Lymphedema

Definitions:
Lymphedema, also known as lymphatic obstruction, is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system.

The lymphatic system returns the interstitial fluid to the thoracic duct and then to the bloodstream, where it is recirculated back to the tissues.

Tissues with lymphedema are at risk of infection.

Causes

Lymphedema may be inherited (primary) or caused by injury to the lymphatic vessels (secondary). It is most frequently seen after lymph node dissection, surgery and/or radiation therapy, in which damage to the lymphatic system is caused during the treatment of cancer, most notably breast cancer. In many patients with cancer this condition does not develop until months or even years after therapy has concluded. Lymphedema may also be associated with accidents or certain diseases or problems that may inhibit the lymphatic system from functioning properly. In tropical areas of the world, a common cause of secondary lymphedema is filariasis, a parasitic infection. It can also be caused by a compromising of the lymphatic system resulting from cellulitis.

While the exact cause of primary lymphedema is still unknown, it generally occurs due to poorly-developed or missing lymph nodes and/or channels in the body. Lymphedema may be present at birth, develop at the onset of puberty (praecox), or not become apparent for many years into adulthood (tarda). Some cases of lymphedema may be associated with other vascular abnormalities. Lymphedema affects both men and women. In women, it is most prevalent in the upper limbs after breast cancer surgery and lymph node dissection, occurring in the arm on the side of the body in which the surgery is performed. It may also occur in the lower limbs or groin after surgery for colon, ovarian or uterine cancer in which removal of lymph nodes is required. In men, lower-limb primary lymphedema is most common, occurring in one or both legs. Surgery and/or treatment for prostate, colon and testicular cancers may result in secondary lymphedema, particularly where lymph nodes have been removed or damaged.

The onset of secondary lymphedema in patients who have had cancer surgery has also been linked to aircraft flight (likely due to decreased cabin pressure). For breast cancer survivors, wearing a prescribed and properly-fitted low-compression sleeve and gauntlet may help decrease swelling during flight.

Some cases of lower-limb lymphedema have been associated with the use of Tamoxifen, due to the blood clots and deep vein thrombosis (DVT) that can be caused by this medication.

Symptoms

Symptoms may include severe fatigue, a heavy swollen limb or localized fluid accumulation in other body areas, including the head or neck, discoloration of the skin overlying the lymphedema, and eventually deformity.

Lymphedema should not be confused with edema arising from venous insufficiency, which is not lymphedema. However, untreated venous insufficiency can progress into a combined venous/lymphatic disorder which is treated the same way as lymphedema.

Treatment

Treatment for lymphedema varies depending on the severity of the edema and the degree of fibrosis of the affected limb. Most people with lymphedema follow a daily regimen of treatment as suggested by their physician or certified lymphedema therapist. The most common treatments for lymphedema are a combination of direct lymphatic massage, compression garments or bandaging and the use of intermittent sequential gradient pumps. Complex decongestive physiotherapy is an empiric system of lymphatic massage, skin care, and compressive garments. Although a combination treatment program may be ideal, any of the treatments can be done individually.

Compression

Garments

Elastic compression garments are worn by a person with lymphedema on the affected limb following complete decongestive therapy to maintain edema reduction. Depending on the therapist's discretion, a compression garment may be custom-fit or purchased in over-the-counter, standard sizes. Compression garments are meant to be worn every day to maintain edema reduction and must be replaced on a regular basis.

Bandaging or Wrapping

Compression bandaging, also called wrapping, is the application of several layers of padding and short-stretch bandages to the involved areas. Short-stretch bandages are preferred over long-stretch bandages (such as those normally used to treat sprains), as the long-stretch bandages cannot produce the higher tension necessary to safely reduce Lymphedema and may in fact end up producing a tourniquet effect. During activity, whether exercise or daily activities, the short-stretch bandages enhance the pumping action of the lymph vessels by providing increased resistance for them to push against. This encourages lymphatic flow and helps to soften fluid-swollen areas.

A new adjunct treatment is being taught to therapists using a special type of tape called Kinesio Taping. It is used to help soften edemas.
Sequential Gradient Pump Therapy

Sequential Gradient Pump Therapy has been utilized for over 30 years throughout the world. Compression pump technology utilizes a multi-chambered pneumatic sleeve with overlapping cells, to gently move the lymph fluid. There are several manufacturers of these devices, including Lympha-Press Bio Compression Systems and Flexitouch. These compression devices are recognized treatments and approved by Medicare and most insurance plans in the USA. Patients may often receive treatment on a pump for 10-15 minutes before an MLD (Manual Lymphatic Drainage) session to help break up fibrotic (hard) tissue. By softening or breaking up the fibrosis, the MLD Therapist may be more effective with the given treatment. Many patients may benefit from a pump without ever going to MLD sessions, as they are very beneficial, but not a requirement. However, after completion of the appropriate number of MLD sessions, many patients will benefit from a home use of a sequential pump. A recent Stanford University medical studyshowed that patients receiving the combined modalities of MLD/CDT and pneumatic pumping had a greater overall reduction in limb volume than patients receiving only MLD/CDT.


Complete Decongestive Therapy or Manual Lymph Drainage

Complete decongestive therapy (CDT) is a primary tool in lymphedema management consisting of manual manipulation of the lymphatic ducts, short stretch compression bandaging, therapeutic exercise, and skin care. The technique was pioneered by Emil Vodder in the 1930s for the treatment of chronic sinusitis and other immune disorders. Initially, CDT involves frequent visits to a certified therapist with a doctor's prescription. Once the lymphedema is reduced, increased patient participation is required for ongoing care, along with the use of elastic compression garments and non-elastic directional flow foam garments.

Manual manipulation of the lymphatic ducts consists of gentle, rhythmic massaging of the skin to stimulate the flow of lymph and its return to the blood circulation system. In the blood’s passage through the kidneys, the excess fluid is filtered out and eliminated from the body through urination. The treatment is very gentle and a typical session will involve drainage of the neck, trunk, and involved extremity (in that order), lasting approximately 40 to 60 minutes. CDT is generally effective on non-fibrotic lymphedema and less effective on more fibrotic legs, although it has shown to help break up fibrotic tissue.


Surgical techniques

Surgical techniques for correcting lymphedema may be excisional or physiological. Excisional techniques include:

* Circumferential excision of the lymphedematous tissue followed by skin grafting (Charles technique); longitudinal removal of the affected segment of skin and subcutaneous tissue and primary closure (Homans technique); excision of subcutaneous tissue and tunneling of a dermal flap through the fascia into a muscular compartment of the leg (Thompson technique).

* Lympholymphatic anastomosis (autologous lymphatic grafts to bridge obstructed lymphatic segments); lymphovenous shunt (anastomosis of lymphatic channels to veins); lymphangioplasty enteromesenteric flap omental transfer (pedicled portion of omentum transposed to the affected limb).

* Modified Liposuction has been developed in Sweden in recent years to remove adipose tissue associated with longstanding lymphedema, primarily in the upper region. This technique is meant to reduce the volume of a limb and does not cure lymphedema. Compression garments and compression bandages must still be worn after the operation.