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LYMPHEDEMA TARDA

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Lymphedema Tarda

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Disorder Subdivisions - Lymphedema
        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

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LYMPHEDEMA TARDA

Discussion

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.

Diagnosis

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.

Etiology

The cause of lymphedema tarda is a break in the FOXC2  gene.

Complications

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.

Treatment

Decongestive therapy is the most widely accepted form of treatment.  There is no cure for lymphedema tarda but the condition can be managed by early diagnosis and treatment.

Prognosis

Long term prognosis is excellent is the condition is identified early and treatment begins so after the diagnosis is made.

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Stages Of Lymphedema Tarda

LATENCY STAGE
Lymphatic transport capacity is reduced
No visible/palpable edema
Subjective complaints are possible

STAGE I
(Reversible Lymphedema)
Accumulation of protein rich edema fluid
Pitting edema
Reduces with elevation (no fibrosis)

STAGE II
(Spontaneously Irreversible Lymphedema)
Accumulation of protein rich edema fluid
Pitting becomes progressively more difficult
Connective tissue proliferation (fibrosis)

STAGE III
(Lymphostatic Elephantiasis)
Accumulation of protein rich edema fluid
Non pitting
Fibrosis and sclerosis (severe induration)
Skin changes (papillomas, hyperkeratosis, etc.)

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Lymphedema Tarda and Fibrosis

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, its just satiated with fluid. As the swelling continues and as he fluid changes to that protein-rich fluid referred to a lymphorrea, you enter into stage two. In this stage, the tissue become 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 response 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 serious side affect of stage three lymphedema. This type of tissue increases potential of 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 of this tissue compresses nerves within the limb.

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Lymphedema Tarda and Cellulitis  

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, 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.

The early intervention and treatment with antibiotics will resolve this condition (it usually takes one week 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 Inter venous 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 sleeve or exercise excessively. Avoid the blood pressure and blood to be drawn from the involved arm. Keep the limb elevated as much as possible while resting. Once the symptoms dissipate the treatment MLD/CDP 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 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.

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Decongestive Therapy and Lymphedema Tarda

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 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 fibrosis and fibrotic areas of a lymphodemous limb.

This treatment was created and developed Danish therapists Dr. Emil Vodder and his wife, Estrid, in the 1930's and was introduced in Paris in 1936. They are also credit 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 American 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, compressive 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 pioneered in the United States by Dr. Robert Lerner.

=====External Links=====================

Localized bullous pemphigoid in a patient with primary lymphoedema tarda

http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2230.2009.03722.x/abstract

Images in clinical medicine. Lymphedema tarda.


http://www.nejm.org/doi/full/10.1056/NEJMicm053461


Lymphedema tarda after liver transplantation: a case report and review of the literature.

http://www.ectrx.org/forms/ectrxcontentshow.php?year=2006&volume=4&issue=2&supplement=0&makale_no=0&spage_number=567&content_type=

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Pat O'Connor

Lymphedema People / Advocates for Lymphedema

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For information about Lymphedema

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Lymphedema Glossary

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Lymphedema People

Support Groups

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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.

http://health.groups.yahoo.com/group/childrenwithlymphedema/

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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!

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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.

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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.

http://health.groups.yahoo.com/group/allaboutlymphangiectasia/

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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.

DISCRIPTION

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.

http://health.groups.yahoo.com/group/lymphaticdisorders/

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on lymphedema, edema, and related conditions:

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          Related Medical Conditions

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          Complete Listings of Therapists and Links

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          Wound Information, Care, Treatment

          Skin Care, Conditions and Complications

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Updated Dec. 26, 2011