gives the muscles something to push against, thus promoting lymph pumping even during the slightest muscle motion," Will explained. When no further girth decrease is obtained, a compression garment is ordered. Other components of a therapy program may include exercise (with compression wrap or bandage on), use of a pump to remove lymph fluid from the limb, frequent reassessment of girth measurements, and coordination with Marshfield Clinic's Orthotics and Prosthetics Department to get each patient the proper compression garment. Therapy requires a strong commitment from the patient. "They may come to therapy for an hour or more daily for a few weeks," Gamble said. The frequency of therapy decreases as improvements are made. Self-management techniques such as self-massage, compression wrapping and exercise also are taught. The othotist fits a compression garment once the therapy goals are reached. The garment does not reduce lymphedema, but it does help to maintain the size reduction already attained. For more information on lymphedema or to schedule an appointment, call the Department of Physical and Occupational Therapy at 1-800-782-8581, ext. 7-5529 or 715-387-5529. http://www2. marshfieldclinic.org/CATTAILS/02/JANFEB/LYMPHEDEMA.ASP --- Lymphedema is the buildup of lymph (a fluid that helps fight infection and disease) in the fatty tissues just under the skin. The buildup of lymph causes swelling in specific areas of the body, usually an arm or leg, with an abnormally large amount of tissue proteins, chronic inflammation, and thickening and scarring of tissue under the skin. Lymphedema is a common complication of cancer and cancer treatment and can result in long-term physical, psychological, and social issues for patients. The lymphatic system consists of a network of specialized lymphatic vessels and various tissues and organs throughout the body that contain lymphocytes (white blood cells) and other cells that help the body fight infection and disease. The lymphatic vessels are similar to veins but have thinner walls. Some of these vessels are very close to the skin surface and can be found near veins; others are just under the skin and in the deeper fatty tissues near the muscles and can be found near arteries. Muscles and valves within the walls of the lymphatic vessels near the skin surface help pick up fluid and proteins from tissues throughout the body and move the lymph in one direction, toward the heart. Lymph is slowly moved through larger and larger lymphatic vessels and passes through small bean-shaped structures called lymph nodes. Lymph nodes filter substances that can be harmful to the body and contain lymphocytes and other cells that activate the immune system to fight disease. Eventually, lymph flows into one of two large ducts in the neck region. The right lymphatic duct collects lymph from the right arm and the right side of the head and chest, emptying it into the large vein under the right collarbone. The left lymphatic duct or thoracic duct collects lymph from both legs, the left arm, and the left side of the head and chest, emptying it into the large vein under the left collarbone. The lymphatic system collects excess fluid and proteins from the body tissues and carries them back to the bloodstream. Proteins and substances too big to move through the walls of veins can be picked up by the lymphatic vessels because they have thinner walls. Edema may occur when there is an increase in the amount of fluid, proteins, and other substances in the body tissues because of problems in the blood capillaries and veins or a blockage in the lymphatic system. Lymphedema may be either primary or secondary. Primary lymphedema is a rare inherited condition in which lymph nodes and lymph vessels are absent or abnormal. Secondary lymphedema can be caused by a blockage or cut in the lymphatic system, usually the lymph nodes in the groin area and the armpit. Blockages may be caused by infection, cancer, or scar tissue from radiation therapy or surgical removal of lymph nodes. This summary discusses secondary lymphedema. Acute versus gradual-onset lymphedema There are four types of acute lymphedema. The first type of acute lymphedema is mild and lasts only a short time, occurring a few days after surgery to remove the lymph nodes or after injury to the lymphatic vessels or veins just under the collarbone. The affected limb may be warm and slightly red, but is usually not painful and gets better within a week by keeping the affected arm or leg supported in a raised position and by contracting the muscles in the affected limb (for example, making a fist and releasing it). The second type of acute lymphedema occurs 6 to 8 weeks after surgery or during a course of radiation therapy. This type may be caused by inflammation of either lymphatic vessels or veins. The affected limb is tender, warm or hot, and red and is treated by keeping the limb supported in a raised position and taking anti-inflammatory drugs. The third type of acute lymphedema occurs after an insect bite, minor injury, or burn that causes an infection of the skin and the lymphatic vessels near the skin surface. It may occur on an arm or leg that is chronically swollen. The affected area is red, very tender, and hot and is treated by supporting the affected arm or leg in a raised position and taking antibiotics. A compression pump should not be used and the affected area should not be wrapped with elastic bandages during the early stages of infection. Mild redness may continue after the infection. The fourth and most common type of acute lymphedema develops very slowly and may become noticeable 18 to 24 months after surgery or not until many years after cancer treatment. The patient may experience discomfort of the skin; aching in the neck, shoulders, spine, or hips caused by stretching of the soft tissues or overuse of muscles; or posture changes caused by increased weight of the arm or leg. Temporary versus chronic lymphedema Temporary lymphedema is a condition that lasts less than 6 months. The skin indents when pressed and stays indented, but there is no hardening of the skin. A patient may be more likely to develop lymphedema if he or she has one of the following: Surgical drains that leak protein into the surgical site. Inflammation. Inability to move the limb(s). Temporary loss of lymphatic function. Blockage of a vein by a blood clot or inflammation. Chronic (long-term) lymphedema is the most difficult of all types of edema to treat. The damaged lymphatic system of the affected area is not able to keep up with the increased need for fluid drainage from the body tissues. This may be caused by one of the following: Recurrence or spread of a tumor to the lymph nodes. Infection of and/or injury to the lymphatic vessels. Periods of not being able to move the limbs. Radiation therapy or surgery. Inability to control early signs of lymphedema. Blockage of a vein by a blood clot. A patient who is in the early stages of developing lymphedema will have swelling that indents with pressure and stays indented but remains soft. The swelling may easily improve by supporting the arm or leg in a raised position, gently exercising, and wearing elastic support garments. Continued problems with the lymphatic system cause the lymphatic vessels to expand, allowing lymph to flow back into the body tissues and make the condition worse. Pain, heat, redness, and swelling result as the body tries to get rid of the extra fluid. The skin becomes hard and stiff and no longer improves with raised support of the arm or leg, gentle exercise, or elastic support garments. Patients with chronic lymphedema are at increased risk of infection. No effective treatment is yet available for patients who have advanced chronic lymphedema. Once the body tissues have been repeatedly stretched, lymphedema may recur more easily. Risk factors Factors that can lead to the development of lymphedema include radiation therapy to an area where the lymph nodes were surgically removed, problems after surgery that cause inflammation of the arm or leg, a larger number of lymph nodes removed in surgery, and being older. Risk factors for lymphedema include the following: Breast cancer, if the patient received radiation therapy or had lymph nodes removed. Radiation therapy to the underarm area after surgical removal of the lymph nodes and having a larger number of lymph nodes removed increases the risk of lymphedema. Surgical removal of lymph nodes in the underarm, groin, or pelvic regions. Radiation therapy to the underarm, groin, pelvic, or neck regions. Scar tissue in the lymphatic ducts or veins and under the collarbones, caused by surgery or radiation therapy. Cancer that has spread to the lymph nodes in the neck, chest, underarm, pelvis, or abdomen. Tumors growing in the pelvis or abdomen that involve or put pressure on the lymphatic vessels and/or the large lymphatic duct in the chest and block lymph drainage. Having an inadequate diet or being overweight. These conditions may delay recovery and increase the risk for lymphedema. Diagnosis Specific criteria for diagnosing lymphedema do not yet exist. About half of patients with mild edema describe their affected arm or leg as feeling heavier or fuller than usual. To evaluate a patient for lymphedema, a medical history and physical examination of the patient should be completed. The medical history should include any past surgeries, problems after surgery, and the time between surgery and the onset of symptoms of edema. Any changes in the edema should be determined, as should any history of injury or infection. Knowing which medications a patient is taking is also important for diagnosis OVERVIEW FROM HEALTHFINDER.GOV ---------------------------------- Contrast Media Mol Imaging. 2006 Nov;1(6):230-45. Links Imaging of the lymphatic system: new horizons. Barrett T, Choyke PL, Kobayashi H. Molecular Imaging Program, National Cancer Institute, Building 10, Room 1B40, Bethesda, MD 20892- 1088, USA. The lymphatic system is a complex network of lymph vessels, lymphatic organs and lymph nodes. Traditionally, imaging of the lymphatic system has been based on conventional imaging methods like computed tomography (CT) and magnetic resonance imaging (MRI), whereby enlargement of lymph nodes is considered the primary diagnostic criterion for disease. This is particularly true in oncology, where nodal enlargement can be indicative of nodal metastases or lymphoma. CT and MRI on their own are, however, anatomical imaging methods. Newer imaging methods such as positron emission tomography (PET), dynamic contrast-enhanced MRI (DCE-MRI) and color Doppler ultrasound (CDUS) provide a functional assessment of node status. None of these techniques is capable of detecting flow within the lymphatics and, thus, several intra-lymphatic imaging methods have been developed. Direct lymphangiography is an all-but- extinct method of visualizing the lymphatic drainage from an extremity using oil-based iodine contrast agents. More recently, interstitially injected intra-lymphatic imaging, such as lymphoscintigraphy, has been used for lymphedema assessment and sentinel node detection. Nevertheless, radionuclide-based imaging has the disadvantage of poor resolution. This has lead to the development of novel systemic and interstitial imaging techniques which are minimally invasive and have the potential to provide both structural and functional information; this is a particular advantage for cancer imaging, where anatomical depiction alone often provides insufficient information. At present the respective role each modality plays remains to be determined. Indeed, multi-modal imaging may be more appropriate for certain lymphatic disorders. The field of lymphatic imaging is ever evolving, and technological advances, combined with the development of new contrast agents, continue to improve diagnostic accuracy. PMID: 17191764 [PubMed - in process] --------------------------------- Diagnosing Lymphedema Introduction Approximately 90% of all lymphedema cases are diagnosed on the basis of a medical history and current symptoms. The remaining 10% of lymphedema cases require more complex diagnostic measures. [1] The Family History Primary lymphedema is considered when the patient has a family history of this condition. However even without a family history, primary lymphedema cannot be ruled out when there is swelling of unknown cause present in the legs. The Medical History Secondary lymphedema is considered when the patient’s medical history includes any events that place the patient at risk for lymphedema. See Are You at Risk for Lymphedema? Because of the delayed onset of secondary lymphedema, these risk factors are not necessarily of recent origin. See Delayed Onset of Lymphedema. Swelling due to lymphedema The Physical Examination Swelling is the most obvious indication of lymphedema; however, not all swelling is due to lymphedema! Pitting edema is a diagnostic sign for stage 1 lymphedema. The test for pitting edema is to gently press a finger against the swollen tissue. Pitting edema is present if this leaves an indentation that soon disappears. (Pitting edema can also be a diagnostic sign of other conditions.) In the later stages of lymphedema, when the tissues have become fibrotic and are no longer soft, pitting edema is no longer present as a diagnostic sign. Testing for Stemmer's sign. Stemmer’s sign is a thickened skin fold at the base of the second toe or second finger. The presence of this sign is an early diagnostic indication of primary lymphedema. It develops later in secondary lymphedema. The absence of Stemmer’s sign does not rule out the possibility of lymphedema. To test for Stemmer’s sign, pinch the skin folds on the upper surface of the second toe or finger as shown here. When this skin cannot be lifted, this is considered to be a positive test result that could indicate the presence of lymphedema; however, a negative Stemmer's sign does not exclude lymphedema. [2] Rule Outs Lymphedema is not the only condition that causes abnormal swelling of the tissues and it is important that the healthcare provider rule out these other conditions before reaching a diagnosis of lymphedema. A rule-out is the process of eliminating conditions that could possibly be causing the presenting symptoms. Sudden swelling could be a sign of a blood clot. This is potentially serious and requires immediate attention. Slowly progressive swelling, particularly of the legs, can be due to other conditions, such as lipedema or a heart condition, that impair the circulation.[3] See Chronic Venous Insufficiency and Lymphedema, Lipedema, Lymphedema, and Lipo-Lymphedema, Diabetes and Lymphedema and Obesity and Lymphedema. Rapidly progressive and painful lymphedema-type swelling could be due to a fast-growing tumor that places pressure on lymph nodes and the nerves. This condition is known as malignant lymphedema; however it is the tumor, not the lymphedema, that is malignant.[4] Imaging Lymphoscintigraphy produces an image of the lymph flow and speed of uptake. When necessary, computerized tomography (CT), magnetic resonance imaging (MRI), and ultrasound techniques can be used to image tissues and structures that cannot be seen effectively with lymphoscintigraphy.[5] See Lymphoscintigraphy. Who Diagnoses Lymphedema? Many times your primary caregiver makes the diagnosis of lymphedema. When the condition is related to cancer treatment, your oncologist may make the diagnosis. For more complex cases, you may want to consult a vascular surgeon. Help is available in finding a vascular surgeon by visiting the Society for Vascular Surgery web site and using their physician locator service. References [1] Management of Childhood and Adolescent Lymphedema by J.F. Feldman. Lymph Link Vol 16:2, April- June 2004 p 1-26. [2] A Primer on Lymphedema by D. G Kelly. Prentice Hall, 2002, page 48. [3] Lymphedema National Cancer Institute (NCI) Comprehensive Cancer Information Database. October 2003. [4] Living Well with Lymphedema by A. Ehrlich, A. Vinjé-Harrewijn PT, CLT, and E. McMahon PhD. Lymph Notes 2005, pages 19-26 and 85-96. [5] The Third Circulation: Radionuclide Lymphoscintigraphy in the Evaluation of Lymphedema by A Szuba et al. JNM, Volume 44, Number 1, January 2003. [6] MediFocus Guide: Lymphedema. Medifocus.com, Inc, 2004. [7] The Lymphatic System Pathology by B. Lasinski in "Implications for the Physical Therapists 2-Ed" by C. C Goodman, W.G. Boissonnault, and K.S.Fuller. Saunders 2003, pages 427-508. [8] The Diagnosis and Treatment of Peripheral Lymphedema: Consensus Document of the International Society of Lymphology. Lymphology 36 (2003) 84-91. This information does not replace the advice of a qualified health care professional. http://www.lymphnotes.com/article.php/id/208/ ----------------------------------------------- Lymphedema Test To confirm a diagnosis of lymphedema, your physician may order tests, including one or more of the following: • Lymphoscintigraphy, which uses a low-dose injected radioactive substance to trace the flow of lymph fluid through your lymphatic vessels • Magnetic resonance imaging (MRI), which uses radiowaves and magnetic fields to detect patterns in your internal tissues that are characteristic of lymphedema • Computed tomography (CT) scanning, which creates images of your internal tissues from a series of cross-sectional x-ray • Duplex ultrasound, which uses high-frequency sound waves and Doppler technology to show vessels and real-time blood flow on a screen to rule out a blood clot in your leg • Lymphangiography, which uses contrast (dye) directly injected into the lymphatic vessels, is now used less frequently Source: http://www.vascularweb.org/_CONTRIBUTION_PAGES/Patient_Information/Nor thPoint/Lymphedema.html What is Lymphoscintigraphy? Lymphoscintigraphy provides a view of the workings of the lymphatic system, which is a network of small channels, like arteries and veins, that transport the fluid and cells of the immune system through the lymph nodes and throughout the body. This fluid, called lymph, normally flows slowly from the periphery toward the center of the body and into the general circulation. If lymphatic flow is blocked, the areas of drainage that are affected can become swollen. A scintigram is a type of picture that uses a radiopharmaceutical (a radioactive drug), which is injected or taken orally, that makes the lymphatic system visible to specialized cameras. The study is performed in the Nuclear Medicine section of the hospital, where the radiopharmaceuticals are prepared and the pictures are taken. Lymphoscintigraphy can be helpful for localizing points of blockage and is also important for identifying abnormal lymph nodes and planning a biopsy or surgery for suspicious areas. Generally, the radiation dose is similar to that of a standard x-ray examination. What are some common uses of the procedure? Lymphoscintigraphy can assist the physician in diagnosing diseases. It can help detect tumors, infection and other disorders such as the following: Lymphoscintigraphy can help diagnose lymphedema, a condition in which lymphatic fluid accumulates in soft tissues and may lead to inflammation and obstruction. This nuclear medicine test has all but replaced lymphangiography, a diagnostic x-ray procedure that used an oil-based contrast material that required surgical incisions on both feet to expose and inject the lymphatics directly. Lymph flow in an arm or leg may be evaluated with lymphoscintigraphy by injecting radioactive material into a web space between the fingers or toes and recording images for 60 minutes. Local anesthesia is not necessary. When planning surgery for a breast tumor, it is helpful to assess the lymphatic drainage beforehand to identify the sentinel lymph node (the first lymph node that receives lymph drainage from the tumor site) for excisional biopsy. A radiopharmaceutical is injected either just beneath the skin around the areola (nipple); at two to four sites around the tumor; beneath the skin above the tumor; or into the tumor itself on the day of surgery. Imaging usually is completed within 30 minutes, but may take up to one to two hours. Lymphoscintigraphy of the breast is very safe. Side effects are infrequent, and morbidity is much reduced compared with axillary lymph node dissection, which formerly was the routine staging procedure for patients with breast cancer and no obvious spread (metastasis). Malignant melanoma is an aggressive form of skin cancer that may spread rapidly to distant body sites. Lymphoscintigraphy may be performed preoperatively in order to identify the sentinel lymph node. A tumor-negative sentinel lymph node is strong evidence that there has not been spread of the tumor. This is important for staging the disease and planning treatment management. How should I prepare for the procedure? No special preparation is needed for lymphoscintigraphy. What does the equipment look like? During lymphoscintigraphy you will lie down on a scanning table. Consequently, the only piece of equipment you may notice is the specialized nuclear imaging camera used during the procedure. It is a large, round device enclosed in a metallic housing and suspended over the examination table. The camera sometimes is located within a large, doughnut-shaped structure similar in appearance to a computed tomography (CT) scanner. A nearby computer console, often in an adjacent room, processes the data from the procedure. How does the procedure work? With regular x-ray examinations, an image of the body is made by passing x-rays through the body part from an outside x-ray source. In contrast, with nuclear medicine a radioactive substance called a radiopharmaceutical or radiotracer localizes in a certain body part (or parts) which emits gamma rays that are detected by a gamma camera. The gamma camera sends the information to a computer that develops the image. Scanning times may vary considerably. The most commonly used radiopharmaceutical in the United States for lymphoscintigraphy is called technetium-99m sulfur colloid. It typically loses its radioactivity in less than 24 hours. How is the procedure performed? You will be asked to lie face up beneath or next to a gamma camera. When assessing lymph drainage to identify the sentinel lymph node, as in patients with melanoma, three to five injections of radiopharmaceutical are given into the skin surrounding the site of the melanoma. For breast cancer, the injections are given through the skin near the tumor or around the areola. All injections are made using a very small needle. In lymphoscintigraphy performed for leg or arm edema, the radiopharmaceutical is injected into the skin between the first and second fingers or toes of each hand or foot. Imaging begins immediately after injection and is repeated at five- minute intervals for 45 to 60 minutes. Often, both sides are studied so that the normal and abnormal sides can be compared. You may also be asked to exercise lightly for about 10 minutes, either by walking for leg exams or by doing handgrip or lifting exercises for arm exams. Images are acquired after exercise and, in some cases, delayed images are needed at one- to two-hour intervals for up to six hours, or even up to 24 hours. For patients with breast cancer, images will be made of the underarm regions and breast/chest. For melanoma patients, images will be made of the underarms, head, neck and both groins. In most melanoma patients, imaging will take three to four hours. Markings are made on the skin to show where lymph nodes are located. What will I experience during the procedure? Lymphoscintigraphy is an outpatient procedure. No anesthesia is needed unless a lymph node biopsy is performed in the operating room immediately following lymphoscintigraphy in order to detect involvement by melanoma or breast cancer. It is important that you avoid moving while the images are recorded. Most patients can resume regular activities immediately after the procedure. The small amount of radioactivity in your body will decrease due to the natural process of radioactive decay over several hours. Who interprets the results and how do I get them? Most patients undergo lymphoscintigraphy because their primary care physician or surgeon has recommended it. A radiologist, a physician who has specialized training in nuclear medicine and other medical imaging procedures, will interpret the images and forward a report to your physician. What are the benefits vs. risks? Benefits The functional information provided by nuclear medicine examinations such as lymphoscintigraphy is unique and currently unattainable by using other imaging procedures. For many diseases, nuclear medicine studies yield the most useful information needed to make a diagnosis and to determine appropriate treatment, if any. Sentinel lymph node biopsy following lymphoscintigraphy is a useful means of diagnosing and staging melanoma and learning whether the disease has spread from its primary site. Lymphoscintigraphy and sentinel lymph node biopsy are less traumatic than the alternatives of staging breast cancer or melanoma by surgical lymph node dissections. Computers are involved in the generation of images, making it possible to measure function and quantify it in addition to identifying abnormalities. Because lymphoscintigraphy is generally performed according to standardized protocols, the type of examination done at one hospital is likely to be similar to that performed at other hospitals, making the information easy to understand and easy to transfer to all doctors who may be involved in your care. Risks Because the doses of radiopharmaceutical administered are small, nuclear medicine procedures such as lymphoscintigraphy result in minimal radiation exposure. Nuclear medicine has been used for more than five decades, and there are no known long-term adverse effects from such low-dose exposure. As with all radiological procedures, it is important that you inform your physician and the radiological technologist if you are pregnant. In general, exposure to radiation during pregnancy should be kept to a minimum. Allergic reactions to radiopharmaceuticals may occur but are extremely rare. Injection of the radiopharmaceutical may cause slight pain and redness. What are the limitations of Lymphoscintigraphy? Nuclear medicine procedures such as lymphoscintigraphy are time- consuming. They involve administration of a radiopharmaceutical, acquisition of images, and interpretation of the results. Imaging can take up to an hour and sometimes longer to perform. Source: http://www.radiologyinfo.org/en/info.cfm?pg=lympho&bhcp=1 ------------------------------------------ Breast and Chest Lymphedema: What It Is, and How to Help next ... File Format: Shockwave Flash Anecdotal experiences with post-surgical and reconstruction breast cancer patients also refute this assumption. Chest and Breast Lymphedema May Be Serious www.bellisse.com/swf/presentation.swf ------------------------------------------ There are some options for genital edema, though none are easy. For females, you can get a long line panty shaper (which was formerly called a girdle) and then sew another crotch within the crotch to form a pocket. In this you can place either foam, or a heavy kotex (maternity) which will provide pressure against the labia. Not terribly comfortable, but can be effective. There is also genital pads from Solaris (male and female) which are then held in place by a long leg panty or underarmor for men. The male genitalia can be bandaged and then supported with a scrotal support. The scrotum and penis will reduce, it is more of a challenge for female genital edema. Hope this helps Renee Romero RN, CLT-LANA of Bandages Plus ---------------------------------- I can offer a suggestion that might help. Use an old full (like granny panty) that you don't need anymore, but still elastic, next you pad the genital area with sponge like the sponge padding the therapist use under your bandages during treatment. Ridged sponge even better, or cut uneven sponge chips and sew them on the crotch area of the panty. Then you wear them over your own panty plus a bike shorts for the increased compression gradient. Your massage suggestions are also helpful. Best Regards, Veronica Yap Vodder Lymphoedema Therapist. -------------------------------------------- The Dutch explorer Jan Huygen Linschoten recorded that the descendants of those that killed St. Thomas were "all born with one of their legs and one foot from the knee downwards as thick as an elephants leg" (32). Thereafter, there are numerous references to elephantiasis, especially in Africa but also in Asia, including China, where Manson was later to discover the life cycle of the parasite. Another pathological condition associated with lymphatic filariasis is chyluria, in which the urine appears milky. This condition was recorded by William Prout in his 1849 book On the Nature and Treatment of Stomach and Renal Diseases Handley WS: “Lymphangioplasty: New Method for Relief of Brawny Arm of Breast-Cancer and for Similar Conditions of Lymphatic Oedema” Lancet Mar 1908;1:783-5. Indian works on traditional medicine described elephantiasis with lymphorrhea around 1500 B.C. Ancient Egypt: the Ebers Papyrus circa 1550 B.C., refered to infectious elephantiasis (filariasis) Celsus was probably the first author to use the term elephantiasis (30 B.C. - 50 A.D.) Dropsy andelephantiasis were described by Paul of Aegina and Artetaus of Cappadocia (De Elephante Morbo, 50 A.D.) Galen (138-201 A.D.) included edema in his list of medical diagnoses. Prosper Alpino, physician of the Venetian ambassador to Egypt, described Elephantiasis arabicum around 1541 The association of edema with the lymphatic system was not made until the lymphatics were clearly described. hewson 1744 and Cruikshank 1789 established these connections for the first time. Lymphedema as a complication fo breast cancer was described by horne 1805 and Druitt 1850. Halstead 1921 who pioneered radical mastectormy surgery related lymphedema to axillary node dissection. Milroy provided the first scientific description of congenital lymphedema 1892. History of Milestones and scientific breakthroughs in lymphatic research 1998-2008 1998 Mapping of the gene for Nonne-Milroy disease chromosome 5 Nitric oxide found to inhibit contraction frequency of the lymph pump Rose of nasallymphaticsin the regulation of CSF volume and pressure identified Nitric oxide and prostaglandins found to regulate vasomotor activity of lymphatic vessels 1999 generation of the first animal model devoid of a lymphatic vasculature Identification of Prox-1 as a lymphatic endothelial cell marker LYVE-1 identified as lymphatic marker Mapping of the gene for hereditary lymphedema distichiasis to chromosome 16 Lymphatic found to generate negative pressures to promote lymph formations Identification of Podoplanin as a lymphatic-specific cell marker 2000 Mutations in the VEGFR-3 gene found to be responisble for Nonne-Milroy primary hereditary lymphedema Mutations in the FOXC2 gene found to cause hereditary lymphedema distichiasis or LD syndrome Mapping of gene for cholestasis-lymphedema syndrome (Aagenaes syndrome) to chromosome 15 Calcium activated chloride currents found to play critical role in electrical activity driving spontaneous contractions of the lymph pump. role of lymphatic endothelium in trafficking of T-lymphocytes during inflammation is defined. 2001 VEGF-C found to promote lymphatic growth in the skin of mice with lymphedema. Functional evidence of primary valve system noted in lymphatic endothelium Indirection lymphangiography using water soluble MR contrast agents; this is further refined in 2006. Isolation and charactization of dermal lymphatic and blood endothelial cells found to reveal stable and functionally specialized cell lineages. 2002 Prox-1 expression found to induce the specifications of lymphatic phenotype Specialized cells within ovine lymphatics found to display vimentin and c-Kit, characteristic of electrical pacemakers Shear force generated by lymph flow found to inhibit spontaneous contractions of lymph umpm via endothelial/nitric oxide-dependent mechanism Successful threatment of experimental lymphedema with VEGF-C. 2003 Blod and lympahtic vascular separation during embryonic development found to be mediated by signatling proteins SLP-76 and sky Identifiction of mutations in SOX-18 gene in hereditary hypotrichosis-lymphedema-telangiectasia Interstital fluid channeling found to precede and direct lymphangiogenesis Lymphatic muscle found to inhibit genotypic and phenotypic characteristics of smooth and striated muscle. 2004 Vascular endothelial growth factor C found necessary for sprouting of the first lymphatic vessels from embryonic veins Defective valves and abnormal mural cell recruitment found to underlie lymphatic vascular failure in lymphedema distichiasis Lymphatic pumps from different regions of the rat exhibi variable strengths and stretch shear sensitivities set by local factors Importance androle of th release of intracellular calcium stores in lymph pump pacemakers demonstrated. 2005 Defective lymphatic vasculature found to promote late onset obesity Measurement of lymph flow and lymphocyte flux in animal models Nasal lymphatic identified as critical to regulation of cerebrospinal fluid volume and pressure in primates A genetic Xenopus laevis tadpole model developed to study lymphangiogenesis Isolation and characterization of a novel mouse lymphatic endothelial cell line: SV-LEC. 2006 Identificationof a functional lymphatic vasculature in zebrafish using in vivo live imagining Demonstartion of dimished lymphatic contractile function in model of ileitis Demonstartion that pump generated flow reduces tone to maintain pump efficiency Characterization of lymphangiogenesis in a model of sadult skin regneration Genome-wide transcriptional profiling of the skin in acquired lymphedema Transfection of human hepatocyte growth fctor gene ameliorates secondary lymphedema. 2007 Definitive identification of the origin of mammalian lymphatic vasculature Againg found to produce diminished lymph pumping. Non-invasive procedure for documentation of impairment of the lymph pump in patients with breast cancer related lymphedema Functionally specialized junctions found between endothelial cells of lymphatic vessels. 2008 Spatial arrangements and organization of lymphatics found to determine their role in lymph formation or downstream lymph flow. Impaired lymph drainage of cerebrospinal fluid found to be responsible for severity of disease in animal model of hydrocephalus Near-infrared fluorescence imaging used to demonatrate propulsive lymph flow in normal mice and in mouse models of lymphatic dysfunction. Blocking VEGFR-3 found to suppress angiogenic sprouting and vascular network formation Reversal of lymphedema by insterstitial flow demonstrated in model. Heterogeneity in immunohistochemical, genomic, and biological properties of human lymphatic endothelial cells between initial and collecting lymph vessels. ----------------------------------- Tips from Lymphologists: a. Keep your lymphodematous limb spotlessly clean. A Daily bath is recommended; do use soap but be sure to wash off all soapy residue. WIPE DRY. When drying, be gentle but thorough (a hairdryer may help for difficult areas). Make sure your underclothes and compression garments are regularly washed (in accordance with the manufacturer's instructions). Dust an anti fungal powder between the webs, skin folds and other areas where sweat may accumulate. b. Avoid any trauma (knocks, cuts, sunburn, insect bites). Be careful cutting nails; do not cut the cuticle or push back too hard (this can injure it and allow bacteria to enter, thus leading to infection); ease back gently with a cotton wool covered orange stick. If you are sewing, wear a thimble; if gardening, wear a glove and long sleeves; if bush walking etc. wear boots and protective clothing. Never cut calluses (e.g. heel or sole). Use 10 % urea or Salicylic acid cream may help. Attend to minor burns, cuts, stings, bruises, etc. immediately c. Keep the limb as cool as possible in hot weather. Be careful of the water temperature in showers and spas. (These can be useful in therapy and self massage). If traveling for a long time in a car, drape a white shirt over a limb in the sun. Move it if possible when you have to sit for a long time, flex and stretch fingers or feet. d. (For Arms) Do Not pick up heavy loads with an arm 'at risk', e.g. a case or heavy shopping. Do not carry a heavy bag or handbag on this arm. e. (For Legs) Do Not use heeled shoes. Do not walk barefoot. f. Any redness or other signs of infection like pain brawny hardness should be treated AT ONCE (keep antibiotics in your purse). All infections need antibiotics. Some patients may need these permanently (e.g. Monthly Penicillin injections). However, if warm red patches have often occurred but disappear in 3 days, antibiotics may not be needed unless their frequency or duration increase greatly. Watch out for tinea! (often only a slight redness and a peeling of the skin.) You will need to puff an anti fungal powder into shoes or gloves as well as on the inflamed area (replace rubber gloves often). Redness and/or peeling under a breast or in the groin can also be fungal. Some conditions need oral anti fungicides for many months. Generally weekly ketoconazole (Syscan 150) is best. g. Oral and topical benzo-pyrones, where available, help prevent and treat lymphoedema and infections by removing stagnant protein. h. It is very important to keep the skin supple and moist in winters. Best not to use creams in summer. i. You MUST NOT allow ANYONE to measure blood pressure, to take blood, nor to give an injection in a lymphodematous limb or one at risk. Another limb can be used, even a leg. j. To remove hair, a properly maintained electric razor (new heads, as needed) is better than either safety razors, depilatories, or abrasive mitts. Do not use these. k. If traveling by air it is a good precaution to wear a compression garment (and glove, for arms) or an inflatable splint may be used. If lymphoedema is present, additional pressure bandages may be needed on a long flight. Bandages may be used on fingers, hand or toes if necessary. l. There should be no redness or indentation when you remove clothing, otherwise it is stopping some of the lymphatic drainage that you do have left! Arms: Brassieres should not be too tight around the ribs or over the shoulders. A good supporting one (not too tight!) helps prevent oedema forming in your other breast. A wider shoulder strap is recommended and, if needed, some foam padding under this and the chest band. Tight jewelry also will constrict, and should not be worn. Legs: Clothes (including underpants/panties) should be loose around both waist and thighs. Do not wear a tight belt - use braces to keep your trousers up. m. Try not to lie on an arm at risk when sleeping or resting. n. A normal balanced diet is best (with plenty of vitamins!). Lymphoedema is a high protein oedema, but eating too little protein will not help. Rather it weakens the connective tissue, thus making an edema worse. Dieting will not reduce lymphoedema, but is advisable if a patient is overweight. It is not necessary for patients to have a "low salt" diet or diuretics (unless needed for another disease). Lymphoedema may be temporarily reduced, but not permanently. Patients have enough troubles and do not need pointless diets in addition. Again, some patients are advised not to smoke or drink alcohol, "because these will worsen the lymphoedema". This is completely wrong! Nicotine in fact makes the lymphatic pump better (so does coffee). While too much alcohol causes obesity and it mildly increases the lymphatic load, there is no evidence that it worsens lymphoedema (unless drunk to excess so that the limb is neglected). There may be other reasons to avoid these, but worsening lymphoedema is not one of them. o. If you are under severe psychological stress, concentrate on relaxing the shoulders, back and neck. Extend your spine and 'stand straight'. Constant tension in these areas will worsen lymphoedema or may precipitate it if you are at risk. Tension in the upper body may cause a "balance" tension in the lower limbs, so a leg 'at risk' may also develop lymphoma. If lymphoedema is already present, the above points must be observed, plus: p. Your way of life may need to be modified; but you should not become a 'victim' to the disease. You just need to be aware of the facts about your condition and to be sensible. Do not give up sports or hobbies that you love. Low impact sports should be encouraged, as long as the limb is monitored. After surgery and/or radiotherapy it is perhaps unwise to take up a new sport when you muscles and coordination will be tested and perhaps strained (and therefore add to the lymphatic load) until some expertise is gained. Cycling, aerobics, weight lifting etc. may strain the limbs; swimming, SCUBA diving etc. are helpful. q. It is very well known that patients who are intelligent about their lymphatic deficiencies can keep their limbs reduced and live normal, or almost normal, lives; those who forget details and are careless almost invariably have problems. r. Wear the compression garment at all times including at night. Bandages are often more comfortable at night but only if a patient (or a caretaker) can bandage properly and without causing damage. If a limb reduces at night by itself, you may be able to leave the compression off at night (but not until 6 months after a course of treatment). s. If you are going out for a special occasion, you may be able to leave the compression garment off - just for this time. Try at home, several days in advance, and measure before and after about the same period. If the circumference increases, or if the limb aches, then do not risk it. However do NOT do this until some months have passed after your last Course of treatment, the tissues will not have strengthened very much before then. They can readily open up again! If a garment does not cover your fingers and hand, and they swell, see a therapist at once. Coumarin powder can be safely used under the compression garment to assist with the reduction of lymphoma, and helps you to put the garment on. Do not use the ointment under a garment; use it under bandages, especially over fibrotic areas. t. Measure (or get someone to measure) your limb once a month at least. If there is any real change (up or down) see a therapist. You may need help or a new compression garment. u. Change the compression garment according to the recommendations of your therapist, or if it feels at all loose, or too easy to put on. (Looseness means that either the limb is smaller or the garment is old - either way it will not do its job!). The compression garment must not leave a band at the wrist, elbow or under a limb. It should not chafe at any point; if is does, get help from the Therapist. Wash it regularly according to the manufacturer's instructions. v. DO exercise; but NOT too much (don't make the limb ache with tiredness). If you have a 'limb at risk' and do not need to wear a garment, consider doing so if you know that you are going to use it a lot that day. Wear a compression garment, or bandages, while you exercise. Exercises may be modified or added to according to your own requirements by your therapist. w. It is not easy to remember how to exercise correctly. x. Self massage is important. It consists of regular gentle pressure with rotary motion on the key drainage areas which are - Both groins, both axilla and just above the collar bone in the neck (especially the involved side). -------------------------------- CA Cancer J Clin 2009; 59:25-26 doi: 10.3322/caac.20007 © 2009 American Cancer Society -------------------------------------------------------------------------------- Patient Pages Lymphedema WHAT IS LYMPHEDEMA? Lymphedema is swelling of a body part caused by a build up of lymph fluid. It usually occurs in an arm or leg. Our bodies have a network of lymph vessels that carry lymph fluid to all parts of the body. These lymph vessels are connected to lymph nodes, which are small bean-sized collections of immune system cells. The lymph fluid and lymph nodes both contain white blood cells that help the body fight infections. If the lymph vessels are not working well, lymph fluid can build up in body tissues; this is called lymphedema. Many people with cancer have treatments that involve lymph nodes and lymph vessels. Surgery that removes lymph nodes or radiation treatment to areas that contain lymph nodes can increase the chance of having lymphedema. Having both surgery and radiation increases the chance even more. Having a few lymph nodes removed is less likely to cause lymphedema than having many nodes removed. Sometimes, the cancer can cause a blockage of the lymph system and result in lymphedema. Lymphedema is most often linked to treatments for breast, prostate, uterine, vulvar, or cervical cancers, sarcomas, and melanoma. If lymphedema occurs after breast cancer treatment, swelling can affect the arm on the side of the breast cancer. After treatment for cancer in the abdomen or pelvis, lymphedema may occur in the lower part of the body including the legs. WHAT ARE THE SIGNS AND SYMPTOMS OF LYMPHEDEMA? The signs and symptoms of lymphedema may include: a full or heavy feeling in the arm, leg, or genitals, a tight feeling in the skin of the arm, leg, or genitals, less movement or flexibility in your hand, wrist, or ankle, trouble fitting into clothing or jewelry in one specific area, such as one sleeve of your jacket being tight. Early on, lymphedema may be relieved by raising the affected limb. HOW CAN LYMPHEDEMA BE PREVENTED AND CONTROLLED? There is no cure for lymphedema, so we try to prevent it from starting. If it has started, then we try to improve the symptoms and prevent them from getting worse. Most experts suggest basic steps (listed below) to lower your chance of developing lymphedema or to help delay its start. Protect the Arm or Leg From Injury Protect the arm or leg nearest where the cancer was treated from any burns or injury. Your body responds to an injury by making extra fluid. When lymph nodes and vessels have been damaged or removed by cancer treatments, it is harder to get rid of this extra fluid. This can trigger lymphedema. Keep the arm or leg (on the side of the cancer treatment) clean. Keep your skin and cuticles soft and moist by regular use of a lotion or cream. Do not cut or clip cuticles. Use an electric shaver for removing underarm and leg hair instead of a blade razor or hair removal cream. Use an insect repellent and sunscreen to avoid bug bites and sunburn. Avoid extreme heat or cold. Use standard first aid measures to treat any cuts, scrapes, burns, insect bites, hangnails, or torn cuticles. Ask your doctor or nurse team if you are unsure about what to do. Watch for early signs of infection, such as pus, redness, swelling, increased heat, tenderness, chills, or fever. Call your doctor right away if you think you have an infection. If your arm is affected, try to avoid blood drawing, IVs, or shots on that side. Tell all health care workers that you are at risk for lymphedema. Wear protective gloves when doing household chores and yard work. If your legs are affected, always wear well-fitting, closed shoes instead of sandals or slippers. Wear soft protective socks. Do not go barefoot. Avoid Pressure or Squeezing the Leg or Arm Wearing tight clothing or squeezing the arm or leg can cause increased fluid and swelling. Lymphedema may get worse during air travel because of the changes in cabin pressure. If your arm is affected, do not wear tight jewelry or tight clothing. Avoid using shoulder straps when carrying briefcases and purses. Women should not wear bras that fit tightly or have straps that dig into their shoulder. Do not have your blood pressure taken on the affected side. If your legs are affected, avoid socks, stockings, or undergarments with tight elastic bands. Do not cross your legs for long periods of time when sitting. Ask your doctor or physical therapist about wearing a compression sleeve or compression stocking on the affected arm or leg when you travel by air. Try to move and flex the affected arm or leg often during the trip. Avoid Muscle Strain Being active helps lymph fluid to drain from the arms and legs, but overuse of the arm or leg may cause injury and lymphedema in some people. Exercise regularly but try not to overtire your arm or leg. Ask your doctor, nurse, or physical therapist about what level of activity is right for you and about wearing a compression sleeve or stocking during activities. If your arm or leg starts to ache, lie down and raise it above the level of your heart. Maintain Good General Health Some studies have found that being obese or having high blood pressure increases the chance that arm lymphedema will occur after breast cancer treatment. If you have lymphedema or are at risk for it, you should keep your weight and blood pressure under control. HOW IS LYMPHEDEMA TREATED? Lymphedema treatment helps reduce swelling, prevents it from getting worse, and decreases the chance of complications. The standard treatment is called Complete Decongestive Therapy (CDT). This includes skin care, a specific form of massage, special bandaging, exercises, and fitting for a compression sleeve or stocking. These treatments are prescribed by your doctor and are performed by an experienced therapist who has gone through special training. Most insurance companies will pay for this treatment, but some companies will not cover the cost of compression garments and bandages. If treatment is not started early, it can take longer to get lymphedema under control, and the long-term results may not be as good. So, you should have regular exams by your doctor or nurse to find swelling while it is still mild and more easily treated. If lymphedema does not respond to CDT, there are other treatment options, but we do not know how effective they may be for you. If you are thinking about these treatments, please see a lymphedema specialist who can help you decide what is best for you. OTHER SOURCES OF INFORMATION Information about lymphedema is available from several nonprofit lymphedema organizations. American Cancer Society. Available at: http://www.cancer.org Circle of Hope Lymphedema Foundation, Inc. Available at: http://www.lymphedemacircleofhope.org Lymphedema Research Foundation (LRF). Available at: http://www.lymphaticresearch.org Lymphology Association of North America (LANA). Available at: http://www.clt-lana.org National Cancer Institute (NCI). Available at: http://www.cancer.gov National Lymphedema Network (NLN). Available at: http://www.lymphnet.org http://caonline.amcancersoc.org/cgi/content/full/59/1/25 CA: A Cancer Journal for Clinicians > 2009 > Lymphedema: A Primer on the Identification and Management of a Chronic Condition in Oncologic Treatment CME Activity Information Important dates for this Course Accreditation/CME Credit CME Instructions For Credit Disclosure Privacy Statement Hardware/Software Requirements Released: January 1, 2009 Expires: January 1, 2011 LEARNING OBJECTIVES: Lymphedema: A Primer on the Identification and Management of a Chronic Condition in Oncologic Treatment After completing this activity, the learner should be able to: • Identify the physiology of the lymph system, the pathophysiology of lymphedema, and its causes. • Describe stages, severity, and characteristics of cancer treatment-related lymphedema. • Discuss the management of lymphedema. • Identify approaches to the prevention of lymphedema. The ACS designates this educational activity for a maximum of one AMA PRA Category 1 CreditTM. Physicians should only claim credit commensurate with the extent of their participation in the activity. This continuing nursing education activity was approved by the Georgia Nurses Association, an accredited approver by the American Nurses Credentialing Center's Commission on Accreditation. Contact hours = 1.0. This activity is approved for 1 AAFP Prescribed credit. Credit may be claimed for 2 years from the date of this issue. The physician should claim only those hours of credit that he or she actually spent in the educational activity. The online quiz must be completed by January 1, 2011. Each question has ONE best answer. http://cme.amcancersoc.org/cgi/cme/acscme_course;59/1/8 -------- When time doesn't heal all wounds see a doctor Samuel Kasberg, M.D. Approximately 5 million Americans suffer from chronic wounds that either will not heal, or heal very slowly. In general, a wound can be defined as a break in the skin, or epidermis. Chronic wounds can be painful, diminish one’s quality of life and affect body image. Wound care is a growing medical specialty dedicated to healing these skin breakages as part of disease management and surgical recovery. Many disease processes can lead to a non-healing state. While accidents, trauma and burns may be the initial event for a younger population, a significant number of chronic wounds present in the senior population. Bed-ridden people and debilitated health-care patients also are at risk. Seniors, or those aged 65 and older, are inclined to have more disorders, sometimes two or more, such as diabetes, congestive heart failure, lymphedema, peripheral arterial disease (PAD), venous hypertension or a compromised immune system. Combinations of these types of medical problems increase the risk of complications. Diabetes is of special concern when it comes to wound care. The Center for Disease Control and Prevention (CDC) reports statistics indicating 23.6 percent of the American population has diabetes, and of those, one-third will suffer from lower extremity wounds. Unfortunately, about 60 percent of lower extremity amputations in the United States are in diabetic patients — almost 86,000 per year. When coupled with those who suffer from circulatory problems, such as PAD, The United States has approximately 160,000 to 180,000 lower extremity amputations annually. For those of us who treat wounds, our first priority is to determine the cause of each patient’s inability to resolve the wound. This requires a diligent search to find if it is a circulation problem, and whether it is venous insufficiency or an arterial defect. One technique is a simple procedure, which requires taking a blood pressure reading in the patient’s leg and comparing it to the pressure in the arm. Another is careful examination. We may find a repetitive trauma as the causative factor, such as a poorly-fitting shoe or prosthetic device. Another important concern in skin breakdowns are bed or pressure sores, and these are addressed by relieving the attending pressure. When the cause or origin of the disease has been identified, treatment options are addressed. The removal of any dead tissue (debridement) is the first course of action. The amount of drainage needs to be controlled. It’s important to remember a moist environment is optimal for wound healing. Many people wish to leave the wound open, “so it can get some air,” this practice however, decreases the activities of the body’s own recuperative mechanisms, so much so that almost all healing activities will come to a halt. Different dressing choices can keep the ideal amount of moisture in the wound. Evaluation for evidence of infection is also important. Any area that is red is not necessarily infected. A determination also is made to see if the wound is truly infected or just colonized. Treatment is adjusted accordingly. Abscesses, i.e., an “abscess” or “boil,” (a collection of pus or white blood cells with bacteria) are commonly treated in our outpatient clinic. Wound care therapy is more than just picking out the right bandage. Wound debridement, drainage, moisture-controlled dressings, compression dressings along with hyperbaric oxygen therapy are some of the treatment options. Many hospitals have a wound care team or center, but are part of a much larger team. Close contact with a patient’s primary doctor or provider is essential to controlling blood sugars. While we check blood pressure in patient’s legs, the individual may need arterial treatment with an interventional radiologist, cardiologist, or vascular surgeon. Some procedures are coordinated with general surgeons, orthopedic surgeons, or plastic surgeons. We also work closely with podiatrists, and frequently seek input from rehab, physical therapy and dieticians. A person’s doctor will generally refer them to a wound care center when needed. A treatment plan is individualized for each patient and becomes part of a comprehensive medical plan, which includes their primary care physician. Wound care as a specialty is growing and evolving. Dr. Kasberg is the medical director of the first wound care and hyperbaric treatment center at San Angelo Community Medical Center. http://www.gosanangelo.com/news/2009/jun/29/when-time-doesnt-heal-all-wounds-see-a-doctor/ ========================================= Aetiological factors in chronic swelling of the lower leg Type Aetiology/underlying pathophysiology Primary lymphoedema - intrinsic abnormality in the lymphatic system which may include: - absence (aplasia) or reduction (hypoplasia) in lymphatic vessels - fibrosis of lymph nodes - large, dilated, incompetent lymphatics (hyperplasia) Secondary lymphoedema - obstruction, obliteration, insufficiency in the lymphatic system due to an extrinsic cause such as: - eg cervical, bladder, prostate cancer and malignant melanoma - advanced malignancy infiltrating the lymphatics - trauma to lymphatics eg injury, non-cancer surgery, burns - infection/inflammation eg lymphangitis, lymphangiothrombosis, chronic ulceration - filarial infection Non-specific/ non-lymphatic oedema - immobility, reduced function; chronic venous insufficiency dependent limb - tumour obstruction - hypoproteinaemia which can occur in maliganant ascites and renal disease - congestive cardiac failure - lipoedema -------------- Defining lymphoedema: clinical signs - tissue swelling of > 3 months duration that does not reduce completely on elevation - a positive Stemmer's sign (inability to pinch a fold of skin at the root of the second toe) Dry, flaky skin due to stretching of the tissues Hyperkeratosis scaly, thickened skin due to a build-up of keratin (horny scale) Fibrosis skin and subcutis become hard and non-pitting due to formation of fibrous tissue Papillomatosis a cobblestone appearance of the skin surface due to dilation of the upper dermal lymphatics, followed by fibrosis Lymphangio a Proliferation of lymph vessels on the skin surface producing a wart-like appearance which may rupture and leak lymph fluid Acute inflammatory episodes infection, similar to cellulitis and often referred to as erysipelas which affects the lymphatics, the skin and underlying tissues. Red streaks along the limb indicate lymphangitis Lymphorrhoea Leakage of lymph from the skin in lymphoedema Others Tinea pedis, contact dermatitis (irritant or allergic) are also common in these patients --------------------- Skin and preventative care in lymphoedema (adapted from Lymphoedema Support Network (undated) Swollen feet leaflet) - undertake daily skin washing and moisturising - avoid going barefoot - wear slippers or sandals at home - avoid tight socks - wear well-fitting shoes and keep a look out for corns or blisters - avoid injury to the skin of the limb eg scratch, insect bite, burn, sunburn - take care when cutting toe nails - use nail clippers - use an electric shaver to remove hairs - not a razor - look out for signs of fungal infection between the toes - contact your doctor at the first signs of an acute infection - avoid doing anything that exacerbates the swelling such as high impact sports or standing for long periods - wear hosiery garments for flying -------------------------- Acute inflammatory episodes in lymphoedema Look for: - red, hot, tender limb - sudden onset - 'flu-like symptoms - malaise and nausea - local oedema Managing the acute inflammatory episode - exclude deep venous thrombosis - rest - gentle elevation if possible - stop treatment - avoid compression, manual lymphatic drainage - increase fluid intake - wound swab if indicated - antibiotic therapy -------------------- SPECIFIC SUGGESTIONS FOR LYMPHEDEMA Keep weight as close as possible to your ideal weight. This is the MAJOR factor in controlling symptoms. A diet high in complex carbohydrates and plant fiber, but low in calories, sodium, fat, sugar, protein and cholesterol is recommended. High fiber legumes (vegetables and beans) should be a major component of your daily diet. These include beans, kale, broccoli, artichoke, cabbage, Brussels sprouts, cauliflower, squash, onion, potato, etc. Exercise to your limitations on a consistent basis. No matter what form that exercise takes, make sure it becomes a part of your regular routine. Eight glasses of pure water are essential to reduce weight and help relieve swelling. Pure water is either filtered tap water, spring water or distilled water. Increasing potassium will help reduce fluid retention as well as limiting sodium. Foods high in potassium include: bananas, oranges, tomatoes, dandelion, fresh fruit, wheat germ, lettuce, broccoli, potatoes, celery, nuts, dried apricots, cantaloupes, flounder, chicken, haddock, etc. Natural diuretics will help reduce fluid retention. They are: celery seed, parsley, corn silk, dandelion and/or dandelion extract. Celery seed is an inexpensive, but tasty addition to salads. Dandelion acts as a diuretic that stimulates the liver and kidneys and absorbs toxins. Burdock root relieves congestion of the lymphatic system. It also cleanses the blood by eliminating uric acid and excess waste materials. Digestive enzymes taken on an empty stomach will help dissolve the protein and fat accumulation in the lymph system. Digestive enzymes are called pancreatic enzymes because that is where our bodies manufacture them. They are classified according to their action. Proteases break down protein, amylases break down sugars/starches and lipases break down fat. Bromelain is a specific protein-digesting enzyme that is often used to combat inflammation. Vitamin C supplemented with the bioflavonoid quercitin and/or rutin will help to strengthen the lymph system and reduce inflammation. This combination can be taken throughout the day. Anti-oxidants help strengthen cells by destroying “free radicals” that damage cells. The major anti-oxidants are vitamin C, E, beta-carotene and selenium. Vitamin B6 is the catalyst for hundreds of reactions that metabolize food. B6 should be added to the diet on a daily basis spread throughout the day. Curcumin found in turmeric is a powerful anti-inflammatory agent. Turmeric is a spice used in Indian food. Improving circulation is very beneficial for Lymphedema. There are many ways to accomplish this. Exercise is the best method, but some dietary additions include ginger and red pepper. Garlic and onions have many beneficial properties and should be added to the diet in abundance. Very moderate intake of alcohol is important. Alcohol is an initial stimulant that later depresses the lymphatic system which leads to fluid retention. Caffeine is another substance like alcohol that depresses the lymphatic system. Avoid as much as possible. This includes the caffeine in soft drinks. http://www.acols.com/about_treat.html Treatment of Lymphedema with Complete Decongestive Physiotherapy Author: Joachim E. Zuther (also published in NLN Newsletter Vol II, #2 Summary: Lymphedema, caused by a low output failure, i.e. a reduced transport capacity (TC) of the lymphatic system, which in the case of lymphedema has fallen below the physiological level of the protein and water load, is a common and serious condition worldwide. Complete Decongestive Physiotherapy (CDP) is the treatment of choice, even in the most advanced stages of lymphostatic edema - provided that both a physician with broad experience in clinical lymphology and a specially trained therapist are available. A sufficient lymphatic system is able to return the physiological amount of protein and water load back to the venous system. The lymphatic protein load consists of plasma proteins continuously leaving the blood capillaries. The fraction of water ultrafiltrated in the area of the blood capillaries which is not reabsorbed, is called the lymphatic load of water. In the event of an increase of water and protein a healthy lymphatic system is, for some time, able to prevent the onset of edema by increasing its lymph time volume (LTV), i.e. lymph anions will increase their pulsation frequency and amplitude. This is called the lymphatic safety factor or safety valve function of the lymphatic system. The highest lymph time volume is known as the transport capacity of the lymphatic system which is approximately ten times higher than the lymph time volume under physiological conditions (10). Lymphedema arises due to an imbalance between the normal amount of protein load and the reduced transport capacity of the lymph vascular system. This condition, known as mechanical insufficiency, results in an accumulation of proteins in the interstitial tissue with subsequent fibrosclerotic changes. Since in such cases the lymphatic system is not able to activate its lymphatic safety factor, other pathologic factors that produce an increased level of lymphatic load (inflammation, chronic venous insufficiency), can lead to even more serious complications such as ulcerations. Common causes for mechanical insufficiencies in the case of secondary lymphedema are surgery, radiation, trauma or inflammation. The reason for an insufficient transport capacity in primary lymphedema are congenital malformations of the lymphatic system. Primary lymphedema can be present at birth or develop some time during the course of life (5,12). ETIOLOGY Primary Lymphedema Aplasia Hypoplasia Hyperplasia Fibrosis of lymph nodes Agenesis of lymph nodes Congenital < 35 years of age (Lymphedema Precox) > 35 years of age (Lymphedema tardum) Secondary Lymphema Dissection of lymph nodes Radiation post-traumatic Post-inflammatory Malignancies Self-induced (artificial) Stages of lymphedema If the reduced transport capacity is still sufficient in managing the lymphatic load there is no clinical lymphedema present The time preceding the onset of lymphedema is called the "latency stage" (5). The first stage of lymphedema (reversible stage) is characterized by a smooth texture of the tissue. The area affected with lymphedema is pitting and may vanish more or less over night. If the protein rich swelling persists, fibrosclerotic tissue changes will result in increased hardening of the tissues (stage II), elevation has no effect and in addition patients are prone to developing frequent infections which worsen the condition (4). Typical for the third stage of lymphedema (lymphostatic elephantiasis), is an extreme increase of the swelling, combined with skin changes, loss of function and other complications. Lymphedema, if left untreated, may lead to invalidity or even the development of angiosarcoma (Stewart Treves-Syndrome) (8). LYPHEDEMA Stages Characteristics Latency No swelling, reduced transport capacity, 'normal' consistency Stage I (reversible) Edema is soft ('pitting'), no secondary tissue changes, elevation reduces swelling Stage II (spontaneously irreversible) Fibrosclerotic changes hardening of the tissue (no 'pitting'), frequent infections Stage III (lymphostatic elephantiasis) Extreme increase in volume and texture with typical skin changes (papillomas, deep skin folds) Therapy Since there is no cure for lymphedema (10), the goal of the therapy is to reduce the swelling and to maintain the reduction, i.e. to bring the lymphedema back to a stage of latency. For a majority of patients this can be achieved by the skillful application of Complete Decongestive Physiotherapy, a non-invasive, safe and reliable method that shows good long term results in both primary and secondary lymphedema. CDP is also cost-effective: it transfers the care from the doctor to the patient/family it significantly reduces the risk factors of developing cellulitis attacks, described by Olszewski as "Dermatolymphangioadenitis" (DLA), by improving lymph cysts, lympho cutaneous fistulas, varicose lymphatics or fungal infections (7) Even though the basic steps of CDP had already been described by Winiwarter at the end of the last century, this therapy became widely accepted only during the past two and a half decades(1,2,13). Numerous studies have proven the effectiveness of this therapy which is well established in European countries and is now becoming widely recognized in the United States (1,2,8,10). CDP consists of four basic steps: Skin and nail care, that may also include topical and systemic antimycotic drug treatment (the skin must be free of infections before treatment can be started) Manual Lymph Drainage Compression therapy and Decongestive exercises The treatment itself is done in two phases (8). In phase one the goal is to mobilize the accumulated protein- rich fluid and to initiate the reduction of fibrosclerotic tissues (if present). The average duration of this intensive phase is four weeks. The treatment is done twice a day, five days a week. Another important goal in this first phase is to instruct the patient in techniques designed to maintain and improve the success of the treatment (proper skin care, correct application of bandages, wearing of compression garments, etc.). The first phase of the therapy is immediately followed by phase two, aimed to preserve and also to improve the success achieved in phase one. This phase is for the most part continued at the patients home. With a good patient compliance the volume reduction can not only be maintained but also improved by progressive reduction of fibrosclerotic tissues. In more severe cases it is sometimes necessary to repeat phase one and if lymphedema is associated with other conditions, the individual steps of CDP will be modified accordingly. STAGES OF LYMPHEDEMA AND THERAPEUTIC APPROACH Stages Duration Phase I (decongestion) Phase II (preserve and improve) Latency Stage I 2-3 weeks MLD 1-2x/day short-stretch bandages skin care remedial exercises patient instruction MLD if necessary compression garments skin care remedial exercises Stage II 3-4 weeks MLD 2x/day short-stretch bandages skin care remedial exercises patient instruction MLD as needed (1-2x/week) compression garments bandages at night skin care remedial exercises repeat Phase I (1-2x) Stage III 4-6 weeks MLD 2-3x/day short-stretch bandages skin care remedial exercises patient instruction MLD 1-2x/week compression garments (in combination with bandages) bandages at night skin care remedial exercises repeat Phase I (3-4x) if indicated plastic surgery Manual Lymph Drainage is a gentle manual treatment technique which improves the activity of intact lymph vessels by mild mechanical stretches on the wall of lymph collectors (10). A better filling of lymph capillaries, achieved by a mild increase in tissue pressure during MLD, also results in a higher lymphangiomotoricity. In most of the post-mastectomy patients, lymphedema not only includes the arm but also the ipsilateral trunk quadrant, since the collecting area of the axillary lymph nodes are the upper extremity and the homolateral upper trunk quadrant. In cases of secondary lymphedema of the lower extremities, the lower trunk quadrant and/or the genitalia may be involved in the lymphostasis because the inguinal lymph nodes receive lymph fluid from the leg, the ipsilateral lower quadrant of the trunk and the exterior genitals (10). MLD is therefore performed in steps: the first step is to stimulate the lymph vessels in the non-affected contralateral trunk quadrant which results in a suction effect (6) on the lymphatics of the affected trunk quadrant. In the second step edema fluid is cautiously pushed from the congested quadrant into the quadrant free of edema via tissue channels, initial lymphatics and lymph vessels bridging the watersheds thus creating a connection between regional lymph nodes on the contralateral and ipsilateral sides. After the trunk is decongested, the upper part of the extremity is treated and later on, the distal part and the hand/foot - always making sure not to overwhelm the drainage areas previously stimulated. Many patients we see report that even though they received many treatments in "Manual Lymph Drainage" the lymphedema didn't improve and sometimes the limb size even increased. Asking the patient how the treatment was performed, in many cases we hear that the therapist performed an effleurage beginning at the fingers or toes or used massage techniques on the swollen extremity. As mentioned before, MLD is a very gentle manual technique consisting of four basic strokes and any combination of same. MLD has nothing to do with "classical" or "Swedish" massage and shouldn't be called massage. The word "massage" means "to knead" (Greek: massain), Manual Lymph Drainage does not have kneading elements and is generally applied suprafascially, whereas massage is usually applied to subfascial tissues. Compression Therapy Since the elastic fibers of the skin are damaged in lymphedema, it is mandatory to apply sufficient compression to the affected area in order to prevent reaccumulation of fluid. Compression therapy increases the tissue pressure (TP) which results in lower effective ultrafiltration and better reabsorption on the venous end of the blood capillaries. It also promotes the filling of initial lymph vessels, improves the function of the muscle pumps and helps to reduce fibrosclerotic tissue. In phase I of the therapy compression is applied via short-stretch bandages. Short-stretch bandages have a high working pressure (pressure the bandage exerts on the musculature working underneath) and a low resting pressure (pressure exerted on the tissue while resting). Long-stretch bandages have exactly opposite characteristics and are therefore not indicated in the treatment of lymphostatic edema since they tend to cut into the tissue while resting, causing a tourniquet effect and thus impeding sufficient lymph and blood flow. Long-stretch bandages also fail to produce an effective counterforce to the working muscles. In order to avoid irritation on bony prominences and tendons, padding with cotton bandages or foam is applied underneath the bandages. To enhance the reduction of fibrosclerosis, high density foam is frequently used in combination with short- stretch bandages. Low pH-lotion to keep the skin moist and tubular bandages to avoid allergic reactions and to protect the bandage materials are also applied. During phase I of CDP compression therapy during day and night is achieved by short-stretch bandages. In phase II the patient wears compression garments during the day and applies bandages for the night. Measurements for these elastic support garments should be taken at the end of phase I by the therapist or the supervising physician. An incorrectly fitted sleeve or stocking will have negative effects on the lymphedema itself and on the patients compliance. To achieve the best results with CDP good compliance of the patient is absolutely necessary. The compression class and the type of garment (round or flat knit, style) depends on the severity of the swelling, the patients age and any other relevant factors. In general the pressure of the garment should be as high as the patient can tolerate (14). For lower extremity lymphedema compression classes 111(30-40 mm/Hg) or IV (> 50 mm/Hg), for lymphedema of the upper extremities compression classes 1(10-20 mm/Hg) or II(20-30 mm/Hg), sometimes compression class Ill, are suitable. In some cases it might be necessary to apply even a greater compression than class IV which can be achieved by wearing two stockings on top of each other or by the application of bandages on top of a stocking. To have the maximum effect, garments must be worn every day and replaced after six months. Remedial exercises aid the lymphokinetic effects of joint and muscle pumps and should be performed by the patient wearing the compression bandage or the garment. The exercise program should be customized for each patient depending on the individual capacities. Exercises should be performed slowly and with both, the affected and non-affected extremity. Vigorous movements or exercises causing pain must be avoided. When does CDT fail? Phase I: malignant lymphedema artificial lymphedema improper treatment (MLD as the only treatment, no MLD or improper bandage) associated illnesses lack of compliance Phase II: lack of compliance lack of hygiene reoccurrence of cancer associated illnesses Conclusion: Lymphedema can be treated successfully by a skilled therapist with extensive training in all components of Complete Decongestive Physiotherapy and good patient compliance. The treatment success must be monitored by circumferential and/or volumetric measurements. If phase I of CDT is performed in the early "pitting" stage of lymphedema, total remission of the swelling is possible. In later stages of lymphedema phase I only reduces the swelling but fibrosclerotic tissue changes will still be present. For these more advanced stages phase II of Complete Decongestive Physiotherapy not only preserves the treatment success achieved in the intensive phase but also improves the edema overtime, restoring the limb to a normal or near normal size. ==== ============================================================ = http://www.acols.com/info_lymph.html Information about Lymphedema INTRODUCTION At the end of the last century, Alexander Von Winiwarter, M.D., Professor of Surgery, treated swollen limbs with a special 'massage' technique, compression therapy and elevation. The manual therapy Dr. Winiwarter devised was improved in the 30's by Dr. Vodder from Denmark. He successfully treated various conditions with this technique, known as Manual Lymph Drainage. In the 80's, Professor Michael Foldi, M.D. from Germany considerably advanced lymphedema therapy by combining a variety of techniques into what is now known as Complete Decongestive Therapy (CDT). In 1994 Joachim E. Zuther founded the Academy of Lymphatic Studies and taught the first certification class in the United States according to the European curriculum. MANUAL LYMPH DRAINAGE / COMPLETE DECONGESTIVE THERAPY This non-invasive, painless and very effective therapy for lymphedema and other related conditions has been used in Europe successfully for decades and is now becoming widely recognized in the United States. The treatment consists of four basic steps: 1. Manual Lymph Drainage (MLD) MLD is a gentle manual treatment technique which improves the activity of the lymph vessels by mild mechanical stretches on the wall of lymph collectors. MLD re-routes the lymph flow around the blocked areas into more centrally located lymph vessels that eventually drain into the venous system. 2. Compression Therapy Compression therapy increases the tissue pressure and is applied between treatments in order to prevent reaccumulation of lymph fluid. In Phase I of the treatment compression therapy is applied using short-stretch bandages, in Phase II by custom made garments which the patient has to wear every day. 3. Decongestive Exercises Performed by the patient wearing the compression bandages or garments as well as respiration therapy aid the lymphokinetic effects of the joint and muscle pumps. 4. Skin Care Since infections are very common and serious complications of lymphedema, each patient is taught meticulous skin and nail care. Therapy cannot proceed until all infections, bacterial or fungal, are under control. INDICATIONS 1. Lymphedema Lymphedema , an accumulation of protein-rich fluid in the superficial tissues, is a very common and serious condition worldwide. Left untreated is has important pathological and clinical consequences for the patient. Secondary lymphedema is often caused by surgery, radiation, trauma or infection. The reason for the development of primary lymphedema are congenital malformations of the lymphatic system. In such cases the swelling may appear after simple accidents such as sport injuries which can suddenly overload the transport capacity of the lymphatic system. Today there are millions of patients living in the U.S. suffering from lymphedema (primary or secondary) and its complications. Manual Lymph Drainage and Complete Decongestive Physiotherapy is the only effective treatment for this condition.Other indications are: 2. Chronic Venous Insufficiency 3. Post-surgical swelling 4. Post-traumatic swelling 5. Lipedema, Lipo-Lymphedema 6. Amputee stump edema 7. Migraine headaches ----------------------------------------- http://www.naturalhealthweb.com/articles/Zuther.html UNDERSTANDING LYMPHEDEMA PATHOPHYSIOLOGY AND TREATMENT By: Joachim E. Zuther Lymphedema, a protein rich swelling which usually affects the extremities, is a very common condition worldwide. Complete Decongestive Physiotherapy is done successfully in Europe for decades in the treatment of primary and secondary Lymphedema and is now becoming widely recognized in the U.S. UNDERSTANDING LYMPHEDEMA PATHOPHYSIOLOGY AND TREATMENT Joachim E. Zuther Chronic peripheral lymphedema, an accumulation of protein rich fluid in the superficial tissues, is a very common and serious condition with significant consequences for the patient. One of the main reasons for the development of lymphedema are surgical interventions in combination with lymph node dissections, such as mastectomy or lumpectomy due to breast cancer, which is the number one cause for this disease in the U.S. Although reliable statistics on the overall incidence of lymphedema are not available, conservative numbers estimate the incidence of secondary upper extremity lymphedema to be around 2.5 million. In addition to that there is a large number of patients suffering from primary lymphedema, which usually affects the lower extremities and is caused by congenital malformations of the lymphatic system. Anatomy and Physiology of the Lymphatic System Unlike the blood system the lymphatic system works according to the one way principle, its main purpose is to transport "waste materials" from the interstitial tissues back into the blood system. These materials, also called lymphatic loads, consist of protein, water, cells and fat, are drained by the various vascular structures of the lymphatic system and filtered by a large number of regional and central lymph nodes before they enter the venous system. Part of these waste materials are also cell products and cell residues including foreign materials. Initial lymphatic system: Lymph vessels start in almost every tissue as lymph capillaries. These initial lymphatics are made up of endothelial cells which overlap each other. Capillaries do not have a continuous connection like blood capillary endothelial cells do. A surrounding fiber net, anchoring filaments, arranged around the lymph capillaries, enables these small vessels to stay open, even under high tissue pressure. Lymph capillaries collect lymphatic loads from the interstitial areas and gradually join together into bigger lymph vessels, so-called precollectors which then drain into collectors. Collectors: One segment of a lymph collector is called lymph angion. Contractions of smooth muscles situated in each lymph angion, generate the propulsive force of the lymph flow. The pumping is aided by a large number of valves located inside the collectors which allow the lymph flow in only one direction. After passing a large number of lymph nodes, where foreign substances like bacteria are filtered out and necessary immune reactions are activated, the lymph fluid empties into the venous system, mainly via the thoracic duct. The thoracic duct is the largest lymph vessel of the body. Under physiological conditions approximately 1-2 liters of lymph fluid drain in 24 hours via the thoracic duct into the left venous angle, formed by the left internal jugular and the left subclavian vein. Starling's equilibrium: The amount of water and protein transported via the lymphatic system is depending on forces being active in the area of the blood capillaries. Starling's equilibrium describes the balance of capillary filtration and capillary reabsorption. The transport of fluid through the membrane of blood capillaries depends on four variables: 1. blood capillary pressure (BCP) 2. colloidosmotic pressure (COP) of the plasma proteins 3. colloidosmotic pressure (COP) of the proteins located in the interstitial tissue 4. tissue pressure (TP) Ultrafiltration: blood capillary pressure > COP of plasma proteins Reabsorption: blood capillary pressure < COP of plasma proteins Under physiological conditions 10-15% of the ultrafiltrate remains in the interstitial tissues and is then drained by the lymphatic system. Shifting of Starling's equilibrium towards an increase in ultrafiltration, e.g. increased blood capillary pressure (inflammation, venous hypertension) or decreased colloidosmotic pressure (hypoproteinemia), can cause an increased amount of water and proteins, thus creating a higher burden on the lymphatic system. A healthy lymphatic system is, for some time, able to prevent the onset of edema, under normal conditions the transport capacity (TC) of the lymphatic system is approximately 10 times higher than the physiological amount of the lymphatic loads (LL) of water and protein => functional reserve (FR) of the lymphatic system. Pathophysiology of Lymphedema: As long as the lymphatic loads remains lower than the transport capacity of the lymphatic system, the lymphatic compensation is successful. If the amount of water and protein exceeds the transport capacity, edema will occur. This condition is called dynamic insufficiency of the lymphatic system, the lymph vessels are intact but overwhelmed. The result is an accumulation of fluid in the tissue which is usually treated successfully with elevation of the affected limbs, compression and decongestive exercises. Lymphedema is caused by a mechanical insufficiency, or low-volume insufficiency of the lymphatic system. In this case the transport capacity of the lymphatic system drops below the physiological level of the lymphatic loads of water and protein, that means the lymphatic system is not able to manage its main purpose which is to clear the interstitial spaces from excess water, protein and other chemical, organic and inorganic cell products. The reasons for mechanical insufficiencies are various and mainly caused by surgery, radiation, trauma or inflammation. Accumulation of high protein fluid is the result which is then recognized as lymphedema or lymphostatic edema. Classification of Lymphedema: Secondary Primary 1.Caused by lymph node dissections, 1.caused by malformations of the e.g. axilla, groin, pelvis or neck, lymphatic system (hyperplasia, radiation of lymph nodes, trauma or hypoplasia, aplasia or sclerosis infection ofthe lymphatic system. of lymph nodes) 2.Affects extremities, genitalia, 2.Can be present at birth (congenital abdomen or the face a lymph-edema), develop around the (lymphedema precox) or after age of puberty the age of 35 (lymphedema tardum) Any lymphedema left untreated gradually worsens and will have significant consequences.The first stage of lymphedema (reversible stage) is characterized by a smooth texture of the tissue, the lymphedema is "pitting" and may vanish more or less over night. If protein rich edema persists, the congested protein molecules are replaced by fibrotic tissue, i.e. the lymphedema becomes harder (stage II) and in addition to that patients are prone to developing frequent infections which additionally worsen the condition. Typical for the third stage of lymphedema, lymphostatic elephantiasis, is an extreme increase of the swelling combined with skin changes, loss of function and other complications. Since lymphedema, primary or secondary, is a progressive condition, treatment should begin as early as possible. The goal of the treatment is to remove the excess lymphatic loads of water and protein and to restore the disturbed equilibrium in the interstitial tissues of the affected area. Treatment of Lymphedema with Complete Decongestive Physiotherapy: Complete Decongestive Physiotherapy (CDP), done successfully in Europe for decades, is a non-invasive therapy with long lasting results. CDP, which now becomes widely recognized in the U.S., is superior to all other approaches to treat lymphedema (pumps, medication, surgery) and designed to reduce and to maintain the reduction of the swollen extremity. At the end of the last century Winiwarter, professor of surgery, already described the basic steps of this therapy. The technique was improved in the 1930s by Vodder, a physical therapist from Denmark, who successfully treated lymphedema. In the 1980s Foeldi considerably improved this therapy by developing a technique called Complete Decongestive Physiotherapy, which even in advanced stages of lymphedema shows remarkable results. CDP is done in two phases. The first phase, intensive phase, lasts between two and four weeks (in extreme cases longer), treatments are done twice a day, five days a week. The goal of this phase is to decongest the swollen extremity to a normal or near normal size. Simultaneously the patient is instructed in techniques designed to maintain and even improve the condition after the intensive phase of the therapy. The first phase is immediately followed by phase two, the maintenance and improvement phase which the patients continues at home. For the safety of the patients and to achieve good results it is absolutely mandatory that the therapist is thoroughly trained in all components of CDP. Only certified MLD/CDP therapists have a complete understanding of the pathophysiology of lymphedema and its treatment. CDP consists of four basic steps : 1. Manual Lymph Drainage 2. Compression Therapy 3. Meticulous Skin and Nail Care 4. Remedial Exercises Manual Lymph Drainage is a gentle manual treatment technique which improves the activity of intact lymph vessels by mild mechanical stimuli. The goal of this treatment is to move excess lymphatic loads of water and protein into areas with still sufficient lymphatics. Since the elastic fibers of the skin are destroyed during the course of lymphedema it is mandatory to apply sufficient compression to the affected area in order to prevent reaccumulation of fluid. Compression therapy in phase one is achieved by short-stretch bandages applied after each treatment. After the extremity is decongested the patient is fitted with a sufficient compression garment that needs to be worn during the daytime. At night the patient applies mild compression using bandages. Before treatment can be started the skin has to be free of infections or fungal affections. During the treatment it is mandatory to apply a low-pH lotion to maintain the moisture of the skin. Remedial exercises performed by the patient wearing the compression bandage or garment aid the lymphokinetic effects of the joint and muscle pumps. References: 1. Marvin Boris, et al. Lymphedema Reduction By Noninvasive Complex Lymphedema Therapy Cornell University School of Medicine and Lymphedema Therapy. Woodbury, NY 2. M.Foeldi, S.Kubik Lehrbuch der Lymphologie Gustav Fischer Verlag, Germany 1992 3. L.Clodius, M.Foeldi Therapy For Lymphedema Today Inter. Angio., 3 1984 4. S.Kubik The Lymphatic System Springer, NY 1985 5. M.Foeldi, E.Foeldi Das Lymphoedem Gustav Fischer Verlag, Germany 1991 6. G.Bringezu, O.Schreiner Die Therapieform Manuelle Lymphdrainage Otto Haase Verlag, Germany 1987 7. R.Lerner, J.Petrek Lymphedema Diseases Of The Breast, Lippincott - Raven, 1996 8. J.R. Casley-Smith Lymphedema, The Poor and Benzo-Pyrones: Proposed Amendments To The Consensus Document Lymphology 29, 1996 137-140 9. E.Foeldi Preventions of Dermatolymphangioadenitis By Combined Physiotherapy Of The Swollen Arm After Treatment For Breast Cancer Lymphology 29, 1996 48-49 10. E.Foeldi, M.Foeldi, L.Clodius The Lymphedema Chaos: A Lancet Ann Plast Surg 22:505-515 -------------------------------------------------------------------------------- Bio: Joachim E. Zuther is a certified instructor in Manual Lymph Drainage (Vodder Technique) and Complete Decongestive Physiotherapy. He is an active member of the International Society of Lymphology (ISL), a member of the Medical Advisory Board of the National Lymphedema Network (NLN) and a member of the German Society of Lymphology. He is the Director and Chief Instructor at the Academy of Lymphatic Studies in Ft. Lauderdale, FL and primary instructor at the ulmkolleg/University of Ulm, Germany The Academy of Lymphatic Studies can be reached at (561)589-3355 or www.acols.com Fibrosis Fibrosis due to radiation. Lymph Notes Introduction The term fibrosis is a noun that describes the formation of fine scar-like structures that cause tissues to harden and reduces the flow of fluids through these tissues. The term fibrotic is a verb meaning pertaining to fibrosis or the hardening of tissues. As shown here, radiation is one cause fibrosis. It can also be caused by chemotherapy, burns, and the improper treatment of lymphedema. How Fibrotic Tissues Form In the early stages of lymphedema, the tissues swell with protein-rich lymph and cannot drain properly. These tissues are soft to the touch and pressure leaves an indentation that takes time to fill back in. This is known as pitting edema and you can read more about it by reading How Lymphedema is Diagnosed. If the lymphedema is not treated at this stage, the stagnant lymph causes fibrosis within the affected tissues as the disease progresses to Stages 2 and 3. As fibrosis develops, normal tissues are replaced by scar-like structures that create obstructions and make lymph drainage even more difficult. The amount of swelling is not an indicator as to the risk for developing fibrosis. Fibrosis can occur in tissues that are only moderately swollen. Because this lymph cannot drain properly, the protein molecules accumulate in the tissues and cause more fibrosis and a greater danger of infections. Fibrosis can be felt as the tissues are no longer flexible or soft. As these changes increase, so too do the risks of more swelling, tissue infections, and skin problems. Potential Complications of Fibrosis The tissues are further damaged because the fibrosis prevents tissue fluid from bringing essential nutrients to the cells. This produces increased toxic waste products surrounding the cells within these tissues. Because the lymph cannot flow to the lymph nodes where pathogens would be destroyed and the fluid would be filtered, the danger of infection increase. These tissue changes result in infections within the tissues and open wounds that do not heal properly. Treatment As the tissue hardens different massage techniques, using more pressure, are required as recommended by the patient's lymphedema therapist. References [1] Foundations of Manual Lymph Drainage 3-E by M. Földi and R. StröBenreuther. Elsevier Mosby, 2003. [2] Living Well with Lymphedema by A. Ehrlich, A. Vinjé-Harrewijn PT, CLT-LANA, and E. McMahon PhD. Lymph Notes, 2005. [3] Lymphedema Diagnosis and Therapy 2-E edited by H. Weissleder and C. Schuehhardt. Kagerer Kommunikation, 1997. [4] The Lymphatic System Pathology by B. Lasinski in Implications for the Physical Therapists 2-Ed C.C Goodman, W.G. Boissonnault, and K.S.Fuller. Saunders, 2003. © LymphNotes.com 2009. This information does not replace the advice of a qualified health care professional. Got a question or comment? Post in the 'What is Lymphedema?' forum. Category: What is Lymphedema? Updated: 2009-07-29 http://www.lymphnotes.com/article.php/id/43/ good link http://en.wikipedia.org/wiki/Lymphedema if you look to the left you can click on all diffrent subjects and stuff related to lymphedema http://www.medifocushealth.com/OC030/Understanding-Lymphedema_Introduction-to-Lymphedema.php ---------------------------------------------------------------- ======================================================= ============================================================== compression therapy http://www.nortonschool.com/compressiontherapy.html -- http://www.mayoclinic.com/health/angiosarcoma/AN00841 what is Angiosarcoma Angiosarcomas are cancerous tumors that develop from blood or lymphatic vessels. These tumors can arise in any part of the body. But they occur most often in the arms, legs, neck and head. In most cases, the cause of angiosarcomas isn't known. However, one type of angiosarcoma may occur in an area of the body previously exposed to radiation. This type tends to be aggressive and respond poorly to treatment. Another type (lymphangiosarcoma) may occur in people with chronic lymphedema in an arm or leg. Doctors refer to this rare condition as Stewart-Treves syndrome. Angiosarcomas typically cause no signs or symptoms in the early stages. As a tumor grows, it may cause a lump or swelling. Later it may cause pain if it presses on nerves or muscles. A doctor may suspect an angiosarcoma based on: Physical exam A computerized tomography (CT) scan X-rays A magnetic resonance imaging (MRI) scan The only way to confirm a diagnosis is by a biopsy of affected tissue. Early-stage treatment often includes surgical removal of the tumor combined with chemotherapy and radiation. In later stages of the disease, treatment is more difficult. But it may include chemotherapy and, in some cases, radiation therapy. Prognosis depends on stage of disease. If the cancer is localized, the prognosis is very favorable. http://www.acols.com/about_treat.html Treatment of Lymphedema with Complete Decongestive Physiotherapy Author: Joachim E. Zuther (also published in NLN Newsletter Vol II, #2 Summary: Lymphedema, caused by a low output failure, i.e. a reduced transport capacity (TC) of the lymphatic system, which in the case of lymphedema has fallen below the physiological level of the protein and water load, is a common and serious condition worldwide. Complete Decongestive Physiotherapy (CDP) is the treatment of choice, even in the most advanced stages of lymphostatic edema - provided that both a physician with broad experience in clinical lymphology and a specially trained therapist are available. A sufficient lymphatic system is able to return the physiological amount of protein and water load back to the venous system. The lymphatic protein load consists of plasma proteins continuously leaving the blood capillaries. The fraction of water ultrafiltrated in the area of the blood capillaries which is not reabsorbed, is called the lymphatic load of water. In the event of an increase of water and protein a healthy lymphatic system is, for some time, able to prevent the onset of edema by increasing its lymph time volume (LTV), i.e. lymph anions will increase their pulsation frequency and amplitude. This is called the lymphatic safety factor or safety valve function of the lymphatic system. The highest lymph time volume is known as the transport capacity of the lymphatic system which is approximately ten times higher than the lymph time volume under physiological conditions (10). Lymphedema arises due to an imbalance between the normal amount of protein load and the reduced transport capacity of the lymph vascular system. This condition, known as mechanical insufficiency, results in an accumulation of proteins in the interstitial tissue with subsequent fibrosclerotic changes. Since in such cases the lymphatic system is not able to activate its lymphatic safety factor, other pathologic factors that produce an increased level of lymphatic load (inflammation, chronic venous insufficiency), can lead to even more serious complications such as ulcerations. Common causes for mechanical insufficiencies in the case of secondary lymphedema are surgery, radiation, trauma or inflammation. The reason for an insufficient transport capacity in primary lymphedema are congenital malformations of the lymphatic system. Primary lymphedema can be present at birth or develop some time during the course of life (5,12). ETIOLOGY Primary Lymphedema Aplasia Hypoplasia Hyperplasia Fibrosis of lymph nodes Agenesis of lymph nodes Congenital < 35 years of age (Lymphedema Precox) > 35 years of age (Lymphedema tardum) Secondary Lymphema Dissection of lymph nodes Radiation post-traumatic Post-inflammatory Malignancies Self-induced (artificial) Stages of lymphedema If the reduced transport capacity is still sufficient in managing the lymphatic load there is no clinical lymphedema present The time preceding the onset of lymphedema is called the "latency stage" (5). The first stage of lymphedema (reversible stage) is characterized by a smooth texture of the tissue. The area affected with lymphedema is pitting and may vanish more or less over night. If the protein rich swelling persists, fibrosclerotic tissue changes will result in increased hardening of the tissues (stage II), elevation has no effect and in addition patients are prone to developing frequent infections which worsen the condition (4). Typical for the third stage of lymphedema (lymphostatic elephantiasis), is an extreme increase of the swelling, combined with skin changes, loss of function and other complications. Lymphedema, if left untreated, may lead to invalidity or even the development of angiosarcoma (Stewart Treves-Syndrome) (8). LYPHEDEMA Stages Characteristics Latency No swelling, reduced transport capacity, 'normal' consistency Stage I (reversible) Edema is soft ('pitting'), no secondary tissue changes, elevation reduces swelling Stage II (spontaneously irreversible) Fibrosclerotic changes hardening of the tissue (no 'pitting'), frequent infections Stage III (lymphostatic elephantiasis) Extreme increase in volume and texture with typical skin changes (papillomas, deep skin folds) Therapy Since there is no cure for lymphedema (10), the goal of the therapy is to reduce the swelling and to maintain the reduction, i.e. to bring the lymphedema back to a stage of latency. For a majority of patients this can be achieved by the skillful application of Complete Decongestive Physiotherapy, a non-invasive, safe and reliable method that shows good long term results in both primary and secondary lymphedema. CDP is also cost-effective: it transfers the care from the doctor to the patient/family it significantly reduces the risk factors of developing cellulitis attacks, described by Olszewski as "Dermatolymphangioadenitis" (DLA), by improving lymph cysts, lympho cutaneous fistulas, varicose lymphatics or fungal infections (7) Even though the basic steps of CDP had already been described by Winiwarter at the end of the last century, this therapy became widely accepted only during the past two and a half decades(1,2,13). Numerous studies have proven the effectiveness of this therapy which is well established in European countries and is now becoming widely recognized in the United States (1,2,8,10). CDP consists of four basic steps: Skin and nail care, that may also include topical and systemic antimycotic drug treatment (the skin must be free of infections before treatment can be started) Manual Lymph Drainage Compression therapy and Decongestive exercises The treatment itself is done in two phases (8). In phase one the goal is to mobilize the accumulated protein- rich fluid and to initiate the reduction of fibrosclerotic tissues (if present). The average duration of this intensive phase is four weeks. The treatment is done twice a day, five days a week. Another important goal in this first phase is to instruct the patient in techniques designed to maintain and improve the success of the treatment (proper skin care, correct application of bandages, wearing of compression garments, etc.). The first phase of the therapy is immediately followed by phase two, aimed to preserve and also to improve the success achieved in phase one. This phase is for the most part continued at the patients home. With a good patient compliance the volume reduction can not only be maintained but also improved by progressive reduction of fibrosclerotic tissues. In more severe cases it is sometimes necessary to repeat phase one and if lymphedema is associated with other conditions, the individual steps of CDP will be modified accordingly. STAGES OF LYMPHEDEMA AND THERAPEUTIC APPROACH Stages Duration Phase I (decongestion) Phase II (preserve and improve) Latency Stage I 2-3 weeks MLD 1-2x/day short-stretch bandages skin care remedial exercises patient instruction MLD if necessary compression garments skin care remedial exercises Stage II 3-4 weeks MLD 2x/day short-stretch bandages skin care remedial exercises patient instruction MLD as needed (1-2x/week) compression garments bandages at night skin care remedial exercises repeat Phase I (1-2x) Stage III 4-6 weeks MLD 2-3x/day short-stretch bandages skin care remedial exercises patient instruction MLD 1-2x/week compression garments (in combination with bandages) bandages at night skin care remedial exercises repeat Phase I (3-4x) if indicated plastic surgery Manual Lymph Drainage is a gentle manual treatment technique which improves the activity of intact lymph vessels by mild mechanical stretches on the wall of lymph collectors (10). A better filling of lymph capillaries, achieved by a mild increase in tissue pressure during MLD, also results in a higher lymphangiomotoricity. In most of the post-mastectomy patients, lymphedema not only includes the arm but also the ipsilateral trunk quadrant, since the collecting area of the axillary lymph nodes are the upper extremity and the homolateral upper trunk quadrant. In cases of secondary lymphedema of the lower extremities, the lower trunk quadrant and/or the genitalia may be involved in the lymphostasis because the inguinal lymph nodes receive lymph fluid from the leg, the ipsilateral lower quadrant of the trunk and the exterior genitals (10). MLD is therefore performed in steps: the first step is to stimulate the lymph vessels in the non-affected contralateral trunk quadrant which results in a suction effect (6) on the lymphatics of the affected trunk quadrant. In the second step edema fluid is cautiously pushed from the congested quadrant into the quadrant free of edema via tissue channels, initial lymphatics and lymph vessels bridging the watersheds thus creating a connection between regional lymph nodes on the contralateral and ipsilateral sides. After the trunk is decongested, the upper part of the extremity is treated and later on, the distal part and the hand/foot - always making sure not to overwhelm the drainage areas previously stimulated. Many patients we see report that even though they received many treatments in "Manual Lymph Drainage" the lymphedema didn't improve and sometimes the limb size even increased. Asking the patient how the treatment was performed, in many cases we hear that the therapist performed an effleurage beginning at the fingers or toes or used massage techniques on the swollen extremity. As mentioned before, MLD is a very gentle manual technique consisting of four basic strokes and any combination of same. MLD has nothing to do with "classical" or "Swedish" massage and shouldn't be called massage. The word "massage" means "to knead" (Greek: massain), Manual Lymph Drainage does not have kneading elements and is generally applied suprafascially, whereas massage is usually applied to subfascial tissues. Compression Therapy Since the elastic fibers of the skin are damaged in lymphedema, it is mandatory to apply sufficient compression to the affected area in order to prevent reaccumulation of fluid. Compression therapy increases the tissue pressure (TP) which results in lower effective ultrafiltration and better reabsorption on the venous end of the blood capillaries. It also promotes the filling of initial lymph vessels, improves the function of the muscle pumps and helps to reduce fibrosclerotic tissue. In phase I of the therapy compression is applied via short-stretch bandages. Short-stretch bandages have a high working pressure (pressure the bandage exerts on the musculature working underneath) and a low resting pressure (pressure exerted on the tissue while resting). Long-stretch bandages have exactly opposite characteristics and are therefore not indicated in the treatment of lymphostatic edema since they tend to cut into the tissue while resting, causing a tourniquet effect and thus impeding sufficient lymph and blood flow. Long-stretch bandages also fail to produce an effective counterforce to the working muscles. In order to avoid irritation on bony prominences and tendons, padding with cotton bandages or foam is applied underneath the bandages. To enhance the reduction of fibrosclerosis, high density foam is frequently used in combination with short- stretch bandages. Low pH-lotion to keep the skin moist and tubular bandages to avoid allergic reactions and to protect the bandage materials are also applied. During phase I of CDP compression therapy during day and night is achieved by short-stretch bandages. In phase II the patient wears compression garments during the day and applies bandages for the night. Measurements for these elastic support garments should be taken at the end of phase I by the therapist or the supervising physician. An incorrectly fitted sleeve or stocking will have negative effects on the lymphedema itself and on the patients compliance. To achieve the best results with CDP good compliance of the patient is absolutely necessary. The compression class and the type of garment (round or flat knit, style) depends on the severity of the swelling, the patients age and any other relevant factors. In general the pressure of the garment should be as high as the patient can tolerate (14). For lower extremity lymphedema compression classes 111(30-40 mm/Hg) or IV (> 50 mm/Hg), for lymphedema of the upper extremities compression classes 1(10-20 mm/Hg) or II(20-30 mm/Hg), sometimes compression class Ill, are suitable. In some cases it might be necessary to apply even a greater compression than class IV which can be achieved by wearing two stockings on top of each other or by the application of bandages on top of a stocking. To have the maximum effect, garments must be worn every day and replaced after six months. Remedial exercises aid the lymphokinetic effects of joint and muscle pumps and should be performed by the patient wearing the compression bandage or the garment. The exercise program should be customized for each patient depending on the individual capacities. Exercises should be performed slowly and with both, the affected and non-affected extremity. Vigorous movements or exercises causing pain must be avoided. When does CDT fail? Phase I: malignant lymphedema artificial lymphedema improper treatment (MLD as the only treatment, no MLD or improper bandage) associated illnesses lack of compliance Phase II: lack of compliance lack of hygiene reoccurrence of cancer associated illnesses Conclusion: Lymphedema can be treated successfully by a skilled therapist with extensive training in all components of Complete Decongestive Physiotherapy and good patient compliance. The treatment success must be monitored by circumferential and/or volumetric measurements. If phase I of CDT is performed in the early "pitting" stage of lymphedema, total remission of the swelling is possible. In later stages of lymphedema phase I only reduces the swelling but fibrosclerotic tissue changes will still be present. For these more advanced stages phase II of Complete Decongestive Physiotherapy not only preserves the treatment success achieved in the intensive phase but also improves the edema overtime, restoring the limb to a normal or near normal size. ==== ============================================================ = http://www.acols.com/info_lymph.html Information about Lymphedema INTRODUCTION At the end of the last century, Alexander Von Winiwarter, M.D., Professor of Surgery, treated swollen limbs with a special 'massage' technique, compression therapy and elevation. The manual therapy Dr. Winiwarter devised was improved in the 30's by Dr. Vodder from Denmark. He successfully treated various conditions with this technique, known as Manual Lymph Drainage. In the 80's, Professor Michael Foldi, M.D. from Germany considerably advanced lymphedema therapy by combining a variety of techniques into what is now known as Complete Decongestive Therapy (CDT). In 1994 Joachim E. Zuther founded the Academy of Lymphatic Studies and taught the first certification class in the United States according to the European curriculum. MANUAL LYMPH DRAINAGE / COMPLETE DECONGESTIVE THERAPY This non-invasive, painless and very effective therapy for lymphedema and other related conditions has been used in Europe successfully for decades and is now becoming widely recognized in the United States. The treatment consists of four basic steps: 1. Manual Lymph Drainage (MLD) MLD is a gentle manual treatment technique which improves the activity of the lymph vessels by mild mechanical stretches on the wall of lymph collectors. MLD re-routes the lymph flow around the blocked areas into more centrally located lymph vessels that eventually drain into the venous system. 2. Compression Therapy Compression therapy increases the tissue pressure and is applied between treatments in order to prevent reaccumulation of lymph fluid. In Phase I of the treatment compression therapy is applied using short-stretch bandages, in Phase II by custom made garments which the patient has to wear every day. 3. Decongestive Exercises Performed by the patient wearing the compression bandages or garments as well as respiration therapy aid the lymphokinetic effects of the joint and muscle pumps. 4. Skin Care Since infections are very common and serious complications of lymphedema, each patient is taught meticulous skin and nail care. Therapy cannot proceed until all infections, bacterial or fungal, are under control. INDICATIONS 1. Lymphedema Lymphedema , an accumulation of protein-rich fluid in the superficial tissues, is a very common and serious condition worldwide. Left untreated is has important pathological and clinical consequences for the patient. Secondary lymphedema is often caused by surgery, radiation, trauma or infection. The reason for the development of primary lymphedema are congenital malformations of the lymphatic system. In such cases the swelling may appear after simple accidents such as sport injuries which can suddenly overload the transport capacity of the lymphatic system. Today there are millions of patients living in the U.S. suffering from lymphedema (primary or secondary) and its complications. Manual Lymph Drainage and Complete Decongestive Physiotherapy is the only effective treatment for this condition.Other indications are: 2. Chronic Venous Insufficiency 3. Post-surgical swelling 4. Post-traumatic swelling 5. Lipedema, Lipo-Lymphedema 6. Amputee stump edema 7. Migraine headaches http://www.pon.nic.in/fil-free/Disease.html What is Lymphatic filariasis? Lymphatic Filariasis is the common term for a group of disease caused by lymph dwelling nematode worms such as Wuchereria bancrofti, Brugia malayi and Brugia timori termed as Bancroftian filariasis, Brugian (Malayan) filariasis and Timorian filariasis. Since these parasites affect primarily the lymphatic system of man, the disease caused is also commonly termed as Lymphatic filariasis. Filariasis is mostly a hidden disease. Individuals with filarial infection may be Asymptomatic or Symptomatic Asymptomatic condition: Asymptomatic individuals are those infected but without any detectable clinical symptoms and signs.They may have detectable microfilaraemia or antigenaemia. They may be immune or partially immune to infection. Still others in this group may have subclinical infections(without microfilaraemia), as indicated by the presence of filarial antigens in the blood. Asymptomatic individuals are of two types: Asymptomatic without sub clinical damage Asymptomatic with subclinical damage Sub clinical changes include Haematuria and Lymphatic changes Symptomatic individuals are classified based on the clinical manifestations into ACUTE FILARIASIS The Acute clinical manifestations of lymphatic filariasis are characterized by episodic attacks of lymphadenitis and lymphangitis associated with fever and malaise.Sometimes fever precedes the adenolymphangitis by a few days.Although the fever sometimes precedes adenolymphangitis, fever alone, in the absence of adenolymphangitis, should not be ascribed to filariasis, even when microfilaraemia is present. The main clinical manifestations of acute filariasis are: FILARIAL FEVER Filarial fever is an acute recurrent fever with haedache, malaise, chills, rigors, low to high grade (38-40°), subsiding with sweating and it may closely resemble malaria.Fever persists for 3-5 days. It is usually accompanied by symptomatic lymphangitis and other early signs of filariasis but may occur as fever alone. LYMPHANGITIS AND LYMPHADENITIS Acute lymphadenitis and lymphangitis are characteristic of Bancroftian and Malayan filariasis. The onset is acute, involving a single gland or group of glands within the inguinal, axillary, cervical or epitrochlear regions. This is accompanied by fever; 6-8 hours later retrograde (centrifugal) lymphangitis starts in the glands and spreads peripherally. The lymphatic trunks become painful and cord-like with characteristic red streaks running down the arms or legs. The distal affected limb becomes swollen and oedematous during the attack which may continue for several days accompanied by the fever. Lymphangitis may be confined to the groin glands, testis, spermatic cord (endemic funiculitis) or abdominal varix ruptures, symptoms of peritonitis rapidly develop and may be fatal (abdominal filariais) FILARIAL FUNICULITIS AND EPIDIDYMITIS This is an acute painful inflammation of the spermatic cord and epididymis. This is usually associated with fever and inflammation of testis and painful glands in the groins. Lymphangitis of the spermatic cord gives rise to funiculitis which has been called 'endemic funiculitis'.It is usually accompanied by filarial fever and orchitis. Fluid aspirated from the runica vaginalis is cloudy and contains a number of polymorphonuclear cells and occasional red cells, together with microfilariae. The acute symptoms last a few days but recur at intervals. In sections dead and efferentia causing extensive fibrotic change are found and it is possible, although not yet proved, that this may result in sterility. The end result is a thickened spermatic cord. EPIDIDYMO-ORCHITIS Epididymo-orchitis is an acute painful condition involving the testis and epididymis resulting in some degree of scrotal swelling and redness. It is usually associated with fever, funiculitis and lymphnode elargement in the groin. The onset is sudden with pain radiating to the groin and tendernesss of the testis which may swell to twice its usual size with a boggy feeling on palpation. Orchitis is associated with filarial fever,and recurrent attacks lead sooner or later to hydrocele. FILARIAL ABSCESS Filarial abscesses develop within superficial lymphatics on the limbs or scrotum and at first the symptoms resemble an attack of lymphangitis but the pain and swelling continue, to be followed after some weeks by rupture and a discharge of pus. Pus may be sterile or contain bacteria particularly Beta haemolytic streptococci. Fragments of dead adult worms may be discharged. CHRONIC FILARIASIS Chronic stage of filariasis usually develop 10-15 years from the onset of the first acute attack. The incidence and severity of chronic clinical manifestations tends to increase with age. The main characteristic feature of chronic filariasis is: CHRONIC LYMPHANGITIS Great enlargement of the lymphatic glands with fibrotic changes is common in chronic filariasis. The glands (groin glands usually but, in the Pacific, epitrochlear glands) are enlarged to 5-7.5cm in diameter and may form permanent 'tumours'. On section they resemble an unripe pear, the central portion being fibrotic and the peripheral, glandular. They may contain numerous coiled up adult worms. Varicous groin glands are frequently associated with lymph scrotum chylocele or chyluria. Then a sense of tension or an attack of lymphangitis draws attention to the area where soft swelling are discovered. These swelling may be of insignificant size or they may be as large as a fist. They may be noted on groin and affect the inguinal or femoral glands alone or together. THICKENED LYMPHATIC TRUNK After the initial swelling and inflammation of lymphangitis have subsided a line of induration remains. On excising this thickened tissue and dissecting it, minute cyst-like dilations of the lymphatics have been found containing live or dead adult filariae (but this surgical treatment is not recommended) CHRONIC LYMPHOEDEMA AND ELEPHANTIASIS Swelling of the distal parts of the body appears during acute attacks of filarial lymphangitis and consists of pitting edema which at first subsides completely. After each attack edema increases and subsides more slowly until it finally becomes permanent. The edema eventually ceases to pit and after a period of time becomes firm.The lymphoedema of lower limb is classified into four grades. Grade1: Early oedema completely reversible on elevation. Grade2: Oedema of the limb which is partially reversible on elevation of the limb without thickening of the skin Grade3: Irreversible oedema of the limb with thickening of the skin Grade4: Irreversible oedema of the limb with papillary and nodular growth. When the skin becomes chronically thickened it can be called elephantiasis and this is associated with hypertrophy and fibrous hyperplasia of the subcutaneous tissues. In 95% of cases the lower extremities, either one or both, alone or in combination with the scrotum, or arms are affected. The foot and ankle only, the foot, leg and thigh may each or all be involved, and more rarely the breast, vulva and circumscribed portions of the integuments of the limbs or trunk. LYMPH SCROTUM The scrotum is thickened and the lymphatic varicosities, in the skin are discharging serous serosangineous or milky fluid. Many cases have an associated inguinal or femoral adenopathy. HYDROCELE Hydrocele is the commonest manifestation of bancroftian filariasis among males. Pathologically it is characterized by a distended, generally thickened tunica, vaginalis with hyalinization and fibrosis of the subserosal layer, disorganization of the muscle layers, lymphoid and foreign-body giant cell infiltration, and, in extreme cases, calcification. The hydrocele fluid itself is amber in colour and the sediment shows a characteristic predominance of vacuolated mesothelial cells, fibrin, old blood clots, cholesterol clefts, and calcium dust. Such findings, when associated with epididymal changes, are highly suggestive of a filarial etiology for the hydrocele even without the recovery of W. bancrofti microfilariae from the fluid or adult worms from the cord and epididymal tissues. A majority of these patients do not give any history of ADL attacks in their life time therefore, the progression seems to be a passive phenomenon. CHYLURIA AND LYMPHURIA Chyluria is a condition where the patient complains of passing milky white urine, caused by admixture of lymph with urine due the rupture of lymphatics into the urinary system. Prolonged chyluria may result in the loss of fat in the urine amounting to 15% of lymphatic drainage of the gut. Chyluria will have the same metabloic effects as malabsorption and cause considerable loss of weight with vitamin, electrolyte and other deficiencies. The protein loss in lymphuria may lead to oedema secondary to hypoalbuminaemia. Loss of lymphocytes in prolonged chyluria may lead to low lymphocyte levels which when associated with immunosuppression from drugs may encourage opportunist infections. Chyluria results in pain in the back and aching sensations about the pelvis and groins probably caused by distension of the pre-existing varix. Retention of urine from the presence of chylous or lymphatic clots may be the first indication of trouble. The patient then suddenly becomes aware that he is passing milky urine which may be pink or red; sometimes it is white in the morning and red in the evening or vice versa. Chyluria is likely to occur for the first time, or as a relapse, in pregnancy or after childbirth. The presence of blood is caused by the rupture of small blood vessels into the dilated lymphatics when microfilariae may appear in urine passed during the night time only. OCCULT FILARIASIS The term Occult Filariasis is commonly used to designate filarial infections in which mf are not found in the periphral blood although they may be seen in tissues. However, it has now been shown that in some cases with occult filariasis, mf may actually be found after more careful blood examination despite their low density. Occult filariasis is believed to result from a hypersensitivity reaction to filarial antigens derived from microfilariae.Only a very small proportion of individuals in a community where filariasis is endemic develop occult forms of the disease. The clinical manifestations of Occult filariasis are: TROPICAL MULMONARY EOSINOPHILIA TPE was first described by Frimodty Moller and Barton in 1940. Its main clinical manifestations are: Severe Cough and wheezing (specially at night) Frequent weight loss and fatigue but with minimal or no fever. Restrictive or obstructive lung abnormalities. Abnormal chest radiographs that frequently show diffuse mottled pulmonary interstitial infiltrate. Peripheral blood eosinophilia > 3000 cell/µl Extreme elevation of immunoglobin (IgE) Extreme elevation of anti-filarial antibodies Detection: Using IFAT(Indirect flourescent Antibody Test) Filarial Antibodies are detected. Treatment: Dramatic clinical improvement in response to specific anti-filarial chemotherapy with (DEC) GLOMERULOPATHIES(GLOMERULONEPHRITIS) Glomerulonephritis is associated with lymphatic filariasis. Filarial antibodies have been detected in 2 of 5 children with filariasis and acute glomerulonephritis. Renal biopsy showed diffuse messangial proliferative glomerulonephritis with C3 deposition on the basement membrane. The condition responds well to DEC therapy. ENDOMYOCARDIAL FIBROSIS Endomyocardial fibrosis is a rare diseasee seen in the equatorial belts. The incrimination of filarial infection in its causation is based largely on circumstantial evidence. The geographic distribution of the disease in areas endemic for filariasis, the detection of antibodies to Loa loa in patients with EMF, certain clinical features resembling fialrial infection and the occurance of eosiniphilia and EMF with Loeffler's syndrome have led to the hypothesis of EMF being filarial in origin. Further, Filarial antibodies have been detected in patients with EMF supporting the theory that EMF may be of fialrial origin. FILARIAL ARTHRITIS This is a form of arthritis which usually affects the knee joints and is fairly common in filarial endemic areas. Two types of filarial arthritis have been desbribed clinically 1.Oligoarticular filarial arthritis 2.Filarial pseudo-rheumatism Oligoarticular filarial arthritis: Typically affects just one large joint, most commonly a knee. Synovial fluid from the afffected joint does not contain microfilariae, adult worms or pyogenic organisms. Monoarticular inflammation may reflect a tissue reaction to a filarial worm in the vicinity of the joint. Rarely, lymphatic fistulation into the synovial sac causes chylous arthritis. Filarial pseudo-rheumatism: Less common in lymphatic filariasis than in onchocerciasis. Its pathogenesis involves intra-articular deposition of immune complexes. Intact microfilariae have been detected intra-articularly in some patients with filarial polyarthritis and the local release of proteases by the worms may directly damage synovial tissue. Majority of patients with filarial arthritis do not have fever but a painless swelling of one or more joints (usually the knee). Sometimes the affected joint may be painful, warm and tender with restriction of movement. The symptoms may recur, often in the same joint but occasionally in some other joint and may be mistaken for rhematoid arthritis. It is found that 90% of the patients with filarial arthritis tested are positive for filarial antibodies when tested with mf of W.bancrofti in a fluorescent antibody test These patients show normal or moderately elevated eosiniphil counts and erythtocyte sedimentation rates; X- rays of the involved joints show soft tissue swelling but no bony abnormalities. The antistreptolysin O titre is generally normal. FILARIAL GRANULOMAS IN THE BREAST This manifestaion is particularly prevalent in India and Srilanka where W.bancrofti is the predominant species. It has not been reported from areas endemic for Brugian filariasis. Filarial granulomas present as hard breast lumps attached to the overlying skin and are at times difficult to distinguish from malignant tumours. A histological examination can confirm the diagnosis by the finding of an eosinophilic granulomatous reation around the filarial parasites which are in varying stages of degeneration. Both adult worms and mf have been found in the granulomas. Filarial antibodies have been demonstrated in these patients and the condition responds to DEC therapy which, in many instances, can lead to complete disappearance of the lump. ---- Causes of Lymphoedema Primary Lymphoedema This is a genetic abnormality causing an insufficiency in lymphatic drainage. It can be present at birth, or more commonly occur at puberty or later in life. Pregnancy can cause a later onset or worsen the lymphoedema (because of the extra pressure of the foetus on pelvic lymphatic drainage). Sometimes lymphoedema may be of primary origin but the onset can be triggered by e.g. an injection or drip put into a limb "at risk" e.g. in the case of lymphoedema originally of one leg only, onset in the other leg can be precipitated by this. Sometimes this lymphatic deficiency is almost minimal and limbs can remain 'normal', i.e. in size, for years, but the onset may be triggered later in life by a traumatic incident. Secondary Lymphoedema This is due to an outside factor which reduces the drainage of the once normal lymphatics. Some causes are listed below to lymphatic drainage through the nodes. a. Cancer: This in itself can cause blockage. If a limb, or neck and face suddenly swells, this should be the first thing checked for especially after the age of 30 years. Patients should never be treated without a thorough investigation and diagnosis. b. Secondary metastases: As for cancer. Trauma Accidents: including bad fractures, where vessels may be severed or crushed. Surgery: particularly surgery for carcinomas, (including melanomas) where nodes are removed. Other forms of surgery that can cause lymphoedema are; liposuction, the stripping of varicose veins, knee reconstructions and other operations that may inadvertently damage the lymphatic system, or overload a somewhat reduced system that up to that point was functional. Radiotherapy: This is used to kill the cancer cells nodes, and thereby blocks them and can lead to lymphoedema. It may also cause burn and scarring leading to constriction of and damage to the lymphatics. In rare cases chemotherapy may also cause blockage of nodes. Paralysis:-especially if the patient spends much of the time in a wheel chair with their legs in a downward position Allergic reactions: These can include spiderbites, (especially white tailed or red-back spiders), cat scratch allergy, tampon shock, coral abrasions, and adverse drug reactions (only rarely), and occasionally by severe infections. Self - mutilation: drainage may be impeded and result in lymphoedema if a person applies a tourniquet to a limb. Filariasis Lymphatic vessels and valves are damaged and at a later stage may be blocked by the parasite (transmitted by mosquitoes). Lymphoedema will not be apparent until a number of years after the original infestation. Chronic Venous Insufficiency (CVI): Lymphoedema can occur in the later stages on top of the original venous oedema Lipoedema: Lymphoedema can occur in the more severe stage of this disease but does not always happen. AIDS: Lymphoedema can occur in the later stages. Benign growths: these can affect drainage e.g. lymphadenomas. Some other causes are: Leaking breast implants, (where silica lodges in the nodes) and podoconiosis (through entry via the feet), after deep vein thrombosis, and after severe mastitis where infection was not adequately treated. Where can lymphoedema occur? Whereas lymphoedema is usually diagnosed in limbs, it is often neglected or thought of as unimportant in other areas of the body. This may not seem of great importance to the treating physician or surgeon, but can make a great difference in the comfort, general well-being (including healing) and self-image of everyone who has to cope with this. Lymphoedema can occur in any limb, the face, neck and trunk, in one or both breasts, or in deep organs or the abdominal cavity. i.e. anywhere in the body. It is more easily recognisable if it appears in a limb but, because these drain to particular sets of nodes e.g. arms to axillary (under the arm) nodes and the legs drain to the superficial inguinal (in the groin - crease of the leg between the leg and the trunk) and then on to the deep pelvic nodes, the adjacent superficial regions of the trunk may also be involved. These also drain to the same nodal areas i.e. thoracic (chest) area both anterior and posterior (front and back) drains to the left axillary nodes from the midline to the left side and from the midline to the right axillary nodes on the right side. These include the breasts. Similarly with the superficial abdominal (again anterior and posterior i.e. front and back) which on the left hand side drain to the nodes in the left groin (sup. inguinal) and on the right to the right groin (sup. inguinal). This drainage divides superficial drainage of the trunk into 4 lymphatic quadrants (or lymphotomes i.e. drainage areas to the same outlet or set of nodes). Frequently this swelling is overlooked and sometimes not treated adequately and yet it is often this that causes as much of a problem as the limb swelling, and sometimes this is the only major problem. The "division" lines between these are called watersheds. However when an overload of one area occurs there are small collateral (just under the skin) lymphatic vessels that naturally cross these lines and extra lymph will flow to the adjacent areas (quadrants) thereby compensating for the extra load in one particular area. This may mean that these areas also can become overloaded and start to swell e.g. an opposite side of the chest and a normal breast, and/or the abdominal area below this area on the same side after a post- mastectomy operation. This can usually be easily rectified by improving the deep as well as the surface drainage that these adjacent areas drain to with correct massage. The affected area (lymphotome) can then have a free pathway for redirecting and improving drainage. Swollen or "lumpy" breasts can be quickly reduced especially if treated early before too much fibrosis occurs. This also applies e.g. to a lump or bulge under a scar under the arm on the side of the chest. Other scars (e.g. from completely different and previous operations) may limit where or how this drainage can be achieved. The genital areas also drain to the inguinal (or groin) nodes and if these are affected, swelling of this area may be a problem. Certainly this area is "at risk". Lymphoedema can also manifest as hydroceles i.e. a contained swelling in a particular area. These frequently occur in the genital region but can also be located in other areas. How does the body react to compensate for overload? Many things work to prevent lymphoedema from occurring. These are called 'safety-factors'. The superficial drainage across 'watersheds' which has been discussed above is one of these. At the interstitial level (between the muscles and the skin), both in acute injury and inflammation, or where a person is at risk of lymphoedema a number of factors come into play to try and prevent the onset of lymphoedema. In an inflammatory (or traumatic) situation, there is excessive leakage of water, protein and cells etc. from the blood vessels. The changes in tissue pressure cause an increased uptake by the lymphatic system. Tissues cells (macrophages) become more active and lyse (breakdown) excess protein. Protein is a stimulus for the formation of fibrotic (scar) tissue, so that anything that reduces this acute phase helps to prevent this occurring and thereby blocking the channels between the blood vessels and the lymphatics as well as the transport of oxygen which promotes general tissue health and healing. This is aided by the increased contractions of the collecting lymphatics, which have muscle in their walls, and thereby increase clearance. It is only when these mechanisms become overloaded and the lymphatic drainage cannot cope with the lymphatic load from the tissues that lypmhoedema occurs. Contrary to the theory held for the last 100 years, it would seem that much of the return of water (75-80%) to the blood system occurs at the nodes rather than back via the venous capillaries. If the nodes are either few in number e.g. a) as they may be in primary lymphoedema or b) have been removed by surgery (and the lymphatics thereby severed), then this will greatly affect removal of the excess tissue fluid and cause further and long-lasting pathological changes. Recognition, Diagnosis and Treatment of Lipodema Vs. Lymphoedema Lipodema is a chronic disease of complex causes, many of which we understand little about. These include hormonal imbalance, an inability to metabolise exudate from blood vessels so that those proteins and cells that would normally be metabolised and returned to the circulation are deposited as adipose tissue in the subcutaneous tissue. The blood vessels themselves are affected, venous stasis and vasoconstriction occur and return is diminished especially at the subcutaneous level. This causes arterial constriction which accounts for the cold and often pale skin and leads to an increased lymphatic load(1). The condition worsens over years as tissue channels become progressively narrowed until the condition of a secondary lymphoedema, overlying the original lipodema, may lead to a situation where the patient may be either wheel-chair bound or bedridden. Weight gains can be up to or greater than 250 kgs. At this stage infections and intractable ulcers (or ones that necessitate skin grafts when lack of oxygenation to the skin causes problems with healing) which can be due to the simplest injury e.g. careless donning of a compression garment or other minor trauma, occur with an even higher frequency. This condition occurs predominantly in women, and can in some cases be familial; the rare cases of men with lipodema always seem to include a hormonal imbalance which should be treated if possible. The macrophages themselves become overloaded and cease to play an active role in protein proteolysis and look like fat cells. Lipodema is often misdiagnosed as obesity in its earlier stages but the symptoms are clear and distinctly recognisable clinically from this. In its later stages it may be more difficult to distinguish from lymphoedema, although the case history and distribution of excessive tissue should provide an indication. The overlying condition of lymphoedema may occur in the later stages. Lipodema can occur in the legs, buttocks and also, but not necessarily, in the arms. It does not involve either the feet or hands until the onset of lymphoedema. The mean age of diagnosis is approximately 35-36 years but it usually starts at puberty. Symptoms of onset, however, can start in childhood, and may be clinically detectable in adolescence. Cellulite, which is a very mild form of lipodema, usually occurs towards or after menopause. It has been suggested that a mixture of primary lymphoedema as well as lipodema can occur in some cases. Differential Diagnosis Lipodema There is symmetrical bilateral enlargement of limbs, both in legs (with buttocks involved) or in both legs and arms, but with the obvious enlargement excluding the feet and hands, until the later stages. Enlargement is a gradual but continuous process. There is no pitting with pressure. The tissue feels more like "rubber", and is not as hard and fibrotic as in a grade II lymphoedema. It does not, in the early stages, reduce with elevation. There is pain on pressure, especially in the medial aspect of the thigh and the base of the spine. As the disease progresses, pain is often caused by the lightest of touches to the skin, particularly later in the day. Pain is also caused by "self pressure" e.g. crossing the legs or sitting with pressure on the spine. The abdomen may also be painful to pressure. Stemmer's sign is negative; i.e. a skin fold test done on the second toe. If it comes up as a thin fold test when "pinched", no lymphoedema is present. If it is a "lump" this indicates lymphoedema. Superficial capillaries are easily damaged i.e. the limb bruises easily. Skin temperature is lowered. Hands may feel clammy (damp), but limbs are cold. General nourishment of the skin is also affected and may cause "patchy" skin, dry in one place, oily in others in the early stages. Skin elasticity is reduced and it usually has an "orange peel" appearance. "Pins and needles" are common and movement seems to alleviate these to a large extent. A feeling of dizziness may be present. There seems to be a reduction of venous return in the legs when standing which can lead to fainting; this may be prevented by walking. There is little or no loss of weight with rigorous diet. Many of these patients have eaten low calorie diets for many years. In some cases their stomachs have been stapled because of misdiagnosis. In some of these cases, this seems to have lead to the onset of stomach cancer. It is not the answer to the problem! Obesity, caused by overeating, does respond to a proper dietary regime; lipodema does not. Joint pains (especially in knees) are common. Infection of the limb, either bacterial or mycotic is not normally a problem. Plantar support is reduced i.e. people have fallen arches. The shape of lipodema may vary, from a inverted "pear" shape (like a classical Greek column) to a more bulbous shape from the ankles upward. (Figs. 1-2.) This also usually involves the buttocks. Lymphoscintigraphy i.e. time of clearance of a radio-tracer injected into the feet to the inguinal nodes, is normal. Secondary Lymphoedema Accompanying by Lipodema in the later stages. Stemmer's sign becomes positive. Pressure will cause pitting, and there may be a small reduction with elevation. Folds of skin will further enlarge and feet will swell (Fig. 4.). If arms are affected hands also will swell. If the top of the body is affected the shoulders, thoracic and neck area may be affected as well. Infection may become a problem. Diagnosis can normally be achieved by the taking of a careful case history and clinical observation (see above). In the later stages lymphoscintigraphy may clarify this, but the picture is so different from primary lymphoedema that this should seldom be necessary. Psychological Problems All of these patients present with a variety of these problems ranging from lack of self-confidence to lack of confidence in their medical or health workers, often because of misdiagnosis and lack of sympathy, and then to real depression and anxiety and because of their appearance and the lack of understanding of the condition, particularly as their mobility decreases. The whole problem, of course, becomes worse if the onset of lymphoedema further exacerbates the condition. They are "blamed" for being overweight, told they eat too much or are "cheating" on their prescribed diets. If they are hospitalised for a weight loss program when the situation becomes very serious and they don't lose weight, they are often met by the comment "I am surprised" by their health care professionals and are summarily dismissed as "patients for whom nothing can be done" or "we don't know what is wrong with you" or "you'll just have to live with it". This is not helpful to the patient who should be made aware that the condition is a genetic abnormality and that their obesity is not their fault. Of course, up to a point dieting can help but it will never cure this condition. They obviously, and for good reason, become discouraged and dismayed by their problems, which seem not even to be recognised. When lymphoedema occurs on top of the lipoedema this is a situation which is almost a problem that is so great for them (and again often undiagnosed as such) that they need to be very strong people to cope with it. Sadly, many are not able to do this. Psychological counselling can be helpful; for this poorly understood condition it is seldom offered. Invalid Pensions are not the answer for those that have, until they could not, lead an active and productive life. In many centres in Australia and I am sure worldwide, these patients are turned away from treatment centres (for lymphoedema) as untreatable cases. In some cases they resort to surgery in a final effort to improve their condition. The result of some of these operations (including liposuction and limb reduction) are so appalling that they have to be seen to be believed. Treatment Lipodema can be treated and reduced with careful massage to the normal nodal groups after the truncal areas have been precleared i.e. the superficial inguinal and axillary nodes, then gentle superficial drainage towards these. and compression applied to the legs in the form of bandaging as garments. Although not as easy to treat as is lymphoedema, considerable reduction, easing of pain and improvement in mobility, can be of huge physical and psychological benefit to the patient. Compression bandaging is tolerable, especially after a few days of massage. The overlying lymphoedema, if present, can be greatly reduced. The improved mobility will increase the ability to exercise which will help the calf muscles pump and increase venous and lymphatic return. After the initial decongestion by manual drainage permanent compression causes a significant reduction in adipose tissue and also has a positive influence on the disturbed veno-arterial response. Benzo-pyrones seem to help this condition considerably, presumably by their stimulation of macrophage numbers and activity. Many patients have reported a considerable weight gain when coumarin became unavailable in Australia. Interestingly, the Italian product CellaseneÔ which is recommended for cellulite, contains benzo-pyrones and other plant extracts that work in a similar way to benzo-pyrones, so despite medical scepticism, this may help in these conditions. Unfortunately the cost of these and other available benzo-pyrones are too high for many people who would benefit from them. The only diet which may help is a very low protein only diet (250 mg per day) (and nothing else, except, of course, water), which will put the body into a state of ketosis where some of the excess fat may be metabolised. However this usually results in weight loss in already lean areas e.g. the waist and often the upper body. Operative procedures do not attack the cause of the problem. Careful liposuction may produce immediate reduction but considering the destruction of tissue it causes, long term results have not been clinically proven. Other reduction operations are contraindicated. Pumps are normally intolerable because of the pain they cause, and there is no published evidence of them ever being successful in this condition. Lymphoedema. Causes of secondary lymphoedema are frequently obvious from case histories and have been discussed already. However primary lymphoedema varies from lipodema in the following aspects. 1. Swelling is a-symmetrical. Indeed, often only one limb is affected, and the swelling clinically apparent (Fig. 5.). If a leg is lymphoedematous the foot is involved. The hand is usually involved with primary lymphoedema of the arm. Lymphoedema all over may present as more symmetrical but the feet are involved from onset. 2. In the early stages pitting may be present and it may reduce with elevation. 3. It is not painful on pressure. The only time pain is experienced is during an episode of infection. If swelling is rapid in the early stages of secondary lymphoedema this is frequently painful. 4. Stemmer's sign is positive. 5. The limb does not bruise easily, as it does in lipodema. 6. Skin temperature is higher in the lymphoedematous limb/s. 7. "Pins and Needles" are rare in primary lymphoedema, (although both these and paraesthesia may occur in secondary lymphoedema). Venous return is usually normal. 8. Dieting will not reduce primary lymphoedema. 9. Knee joints may be affected by both lymphoedema and extra leg weight and may cause pain such as arthritis. The condition is often diagnosed as this and under investigation is pathologically similar. 10. Infection (both bacterial and mycotic), especially in the later grades of lymphoedema may be a considerable and ongoing problem, and cause an exacerbation of the lymphoedema. As lymphoedema progresses from Grade I, there is excess fibrotic tissue (collagen), adipose tissue, (especially in primary lymphoedema), and a proliferation of other cellular and interstitial tissue elements. The limb ceases to pit with pressure, feels hard to the touch and much less "rubbery" than pure lipodema. As with lipodema, diagnosis can be clarified both with case histories and lymphoscintigraphy. Psychological Problems Lymphoedema can cause psychological problems as well as lipodema. These range again from concern and depression about appearance, to anxiety about the worsening of the condition and of infection, to depression, break-up of relationships with partners, especially if genital lymphoedema is involved etc. etc. This is balanced by the fact that many patients are now aware that some forms of treatment can be obtained, especially if they can afford it, or have Health Cover to assist them. In many areas the public patient is very poorly catered for. Despite these problems lymphoedema patients are usually less "psychologically fragile" than those with lipodema, partly due to the psychological abuse and mockery that many of the latter have suffered. Treatment Complex decongestive therapy (skin care, lymphatic drainage by massage, compression and exercises) are accepted as the best treatment for lymphoedema. Because of the inadequate lymphatic drainage and lack of nodes and with abnormal and fewer lymphatic vessels, drainage needs to be taken to truncal quadrants where lymphatic drainage is more normal. This of course depends on the individuals situation. What drainage that does exist in a limb should also be enhanced by massage. Pumps should never be used in primary lymphoedema. Surgery, unless in very skilled hands, is seldom beneficial in the long term. The following figures illustrate a few of the typical differences between lipodema in its various stages (Fig. 1- 3.), and primary lymphoedema of one leg (Fig. 4.). Arm illustrations are not included but for lipodema show bilateral symmetrical swelling as against a uni or bilateral a-symmetrical condition in primary lymphoedema. 1. Lipodema- early stage. Sometimes this shape simply enlarges with the medial aspect of the thigh and buttocks increasing more than the lower leg. 2. Lipodema- later stage. 3. Lipodema with overlying lymphoedema. At this stage the genital area may also be affected. 4. Primary lymphoedema of one leg only. In some cases both legs may be affected but swelling is a- symmetrical. It may or may not include the gluteal region. New Advances In Understanding Primary Lymphoedema DeCourcy Squire, P.T. Lymphedema Therapy Centre, Inc U.S.A. A child falls and develops swelling which does not go away. A teenager thinks he has a sprain–but gradually his whole leg becomes swollen and the other ankle starts to swell too. A mother notices that during each pregnancy her legs swell–but after the third pregnancy, the swelling remains, and after tests to rule out a blood clot, the swelling worsens significantly. Primary lymphoedema–sometimes called "idiopathic" (of unknown origin)–has for many years been the least understood and most often undiagnosed or misdiagnosed form of lymphoedema. Now, exciting breakthroughs in medical research are helping us to identify and understand some of the underlying genetic causes of certain types of primary lymphoedema. What is Primary Lymphoedema? Primary lymphoedema differs from the more common secondary lymphoedema. Secondary lymphoedema is acquired: the cause is an injury to the lymphatic system (from cancer treatment where lymph nodes are removed or radiated; from filariasis, where parasitic worms get into the lymph vessels; from severe traumatic injuries; from longstanding venous problems, etc.) In primary lymphoedema, the problem is one that is present from birth: an undeveloped or malformed lymphatic system. *An estimated 1 person out of 6000 is born with primary lymphoedema. *Swelling may be obvious at birth, but for about 75-80% of the people with primary lymphoedema, it does not develop until later. When it develops in childhood, adolescence, or early adulthood, it is termed "lymphoedema praecox." After the age of 35, it is known as "lymphoedema tarda." *Sometimes the onset is gradual and sometimes it is sudden, triggered by a seemingly minor injury or infection. *It can run in families ("Oh, you just have Aunt Mildred’s legs,") but more often (an estimated 80% of the time) it can appear out of the blue, with no traceable family history. *It affects twice as many women as men *It appears in legs four times more often than arms *In about 70% of the cases, it affects only one leg, usually starting in the foot, ankle, and then calf. However, it can affect both legs, including the thighs, as well as the trunk, genitals, arms, and face. A study (Casley-Smith and Casley-Smith, 1995) found that primary lymphoedema of the leg and secondary lymphoedema of the leg tended to get larger at about the same rate (which was slower than the progression of swelling of the arm). However a leg with primary lymphoedema often did not become fibrotic as quickly as a limb (arm or leg) with secondary lymphoedema. It’s possible a limb with primary lymphoedema may have more fatty tissue. What Causes the Swelling in Primary Lymphoedema? The lymphatic system varies a little in everyone: the number and size of lymph nodes, the number, exact location and specific direction of lymph vessels, and so on. When these variations become more extreme, a person can be at risk for lymphoedema. Some of the problems found in primary lymphoedema (whether hereditary or not) are as follows: 1. Too few initial lymphatics. The initial lymphatics are the start of the lymphatic system. They are tiny finger like projections in the dermis. They have flap-like valves that open and close to allow tissue fluid, proteins, and other large molecules, cells, germs, and debris to enter. The initial lymphatics are arranged in a mesh in the skin all over the body. They lead to a network of pre-collectors which connect the initial lymphatics to the collecting vessels (collectors). When there are too few initial lymphatics in a particular area of the body, the tissue fluid and its components, including protein, is not able to enter the lymphatic system. It stagnates in the tissues causing a high-protein oedema. 2. Too few collectors (or too small). The collectors are in the tissue just below the skin. They travel up the limb to the lymph nodes, which are located throughout the body. When there are not enough collectors, the lymph fluid gets backed up in the pre-collectors and can reflux out the initial lymphatics and into the tissues again. 3. Too many collectors (or too large). Although it doesn’t seem as if this could be a problem, it is when the collectors become a tangled winding mass, not really leading anywhere. The collectors are made up of units called lymphangions. At the beginning and end of each lymphangion is pair of valves which open to allow lymph fluid to pass from one lymphangion to the next and which prevents backflow of lymph fluid. With an enlarged collector, these valves may not be able to meet and close. If the valves can’t work, then instead of lymph fluid moving to the nodes, it can also move backwards and reflux into the tissues. 4. Too few lymph nodes, or malformed nodes. The purpose of the nodes is to filter the lymph fluid, removing and destroying anything that is harmful, before the lymph fluid is returned to the blood stream. A blockage here will back fluid up through the collectors which become distended and incompetent. This eventually leads to reflux into the tissues which results in swelling. An examination of 46 people with primary lymphoedema of the leg (Fox et al) found that 41/46 had problems in the lymph nodes located behind the knee. These were found to be hardened and malformed. This would seriously affect lymph flow from the calf and lateral ankle. A lack of functional nodes would also affect the effectiveness of the immune system, leaving the person more prone to infections. A vicious cycle can begin. Persistent local infections of a limb can damage or destroy lymph collectors and eventually the lymph nodes. It can make the tissue in the area of the infection hard and fibrotic. This leads to less fluid being carried to the nodes and fewer nodes to filter it, thus hastening the onset of lymphoedema or worsening it. 5. A problem with abdominal lymphatics or a constriction/blockage at the thoracic duct. There are also initial lymphatics (called lacteals) and collectors in the intestinal tract and the other organs. The collectors mostly go to the largest lymph trunk in the body, the thoracic duct. The thoracic duct passes through the diaphragm, up the body to the supraclavicular nodes. There the lymph is returned to the bloodstream through a connection with the subclavian vein. Along the way from the abdomen, the lymph is filtered by numerous nodes. The thoracic duct carries not only the lymph from the various organs but also lymph from both legs, the lower trunk of the body, and the upper left half of the body, including left arm and left side of the head. A problem with the abdominal lymphatics or with the thoracic duct can have much more serious consequences than lymphoedema of the limb. It can lead to conditions of chylous reflux (the backflow of lymphatic fluid from the intestines to other areas of the body) and protein-losing enteropathy (PLE). In PLE instead of plasma proteins being returned to the blood stream, they are lost in the intestinal tract and excreted. This leads to a lymphoedema of the intestinal wall and a generalized swelling of the body. It is a critical condition which requires medical intervention and a special diet. What Are the Different Kinds of Primary Lymphoedema? There are many different kinds of primary lymphoedema. Some are hereditary, but most are not. The list is far from complete. There are many syndromes which include lymphoedema, but they are rare syndromes. Listed below are a few of the identified syndromes. Idiopathic Lymphoedema -Not Running in Families This is the most common form of primary lymphoedema. As the name implies, this is primary lymphoedema of an unknown origin, and is not a part of a syndrome of other symptoms. It appears at birth or later on in an individual (more often female). Although it most commonly affects one lower extremity, it can affect any part of the body. It is not found in other family members because it is not hereditary and cannot be passed on to children. Lymphoscintigram findings will not show an obstruction as it would in acquired/secondary lymphoedema. Instead usually it will indicate a hypoplasia (underdevelopment of lymphatic vessels). Primary Hereditary Lymphoedemas: Lymphoedemas Which Run in Families Milroy’s Syndrome [also called Nonne-Milroy Syndrome]: Lymphoedema present at birth. Often the problem is a lack of initial lymphatics. [Autosomal Dominant,* chromosome 5] Meige’s Syndrome : Like Milroy’s, but the lymphoedema does not appear until later: lymphoedema praecox (before 35) or lymphoedema tarda (after 35). [Autosomal Dominant] Primary Hereditary Lymphoedemas with other symptoms Yellow Nail Syndrome [Samman-White Syndrome]: Syndrome which includes discoloured thickened nails, pulmonary problems, and lymphoedema. Onset is usually childhood or early adulthood. [Autosomal Dominant] Sharp-Aagenaes Syndrome: Distinguished by neonatal cholestasis (stoppage of bile excretion) with jaundice. The lymphoedema develops in early childhood and is equally seen in males and females. [Autosomal Recessive,* chromosome 15] Lymphoedema with Distichiasis [Falls-Kertesz Syndrome]: As well as lymphoedema, there is an extra row of eyelashes (distichiasis); problems also include a widened spinal canal and other related problems. Onset is usually adolescence. [Autosomal Dominant, chromosome 16] Avasthey-Roy Syndrome: As well as lymphoedema, there are arteriovenous malformations and pulmonary hypertension. Onset is usually adolescence. [Autosomal Dominant] Hennekam’s Syndrome: Lymphoedema of face, genitals, and limbs; the face and nose are flat, the mouth narrow, the chin large, the ear malformed, the eyes protruding, the fingers can be webbed, the thumb large. There can be mild mental retardation. [Autosomal Recessive] Noonan’s Syndrome: Includes webbed neck, protruding upper chest, receding lower chest, cardiomyopathy, short stature. Appears very similar to Turner’s Syndrome, below, except is not sex- linked.. [Autosomal dominant, chromosome 12] Jeken’s Syndrome: Includes mental retardation, abnormal fat distribution at buttocks, and ataxia. Lymphoedema onset is during infancy. [Autosomal recessive, chromosome 16] Figueroa Syndrome: Cleft palate; lymphoedema starts during childhood or adolescence. [Autosomal dominant] Primary Lymphoedemas Associated with other Syndromes- Do Not Run In Families Klippel-Trenaunay-[Weber] Syndrome: Venous and arterial alterations are present; when the lymph vessels are involved, they are frequently varicose. In Weber’s Syndrome, the bones of a limb are also hypertrophied (larger).[not hereditary] Turner Syndrome: Affects only females because it is sex-linked.* Frequently the lymphatic system, (specifically the valves), is underdeveloped, resulting in childhood lymphoedema. This sometimes resolves by adolescence. Other features of Turner’s Syndrome include short stature, infertility, and sometimes problems with the heart, kidney, or thyroid.(XO instead of XX chromosome; not hereditary] *Some Basic Genetics Terms: Autosomal: a gene that is not on the sex-linked chromosome(x and y are the sex-linked chromosomes) Dominant: only one of these genes must be present for the trait to exhibit itself Recessive: the gene must be present from both parents for the trait to exhibit itself; a person with only one of the genes is said to be a carrier. Incomplete penetrance: the situation when the dominant gene or two recessive genes are present, so the person should exhibit the trait, but in a certain percentage of cases, this does not happen. What is the Treatment for Primary Lymphoedema? Currently, the best treatment for primary lymphoedema is the same as for secondary lymphoedema. While problems such as chylous reflux and protein-losing enteropathy (see #5 above, Causes of Lymphoedema) may require surgical and other medical intervention, problems 1-4 can be effectively treated using Complex Lymphatic Therapy (also called Complete Decongestive Therapy and a number of other phrases). CLT consists of the following components: 1. Meticulous skin care and precautions to prevent infections and further damage to the lymphatic system; 2. MLD (manual lymph drainage), a gentle massage-like manual technique to move fluid out of swollen areas into regions with working lymph nodes; 3. Gradient compression wrapping with low stretch bandages and padding, to help soften tissue and to keep fluid from refilling, after it has been moved out with MLD; 4. The Lymphoedema Association of Australia exercises, which help stimulate lymph circulation; 5. The use of benzo-pyrones, where allowable; 6. Instruction in a home program. The home program includes similar elements to CLT: a. A continuation of skin care and precautions; b. Self- or carer-MLD; c. Compression around the clock, but now a compression sleeve or stocking can be worn during the day instead of the bulky compression wrapping; d. The LAA exercises; e. The use of benzo-pyrones, where allowed; f. Follow-up visits with the therapist to monitor progress The main difference from treatment for secondary lymphoedema is during MLD. If there are functional regional lymph nodes, these can be used, in addition to using MLD to move some of the excess fluid to lymph nodes in other areas of the body. When many different parts of the body are affected ("lymphoedema- all-over") the emphasis will usually be to use the normal pathways to clear the trunk extensively. What is New in Research on Primary Lymphoedema? In the last few years, more information about primary hereditary lymphoedema has been emerging which may have hopeful implications for the treatment of all lymphoedemas. Now advances in DNA technology have enabled researchers to make much more detailed findings than was possible before. What the researchers do first is to register and gather data on families with lymphoedema. A family tree of as many generations and members as possible is composed, with information about whether there was no swelling, mild swelling, or marked swelling (which tends to be variable within families), age of onset of the lymphoedema (which tends to be similar within families), site of swelling, associated medical problems and physical anomalies, etc. In the past, research has been made more complicated because the primary hereditary lymphoedemas express themselves in many different ways. Even in the same family, one member may have only one foot swollen, another may have both legs involved, and still others may not exhibit any symptoms, even though some of them carry the genes. From family to family with primary lymphoedema the causative gene may differ and the other symptoms associated with the lymphoedema may be different. [See above for a list of some of the identified syndromes of primary lymphoedema.] Now researchers are able to use DNA to check for linkages. By comparing the DNA samples of members with and without lymphoedema, they are able to trace which chromosome is involved, and the location of the specific gene on the chromosome. Thus, researchers have been able to pinpoint the gene for Milroy’s Syndrome (primary hereditary lymphoedema, with swelling present at birth) to a specific location on Chromosome 5 and to identify the mutation that caused lymphoedema to the Vascular Endothelial Growth Factor Receptor 3 (VEGFR-3.) The chromosomes and genes for other syndromes are slowly being uncovered as well. What this opens the way for is the possibility in the future of a genetic test for lymphoedema. This could help identify which family members are at risk for developing lymphoedema or for passing it on. This could greatly help those at risk for it take precautions before developing it, and those definitely not at risk would not have to worry about such things as overheating, air travel, and over-exertion. In the long run, there is the exciting potential to develop drugs that would stimulate the specific receptors and growth factors responsible for the lymphatic system. This could lead to a regrowth of underdeveloped or damaged lymphatic vessels. Separate research is currently underway looking at the potential for lymphangiogenesis: the stimulating of the (re) growth of lymph vessels. Advances in these areas could be helpful not only for hereditary lymphoedema, or even just primary lymphoedema, but for all lymphoedemas. Although it is still many years away, for the first time there is the real hope in the future for a cure for lymphoedema! Resource List: Casley-Smith, J.R., and Casley-Smith, J.R. Modern Treatment for Lymphoedema 5th Ed., Lymphoedema Association of Australia, 1997, pp. 46-50, 74-75, 94, 147, 148-149 Evans,, A, Brice, G, Sotirova, V, Mortimer, P, Bennison, J, Burnand, KL, Rosbotham, J, Child, A, Sarfarazi, M, "Mapping of primary congenital lymphedema to the 5q35.3 region." American Journal of Human Genetics, Vol.64, pp. 547-555, 1999. Finegold, D. "New Developments in Genetic Research in Lymphedema", presented at the NLN Conference, Orlando, 2000. Foldi, M., and Casley-Smith, J.R., eds. Lymphangiology, Schattauer, 1983, pp. 235-239;263-267;772; 777-782 Hennekam, R. "Syndromic Lymphatic Maldevelopment", presented at the NLN Conference, Orlando, 2000. Levinson, K, "Recent Advances in the Genetics of Lymphedema," Lymph Link, Vol. 12 #4, Oct.-Dec. 2000 Olszewski, W., Ed. Lymph Stasis: Pathophysiology, Diagnosis, and Treatment, CRC Press, 1991, pp.379- 386 Papendieck, C.M., "Angiodysplasia of etheLymphatic System in Pediatrics: Classificaiton, Signs and Syndromes", Presented at NLN conference, Orlando, 2000. Weissleder, H. and Schuchhardt, C., eds. Lymphedema: Diagnosis and Therapy 2nd Ed., Kagerer Kommunikation, 1997 pp. 75-79, 222-234 -------------------------------------------------------------------------------- BENZO-PYRONES The benzo-pyrones comprise a large group of organic compounds (Vitamin P). They all work the same way in the reduction of oedema by reactivating the macrophages and increasing the number of these cells that migrate to the site and lyse protein. The different compounds in this group are metabolised through different liver pathways; some have other effects as well. Dicoumarol, for example, is used as an anticoagulant agent (and although it has been proven to reduce oedema) clearly should never be used for this. An update was done on the availability of these in the 1999 Newsletter. Since then we are delighted that topical coumarin (i.e. applied to the skin) has become available on the Australian and Italian markets. No serious side effects have ever been reported with topical application. (In all the time it was previously available, only 2 patients reported a skin reaction to the product). The metabolism is different from the oral form. A topical product was withdrawn, not because of any contraindications, but because, as a prescription item, the government wanted more efficacy trials done and this was impossible for us to organise at the time. In Australia it is now available without prescription as Lymphoedema cream 75 ml. and Lymphoedema powder 50 gm. from: Poppy Lane Skin Care and Lymphoedema Clinic, Phone (02) 9723 5402 Fax: (02) 9726 3322, email okino@rivernet.com.au The web purchase site for powder & creams is http://member.rivernet.com.au/okino/products.html From Italy, the powder only "Linfovenodren" (Coumarin) 50gr powder is available with prescription from SMA s.a., Via Tre Settembre, 11-Dogana (Rep. San Marino); tel: 00378-909532; fax 00378-909547 The new change of email for Pharm Products who supply 'Lypedim' ä (200 mg. coumarin tablets) is ppplm@md4.vsnl.net.in and pharm@tr.dot.net.in Bandage Winders (made of stainless steel) are available from: Ken Retallick Engineering Mobile Australia, 0417 218 906 redlinek@hotmail.com NEW PRODUCTS Garments are available in India from: Norma D.N.D. Products, 1111, Vikas Kunj, Vikas Puri, New Delhi - 110 018 Ph 559 5287, 554 5324, 552 1239, 554 6708 Fax: 011-552 1239. email: normadnd@vsnl.com Sutherland Medical make a full range of bandages suitable for lymphoedema at very competitive prices. They will export to New Zealand and Asia. PO Box 1194 Huntingdale, Vic 3166. Freecall 1800 677 054, Freefax 1800 356 338, email suthmed@ocean.com.au, Http://www.suthmed.com.au Custom made shoes are available if needed in Australia from Doug and Joan Newlyn, 23 Brenda Tce, Largs North SA 5016 ph/fax (08) 8248 4028 and from Custom Fit Australia P/L 864 Nepean Hwy., Moorabbin, Vic. 3189 ph (03) 9532 2611, fax (03) 9532 1289. Tracey Goodrich makes a very soft sheepskin car seat belt cover that is wonderful for people with either chest and arm or abdominal problems. She originally made them for comfort during pregnancy, but they are a bonus for many people, especially the elderly and are a safety factor in preventing injury to older children under accident condition. She has offered a donation on sale to the L.A.A. if you mention this when ordering. The names and packaging of Hamilton Laboratories (Australia) Skin care range of products have changed. Contact your local pharmacies for updated information. http://www.lymphoedema.org.au/news_let.html Lymphedema is a chronic condition that many doctors do not even diagnose properly for years. Usually the patient goes untreated, worsens to the point of being disabled, and winds up looking for answers online. The specialists who treat lymphedema are called lymphologists, and there are not enough of them in the world to treat patients. Often many of them wind up teaching therapists how to treat patients. If you have lymphedema, know you are not alone. The basic ways to take care of your condition are: 1. Elevate your legs and arms as much as possible. If you have lymphedema in the trunk of your body or neck, laying flat is the best position because it allows the fluid to circulate better. When you have a limb that is swollen you want the fluid to move out of that area and circulate, for middle parts of the body or neck you want the fluid to even out and circulate evenly, so laying flat is best. 2. Keep clean! You want to your swollen areas bacteria and germ free. You have alot of stagnant fluid in those parts so letting more bacteria build on them is the perfect set up for infiltration into the pores and thus a cellulitis (staph or strep infection) episode to develop. 3. Be careful of injuries. Injuries can spead lymphedema. If you have lymphedema in your left foot and you injure your left knee, that's a set up for more lymphatic obstruction. The knee will swell and restict fluid movements and the popiteal lymph node behind the knee will become overloaded and maybe damaged thus setting up lymphedema to spread now up to the knee or beyond. 4. Infections! If you think you have any type of infection, get to the doctor, ER, or immediate care center pronto. Infections in stagnant areas can be strong ones called cellulitis and can spread very quickly to the bloodstream and thus be fatal. 5. Try to get therapy. Therapy is the gold standard treatment for lymphedema. A therapist actually works on your body with a back and forth routine to different lymph drainage patterns and manually moves the fluid out of the stagnant areas. It's called manual lymph drainage which is part of decongestive therapy. Therapy also includes wrapping your leg, the skin care mentioned above, special exercises that are designed to move fluid out of stagnant areas, and self massage at home in which you learn to move the fluid yourself. With doing these few things you can dramatically improve your quality of living with lymphedema. |