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About Lymphedema

LYMPHEDEMA

Lymphedema is a disorder of the lymphatic system. While lymphedema can occur spontaneously, most cases of lymphedema in the United States result from treatment for cancer with surgery, radiation or both.

Lymphedema manifests swelling in either an arm or a leg in most cases. In the cases of arm swelling, lymphedema tends to occur after treatment for breast cancer. It’s estimated that between 5% to over 50% of patients who have treatment for breast cancer, which involves surgery to remove the lymph nodes as well as radiation therapy, develop lymphedema in the arm to some degree.

Lymphedema swelling can also occur in the leg. This is most commonly due to treatment for a gynecological cancer, such as a cervical cancer. This also includes cervical cancer treatment with radiation, surgery to remove lymph nodes or both. It’s estimated that approximately twenty percent of patients that have surgery to remove lymph nodes and also radiation, will develop lymphedema of the leg to some extent.

Initially, the swelling in lymphedema occurs because of blockage of the lymphatic system. The lymphatic system is related to the immune system. The lymphatic system involves movement of clear fluid, known as lymph, in the arms and legs. When this fluid can no longer drain properly, such as after treatment with surgery or radiation therapy, then the fluid builds up and swelling occurs.

Initially, most or all the swelling is due to the collection of excess fluid. This fluid is quite inflammatory, and eventually additional solids and fat deposit into the swollen area. The swollen limb then goes from a fluid-predominant state to a more chronic, solid-predominant state.

The traditional therapy for lymphedema has been non-surgical and involves a course of complete decongestive therapy (CDT) administered by a trained lymphedema therapist. This involves specialized manual lymphatic drainage, specialized massage, exercises, skin care and compression. Additional non-surgical therapy can include the use of compression garments and other modalities to further reduce the swelling in the limb.

Conservative therapy tends to be more effective early in the course of the disease when fluid is predominant.

Surgical options to treat lymphedema are available today. These include: vascularized lymph node transfer (VLNT), lymphatic venous anastomosis (LVA) and suction-assisted protein lipectomy (SAPL).

There is no one procedure that is best suited to all presentations of lymphedema. In the early, fluid-predominant stages, VNLT and LVAs tend to be most effective. These procedures typically improve the fluid drainage of the limb and are thus most effective early on when swelling is still mostly fluid.

In later stages, when the swelling is mostly solid, the SAPL procedure is more effective. This allows the removal of the excess fat, proteins and other solids that have deposited over months or years in the swollen extremity.

VLNT involves the transfer of lymph nodes from the portion of the body where they are in excess, to another part of the body that is affected by lymphedema. The lymph nodes are moved with a vascular circulation involving the reattachment of a connected artery and vein to lymph nodes and surrounding fatty tissues allowing them to survive and integrate into the site where they are needed. These transferred lymph nodes allow the built-up fluid to drain and allow the healing of lymphatics in the recipient area into the lymph nodes for additional improved drainage. In some cases, scar release can be performed at the same time as the VLNT, which can further improve drainage of the limb.

LVAs involve the connection of the lymphatics directly into the veins. The lymphatics are quite small, from 0.1 mm up to approximately 0.6 mm in size. The lymphatics are connected to veins that have one-way valves in them to allow excess lymphatic fluid that is returned directly to the venous system, bypassing the areas of blockage. The venous system, even in healthy individuals, is the ultimate outlet for the lymphatic fluid in any case.

SAPL involves the removal of excess fats, proteins and other solids from the effected limb. This is usually performed for patients whose lymphedema has progressed to a more chronic and solid state. Small incisions are made and a long, thin cannula is introduced to allow removal of the solids. The method has been called “lymphatic liposuction.” Lifelong use of compression garments is required after the performance of SAPL to prevent to recurrence of swelling.

It is important to note that this procedure is quite different from cosmetic liposuction. It involves very close work with a trained lymphedema therapist, the “dry technique,” and the use of a sterile, surgical tourniquet. Specially fitting, custom-compression garments are also required and must be worn afterward to prevent to re-accumulation of fluid.

Dr. Granzow has pioneered the use of a VLNT after SAPL to reduce the need of post-operative compression garments. VLNT was introduced by Dr. Granzow after SAPL in selected patients to reduce the need for continuous, compression-garment use. The results were successful and have been published in major medical journals.

Again, it is to be emphasized that not one procedure will help all patients. The procedures must be carefully selected for the right patient at the right time for the best results possible.

Lymphedema describes the accumulation of fluids, proteins and fat that can result from a defect in the lymphatic system. This causes an affected extremity slowly to swell, decreasing function, altering appearance and increasing the likelihood of significant cellulitis and infection. The effects can be quite debilitating for affected patients.

In the United States, lymphedema is usually secondary and most commonly the result of cancer treatment with lymphadenectomy and radiation therapy. It is estimated that up to 10-40% of patients who have undergone axillary lymph node dissection followed by radiation therapy develop some degree of ipsilateral upper extremity lymphedema. Outside the United States, an estimated 140-250 million cases of lymphedema exist, mostly caused by filariasis (a type of infection) in developing countries.

TYPES OF LYMPHEDEMA

Lymphedema may be classified as primary or secondary, based on underlying cause.
Primary lymphedema results from abnormal development of the lymphatic system. Most cases are sporadic and have no family history, and are thought to occur secondary to an underlying developmental abnormality that manifests at some point later in life. Secondary lymphedema describes lymphedema that occurs through damage or dysfunction of normal lymphatics.

The most common causes of lymphedema in developed countries are lymphatic damage from lymphadenectomy (lymph node dissection), radiation, infection, compression, trauma, burns, insect bites, vascular surgery or tumor invasion. Other causes include vein stripping, peripheral vascular surgery, lipectomy, burns and burn scar excision.

Worldwide, filariasis is the most common cause with damage to the lymphatics. Filariasis is caused by invasion by an offending parasite called Wuchereria bancrofti.

STANDARD NON-SURGICAL TREATMENT

Currently lymphedema is thought by many authorities to be a permanent problem with little hope for a permanent cure. The advanced surgical procedures offered by Dr. Granzow represent a significant advance in the treatment of this condition.

The mainstay of lymphedema treatment has been non-surgical, complete decongestive therapy (CDT) administered by a trained lymphedema therapist. This can be performed by a physical, occupational or massage therapist with special lymphedema training and may involve the use of compression garments, bandages, specialized massage, and special sequential pumps and devices.

Manual lymphatic drainage (MLD) is a massage type therapy designed to move the lymphatic fluid and proteins out of the affected area and back into the circulation.
Complete decongestive therapy (CDT) consists of patient education on skin care, MLD, bandaging and compression garment therapy, breathing exercises and dietary measures.

SURGICAL TREATMENT – PAST HISTORY

Unlike the current, effective surgical procedures, previous, surgical methods to treat lymphedema have been radical and often ineffective. The resulting deformity many times was significantly worse than the original lymphedema. Complications were also numerous and included necrosis of the transferred skin flaps, recurrence or worsening of the lymphedema, permanent nerve damage, loss of extremity function and amputation.

The Charles procedure involved debulking of the extremity by debridement of the edematous skin and boggy subcutaneous tissue down to muscle and fascia. The overlying resected skin was used as a skin graft over the affected area. The resulting deformity often was worse than the original lymphedema.

The Thompson procedure was similar but also involved retaining a debulked flap of skin in the extremity. This flap was thought to contain subdermal lymphatics to “wick” fluid to the deeper lymph channels.

SURGICAL TREATMENT – CURRENT AND FUTURE

Effective surgical treatments now exist for lymphedema. The FLO System (Functional Lymphedema Operations) includes procedures which have been shown to be safe and effective in the treatment of lymphedema. Currently, the FLO system includes vascularized lymph node transfers (VLNT), lymphaticovenous anastomoses (LVA), and suction-assisted protein lipectomy (SAPL).

Microsurgery procedures to drain the excess lymphatic fluid, such as vascularized lymph node transfers and lymphaticovenous anastomoses, have been improved significantly and can offer moderate to excellent improvements in the symptoms of lymphedema. Suction-assisted protein lipectomy also now offers the possibility to treat the solid portion of a lymphedema-affected extremity as well.

These specialized techniques are performed by only a small handful of highly specialized microsurgeons worldwide. Dr. Granzow is a pioneer in this area and is the first surgeon who is proficient in all three modern types of surgery. He was also the first to combine the procedures to allow both effective large-volume reduction and decreased compression garment use in the same patient suffering from long-term lymphedema. He works with colleagues from around the world continually to further refine these procedures.

Vascularized lymph node transfers and lymphaticovenous anastomoses are microsurgical procedures that are most effective in patients whose extremity circumference reduces significantly with compression wrapping, indicating most of the edema is fluid.

Patients who do not respond to compression are less likely to fare well with vascularized lymph node transfers and lymphaticovenous anastomoses because a greater amount of their increased extremity volume consists of fibrotic tissue, protein or fat. The non-compressible limbs of these patients may respond to effective volume reduction through specially performed suction-assisted protein lipectomy instead.

Lymphedema vs Lipoedema (Lipedema)

Lymphedema and lipoedema (lipedema) are two distinct disorders even though both involve swelling in the arms and legs. In short, lymphedema is a disorder of the lymphatic system and is commonly caused by dysfunction in the flow of lymph fluid through the arms or legs. Lipoedema, in contrast, does not involve the lymphatic system, but a pathologic, symmetric deposition of fat that most often effects the lower extremities and almost exclusively occurs in women.

Lymphedema is a disorder of the lymphatic system. It involves circulation of lymph fluid and is related to the immune system. When blockages occur in the circulation of lymphatic fluid, then swelling in an arm or a leg can result. In most cases, lymphedema in the United States occurs following radiation therapy and/or removal of lymph nodes as cancer treatment. In a small number of cases, lymphedema can be spontaneous in onset or congenital. Typically, one extremity, either and arm or a leg, is effected. Lymphedema in the arms typically occurs after treatment for breast cancer and lymphedema in the legs typically occurs after treatment for gynecologic malignancies such as cervical cancer.

Lymphedema is treated with conservative therapy such as complete decongestive therapy, which is administered by a trained lymphedema therapist. Highly technical surgeries can also be used to treat lymphedema. Fluid-based lymphedema can be treated with vascularized lymph node transfer (VLNT) or lymphatic venous anastomosis (LVA). In chronic, solid-predominant cases, lymphedema can be treated with suction-assisted protein lipectomy (SAPL). These procedures have been shown to be very effective in significantly decreasing excess volume, compression garment use and therapy required for patients with lymphedema in the arms or legs. The type of procedure must be carefully matched to the type of patient for the best result.

In contrast, lipoedema is a disorder that involves the deposition of pathologic fat mostly in the legs. The fat distribution is disproportionate to the normal distribution of fat. These fatty areas often resemble fatty tumors and are typically quite painful to the touch. The fat mainly deposits on the insides of the legs and knees, causing pain when the legs touch and rub together. This causes the legs to be pushed further and further out during walking, causing eventual damage to the knees and ankles, with significant wear on the insides of the feet and shoes.

The typical treatment for lipoedema is initially conservative therapy with complete decongestive therapy by a trained lymphedema therapist. Otherwise, some patients may be candidates for tumescent liposuction aspiration and removal of the pathologic fat.

In some cases, lipoedema and lymphedema disorders may overlap and the excess fat deposited in lipoedema may even cause lymphedema. Therefore, some patients who have been treated for lipoedema with either complete decongestive therapy or tumescent liposuction may require long-term treatment with compression garments as well.

FACTS

Lymphedema is characterized by the excess accumulation of fluid, fat and protein in an area of the body. Most commonly, lymphedema can occur after the treatment of cancer of the breast or other areas with surgery and / or radiation therapy. In some cases, the condition may be congenital, or occur spontaneously. Lymphedema has different presentations that require different types of surgical and non-surgical treatment. There is no single method of treatment to address all types of lymphedema.

Conservative therapy has been the mainstay of lymphedema treatment. All patients with lymphedema should be seen by an experienced therapist specifically trained in lymphedema therapy.

Microsurgery is used by specialized surgeons to manipulate tiny blood and lymphatic vessels. Few plastic surgeons routinely perform advanced microsurgery, and only a small handful have been trained to treat lymphedema.

Three modern surgical methods exist to effectively treat lymphedema. These include Vascularized Lymph Node Transfer (VLNT), lymphaticovenous anastomosis (LVA) and Suction-Assisted Protein Lipectomy (SAPL). Dr. Granzow is trained in all three of these methods. It is important to note that these surgical procedures are not related to the previously ineffective lymphatic debulking procedures of the past.

In lymphedema-affected extremities that are early in their presentation and still contain significant amounts of fluid, drainage can be accomplished by means of VLNT and LVA. In more advanced cases of lymphedema, lymphatic fluid has been replaced by fat and protein. These limbs respond poorly to conservative compression therapy and may be soft from fat or firm from protein and fibrosis. Suction-Assisted Protein Lipectomy is the best method used to such cases.

During your consultation, Dr. Granzow will help you determine which type of edema is present and which procedure will offer the most long-term benefit.

VASCULARIZED LYMPH NODE TRANSFER (VLNT):

Vascularized Lymph Node Transfer replaces the nodes lost through surgery and/or radiation with healthy, functioning lymph nodes takes from elsewhere in the body. Dr. Corinne Becker from Paris, France, initially pioneered this type of procedure. Lymph nodes are usually taken from the groin area with their supporting blood vessels and moved to a new location in the axilla (armpit) to treat upper extremity lymphedema. Using microsurgical techniques, the blood vessels are reconnected to vessels in the chest or armpit to support the transferred lymph nodes while they heal in the new area.

Most often, this method of lymph node transfer is performed together with a DIEP flap breast reconstruction. In some cases, the lymph nodes alone, without breast reconstruction, may be transferred and reconnected for improved drainage. This technique functions best when a patient’s arm is still soft and compressible with lymphatic fluid.
 Paris Becker OR


Dr. Granzow in the operating room with Dr. Corinne Becker in Paris, France

LYMPHATICOVENOUS ANASTOMOSIS (LVA):

Lymphaticovenous anastomosis is a method of directly connecting the lymphatic vessels in the affected area of the body to the tiny veins nearby. This allows the backed-up lymphatic fluid to drain directly into the vein and be returned to the body’s natural circulation. The goal of the procedure is to reduce extremity swelling, pain and discomfort and decrease or eliminate the need for further use of compression garments. In our experience, most patients have results which range from a moderate to a significant improvement of their extremity swelling. To be a candidate for this procedure the patient’s edema must be soft and compressible. If the edema is firm and fibrotic the patient is more likely to be a candidate for suction-assisted protein lipectomy (SAPL).


Dr.Jay Granzow and Dr. Isao Koshima performing lymphaticovenous anastomoses in Japan in 2005

SUCTION-ASSISTED PROTEIN LIPECTOMY (also known as LYMPHATIC LIPOSUCTION):

The liposuction for lymphedema technique is different from the normal cosmetic liposuction and has been pioneered by Dr. Hakan Brorson in Malmo, Sweden. This procedure will reduce the size of the affected arm or leg to approximate the normal, unaffected side. The technique also has been proven to decrease the incidence of cellulitis in the affected limb.

Dr. Granzow has trained with Dr. Brorson and leads one of three teams located in the United States. This technique should not be performed by surgeons who are not specifically trained in this specialized type of liposuction. A team approach between surgeon and lymphedema therapist is required.

Patients that are candidates for this procedure have advanced disease where the lymphatic fluid in the affected limb has been replaced by fat and protein. At this point the limb may no longer respond well to conservative treatment including the use of lymphatic compressive therapy. The skin of patient’s affected limb will leave only a small impression when prolonged pressure is exerted in the location of the edema.

Patients must continue to follow lifelong use of compression garments after this procedure to prevent recurrence. Studies have shown that suction-assisted protein lipectomy does not appear to further damage the already damaged lymphatics.


Dr. Granzow in surgery with Dr. Hakan Brorson in Malmo, Sweden