Related terms: lymphatic obstruction, swelling, edema, Milroy's Disease or Milroys Syndrome, Lymphedema Praecox Meige Syndrome, Lymphedema Tarda, Foot lymphedema, hand lymphedema; cellulitis, lymphedema, manual decongestive therapy, complex decongestive therapy, Hereditary Lymphedema 1, Hereditary Lymphedema 2, primary lymphedema, secondary lymphedema, congenital lymphedema, lymphoedema.
Lymphedema is defined as the swelling of a leg, foot, arm, hand or even abdomen and chest as a result of damages to or devlopment of an incomplete lymphatic system.
It is estimated that 120 million people throughout the world suffer from this condition in one form or another. Furthermore, tens of thousands are added each day. Let's explore and learn together about one of the least understood, but most prevalent, medical conditions that exists today. The great news on Lymphatic Filariasis is that a global action program is having extensive success in helping to eliminate this particular type of lymphedema through massive antibiotic “inoculaton” program.
As you study this page and find you have undergone any of the any of the risk factors for lymphedema, you will want to also immediately familiarize yourself with the warning signs of lymphedema. You should then, immediately get a referral to a certified lymphedema therapist for a complete examination and evaluation.
Lymphedema is manageable, treatable and you can live a long, meaningful life with it. One of the saddest things to me, is that someone is so overwhelmed upon finding out they have lymphedema (LE) that they completely withdraw from the enjoyment of life and isolate themselves.
Life is a celebration of that which we can do, not a requiem for that which we can not do.
Updated Jan. 22, 2012
The lymph system is one of your body's circulatory systems. It is composed of lymph vessels, lymph nodes, and organs such as the bone marrow, spleen, and thymus, and is also believed to include tonsils. Its function includes the absorption and elimination of excess fluids and it assists in the absorption of fat. Another very important function is that it is critical to our body's ability to fight infections and is critical in the immune system functions. When this system is not formed correctly, is damaged through injury or infection, or is removed (nodes), then lymphedema is a very real possibility.
To understand our immune system, see The Lymph System and Immunity.
Please see what causes the swelling in primary lymphedema for a detailed explanation.
As mentioned previously, lymphedema is a condition that occurs from a damaged or dysfunctional lymphatic system. There are two different types of lymphedema: primary and secondary. Lymphedema can affect not only the legs and arms, but also the abdomen, neck, head, face, eyelids, and even from scar formation.
Lymphedema for the newly diagnosed is usually traumatic, confusing (if not be bewildering), and frustrating as you seek to discover why you have it, how do you treat it, and how do you live with it.
In addition to our information on lymphedema and lymphedema treatment, you may want to become a lymphedema advocate right in your own community by learning how to start a lymphedema support group and by learning how to plan a local lymphedema event.
It is incredibly important for lymphedema patients to take the time and interest to learn about this condition. See Lymphedema Patient Education for some thoughts on the importance of patient empowerment through solid evidence-based medicine.
See also: An Etiological Study of Lymphedema for a study done on the distribution of lymphedema between primary and secondary.
Primary lymphedema can be hereditary. Milroy's Disease or Syndrome will generally express itself at birth or in the very early years. Meige Lymphedema, also known as lymphedema praecox, generally begins sometime during puberty. Lymphedema tarda begins in or around middle age. Lymphedema that has not expressed itself in an active condition is referred to a latent lymphedema. Primary lymphedema can also be congenital. This means something, either in utero or during birth, caused lymphatic damage.
For more comprehensive information please see: What are the Different Kinds of Primary Lymphedema
Secondary lymphedema is generally caused by an obstruction, damage to, or injury to the lymphatic system that leads to an interruption of the normal lymphatic flow.
Hereditary lymphedema is an inherited disorder of the lymphatic system that is characterized by abnormal swelling of certain parts of the body. The lymphatic system is a circulatory network of vessels, ducts, and nodes that filter and distribute certain fluids (lymph) and blood cells throughout the body. Lymphatic fluid collects in the soft tissues in and under the skin (subcutaneous) due to the obstruction, malformation, or underdevelopment (hypoplasia) of various lymphatic vessels.
There are three forms of hereditary lymphedema: Congenital Hereditary Lymphedema or Milroys Syndrome (Disease), Lymphedema Praecox or Meige Disease, and Lymphedema Tarda. In most cases, hereditary lymphedema is inherited as an autosomal dominant genetic trait.
The cause of hereditary primary lymphedema has been isolated to a malformation or break in two known genes. These are the FOXC2 and VEGFC genes. There are now identified several genes involved in causing lymphedema. These include not only FOXC2 and VEGFC, but CCBE1, FLT4, GATA2, GJC2 and SOX18. There are more that have yet to be identified. Causes of congenital primary lymphedema can be a developmental disorder of the lymphatics, in utero infection, or injury and/or delivery difficulties.
The causes of secondary lymphedema are multiple. Infections from insect bites, serious wounds, or burns can cause lymphedema when they damage or destroy lymphatics. Any type of surgery, serious injury, or radiation for cancer treatments can also cause the onset of the disease. Outside the tropics, the number one cause of secondary lymphedema is the removal of lymph nodes for cancer biopsies. For these patients, it can begin as a slight arm or leg swelling after cancer. Left untreated, it can become a serious and debilitating medical condition. Hopefully, with the improved techniques of performing a small needle biopsy, radiological diagnostic improvements, and site-specific node biopsies, we will see a marked decrease in this type of lymphedema.
In the tropical climates, the most common cause of lymphedma is infection from filarial worms. The worms are usually transmitted to humans by mosquito bites. This parasite then grows, eventually blocking and destroying the lymphatic system. This is called Lymphatic Filariasis.
It is important to distinguish the difference between secondary lymphedema and malignant lymphedema.
Who is at risk for lymphedema? What are the risk factors for lymphedema? Is there an answer to how to prevent lymphedema? Anyone who has one or more of the following risk factors has the potential to develop lymphedema.
If you are a person with any of these risk factors, you will want to learn the warning signs of lymphedema.
The question of how to diagnose lymphedema is fairly simple and I am always dismayed at the frustration, emotional upheaval, and often downright degrading experiences of lymphedema patients seeking that diagnosis.
The path to diagnosis should involve several steps.
First, is the physical examination itself. If a patient presents with unexplained limb swelling, then it should be self-evident that there is a medical problem that needs and requires answers and resolution. There is something called the “Stemmer's Sign.” This is a thickened skin fold at the base of the second toe (or finger). The presence of this sign is an early diagnostic indicator 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 test does not exclude lymphedema.
Another diagnostic method is evaluating the patient for pitting edema. This is a test where the physician presses a finger against the swollen limb. If it leaves an indentation, then that is a good indication of stage one lymphedema.
Other physical indications could include papillomas on the limb, placques, or nodules and even a cobblestone skin appearance. These are common with lymphedema.
The physical examination is also critical to rule out other medical conditions that can cause edema (swelling) that is not lymphedema.
The second step should be a complete medical history. The physcian should find out if there has been any history of cancer and/or its treatment, insect bites, traumatic injuries, or serious infections. These are all documented causes of lymphedema, as is morbid obesity.
Third should come a complete family history. The physician needs to ask if the patient knows of any family members (even extended family) who had what is called milk leg, stump leg, or elephant leg. These are old archaic terms people often used when describing lymphedema.
There are also many radiological tests that can be done to both rule out other conditions and/or to confirm lymphedema. These include ultrasounds, venograms, magnetic resonance imaging (MRI), and computed axial tomography (CAT scans). There is also the gold standard of lymphatic imaging tests called the lymphoscintigraphy. Lymphoscintigraphy replaced the old outdated test called a lymphangiogram. In the lymphoscintigraph, a tracer agent is injected between the toes and is traced as it journeys up the lymph system. This can pinpoint missing nodes, damaged vessels, or lymphatic blockages.
The preferred treatment today is decongestive therapy. The forms of therapy are Complete Decongestive Therapy (CDT) or Manual Decongestive Therapy (MDT). There are variances, but most involve these two types of treatment.
With these massage treatments, swelling is reduced and then the patient is fitted with a pre-measured custom pressure garment to keep the swelling down. Some patients are able to use ready-made sleeves, gauntlets, or hose that substantially reduce the cost of acquiring and replacing garments.
Manual Lymphatic Drainage (MLD) is a unique, therapeutic method of stimulating the movement of fluids in the tissues. The gentle, rhythmic, pumping, massage movements follow the natural direction of lymph flow and produce rapid results. It assists the cutaneous lymphatics in picking up and removing not just fluids, but all the waste products, protein particles, and debris from our system. It also is successful in breaking up fibrosis and fibrotic areas of a lymphodemous limb.
This treatment was created and developed by Danish therapists Dr. Emil Vodder and his wife, Estrid, in the 1930s, and was introduced in Paris in 1936. They are also credited with creating a specialty of medicine called Lymphology.
First brought to North America in 1982, the school is located in Victoria, British Columbia, Canada. Before it was introduced, the standard treatment course in North America was either a surgery called debulking or the use of compression machines wherein the limb was literally squeezed by pneumatic air pressure.
Comprehensive Decongestive Therapy (CDT) is used primarily in the treatment of lymphedema and venous insufficiency edema. It is a combination of MLD, bandaging, exercises, and skin care. CDT may also involve breathing exercises, compression garments, and dietary measures. A frequent indication for CDT is lymphedema caused by irradiation or surgery due to cancer. It can relieve edema, fibrosis, and the accompanying pain and discomfort.
Also known as Complete Decongestive Physiotherapy (CDP), this treatment therapy was pionered in the United States by Dr. Robert Lerner.
To ensure you receive the proper treatment by a certified therapist, you will need to know how to choose a lymphedema therapist, including what questions to ask and what certifications and training he has received.
One common medicine that lymphedema patients speak of using is a diuretic. It is important to understand that diuretics are not for lymphedema.
Pneumatic compression pumps have long been used for the treatment of lymphedema. However, clinical studies have indicated that there are two main complications involved with compression pump therapy for leg lymphedema.
Secondly, it has been demonstrated that 35 percent of patients with lower extremity lymphedema, male and female, will develop genital lymphedema as a result of pump use. The reason for this is that the fluid is pushed into the abdomen and can overload the abdominal lymphatics and thus the fluid is NOT removed from the body.
The pneumatic compression pump is not recommended for leg lymphedema.
Lymphedema bandaging is one of the most powerful components in the treatment process. When a good bandage is applied, it will function as a custom-made compression garment each and every time. While the extremity is reducing in size, the bandage is adapting to the new dimensions. A pre-made elastic garment will never have this adaptability, instead, lymphedema patients should use what is referred to as short stretch bandages.
It is said that compression bandaging contributes to approximately 50 percent of the reduction achieved with CDT. With this in mind, it is crucial that the patient must become proficient in the bandaging techniques to achieve the best possible outcomes.
The compression bandage has the following effects:
Sometimes too, the process we must go through to get our treatment covered is maddening, to say the least. You may need to learn how to file a health insurance appeal to reverse a coverage or treatment denial. In some cases, you may even have to learn how to file a complaint against your insurance company with your state's insurance commissioner.
Seeking proper medical care for lymphedema is important. You should know how to choose a primary care doctor who understands or at least acknowledges the existence of lymphedema and is willing to learn about it and help you make the best treatment decisions. What type of doctor should I seek out?.
This page will help you find a certified lymphedema therapist: How to Find a Lymphedema Therapist
Finally, both diet and exercise are very important parts of managing and controlling lymphedema. We need to understand the importance of proper nutrition in our overall health, wound healing, immune system function, and weight control.
Exercise is not only vital for our overall health, but it helps with weight control and is important for the movement of lymph fluid through our body. No matter the stage of lymphedema, underlying medical conditions, or age, every one of us should have an exercise plan that is specifically designed to aid in the management of lymphedema.
There are three basic stages active of lymphedema. The sooner lymphedema is recognized and diagnosed, the easier it is to successfully treat it and to avoid many of the complications.
It is important to be aware that when you have lymphedema, even in one limb, there is always the possibility of another limb being affected at some later time. This “inactive” period referred to as the latency stage. It is associated with hereditary forms of lymphedema.
Spontaneously Irreversible Lymphedema
There have been some voices raised about a Stage 4 classsification for lymphedema. From my personal experiences, I concur. Both my legs are classified as Stage 3, yet the difference between the two is very dramatic.
Stage IV Classification
There is no treatment option for Stage IV. The complete focus is on management, containment of infections, and prevention of amputation (if at all possible).
Long-standing lymphedema causes a condition known as fibrosis. As the fluid continually collects in a limb, it becomes hard and dense. With each stage of lymphedema there is also a change in the tissue texture of a limb.
With stage one, the tissue is still much like normal tissue–it's just saturated with fluid. As the swelling continues and as the fluid changes to that protein-rich fluid referred to as lymphorrea, you enter into stage two. In this stage, the tissue becomes very similar to a grape (best image I can think of). Already it is becoming much more difficult for antibiotics to reach bacteria and it becomes less responsive to the decongestive therapy.
At stage three, the tissue become similar to one of those old synthetic kitchen sponges, the ones that become rock hard when they are dry. This is the very real and serious side effect of stage three lymphedema. Having this type of tissue increases the potential risk of having persistent and very hard to treat cellulitis or lymphangitis.
The denseness of the limb prohibits antibiotics from reaching the infecting bacterium and it is often able to survive in pockets of fibrotic tissue. These pockets act as a septic foci and after antibiotic treatment is completed, the infections will reappear.
Generally at this stage, it is going to take IV antibiotics to deal with any infection because oral antibiotics just are not able to penetrate this mass of hard tissue.
Also, as the fibrosis intensifies you become more susceptible to deep venous thrombosis (DVT) and other circulatory problems. You may also start to experience neuropathy as the pressure in this tissue compresses nerves within the limb.
Why is it so important to prevent, treat, and control fibrosis?
If you have lymphedema, it must be your priority to seek treatment and manage your condition with the goal of preventing fibrosis.
Acute cellulitis is one of the complications of lymphedema. The patient may not be aware of the source of the etiology. Sometimes it may be a cut, mosquito bite, an open wound, or other infection in the body.
The first sign is increased or different quality of PAIN involving the lymphedema limb. The patients often describe this as a “flu-like symptom or an ache” involving the lymphedema arm or leg. This is usually followed by sudden onset of ERYTHEMA (redness, red streaks or blotches) on the involved limb. The HYPERTHERMIA (lymphedema limb becomes warm, hot) will follow and the patient may experience the CHILLS and even HIGH FEVER.
Early intervention and treatment with antibiotics will resolve this condition (it usually takes a minimum 10-day course of antibiotics). Only a medical doctor will be able to prescribe the antibiotics, thus a consultation with a doctor is necessary. Severe cellulitis may require intravenous antibiotic treatment and hospitalization. Again, elevation of the affected limb is important.
During that phase, the patient should NOT massage the lymphedema limb, bandage, apply the pump, wear tight elastic sleeves, or exercise excessively. Avoid the blood pressure cuff and blood draws from the involved arm. Keep the limb elevated as much as possible while resting. Once the symptoms dissipate, the MLD/CDP treatment should be initiated.
How do we prevent this infection? The patient should be careful with daily activities and take all precautions to protect the skin (wear gloves when gardening, cleaning with detergents, etc.. ).
If an injury to the skin occurs on the lymphedema limb, it is necessary to clean the wound with alcohol or hydrogen peroxide and apply Neosporin/Polysporin antibiotic ointment. If the symptoms progress, seek the attention of a physician immediately.
It is so very important to avoid getting cellulitus as it further destroys the lymphatic system. If allowed to spread or continue, it can become systemic and can lead to gangrene, amputation of the limb, or even death.
Though cellulitis is the most commonly associated infection that comes with lymphedema, it is important to realize that not all our infections can accurately be called cellulitis. There are numerous infections associated with lymphedema. Because the lymphemdema limb is immunocompromised, we may experience and be susceptible to other infections such as erysipelas, impetigo, folliculitis, necrotizing fasciitis, MRSA Methicillin Resistant Staphylococcus Aureus, carbuncles boils, lymphangitis, cutaneous abscesses, scalded skin syndrome, and even cat scratch fever. We can also be more readily susceptible to sepsis, septicemia, septic shock, bacteremia, gangrene, lymphadenitis. It is critical, therefore, that infections be recognized quickly and proper treatment initiated immediately.
What is Lymphorrhea?
Lymphorrhea is the light amber-colored fluid that drains from open skin areas (wounds) on a lymphodemous limb. It is not normal plasma, but is a protein-rich substance that can lead to serious complications for the lymphedema patient.
The cause of lymphorrhea drainage is any open area or break in the skin of the lymphodemous limb. Any opening, no matter how small, will cause this fluid to weep or drain. Insect bites, cuts, abrasions, cracks in the skin from dryness, or wounds of any type, become a source for leakage of this fluid.
There are two serious complications that arise from lymphorrhea.
Lymphorrhea is highly caustic to the skin tissue that it come into contact with. Untreated wounds with this drainage can very quickly become large gaping wounds that may eventually lead to the need for skin grafts.
Little is said about the psychological affects of lymphedema, despite its profound impact on the person dealing with it.
Lymphedema can cause pronounced social inhibitions because of being self conscious, it can cause depression, embarrassment, disfigurement, feelings of hopelessness and helplessness. You often go through tremendous life style changes and feel worthless and useless. It can cause sexual dysfunction as both male and female lymphedema patients can acquire genital lymphedema.
You go through periods of struggling with feeling humiliation, despair and you can experience terrific pain.
You also go through a lot of anger. Anger at why you have this, why the medical world knows so little about this condition and sometimes seems to care little either.
For cancer patients, its a double impact. While you are dealing with cancer, its side affects, whether or not you are going to live, the affects of radiation and chemo… now all of a sudden your body starts swelling out of control. What is going on???
If you are going through these emotions, please don't be afraid to let your doctor know. You may need professional help to get through this. You may also find a lot of comfort and encouragement in joining a support group. Contact the National Lymphedema Foundation to see if there is one in your area.
If there are no support groups in your area, you may also be interested in learning how to start a lymphedema support group.
You may also wish to join an online support group. There are several mentioned in our site. As a member, myself, I have found a wonderful and supportive family of fellow lymphedema people. I cannot tell you just how much my new family has come to mean to me and how much I have gained by being a part of these groups.
THERE IS HOPE……..AND YOU ARE NOT ALONE!!
Vojáčková N, Fialová J, Hercogová J.
Dermatovenereology Department, Charles University 2nd Medical School and Bulovka Hospital, Prague, Czech Republic.
Keywords: complex decongestive physical therapy; compression; manual lymph drainage
The basic principle of the management of lymphedema is so called complex decongestive physical therapy. This therapy is divided into two phases: (i) edema reduction phase - an initial intensive treatment phase aiming for limb volume reduction; and (ii) maintenance phase - following long-term phase to sustain a manageable limb volume. The first phase consists of a number of physical therapeutic approaches which are: manual lymph drainage, pneumatic pump drainage, low-stretch bandaging, exercises, and skin care. Long-term maintenance phase consists of self-lymph drainage, low-stretch bandaging, or compressive garments, and sometimes when indicated pneumatic pump drainage, exercises, and skin care.
PRIMARY LYMPHEDEMA can be hereditary. Milroys Disease or Syndrome will generally express itself at birth or in the very early years. Meige Lymphedema, also known as lymphedema praecox generally begins sometime during puberty. Lymphedema tarda begins in or around middle age. Lymphedema that has not expressed itself in an active condition is referred to a latent lymphedema. Primary lymphedema can also be congenital. This means some either in utero or during birth caused lymphatic damage.
TYPES OF PRIMARY LYMPHEDEMA
Related Terms: Nonne-Milroy lymphedema, Milroy's Disease, Primary congenital hereditary lymphedema, hereditary lymphedema I, Nonne-Milroy-Meige disease
Milroy's Syndrome is an old term used to describe hereditary congenital lymphedema. It is a congenital familial primary lymphedema which results from vertical autosomal inheritance of a single gene. The gene has been identified as VEGFR3. The condition usually presents itself at birth with the swelling of one or even both legs.
If the condition is unilateral (single leg), the other leg may continue in the latency stage for years before expressing itself. The same is indicated for arm lymphedema.
It is the rarest of the inherited lymphedema, accounting for approximately 2% of hereditary lymphedemas.
Hereditary lymphedema was first described by Nonne in 1891, however in 1892 Dr. William F. Milroy described a missionary who had returned from work in India who had swollen legs his entire life. His mother likewise was afflicted with the same condition. Milroy had also, previously studied the 250 year history of a family and had been able to identify 22 persons with this condition through 6 generations. He was also able to pinpoint when the condition entered the family through a marriage in 1768.
Basic diagnosis can be made by the fact that swelling (generally of the legs) presents at birth and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.
The cause of Milroy's Syndrome is a break in the VEGFR3 gene.
The usual complications involved with the condition include fibrosis of the limb tissues, cellulitis (and or lymphangitis and erysipelas infections). Other complications made include involvement of the genitalia, pain, skin conditions and in very rare situations lymphangiosarcoma.
Decongestive therapy is the most widely accepted form of treatment. There is no cure for Milroy's but the condition can be managed by early diagnosis and treatment.
Long term prognosis is excellent is the condition is identified early and treatment begins so after the diagnosis is made.
See this page for special pointers and information relating to Lymphedema and Pregnancy
MEIGE LYMPHEDEMA SYNDROME
See also: Nonne-Milroy-Meige Syndrome, Meige's lymphedema, Hereditary lymphedema II, familial lymphedema praecox
Form of primary hereditary lymphedema that starts at or around the time of puberty. The affected limbs are generally the legs.
Named after French physician Dr. Henri Meige who first described hereditary lymphedema in 1891. This form of lymphedema which usually presents itself at or during puberty is the most common of the hereditary lymphedemas, account for 65-80% of all diagnosed cases.
Also known as Lymphedema II, this syndrome is similar to Lymphedema I but the onset of peripheral edema occurs during the second to the fifth decades. The legs are the most commonly involved, and lymphangiography reveals hypoplasia of peripheral lymphatics with dilation of lymphatic trunks.
Basic diagnosis can be made by the fact that swelling (generally of the legs) presents during puberty and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.
The cause of Lymphedema Praecox is a break in the FOXC2 gene. The gene FOXC2 is implicated and there is also suspect third lymphedema gene.
The usual complications involved with the condition include fibrosis of the limb tissues, cellulitis (and or lymphangitis and erysipelas infections). Other complications made include involvement of the genitalia, pain, skin conditions and in very rare situations lymphangiosarcoma.
Decongestive therapy is the most widely accepted form of treatment. There is no cure for Lymphedema praecox but the condition can be managed by early diagnosis and treatment.
Long term prognosis is excellent if the condition is identified early and treatment begins so after the diagnosis is made.
Form of primary hereditary lymphedema that expresses itself during middle age (generally onset 35+ years). Swelling generally occurs in the legs and may involve either one or both limbs. There is a higher incidence of lymphedema tarda among females than males.
This form of inherited lymphedema accounts for approximately 10% of those with primary lymphedema.
Basic diagnosis can be made by the fact that swelling (generally of the legs) unexpectedly and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.
The cause of lymphedema tarda is a break in the FOXC2 gene.
The usual complications involved with the condition include fibrosis of the limb tissues, cellulitis (and or lymphangitis and erysipelas infections). Other complications made include involvement of the genitalia, pain, skin conditions and in very rare situations lymphangiosarcoma.
Long term prognosis is excellent is the condition is identified early and treatment begins so after the diagnosis is made.
Does Lymphedema Spread to Other Areas of Your Body?
Can lymphedema spread from your legs to your abdomen? Can it spread to your arms? This question has been asked on every online support group I am in, and it has been asked many times. From the life experience of those of us who have experienced this, we would have to answer with a resounding yes, there is such a thing as spreading lymphedema!
I am putting a disclaimer on this article only because I am not a doctor and neither are the lymphedema patients who have asked this question on the various list to which I am a member. We are only people attempting to understand what in the world is going on with our bodies and attempting to answer a question that the medical world is either silent about or oblivious to.
Primary lymphedema patients are always at risk for lymphedema to present in unaffected limbs. This is because there is already a damaged lymphatic system in place at birth. Whether this occurs from an in-utero problem or is genetically based the possibility is there.
A clear example of this are my own arms. I was born with bi-lateral lymphedema of the legs. As a teenager, I began to have lymphedema in my right hand. Last year as a result of months of IV and piclines antibiotics in my left arm, it now swells. This year after the insertion of a port a cath in my chest, it seems I am now having “bloating” of the abdomen that I have never had. Other causes for primary and secondary lymphedma patients to experience new swelling in unaffected areas include infections, IV therapy in the arms, fluid overload of the lymphatics in existing lymphedema areas causing the fluid to “backup” (i.e. from legs to abdomen), further damage to existing lymphaics due to fibrosis and even an injury in an unaffected area.
How Can I Tell if My Lymphedema Has Spread?
For limb lymphedema, the answer is surprisingly simple. If you have a limb that is normal and it begins to swell with no apparent reason, you should immediately suspect lymphedema. It may be that you need a lymphoscintigraphy test of that limb. For the abdomen, the answer is not so simple. If you have sudden swelling of the abdomen with no change in your dietary routine and the swelling is firm, you should suspect lymphedema. Abdominal fat (overweight from dietary) is much much softer than fluid accumulation due to edema.
If you suspect this has occurred with you, you should immediately discuss the problem with your therapis and doctor and begin the appropriate treatment.
HERE IS AN EXCELLANT ARTICLE FROM THE NATIONAL CANCER INSTITUTE INFORMATION FROM PDQCancerMail from the National Cancer Institute Information from PDQ for Patients
INTRODUCTION This patient summary is adapted from the summary on lymphedema written by cancer experts for health professionals. This and other credible information about cancer treatment, screening, prevention, supportive care, and ongoing trials, is available from the National Cancer Institute. 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 high 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.
OVERVIEW 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 and empties into the large vein under the right collar bone. The left lymphatic duct or thoracic duct collects lymph from both legs, the left arm and the left side of the head and chest and empties into the large vein under the left collar bone. 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 arm pit. 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 within a few days after surgery to remove the lymph nodes or injury to the lymphatic vessels or veins just under the collar bone. The affected limb may be warm and slightly red, but is usually not painful and gets better within a week after 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. Use of a compression pump or wrapping the affected area with elastic bandages should not be done 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 or aching in the neck and shoulders or spine and hips caused by stretching of the soft tissues, overuse of muscles, or posture changes caused by increased weight of the arm or leg.
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: - surgical drains that leak protein into the surgical site - inflammation - an inability to move the limb(s) - temporary loss of lymphatic function - blockage of a vein by a blood clot or inflammation of a vein.
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 happen: - after a tumor recurs or spreads to the lymph nodes - after an infection and/or injury of the lymphatic vessels - after periods of not being able to move the limbs - after radiation therapy or surgery - when early signs of lymphedema have not been able to be controlled - when a vein is blocked 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 and lymph flows back into the body tissues, making the condition worse. This causes pain, heat, redness, and swelling 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.
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, the number of lymph nodes removed in surgery, and being elderly.
Patients who are at risk for lymphedema are those with: - Breast cancer if they have received radiation therapy or had lymph nodes removed. Radiation therapy to the underarm area after surgical removal of the lymph nodes and the 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, under the collar bones, 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. - An inadequate diet or those who are overweight. These conditions may delay recovery and increase the risk for lymphedema.
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 well as any history of injury or infection. Knowing the medications a patient is taking is also important for diagnosis.
Patients at risk for lymphedema should be identified early, monitored, and taught self-care. A patient may be more likely to develop lymphedema if he or she eats an inadequate diet, is overweight, is inactive, or has other medical problems. To detect the condition early, the following should be examined: - comparison of actual weight to ideal weight - measurements of the arms and legs - protein levels in the blood - ability to perform activities of daily living - history of edema, previous radiation therapy, or surgery - other medical illnesses, such as diabetes, high blood pressure, kidney disease, heart disease, or phlebitis (inflammation of the veins). It is important that the patient know about his or her disease and the risk of developing lymphedema. Poor drainage of the lymphatic system due to surgery to remove the lymph nodes or radiation therapy may make the affected arm or leg more susceptible to serious infection. Even a small infection may lead to serious lymphedema. Patients should be taught about arm, leg, and skin care after surgery and/or radiation (see Table 1 below). It is important that patients take precautions to prevent injury and infection in the affected arm or leg, since lymphedema can occur 30 or more years after surgery. Breast cancer patients who follow instructions about skin care and proper exercise after mastectomy are less likely to experience lymphedema. Lymphatic drainage is improved during exercise, therefore exercise is important in preventing lymphedema. Breast cancer patients should do hand and arm exercises as instructed after mastectomy. Patients who have surgery that affects pelvic lymph node drainage should do leg and foot exercises as instructed. The doctor decides how soon patients should start exercising after surgery. Physiatrists (doctors who specialize in physical medicine and rehabilitation) or physical therapists should develop an individualized exercise program for the patient.
It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms None Disorder Subdivisions Hereditary Lymphedema, Type I Congenital Hereditary Lymphedema Milroy Disease Nonne-Milroy-Meige Syndrome Hereditary Lymphedema, Type II Meige's Lymphedema Familial Lymphedema Praecox Hereditary Lymphedema Tarda Related Disorders List Information on the following diseases can be found in the Related Disorders section of this report: Hereditary Angioedema Lymphedema (Traumatic) Elephantiasis Distichiasis-Lymphedema Syndrome Lymphedema and Ptosis
Hereditary Lymphedema is an inherited disorder of the lymphatic system that is characterized by abnormal swelling of certain parts of the body. The lymphatic system is a circulatory network of vessels, ducts, and nodes that filter and distribute certain fluid (lymph) and blood cells throughout the body. Lymphatic fluid collects in the soft tissues in and under the skin (subcutaneous) due to the obstruction, malformation, or underdevelopment (hypoplasia) of various lymphatic vessels.
There are three forms of Hereditary Lymphedema: Congenital Hereditary Lymphedema or Milroy Disease, Lymphedema Praecox or Meige Disease, and Lymphedema Tarda. In most cases, Hereditary Lymphedema is inherited as an autosomal dominant genetic trait.
The primary symptom of Hereditary Lymphedema is swelling or puffiness in different parts of the body due to the accumulation of lymphatic fluid in the soft layers of tissue under the skin (lymphedema). Swelling frequently occurs below the waist, especially in the legs, but may also be present in the face, voice box (larynx), and arms. When lymphedema develops in the legs, swelling may begin in the foot and move upward. In some cases, swelling may cause discomfort and unusual tingling sensations (paresthesias) in the affected areas. Poor healing, following even minor trauma (e.g., cut or insect bite), may also occur; permanent structural changes in the skin, with abnormal thickening, often follow longstanding edema.
Infants with Hereditary Lymphedema Type I, also known as Congenital Hereditary Lymphedema or Milroy Disease, have areas of swelling at birth. The swelling tends to slowly worsen with advancing age.
Hereditary Lymphedema Type II or Meige Disease usually develops during childhood, adolescence, or early adulthood. This form of the disease usually produces severe swelling in areas below the waist. The first symptoms of Hereditary Lymphedema Type II usually include red skin over areas of swelling and associated discomfort and/or inflammation.
Hereditary Lymphedema Tarda usually occurs after the age of 35 years. The symptoms are similar to those of Hereditary Lymphedema Type II.
Complications of Hereditary Lymphedema may include inflammation of lymphatic vessels (lymphangitis) and infection of the skin (cellulitis) characterized by areas of warm and painful reddened skin that are hot to the touch. Red skin “streaks” may also develop. A general feeling of ill health (malaise), fever, chills, and/or headaches may also occur. If left untreated, cellulitis can lead to skin abscesses, areas of ulceration, and/or tissue damage (necrosis). Some people with this condition may develop persistent fluid accumulation in the lungs (pleural effusion). The most serious long-term complication of all forms of Hereditary Lymphedema is a minor increased risk for the development of a malignancy in the affected area (i.e., lymphangiosarcoma).
Most cases of Hereditary Lymphedema are inherited as an autosomal dominant trait. Human traits, including the classic genetic diseases, are the product of the interaction of two genes, one received from the father and one from the mother.
In dominant disorders, a single copy of the disease gene (received from either the mother or father) will be expressed “dominating” the other normal gene and resulting in the appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is 50 percent for each pregnancy regardless of the sex of the resulting child.
In some rare cases, Hereditary Lymphedema is thought to be inherited as an autosomal recessive or X-linked recessive genetic disorder.
In recessive disorders, the condition does not appear unless a person inherits the same defective gene for the same trait from each parent. If an individual receives one normal gene and one gene for the disease, the person will be a carrier for the disease, but usually will not show symptoms. The risk of transmitting the disease to the children of a couple, both of whom are carriers for a recessive disorder, is 25 percent. Fifty percent of their children risk being carriers of the disease, but generally will not show symptoms of the disorder. Twenty-five percent of their children may receive both normal genes, one from each parent, and will be genetically normal (for that particular trait). The risk is the same for each pregnancy.
X-linked recessive disorders are conditions that are coded on the X chromosome. Females have two X chromosomes, but males have one X chromosome and one Y chromosome. Therefore, in females, disease traits on the X chromosome can be masked by the normal gene on the other X chromosome. Since males only have one X chromosome, if they inherit a gene for a disease present on the X, it will be expressed. Men with X-linked disorders transmit the gene to all their daughters, who are carriers, but never to their sons. Women who are carriers of an X-linked disorder have a 50 percent risk of transmitting the carrier condition to their daughters, and a 50 percent risk of transmitting the disease to their sons.
Some cases of Hereditary Lymphedema may occur because of a spontaneous change in genetic material early in fetal development (sporadic).
The symptoms of Hereditary Lymphedema develop because of obstruction of the lymphatic vessels due to multiple malformations of the lymphatic vascular system. In some cases, there may be a reduction in the number of lymphatic vessels or the vessels may be underdeveloped (hypoplastic). However, in some cases, the reverse is true and lymphatic vessels may be unusually large (hyperplastic) and numerous.
Congenital Hereditary Lymphedema (Type I) affects more females than males. Hereditary Lymphedema Type II (Meige Disease) affects males and females in equal numbers.
Approximately 1 in 6,000 people in the United States are affected by Hereditary Lymphedema. The symptoms of Congenital Hereditary Lymphedema (Type I) are typically present at birth. The onset of Hereditary Lymphedema Type II can occur during childhood, adolescence, or adulthood (i.e., between the ages of 10 to 35 years). The symptoms of Hereditary Lymphedema Tarda generally occur after the age of 35 years.
Symptoms of the following disorders can be similar to those of Hereditary Lymphedema. Comparisons may be useful for a differential diagnosis:
Hereditary Angioedema is a rare inherited vascular disorder characterized by the excessive accumulation of body fluids in lymphatic vessels or veins. The outstanding symptom of this disorder is swelling (edema) on the back of the hands or feet, eyelids, lips, and/or genitals. Edema may also occur in the mucous membranes that line the respiratory and digestive tracts. The areas of swelling may be hard and painful, but they are typically not red or itchy (pruritic). A skin rash (urticaria) is rarely present. Hereditary Angioedema is inherited as an autosomal dominant genetic trait. (For more information on this disorder, choose “Angioedema” as your search term in the Rare Disease Database.)
Traumatic Lymphedema is caused by injury to the lymph system or blunt trauma (bruising). Localized lymphedema may be due to postinfectious syndromes, post-radiation fibrosis, tumor growth, and/or surgery (e.g., mastectomy). Symptoms may include swelling, pitting, redness, discomfort, and/or tingling sensations.
Elephantiasis is an infectious tropical disease of the lymphatic system and is characterized by gross enlargement of an arm or leg or other areas of the trunk or head. The skin develops a thickened, pebbly appearance and may become ulcerated and darkened. Fever, chills, and a general feeling of ill health (malaise) may also be present. Inflammation of the lymphatic vessels causes extreme enlargement of the affected areas. This condition occurs most commonly in tropical regions and particularly in parts of Africa. (For more information on this disorder, choose “Elephantiasis” as your search term in the Rare Disease Database.)
The following disorders may occur in association with Hereditary Lymphedema as secondary characteristics. They are not necessary for a differential diagnosis:
Yellow Nail Syndrome is a rare disorder characterized by yellow, thickened, and curved nails with almost complete stoppage of nail growth. A loss of cuticles may also be associated with this syndrome. Loosening of the nails (onycholysis) may cause loss of some nails. This condition is usually associated with the presence of fluid in the lungs (plural effusion) and swelling of the arms and legs (lymphedema). Other respiratory problems may occur such as chronic inflammation of the bronchi and bronchioles (bronchiectasis), chronic bronchitis, and/or ongoing inflammation of the membranes that line the sinus cavities (sinusitis). The exact cause of this disease is not known. (For more information on this disorder, choose “Yellow Nail” as your search term in the Rare Disease Database.)
Distichiasis-Lymphedema Syndrome is a rare inherited disorder characterized by the presence of extra eyelashes (distichiasis) and swelling of the arms and legs (lymphedema). Swelling of the legs, especially below the knees, and eye irritation are common in people with this disorder. Occasionally, cysts on the spine (epidural) and other abnormalities of the spinal column may also occur. Distichiasis-Lymphedema Syndrome is inherited as an autosomal dominant genetic trait.
Lymphedema and Ptosis is an extremely rare inherited condition and is characterized by droopy eyelids (ptosis) and swelling (lymphedema), especially in the legs.
The diagnosis of Hereditary Lymphedema may be confirmed by a thorough clinical evaluation and specialized imaging tests. The structure of the lymphatic system may be investigated with special nuclear studies (i.e., indirect radio isotope, lymphoscintigraphy, magnetic resonance imaging (MRI) or CT scanning may yield information that supports the diagnosis.
Complete decongestive therapy (CDT) is a form of treatment in which specialized massage techniques are coupled with therapeutic bandaging, meticulous skin care, exercise, and the use of well-fitted compression garments.
Occasionally, drugs that promote excessive urinary output (i.e., diuretics) may be somewhat helpful for people with Hereditary Lymphedema. These medications increase urinary output and may help to reduce swelling in some affected individuals. However, diuretics have not been uniformly successful in reducing the swelling associated with this disorder. The prolonged use of diuretics for the treatment of Hereditary Lymphedema should be carefully directed by a physician as these medications may have several long-term side effects.
In some cases, the surgical joining of small lymphatic vessels to nearby small veins (microsurgical anastomosis) has had some limited success in people with Lymphedema. The goal of this surgery is to reduce swelling by creating new pathways for lymphatic fluid flow and “rechanneling” this flow into the venous system.
Genetic counseling will benefit people with Hereditary Lymphedema and their families.
Research on genetic disorders and their causes is ongoing. The National Institutes of Health (NIH) is sponsoring the Human Genome Project which is aimed at mapping every gene in the human body and learning why they sometimes malfunction. It is hoped that this new knowledge will lead to prevention and treatment of genetic and familial disorders in the future.
ONLINE MENDELIAN INHERITANCE IN MAN (OMIM). Victor A. McKusick, Editor; Johns Hopkins University, last edit date 4/18/95. Entry Number 153200; last edit date 3/9/99, Entry Number 153100; last edit date 11/6/94, Entry Number 153000. CECIL TEXTBOOK OF MEDICINE, 20th Ed.: J. Claude Bennett and Fred Plum, Editors; W.B. Saunders Co., 1996. P. 357. HARRISON'S PRINCIPLES OF INTERNAL MEDICINE, 14th Ed.: Kurt J. Isselbacher, M.D. et al., Editors; McGraw-Hill, Inc., 1998. Pp. 1405-06. THE MERCK MANUAL, 17th Ed.: Robert Berkow and Mark Beers, Editors; Merck Research Laboratories; 1999. P. 1798. TEXTBOOK OF DERMATOLOGY, 5th Ed.: R.H. Champion et al., Editors; Blackwell Scientific Publications, 1992. Pp. 2018-23. BIRTH DEFECTS ENCYCLOPEDIA, Mary Louise Buyse, Editor-In-Chief; Blackwell Scientific Publications, 1990. Pp. 1087-89. NELSON TEXTBOOK OF PEDIATRICS, 15th Ed.: Richard E. Behrman, Editor; W.B. Saunders Company, 1996. Pp. 1440-41. DICTIONARY OF MEDICAL SYNDROMES, 3rd Ed.: Sergio I. Magalini, Sabina C. Magalini, and Giovanni de Francisci, Editors; J.B. Lippincott Company, 1990. Pp. 637-38. 18 STEPS TO PREVENTION FOR LOWER EXTREMITIES. S. Thiadens, R.N.; National Lymphadema Network Special Circular (1998). CDP TREATMENT OF SMALL CHILDREN AND INFANTS WITH PRIMARY (CONGENITAL) LYMPHEDEMA. G. Klose; National Lymphedema Network Newsletter (Jan-Mar 1998; 10(1)). Pp. 1-5. TREATMENT OF LYMPHEDEMA WITH COMPLETE DECONGESTIVE PHYSIOTHERAPY. J.E. Zuther; National Lymphedema Network Newsletter (Apr-Jun 1999; 11(2)). Pp. 3-8. PRIMARY LYMPHEDEMA. S.G. Rockson, Author; In: Currenty Therapy in Vascular Surgery, 4th Ed.: C. B. Ernst et al., Editors; Mosby. LYMPHEDEMA: A REVIEW OF THE RELEVANT ANATOMY AND PHYSIOLOGY OF THE LYMPHATICS. A. Szuba et al.; Vascular Medicine (1997; 2). Pp. 321-26 LYMPHEDEMA: A REVIEW OF DIAGNOSTIC TECHNIQUES AND THERAPEUTIC OPTIONS. A. Szuba et al.; Vascular Medicine (1998; 3). Pp. 145-56. HEREDITARY LATE-ONSET LYMPHEDEMA WITH PLEURAL EFFUSION AND LARYNGEAL EDEMA. F.A. Herbert et al.; Arch Intern Med (May 1983; 143(5)). Pp. 913-15. HEREDITARY LYMPHEDEMA AND DISTICHIASIS. T. Kolin et al.; Arch Ophthalmol (Jul 1991; 109(7)). Pp. 980-81. LYMPHANGIOSARCOMA IN CHRONIC HEREDITARY OEDEMA (MILROY'S DISEASE). L.A. Brostrom et al.; Ann Chir Gynaecol (1989; 78(4)). Pp. 320-23. CONGENITAL HEREDITARY LYMPHEDEMA (NONNE/MILROY). D.D. Farhud et al.; Padiatrpa Padol (1989; 24(4)). Pp. 305-07.
There is finally much research going on regarding genetics and lymphedema. The specific gene (FOXC2) that is responsible for LE has been identified and experiments are being conducted in gene therapy with mice.
The FOXC2 is referred to as a forkhead gene, one of 17 thus far identified in humans. Because it is a pleiotrophic developmental gene, a mutation can cause multiple effects.
While this research is in its infancy, it does bring a very big light of hope that one day primary lymphedema can be stopped or prevented.
Novel Discoveries Leading To Targeted Treatment Of Lymphatic Diseases
A gene responsible for lymphatic vessel formation
Novel discoveries at the University of Helsinki, Finland, about the development of the lymphatic network may help researchers to better understand the mechanisms of cancer and its metastasis, and also diseases such as lymphedema, wound healing and inflammatory and autoimmune disorders.
Lymphatic vascular network is essential in transporting the tissue fluids and immune cells from tissues to the nearby lymph nodes and back to the blood circulation. Thus, this network of vessels is crucial in provoking body's immune defense mechanisms. A study published as advance online publication of `Nature Immunology' (web edition 23.11.2003) describes VEGF-C as an essential regulator of lymphatic vessel development. Dr. Karkkainen and collaborators from the University of Helsinki show that in the absence of this growth factor the lymphatic development is interrupted, which results in fluid accumulation in tissues and embryonic lethality. VEGF-C haploinsufficiency gene results in delayed and abnormal lymphatic development and swelling of the limbs, in a disease called lymphedema.
Within the past couple of years, there has been an unprecedented explosion of lymphatic biology research. The current study was done in one of the leading laboratories in the field of lymphangiogenesis, in the group lead by Dr. Kari Alitalo from the University of Helsinki, which has reported major advances in the fields of angiogenesis, lymphangiogenesis and cancer biology in recent years. The authors believe that the current study helps in developing more targeted treatments of various lymphatic diseases. Now, for the first time there are exciting new developments making treatment possible for the over one hundred million people worldwide who suffer from diseases related to the lymphatic system.
We have a number of info pages on genetics. These pages include FOXC2 GENE - VEGFC2 / VEGFR3 - SOX18 - GJC2 - FLT4 - GATA2 - CCBE1 - FLT4.
Aagenaes Syndrome; Aarskog Syndrome; Alpha Galactosidase B Deficiency; Atrial Septal Defect; Avasthey Roy syndrome; Campomelia Cumming Syndrome; Cholestasis syndrome; Congenital Disorder of Glycosylation Type 1B; Congenital Recessive; Distichiasis; Dysplasia Anhidrotic Immunodeficiency Oledaid; Ectodermal Dysplasia Hypohidrotic Immune Deficiency; Fabry's Disease; Figueroa Syndrome; Hennekam Lymphangiectasia; Intestinal LymphangiectasiaHypoparathyroidism; Hypotrichosis Lymphedema; Idiopathic Hydrops Fetalis;Jekens Syndrome; Kasabach Merritt Syndrome; Klinefelter Syndrome; Lissencephaly Syndrome; Maffuccis Syndrome; Melkersson Rosenthal Syndrome; Micropehaly Chorioretinopathy Syndrome; Nevo Syndrome; Noonan's Syndrome; Osteopetrosis Ectodermal; Pulmonary Lymphagiectasia; Peho Syndrome; Prolidase Deficiency; Ptosis Syndrome; Pulmonary Cystic Lymphangiectasis; Sharp Aagenaes Syndrome; Stewart Treves Syndrome; Swyer Syndrome; Trisomy 10; Trisomy 13; Trisomy 18; Trisomy 21; Trisomy 22; Trisomy Disorders; Turner Syndrome; Von Recklinghausen Neurofibromatosis; yellow_nail_syndromeLipedema; Omphalocele III; Reflex Sympathetic Dystrophy (RSD); German Syndrome;Prader Willi Syndrome
Syndromes Associated with Lymphatic Dysplasia gives a brief overview of most of these conditions. For further information, please consult the individual pages linked above.
Castleman's Disease; Cystic hygroma; Klippel Trenaunay Weber Syndrome; Lipedema; Lymphangiomatosis; Lymphoceles; Lymphadenopathy; Lymphangiomas; Lymphatic Malformations; Prader-Willi Syndrome; Amniotic Band Syndrome; Hidradenitis Suppurativa; Crohn's Disease, Panniculitis; Lymphangioleiomyomatosis (LAM); Omphalocele III; Phelan-McDermid Syndrome - 22q13 deletion; Lipoma and LipomasLymphadenitis Proteus Syndrome, Cardiofaciocutaneous syndrome
Not all swelling is lymphedema, some is edema caused by an underlying medical condition.
Below is a list of medical conditions that cause or are involved with edema:
Causes of Edema
Premenstrual syndrome; Pregnancy; Heart failure; Kidney conditions; Glomerulonephritis; Nephrotic syndrome; Kidney failure; Liver condition; Cirrhosis of the liver; Varicose veins; Protein deficiency (type of Nutritional deficiency); Thyroid condition; Certain medications; Capillary Leak Syndrome Puffy Hand Syndrome Thrombophlebitis Venous Pooling Chronic Venous Insufficiency
Edema as a complication
Edema as a symptom
Acute rheumatic fever; Angioedema; Anaphylaxis; Aortic coarctation; Cardiomyopathy; Chronic kidney failure; Cirrhosis of the liver; Common migraine; Eclampsia; Edema; Glomerular Disease; Glomerulonephritis; Goodpasture syndrome; Hemolytic disease of the newborn; Hemolytic uremic syndrome; Hepatitis; Hodgkin's Disease; Liver cancer; Nephritis; Nephrotic syndrome; Polyarteritis nodosa; Preeclampsia; Premenstrual syndrome; Primary biliary cirrhosis; Pulmonary valve stenosis; Restrictive cardiomyopathy; Rheumatic fever; Trichinosis; Vitamin E deficiency Diabetes
The lymph system is a network of organs, lymph nodes, lymphatic ducts, and lymphatic vessels that produce and transport lymph from tissues to the bloodstream. The lymph system is a major component of the body's immune system.
The lymphatic system filters fluid from around cells. It is an important part of the immune system. When people refer to swollen glands in the neck, they are usually referring to swollen lymph nodes. Common areas where lymph nodes can be easily felt, especially if they are enlarged, are: the groin, armpits (axilla), above the clavicle ( supraclavicular), in the neck (cervical), and the back of the head just above hairline (occipital).
Lymph is a clear-to-white fluid made of:
Fluid from the intestines called chyle, which contains proteins and fats Red blood cells White blood cells, especially lymphocytes, the cells that attack bacteria in the blood Lymph nodes are small, bean-shaped, soft nodules. They can not usually be seen or easily felt. They are located in clusters in various parts of the body, such as the neck, armpit, and groin.
Lymph nodes produce immune cells that help the body fight infection. They also filter the lymph fluid and remove foreign material, such as bacteria and cancer cells. When bacteria are recognized in the lymph fluid, the lymph nodes produce more infection-fighting white blood cells, which causes the nodes to swell. (see lymphadenopathy)
125.0-125.9 Filarial lymphedema 457.0 Post-mastectomy lymphedema syndrome 457.1 Other lymphedema (praecox, secondary, acquired/chronic, elephantiasis) 457.2 Lymphangitis 457.8 Other noninfectious disorders of lymphatic channels (chylous disorders) 624.8 Vulvar lymphedema 729.81 Swelling of limb 757.0 Congenital lymphedema (of legs), chronic hereditary, ideopathic hereditary 782.3 Edema of Legs-Acute traumatic edema
Procedure A manipulation of the body to give a treatment or perform a test; more broadly, any distinct service a doctor renders to a patient. All distinct physician services have ‘procedure codes’ in various payment schemes.
97001 or 97003 initial evaluation by a physical or an occupational therapist, or an Evaluation and Management CPT Code for physicians. 97002 or 97004 re-evaluation by a physical or an occupational therapist, or an E valuation and Management CPT Code for physicians. 97110 Therapeutic exercises 97016 Vasopneumatic Pump 97124 Massage therapy for edema of an extremity 97140 Manual therapy, manual lymphatic drainage (15 minute units) 97150 Group therapy 97504 Orthotic training/fitting 97530 Therapeutic activities, restoration of impaired function 97535 Self-care home management training, instruction on bandaging, exercises, and self-care 97703 Checkout for orthotic or prosthetic use
Cellulitis ICD-9 codes for Outpatient Treatment: Infections of skin and subcutaneous tissue (680-686)
Cellulitis and abscess (681.00-682.9) 682.0 Other cellulitis and abscess, face 682.1 Other cellulitis and abscess, neck 682.2 Other cellulitis and abscess, trunk 682.3 Other cellulitis and abscess, upper arm and forearm 682.4 Other cellulitis and abscess, hand, except fingers and thumb 682.5 Other cellulitis and abscess, buttock 682.6 Other cellulitis and abscess, leg, except foot 682.7 Other cellulitis and abscess, foot, except toes 682.8 Other cellulitis and abscess, other specified sites 682.9 Other cellulitis and abscess, unspecified site (diffuse) (with lymphangitis)
The items and supplies listed below are considered “incident to” a physician service and are not separately reimbursable. However, if these supplies are given to a patient as a take home supply, the claim should be submitted to the DMERC.
A4454 Tape A4460 Elastic bandage (e.g. compression bandage). Use this code to report compression bandages associated with lymphatic drainage (CIM 60-9, MCM 2133, ASC) A4465 Non-elastic binder for extremity. Use for Reid, CircAid, ArmAssist, etc manually-adjustable sleeves and leggings. Medicare jurisdiction DME regional carrier (CIM 60-9, MCM 2133, ASC) A4490-4510 Surgical Stockings A4490 Surgical Stockings above knee length (each) A4495 Surgical Stockings thigh length (each) A4500 Surgical Stockings below knee length (each) A4510 Surgical Stockings full length (each) A4649 Miscellaneous Surgical Supplies, Compression bandaging kit E0650-0652 Pneumatic Compressor and Appliances E0650 Pneumatic Compressor, non-segmental home model E0651 Pneumatic Compressor, segmental home model, without calibrated gradient pressure E0652 Pneumatic Compressor, segmental home model, with calibrated gradient pressure E0655-0673 Arm and Leg Appliances used with Pneumatic Compressor L0100-L4398 Orthotics L2999 Lower Limb Orthosis, not otherwise specified L3999 Upper Limb Orthosis, not otherwise specified L4205 Repair of orthotic device, labor, per 15 minutes L4210 Repair of orthotic device, repair or replace minor parts L5000-L5999 Lower Limb L6000-L7499 Upper Limb L8000-8490 Prosthetics L8010 Mastectomy Sleeve, Ready-Made L8100-L8239 Elastic supports L8100-8195 Elastic Supports, elastic stockings various lengths & weights L8210 Gradient compression stocking, custom made L8220 Gradient compression stocking/sleeve, Lymphedema, Custom L8239 Gradient stocking, not otherwise specified. Carrier discretion.
Hereditary Lymphedema, Type I; Congenital Hereditary Lymphedema Milroy Disease,Nonne-Milroy-Meige Syndrome, Hereditary Lymphedema, Type II, Meige's Lymphedema, Familial Lymphedema Praecox, Lymphedema Praecox Meige Syndrome, Hereditary Lymphedema Tarda, Hereditary Angioedema, Traumatic Lymphedema Elephantiasis, Distichiasis-Lymphedema Syndrome Lymphedema and Ptosis, The lymph system, Lymphorrhea, Lymphedema wounds, Weeping lymphedema, Cellulitis, Lymphangitis, Secondary lymphedema, Lymphedema Stages, treatment for lymphedema, Complete Decongestive therapy, Risk factors for lymphedema, lymphedema fibrosis, Lymphedema complications, Lymphedema Praecox, Temporary lymphedema, chronic lymphedema, Autosomal genetic trait, Emotional impact of lymphedema, Spreading lymphedema, arm lymphedema, leg lymphedema, Pathophysiology of Lymphedema, Lymphedema Advocacy, Lymphedema in Children, Sentinel Node Biopsy, Lymphoscintigraphy, Axillary Node Biopsy, Complications of Lymphedema, Arm and Leg Swelling in A Baby, Travel Tips for Lymphedema, Male Genital Lymphedema, Female Genital Lymphedema, Lymph Fluid, Magnetic Resonance Imaging, Interstitial Fluid, Massive Localized Lymphedema, Lymphatic Filariasis
The book is compiled so that people who have been touched by lymphedema can share their stories; their trials and tribulations, their sadness and disappointments, their strength and hopes. It is intended to encourage, educate and inspire patients and loved ones, who can often feel isolated and uninformed. We hope to increase awareness and general knowledge of a condition that is often overlooked and misdiagnosed, yet which can have monumental physical and emotional impact on the lives that it affects.
Not every story has a happy ending, yet there is hope. By sharing these stories with one another, we can learn from the struggles and successes that others have experienced, and can help each other to live well with lymphedema.
THE PUZZLE - An Inside Glimpse of Lymphedema
Written by a host of therapists AND patients. I also contributed several articles, including my own history with lymphedema.
How do I get this book ???
Lighthouse Lymphedema Network Store
For Information about Lymphedema Complications
For Lymphedema Personal Stories
For information about Lymphedema Wounds
For information about Lymphedema Treatment Options
For information about Lymphedema and Exercises
For information on Infections Related to Lymphedema
For Information on Children's Lymphedema
For Information on Lymphedema and Cancer
For Information in Spanish - Linfedema
For Information in French - Lymphoedème
Join us as we work for lymphedema patients everywhere:
Advocates for Lymphedema
Dedicated to be an advocacy group for lymphedema patients. Working towards education, legal reform, changing insurance practices, promoting research, reaching for a cure.
Lymphedema People / Advocates for Lymphedema
Children with Lymphedema
The time has come for families, parents, caregivers to have a support group of their own. Support group for parents, families and caregivers of chilren with lymphedema. Sharing information on coping, diagnosis, treatment and prognosis. Sponsored by Lymphedema People.
Lipedema Lipodema Lipoedema
No matter how you spell it, this is another very little understood and totally frustrating conditions out there. This will be a support group for those suffering with lipedema/lipodema. A place for information, sharing experiences, exploring treatment options and coping.
Come join, be a part of the family!
MEN WITH LYMPHEDEMA
If you are a man with lymphedema; a man with a loved one with lymphedema who you are trying to help and understand come join us and discover what it is to be the master instead of the sufferer of lymphedema.
All About Lymphangiectasia
Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.
Lymphatic Disorders Support Group @ Yahoo Groups
While we have a number of support groups for lymphedema… there is nothing out there for other lymphatic disorders. Because we have one of the most comprehensive information sites on all lymphatic disorders, I thought perhaps, it is time that one be offered.
Information and support for rare and unusual disorders affecting the lymph system. Includes lymphangiomas, lymphatic malformations, telangiectasia, hennekam's syndrome, distichiasis, Figueroa syndrome, ptosis syndrome, plus many more. Extensive database of information available through sister site Lymphedema People.
Teens with Lymphedema
All About Lymphoedema - Australia
Mid-Missouri Lymphedema Network Ellis Fischel Cancer Center Columbia, MO 65203 US Tel: 1-573-884-3827
PHCMC Lymphedema Support Group 15031 Rinaldi St. Mission Hills, CA 91345-1207 US
Lymphedema Education and Support Program Boca Raton, Florida
My Life with Lymphedema
Developmental Disorders of the Lymphatics
Edema and Related Medical Conditions
Lymphedemaville - AOL Blog
Disorders of the Lymph System
Clinical Trials Glossary
Immune System Glossary
In our forums section, you will find over 3,000 posts with hundreds of additional articles encompassing every aspect of lymphedema you can imagine. Information, questions and answers that will enable you to discover not only how to live with lymphedema, but to manage it, overcome it and lead a life that is rich, meaningful and rewarding.
Visitors can read the forums, but only members can post and answer questions. Come and be a part of the largest lymphedema online network in the world.
Here is a list of the forums:
Membership Rules in New Forums
Lymphedema People Administrative, Management
Registry of Lymphedema Doctors
Lighthouse Lymphedema Network Lymphedema Education & Awareness Program
Daily Lives with Lymphedema
Children and Lymphedema
Young People with Lymphedema
Related Medical Conditions (often associated with lymphedema)
Complications of Lymphedema
Treatment Information for Lymphedema
Therapists, Clinics and Hospitals
Skin care, conditions and complications
Lymphedema and Cancer
Advocacy and Governmental Resources
Genetics, Research, Lymphangiogenesis, Angiogenesis
Organizations, Support Groups, Vendors
Thanks to our super IT manager and cofounder, Brad, you can now “like” the forum page and you can also, now send a note to Facebook… Awesome!!! More changes ahead as we try to improve our service to our very special lymphedema family.
Reviewed Feb. 17, 2013