Lymphedema Lymphatic obstruction


LYMPHEDEMA (LYMPHATIC OBSTRUCTION)

Description:

Swelling (especially in subcutaneous tissues) as a result of obstruction of lymphatic vessels or lymph nodes and the accumulation of large amounts of lymph in the affected region.

The lymphatic system helps coordinate the immune system's function to protect the body from foreign substances and includes an extensive network of lymph vessels and lymph nodes. Here's how the lymphatic system works:

Excess fluid is collected from the space between tissues in the body and moves through the lymph vessels. The fluid (now called lymph) isn't pumped through the body like blood, but instead is "pushed" through the lymph system as the vessels are compressed by surrounding muscles.

Filters called lymph nodes remove certain harmful substances from the lymph fluid, such as bacteria and debris. The fluid from most tissues or organs is filtered through one or more lymph nodes before draining into the bloodstream.

Lymphedema is an abnormal buildup of fluid that causes swelling, most often in the arms or legs. The condition develops when lymph vessels or lymph nodes are missing, impaired, damaged, or removed.

There are two types of lymphedema: primary and secondary. Primary lymphedema is rare and is caused by the absence of certain lymph vessels at birth, or abnormalities in the lymphatic vessels. Secondary lymphedema occurs as a result of a blockage or interruption that alters the flow of lymph through the lymphatic system and can develop from an infection, malignancy, surgery, scar tissue formation, trauma, deep vein thrombosis (blood clot in a vein), radiation, or other cancer treatment.

Lymphedema is not the same as edema, which is another condition that causes arm or leg swelling. In both problems there is too much fluid in the limb, but only lymphedema results from blocked drainage. Without a blockage, the fluid can be pushed forward in your lymphatic system, so you can see pitting small temporary indentations left on the skin after you press on the swollen area. Pitting does not happen when you press on skin if you have lymphedema.

In most cases of lymphedema, the lymphatic system has been injured so that the flow of lymph is blocked either temporarily or permanently. Common causes include:

    Surgical Damage: Surgical cuts and the removal of lymph nodes can interfere with normal lymph flow. Sometimes, lymphedema appears immediately after surgery and goes away quickly. In other cases, lymphedema develops from one month to 15 years after a surgical procedure. Lymphedema is most commonly seen in people who have had surgery for breast cancer. It develops in up to 25% of those who have a breast removed (mastectomy) along with the lymph nodes under the arm. The risk doubles for those who also receive radiation treatments to the underarm area. Lymphedema also may occur after surgery for prostate or testicular cancer, melanoma and cancer in the lower abdomen.

  • An infection involving the lymphatic vessels: Rarely, a bacterial infection that causes a red stripe on the arm or leg (lymphangitis) can be severe enough to cause lymphedema. In areas of the tropics and subtropics, such as South American, the Caribbean, Africa, Asia and the South Pacific, parasites commonly cause lymphedema. Filariasis, a parasitic worm infection, blocks the lymph channels and causes swelling and thickening below the skin, usually in the legs. Filariasis rarely is seen in the United States, except in people who emigrated from tropical areas.

  • Cancer Lymphoma: A cancer that starts in the lymph nodes, or other types of cancer that spread to the lymph nodes may block lymph vessels.

  • Radiation therapy for cancer: This treatment can cause scar tissue to develop and block the lymphatic vessels.

In a rare form of lymphedema present at birth, the lymphatic vessels are absent or do not function properly. This type of lymphedema is called primary lymphedema. Doctors diagnose three types of primary lymphedema according to when symptoms first appear: at birth, at puberty or, for most others with this condition, after age 35. Some types are inherited and linked to genetic abnormalities.

Stages of Lymphedema:

Latency Stage: There is no visible swelling, but there is a feeling of heaviness, fullness or discomfort.

Stage I:

Spontaneously Reversible: Swelling may decrease with elevation. The swelling causes indentation in the skin after pressure is applied.

Stage II:

Spontaneously Irreversible: Pitting becomes progressively more difficult. Tissue under the skin begins to harden.

Stage III:

Lymphostatic Elephantiasis: A tremendous increase in volume is present; the tissue is very hard and skin changes are beginning to occur.

Lymphedema is a very serious condition. There is no cure for lymphedema, and once it develops, it can be a long-term, uncomfortable, and sometimes painful condition requiring daily treatment. When lymphedema is not treated, the protein-rich fluid continues to accumulate, leading to even more swelling and hardening (referred to as fibrosis) of the tissues. This fluid is a good culture medium for bacteria, thus resulting in reoccurring infections when there are injuries to the skin, decrease or loss of functioning of the affected limbs, and skin breakdown. Infections, referred to lymphangitis, can affect the connective tissue under the skin. Repeated infections may result in scarring, which, in turn, makes the tissue susceptible to more swelling and infection. Over time, these infections result in tissue hardening (i.e., fibrosis), which is a characteristic of advanced chronic lymphedema. In very severe cases, untreated lymphedema may even result in a rare form of lymphatic cancer called lymphangiosarcoma.

Lymphedema affects approximately 100 million people worldwide, including at least 3 million people in the United States.

Patients with chronic lymphedema for 10 years have a 10% risk of developing lymphangiosarcoma, the most dreaded complication of this disease. Patients with this tumor commonly present with a reddish purple discoloration or nodule that tends to form satellite lesions. It may be confused with Kaposi sarcoma or traumatic ecchymosis. This tumor is highly aggressive, requires radical amputation of the involved extremity, and has a very poor prognosis. The 5-year survival rate is less than 10%, and the average survival following diagnosis is 19 months. This malignant degeneration is most commonly observed in patients with postmastectomy lymphedema (Stewart-Treves syndrome), where incidence is estimated to be 0.5%.

Other complications of lymphedema include recurrent bouts of cellulitis and/or lymphangitis, deep venous thrombosis, severe functional impairment, cosmetic embarrassment, and necessary amputation. Complications following surgery are common and include partial wound separation, seroma, hematoma, skin necrosis, and exacerbation of foot or hand edema.

Symptoms:

Lymphedema causes swelling with a feeling of heaviness, tightness or fullness, usually in an arm or leg. In most cases, only one arm or leg is affected. Swelling in the leg usually begins at the foot, and then moves up if it worsens to include the ankle, calf and knee. Additional symptoms can include:

    A dull ache in the affected limb.

  • A feeling of tightness in the skin of the affected limb.

  • Difficulty moving a limb or bending at a joint because of swelling and skin tightness.

  • Shoes, rings or watches that suddenly feel too tight.

Lymphedema can make it easier to develop a skin infection. Signs of infection include fever, pain, heat and redness. If lymphedema becomes chronic (long lasting), the skin in the affected area often becomes thickened and hard.

Causes and Risk factors:

Primary lymphedema is an inherited condition where the patient is born without lymph vessels and nodes. The swelling associated with primary lymphedema usually occurs during adolescence and affects the foot or calf. A rare form of primary lymphedema, called Milroy's disease, occurs in pregnancy. However, secondary lymphedema, or acquired lymphedema, develops as a result of an injury to the lymph system. Specific causes include surgical treatments for certain types of cancers, especially those cancers that currently require the removal of lymph nodes. Radiation treatment for cancer or for some AIDS-related diseases such as Kaposi's sarcoma may also result in lymphedema, as radiation may damage or destroy lymph nodes or cause the formation of scar tissue that can interrupt the normal flow of the lymphatic fluid. Specific cancers and their treatment that may result in lymphedema include malignant melanoma, breast (in both women and men), gynecological, head and neck, prostate, testicular, bladder, and colon cancer. Other causes of lymphedema include trauma to the lymphatic system from burns, liposuction, tattooing, injuries, surgery, radiation, obesity, heart or circulatory disease, and multiple sclerosis. People at risk may not develop the condition immediately, but develop the condition weeks, months, or even years later. Aircraft travel has been linked to the development of lymphedema in patients after cancer surgery, possible due to the decreased cabin pressure.

In Western countries, one of the most common causes of lymphedema is mastectomy with axillary dissection (removal of the breast and underarm lymph tissue for treatment of breast cancer), which may result in lymphedema of the breast, underarm, or arm on the side of the surgery in 10-20% of patients. This occurs because the lymphatic drainage of the arm passes through the axilla (armpit), and tissue in the axilla is removed during the mastectomy. To reduce the risk of developing lymphedema after breast cancer treatment, there is an alternative treatment that avoids axillary lymph node dissection. Sentinel lymph node biopsy is a new diagnostic procedure used to determine whether the breast cancer has spread (metastasized) to axillary lymph nodes. A sentinel lymph node biopsy requires the removal of only one to three lymph nodes for close review by a pathologist. If the sentinel nodes do not contain tumor (cancer) cells, this may eliminate the need to remove additional lymph nodes in the axillary area. Early research on this technique indicates that sentinel lymph node biopsy may be associated with less pain and fewer complications than standard axillary dissection. Because the procedure is so new, long-term data are not yet available. However, there is still a risk of developing lymphedema because of follow-up radiation treatments or chemotherapy, which may also damage the lymph nodes.

People who have developed lymphedema after cancer treatment should be checked for a possible recurrence of cancer if they experience a sudden increase in swelling; the tumor growth may be responsible for blocking lymphatic flow.

Risk factors for lymphedema include the following:

    Breast cancer, if the patient received radiation therapy or had lymph nodes removed. Radiation therapy to the underarm area after surgical removal of the lymph nodes and having a larger number of lymph nodes removed increases the risk of lymphedema.

  • Surgical removal of lymph nodes in the underarm, groin, or pelvic regions.

  • Radiation therapy to the underarm, groin, pelvic, or neck regions.

  • Scar tissue in the lymphatic ducts or veins and under the collarbones, caused by surgery or radiation therapy.

  • Cancer that has spread to the lymph nodes in the neck, chest, underarm, pelvis, or abdomen.

  • Tumors growing in the pelvis or abdomen that involve or put pressure on the lymphatic vessels and/or the large lymphatic duct in the chest and block lymph drainage.

  • Having an inadequate diet or being overweight. These conditions may delay recovery and increase the risk for lymphedema.

Diagnosis:

Your doctor will ask you whether you have had any surgery, radiation treatments, or infections in the affected area. The doctor may ask if you have ever had a blood clot. If a child has lymphedema, the doctor will ask if anyone in your family had leg swelling starting at a young age. This may indicate an inherited disorder.

Your doctor will examine the swollen area and press on the affected skin to look for a fingertip indentation (pitting). Your doctor may measure the circumference of the affected arm or leg to determine how swollen it is compared to the other one. The doctor will look for signs of infection, including fever, redness, warmth and tenderness.

Usually, no specific testing is necessary to diagnose lymphedema. But tests may be ordered if there is no obvious cause for your condition:

    A blood count can look for a high level of white cells, which means you might have an infection.

  • An ultrasound can look for blood clots, which can cause an arm or leg to swell.

  • A computed tomography (CT) scan looks for a mass or tumor that could be blocking lymph vessels in the swollen arm or leg.

  • Lymphoscintigraphy and lymphangiography can identify blocked areas of lymph flow and detect any abnormalities in the lymph vessels.

Treatment:

Medical Therapy: The goal of conservative therapy is to eliminate protein stagnation and to restore normal lymphatic circulation. Initiate therapy as early as possible before extensive irreversible fibrosclerotic changes occur in the interstitium. These techniques are often cumbersome, uncomfortable, inconvenient, and time-consuming. Strict compliance is essential, and treatment lasts throughout the lifetime of the individual. The majority of compliant patients can be treated successfully with conservative measures.

Meticulous hygiene is necessary to remove keratinaceous debris and bacteria. Cleanse the skin regularly and dry thoroughly. Regular inspection is necessary to identify any open wounds or developing cellulitis. Bland skin moisturizers applied conservatively may ameliorate cracking and furrowing. Even with excellent skin care, chronic cellulitis may occur. At the earliest signs of infection, institute topical or systemic antifungal or antimicrobial therapy to prevent the development of sepsis. Long-term prophylactic antimicrobial treatment with agents, such as penicillin, cephalexin, or erythromycin, may be required in 15-25% of patients experiencing recurrent lymphangitis or cellulitis. Although relatively uncommon in this country, filariasis is treated with diethylcarbamazine.

Encourage patients to lose weight, avoid even minor trauma, and avoid constrictive clothing that might have a tourniquet effect. Encourage elevation of the affected extremity whenever possible, particularly at night. For lower extremity lymphedema, this may be accomplished by elevating the foot of the bed to an appropriate level.

Patients should use compression garments continuously during the day. They may be removed at night when the extremity is elevated in bed, but they should be replaced promptly each morning. To encourage compliance, the elastic compression garments must fit appropriately. Garments should be custom fit when the extremity is decompressed, they should be comfortable, and they should not have a tourniquet effect. They should also have graduated compression, increasing from distal to proximal, on the affected extremity.

Intermittent pneumatic pump compression therapy may also be instituted on an outpatient basis or in the home. These manual lymphatic devices are most appropriate prior to fibrosclerotic evolution, and they assist in preventing fibrosclerotic evolution of the condition. These devices provide sequential active compression from distal to proximal, effectively milking the lymph from the extremity. In Europe, the best available nonsurgical therapy is manual lymphatic drainage according to the Vodder and/or Leduc techniques. Compression garments are essential between treatments. Contraindications to this therapy include congestive heart failure, deep vein thrombosis, and active infection. Similarly, other authors advocate manual massage of the affected extremity to recruit collateral vessels so that the accumulated lymph can be drained into neighboring regions with normally functioning lymphatics.

Diuretics have no role in the treatment of lymphedema.

Benzopyrenes, including flavonoids and coumarin, have become a useful adjuvant in other countries but are currently not available for clinical use in the United States. These drugs bind to accumulated interstitial proteins, inducing macrophage phagocytosis and proteolysis. The resulting protein fragments pass more readily into the venous capillaries and are removed by the vascular system.

Surgical Therapy: Surgical treatment is palliative, not curative, and it does not obviate the need for continued medical therapy. Moreover, it is rarely indicated as the primary treatment modality. Rather, reserve surgical treatment for those who do not improve with conservative measures or in cases where the extremity is so large that it impairs daily activities and prevents successful conservative management. The goals of surgical therapy are volume reduction to improve function, facilitation of conservative therapy, and prevention of complications. A myriad of surgical procedures have been advocated, reflecting a lack of clear superiority of one procedure over the others. In general, surgical procedures are classified as physiologic or excisional.

Physiologic procedures attempt to improve lymphatic drainage. Multiple techniques have been described, including omental transposition, buried dermal flaps, enteromesenteric bridging, lymphangioplasty, and microvascular lympholymphatic or lymphovenous anastomoses. None of these techniques has clearly documented favorable long-term results. Further evaluation is necessary. Moreover, many of these physiologic techniques also include an excisional component, making it difficult to distinguish between the two approaches.

Excisional techniques remove the affected tissues, thus reducing the lymphedema load. Some authors advocate suction-assisted removal of subcutaneous tissues, but this technique is difficult because of the extensive subcutaneous fibrosis that is present. Additionally, this approach does not reduce the skin envelope, and the lymphedema often rapidly recurs. Suction-assisted removal of subcutaneous tissue followed by excision of the excess skin envelope has no clear advantage over direct excisional techniques alone.

The Charles procedure is another quite radical excisional technique. This procedure involves the total excision of all skin and subcutaneous tissue from the affected extremity. The underlying fascia is then grafted, using the skin that has been excised. This technique is extreme and is reserved for only the most severe cases. Complications include ulceration, hyperkeratosis, keloid formation, hyperpigmentation, weeping dermatitis, and severe cosmetic deformity.

Staged excision has become the option of choice for many authors. This procedure involves removing only a portion of skin and subcutaneous tissue, followed by primary closure. After approximately 3 months, the procedure is repeated on a different area of the extremity. This procedure is safe, reliable, and demonstrates the most consistent improvement with the lowest incidence of complications.

Medicine and medications:

Medication is also commonly tried to treat the swelling caused by lymphedema chronic, abnormal swelling of a part of the body. It is an accumulation of protein-rich fluid in the superficial tissues that most commonly occurs in the arms or legs but can affect any part of the body. Diuretics are most commonly prescribed. They are designed to stimulate the kidneys to remove excess water from the bloodstream. They, however, do not stimulate the removal of stagnant lymph in soft tissue and can cause dehydration of lymph fluid, causing an even higher content of protein in the tissue space, causing the advancement of lymphedema. It is, however, appropriate to use diuretics in conjunction with treatment for lymphedema if the patient needs treatment for other medical conditions that are normally treated with diuretics.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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