Lymphedema Strategies for Investigation and Treatment: A Review

Lymphedema Strategies for Investigation and Treatment: A Review

Oct/Dec 2012

Pankaj Tiwari MD 
Michelle Coriddi MD 
Susan Lamp BSN, RN, CPSN 

Plastic Surgical Nursing

**For information only.  There is much controversery regarding surgical treatment for lymphedema**

Abstract

The goal of this article was to define lymphedema as a disease entity, to introduce the American Lymphedema Framework Project, and to summarize current surgical strategies on the horizon in the surgical treatment of lymphedema.

LYMPHEDEMA DEFINED

Alongside the arterial and venous vasculature, the lymphatic system is a part of the circulatory system. Lymphatic channels primarily regulate the flow of fluid in the interstitium (Ellis, 2006). Under normal conditions, venous capillaries reabsorb 90% of the fluid in the tissues, and lymphatic channels absorb the remaining 10% of lymph fluid, proteins, and other molecules (Warren, Brorson, Borud, & Slavin, 2007). Lymphatic fluid passes to regional lymph node basins. Ultimately, the lymphatic fluid is transported back into the subclavian vein to enter the venous system via the thoracic duct.

Lymphedema is an external or internal manifestation of lymphatic insufficiency and deranged lymph transport (International Society of Lymphology, 2009). This insufficiency causes an accumulation of protein-rich interstitial fluid, leading to distention, proliferation of fatty tissue, and progressive fibrosis. Skin changes such as thickening and hair loss may occur. Progressive lymphedema without adequate management can lead to functional impairment, compromised quality of life, and deformity. Clinically, lymphedema is noted as swelling of the involved extremity. The head and neck, breast, or genitalia may also be affected (McWayne, & Heiney, 2005; Rockson, 2010; Smeltzer, & Stickler, 1985).

Lymphedema is generally classified as either primary or secondary. Primary lymphedema (hereditary) is related to congenital malformation of the lymphatic channels. Secondary lymphedema results from disruption to the lymphatic system. Primary lymphedema can result from any one of a number of disorders that may be sporadic or hereditary. Syndromes such as Milroy's disease and Prader-Willi syndrome have lymphedema as an element of their clinical manifestations to varying degrees. The estimated prevalence of primary lymphedema is 1.15 in 100,000 persons under the age of 20 years Milroy's Disease (Smeltzer & Stickler, 1985). In children, the two main causes are Milroy's disease and lymphedema distichiasis (International Society of Lymphology, 2009).

Secondary lymphedema is a consequence of removal or damage to lymph nodes, fibrosis of the nodes (postradiotherapy), and trauma or infection secondary_lymphedema Rockson, 2010). Upper extremity lymphedema is commonly associated with the treatment of breast cancer. The degree of lymphedema has been well recognized to correlate with the number of lymph nodes that have been removed and the extent of radiotherapy to the axillary region. Lower extremity lymphedema is most often seen in survivors of uterine and prostate cancer, as well as melanoma and lymphoma survivors (Meneses & McNees, 2007). Most cancer survivors develop lymphedema within 3 years of treatment (Petrek, Senie, Peters, & Rosen, 2001).

In addition to cancer ablation, side effects of advanced diseases such as congestive heart failure, neurological and liver disease, and end-stage renal disease can cause chronic edema. An increase in the bariatric population has also seen an increase in lymphedema incidence. Lympedema caused by the parasite wucheria bancrofti and transmitted by mosquitoes remains the most common cause of lymphedema worldwide. Unfortunately, no strategies employed to prevent the onset of lymphedema have proven fruitful to date. The term chronic edema has been adopted by European investigators to define a population of patients with long-standing edema (>3 months). Prevalence estimates for chronic edema are between 1.3 and 1.5 per thousand.

New clinical data suggest that some patients may have a primary disposition to lymphedema but that this first becomes clinically evident after a secondary eliciting event (Rockson, 2010). Lymphedema tarda is defined as debut after the age of 35 years. It is often associated with an eliciting factor such as trauma or an inflammatory reaction (Kerchner, Fleischer, & Yosipovitch, 2008).

Complete text:

Nursing Center

A newly designed SIPC device for management of lymphoedema.

WARNING TO ALL LYMPHEDEMA PATIENTS

This abstract is a prime example of just how bad information can be that is presented even through PubMed.

Lymphology 101 clearly shows that high pressure pneumatic devices can cause serious damage to the good lymphatics, making lymphedema even worse.

Read this for education, but please, please, please, what ever you do never ever ever set the compression level on high if you use a pneumatic device.

A newly designed SIPC device for management of lymphoedema.

PubMed

Pat

Lymphedema and the Use of Diuretics

Lymphedema and the Use of Diuretics

Because of the incredible lack of awareness and/or knowledge of lymphedema in the medical community, it is very common to hear of lymphedema patients speak of being prescribed diuretics (water pils) by their physcians in attempting to treat this condition.

It is important to understand not only that diuretics are not for lymphedema, but to understand why.

The long term use of diuretics will eventually lead to a serious worsening of lymphedema and will help set the stage for increased complications such as tissue fibrosis.

Hopefully, this page will clear up questions regarding this and will help patients better manage their lymphedema.

Pat

Diuretics Definition

A class of drugs or other substance that promotes the formation and release of urine. Diuretics are used to decease the fluid volume in the treatment of many underlying medical conditions. Most people commonly call diuretics a “water pill.”

Anything that promotes the formation of urine by the kidney. (The word “diuretic” comes from a combination of the Greek “dia-”, thoroughly + “ourein”, to urinate = to urinate thoroughly).

Diuresis may be due to a huge number of causes including metabolic conditions such as diabetes mellitus (in which the increased glucose level in the blood causes water to be lost in the urine); substances in food and drink (such as coffee, tea, and alcoholic beverages); and specific diuretic drugs.

All diuretic drugs – which are usually called, more simply, diuretics – cause a person to “lose water” but they do so by diverse means, including:

Inhibiting the kidney's ability to reabsorb sodium, thus enhancing the loss of sodium in the urine. And when sodium is lost in the urine, water goes with it. (This type of diuretic is called a high-ceiling diuretic or a loop diuretic).

Enhancing the excretion of both sodium and chloride in the urine so that water is excreted with them. This is how the thiazide diuretics work. Blocking the exchange of sodium for potassium, resulting in excretion of sodium and potassium but relatively little loss of potassium. These diuretics are therefore termed potassium sparing diuretics. Some diuretics work by still other mechanisms. And some diuretics have other effects and uses such as in treating hypertension. (3)

Lymphedema or Edema?

To understand why these agents are not recommended for the treatment of lymphedema we must first start with a basic definition of edema and lymphedema.

Edema: Abnormal pooling or build up of fluid in tissues. Generally caused by underlying medical conditions such as hypertension, varicose veins, thrombophlebitis, congestive heart failure, kidney failure, steroid therapy, inflammatory reactions, or injury or trauma.

Diuretics are used to assist in the correction of the base problem causing the edema.

Lymphedema: A disorder in which “lymph” collects usually in a limb as a result of a congenital, hereditary malformation of the lymphatic system, or is a result secondary conditions such as node removal for biopsies, damage to the lymphatics from radiation, injury or trauma or by parasitic infection. Diuretics cannot help the underlying condition of lymphedema.

Why not use diuretics for lymphedema?

Perhaps the single most important consideration is in the composition of the lymph fluid caused by lymphedema. The fluid i a protein-rich substance that provides a breeding and nourishment center for bacteria. This has to be removed to lesson the risks of cellulitis and lymphangitis.

This fluid also has to be removed so as to prevent the formation and progression of fibrosis as lymphedema advances through the various stages.

Diuretics remove none of this waste fluid as it is a heavier molecule fluid then just water itself..

Diuretics and Lymphedema

A question that came up is whether diuretics should be used in the treatment of lymphedema. In my opinion, the answer is usually no. There are some exceptions and I will explain.

Diuretics are one of the best treatments for patients suffering from edema of the legs due to congestive heart failure. When the right side of the heart does not work efficiently, the pressure in the venous system increases and this, in turn, results in increased pressure in the tissues and edema results. The edema is not from the lymphatic system and is not lymphedema.

Diuretics, such as lasix, cause the kidney to eliminate water from the blood. This in turn reduces the pressure in the venous system and allows the edema to drain into the venous system. Unfortunately, when someone drinks additional water the fluid and edema returns and so many patients require fluid and salt restriction to have the best results. Diuretics must be given regularly to eliminate as much water from the blood system as possible and control the edema. In some case, even when high doses of diuretics are given, the edema cannot be controlled by drugs alone and compression garments can be of additional benefit for these patients. The lymphatic system can be completely normal and patients will still develop edema due to congestive heart failure.

The lymphatic system drains through the lymph nodes and lymphedema generally arises due to an obstruction in the lymphatic system. This can occur due to surgery, radiation or trauma. Decreasing the pressure in the venous system by removing water from the venous system does not help reduce lymphedema. In fact, patients with normal cardiac function do not have excess tissue edema. As a result, fluid removed by diuretics must be replaced by oral intake to maintain a normal fluid balance and any reduction in fluid due to diuretics in normal people is temporary. Diuretics have no value for the treatment of lymphedema in patients who do not have edema due to congestive heart failure or other similar conditions.

A patient could have a mixed condition where lymphedema is complicated by edema due to congestive heart failure. These patients may benefit from treatment with diuretics because of the mixed condition. Check with your doctor to see if you have some component of edema.

Dr. Mortimer addressed this question in a recent publication in Angiology 48:87-91, 1997. He said,

“Lymphedema, regardless of etiology, is essentially incurable but different therapy approaches exist which serve to contain swelling. The objectives of treatment are to reduce swelling, restore shape, and prevent inflammatory episodes, eg, recurrent cellulitis. There are essentially three main approaches to lymphedema treatment: physical therapy, drug therapy, and surgery. Any edema arises from an imbalance between capillary filtration and lymph drainage. The principle of physical therapy is to a) reduce excessive capillary filtration and b) improve drainage of interstitial fluid and macromolecules from congested regions to normally draining lymph node sites. This is achieved through a combination of compression, exercise, and if possible, massage. Control of recurrent inflammatory episodes can only be achieved through diabetic type skin care, a reduction in swelling, and if necessary, prophylactic antibiotics. Drug therapy comprises diuretics or the coumarin/flavonoid group of drugs. The use of diuretics for pure lymphedema is physiologically unsound but may be of use in edema of mixed origin and in palliative (cancer) circumstances.”

Tony Reid MD Ph.D

Penninsula Medical Diuretics Page

Acknowledgment

Penninsula Medical - “The Reidsleeve People”