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Lymphedema

Edema Lymphatic

Lymphedema is an abnormal collection of fluid in the interstitium resulting from conditions affecting lymphatic drainage.


Presentation

Patients manifest with a chronic swelling and fullness of the affected limb. Most commonly it is the lower limb and the upper extremity that are affected; trunk and genitalia are less frequently affected. The patient notices asymmetry of the extremity. This progresses to increased heaviness of the limb, fatigue and difficulty in wearing clothes. There is severe deterioration in performing daily activities. The swelling of the limb is usually painless though some patients do report of constant aching in that area.

As the edema becomes chronic and long standing it develops discoloration, the skin becomes dense and appears thick. The texture appears as that of an orange peel. There is fibrous tissue and scar formation.

In addition to this, recurrent fever with chills, weakness, and repeated cellulitis and infections are common. Locally, there might be fissuring, recurrent ulcer formation, and lymphorrhea [6]. Lymphorrhea constitutes of oozing of clear or straw colored fluid from the affected limb. Inability to pinch the affected skin in between two fingers is called as the positive Stemmer test, and is useful in eliciting the diagnosis for lymphedema.

The degree of severity, from mild to severe forms is accessed through a staging method introduced by the Lee, Morgan and Bergan through the American society of lymphology. This method benefits the physician in indentifying the severity of the case and in providing the necessary intervention accordingly.

  • Stage 0: Absent lymphedema. This is a latent stage. Here, the lymphatic vessels are destroyed to a mild degree and hence it is not yet apparent.
  • Stage 1: The edema is visible but is reversible. It resolves on elevating the limb. Also, the affected area is normal in morning and swelling appears as the day progresses. 
  • Stage 2: The tissues are affected. They become non-pitting on pressure. Lymphedema is evident with increasing swelling and hardening of the skin. 
  • Stage 3: Irreversible swelling is seen in this stage. The limb tissue becomes hard and non tender. There is marked heaviness and disability of the limb. There is complete blockage of the lymphatic vessels. This is also called as stage of elephantiasis [4] [7].
Lymphedema
  • Lymphedema of limb Lymphedema of limbs due to immobility, chronic dependency and/or venous insufficiency Lymphedema of lip Lymphedema of lower extremity Lymphedema of penis Lymphedema of scrotum Lymphedema of uncertain etiology Lymphedema of vulva Lymphedema[icd9data.com]
  • lymphedema or had previously met lymphedema diagnostic criteria and undergone treatment.[ncbi.nlm.nih.gov]
  • Exercise and types of lymphedema: Lymphedema has many causes.[web.archive.org]
  • The current mainstay of lymphedema therapy has been conservative nonsurgical treatment. However, surgical options for lymphedema have been reported for over a century.[ncbi.nlm.nih.gov]
  • Lymphedema praecox, also known as Meige disease, is the most common form of primary lymphedema.[emedicine.medscape.com]
Leg Swelling
  • Symptoms of lymphedema Lymphedema symptoms include: tight-fitting clothing or jewelry heavy, tight feeling in your arm or leg tingling feeling in your arms or legs swelling of body parts aching feeling in your arm or leg or an arm or leg that becomes[allinahealth.org]
  • Lymphedema and any other type of leg swelling greatly increase the risk of infection in the skin and fatty tissues (cellulitis) of the extremity.[tennesseeveincare.com]
  • 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.[drugs.com]
  • Keep the swelling down with simple techniques Lymphedema is an excess of fluid in body tissues that causes abnormal swelling of an arm or leg. Swelling can also occur in other parts of the body, depending on the type of cancer and the treatment.[patientresource.com]
  • For leg swelling, wear comfortable, supportive shoes and wash your feet daily in warm – not hot – water. Some patients may need to wear a long sleeve to lightly compress the affected arm, especially when traveling by airplane.[health.clevelandclinic.org]
Raynaud Phenomenon
  • She also presented Raynaud's phenomenon, a scleroderma pattern in nailfold capillaroscopy, cutaneous thickening and anticentromere antibodies, which together resulted in a new diagnosis of limited cutaneous SSc.[ncbi.nlm.nih.gov]
Advanced Bone Age
  • Abstract Sotos syndrome is a childhood overgrowth syndrome characterized clinically by a distinctive facial gestalt, advanced bone age, childhood overgrowth, and non-progressive developmental delay; and genetically by haploinsufficiency of the Nuclear[ncbi.nlm.nih.gov]
Joint Effusion
  • Joint effusion was identified and treated using a splint that restricted blood flow leading to edema of the distal third of the forearm. Since then the patient was treated however her condition worsened resulting in edema of the entire arm.[ncbi.nlm.nih.gov]
Hyperkeratosis
  • […] insufficiency Lymphedema of lip Lymphedema of lower extremity Lymphedema of penis Lymphedema of scrotum Lymphedema of uncertain etiology Lymphedema of vulva Lymphedema praecox Lymphedema, after surgery Lymphedema, limb Lymphedema, lower extremity Lymphedematous hyperkeratosis[icd9data.com]
  • Hyperkeratosis and papillomas may also be seen in someone with lymphedema. Hyperkeratosis is an overgrowth of the skin like a very thick callus.[oncolink.org]
  • Hyperkeratosis - an increase in the thickness of the outer layer of the skin - may develop. These changes in the texture of the skin are disfiguring and can limit mobility.[jobst.com]
  • Stage III lymphedema is characterized by a tremendous increase in volume, hardening of the dermal tissues, hyperkeratosis, and papillomas of the skin.[nortonschool.com]
  • Dermal changes that follow include dry or flaky skin, hyperkeratosis, skin creases, fibromas, lymphangiomas, and papillomas (see Figures 7 to 10); these will be evident on physical examination when lymphedema is more advanced.[o-wm.com]

Workup

Blood tests include complete blood count, liver function tests, urine examination, creatinine and urea levels including electrolytes. These tests help in identifying the underlying infectious etiology.

Imaging studies such as lymphangiography might be done; it is an invasive technique that evaluates the lymphatic channels and their patency. This technique causes a lot of adverse reactions such as atrophy, fibrosis and obliteration; thus it is not advised frequently. Ultrasonography identifies structural and capacitative changes within the lymphatic vessels. A CT scan and MRI are done in cases of a suspected malignancy [8] [9].

Nephrolithiasis
  • Abdominal ultrasonography detected a large incidental mass in the right renal sinus during his investigation for nephrolithiasis.[ncbi.nlm.nih.gov]
Pericardial Effusion
  • The anticancer drug docetaxel (Taxotere) can lead to fluid retention, which is the main cause of peripheral edema of the extremities, pleural effusion, pericardial effusion, and ascites in patients undergoing chemotherapy.[ncbi.nlm.nih.gov]

Treatment

Treatment comprises of use of compression bandages and garments including stockings. These are applied for several days so that tension is maintained in order to reduce lymphedema. These bandages should be costume fit and should not produce a tourniquet effect. These should be worn during the day and during activities short bandages are preferred that improve the pumping activities against increasing resistance [5] [9].

CDT is complete decongestive therapy and consists of a combination of exercises, bandaging and after skin care. Here, under expert guidance, manual massaging of the limb is done to improve circulation and lymph flow. The treatment is highly effective and enhances drainage.

Intermittent pump therapy involves use of multi-chambered pump compression that causes effective drainage of the lymph.

Surgery offers palliation. It helps to reduce the load of the swelling and is useful for patients who did not respond to the conservative treatment method. Appropriate hygiene is very important to observe in cases of lymphedema. Repeatedly cleansing of the area is recommended in order to eliminate dead skin and debris and prevent the invasion of bacterial or other microbial infections. Patients are advised to lose weight, monitor the limb regularly and to wear proper clothing and comfortable foot wear [10].

Prognosis

Prognosis depends on the severity of the lymphedema, the general health of the patient and the underlying conditions that have caused it.

Long standing lymphedema may develop into a lymphangiosarcoma. This tumor has a very poor prognosis and requires amputation at its earliest. Kaposi sarcoma, B-cell lymphoma are other tumors found with chronic lymphedema.

Lymphedema may also lead to complications such as cellulitis, bacterial infections or deep vein thrombosis leading to functional impairment and disability [5].

Etiology

Based on the causes, lymphedema can be categorized into primary and secondary lymphedema.

Primary causes are related to congenital or developmental arrest of the lymphatic system. In congenital lymphedema, there is failure of the lymphatic channels to develop. This is an autosomal-dominant disorder. The genetic defect mutated and is mapped into the telomeric part of chromosome 5. This most commonly affects bilateral lower extremities. Meige disease or lymphedema praecox is a common form of primary lymphedema. This is more frequently found after a birth defect. Lymphedema tarda is due to valvular incompetency. This is found in individuals older than 35 years [2].

Secondary lymphedema is obtained due to underlying conditions such as infection, trauma, obesity or a tumor mass. This condition obstructs the lymph flow or damages the vessels carrying the lymphatic fluid, leading to an abnormal collection, producing a swelling and edema of the affected arm or leg.

Epidemiology

Studies reveal that lymphedema usually follows after a breast cancer surgery. Around 80% of the cases show congenital onset. Demographics reveal that lymphedema that is primary in nature is seen from birth up to 1 year of age and the secondary ones can occur at any age.

Amongst the infections, filariasis is the most frequent cause leading to lymphedema. Obesity has been highly associated with lymphedema.

Sex distribution
Age distribution

Pathophysiology

Lymphatics normally drain proteins, lipids and water from the interstial tissues into the circulation filtering via the lymph nodes. This occurs through osmotic mechanism. The flow of the lymph is controlled by the valves within the vessels to prevent the back flow of the fluid, also the surrounding muscle contraction and the pulse pressure are important factors involved with the lymph flow.

In case of lymphedema, the transport volume is reduced. The amount of fluid formation exceeds to the volume of fluid returned [3]. Consequently there is stagnation, edema formation with high concentration of proteins per ml. Eventually, the lymphatic vessels undergo changes in the form of fibrosis, thrombus formation and shunt formation.

Within the interstitial spaces, fluid accumulation leads to inflammation producing increased macrophage activity and collagen deposition. This eventually progresses to a suppressed immune activity that increases probabilities of infection and degeneration.

Locally, the skin appears congested and thick; the characteristic appearance is that of an orange skin called peau d’orange appearance. The epidermal layer of the skin gets destroyed and develops fissures, resulting in leakage of the lymph on the surface of the skin. Bacteria enter and multiply leading to a condition called elephantiasis verrucosa [4].

Prevention

Those at a risk for developing lymphedema should observe special precautions especially after undergoing a cancer surgery. Injury to the limb from sharp objects should be avoided. Regular exercise and elevation of the affected limb during rest is advisable. Tight garments hamper circulation and cause constriction; hence comfortable size garments should be worn. Lastly, skin care and hygiene must be observed regularly to avoid infection [1] [11].

Summary

The lymphatic fluid and vessels essay a very vital role in the body’s immune system. The lymphatic circulation consists of many lymph nodes and lymphatic vessels through which the lymphatic fluids pass [1]. It acts by draining the unwanted lymphatic fluid from tissues of the body in the blood circulation for removal.

When this lymphatic fluid is abnormally accumulated within the interstitium, it results in swelling under the skin leading to edema called as lymphedema.

Patient Information

Lymphedema develops in cases where normal lymph drainage is obstructed due to congenital causes or underlying tumors, after surgery or trauma, the fluid builds up causing increased swelling and discomfort.

Long standing lymphedema increases the risk of infection, cellulitis and fibrosis. Treatment includes use of compression bandages and garments like wearing stockings and bandages. These help in improving lymph drainage and circulation. Secondly, gentle exercises, elevation of the limb while resting are advised to enhance lymph drainage. Surgery is advised in cases when conservative treatment does not respond. Other precautions observed are protection of the limb from any injury especially from sharp objects and needles.

References

Article

  1. Foldi E, et al. The Science of Lymphoedema Bandaging in Calne, S. Editor. European Wound Management 2005Association (EWMA). Focus Document: Lymphoedema Bandaging in Practice. London: MEP Ltd, 2004.
  2. Casley-Smith JR. Alterations of untreated lymphedema and its grades over time. Lymphology. 1995 Dec; 28(4):174-185
  3. MacLaren JA. Skin changes in lymphoedema: pathophysiology and management options. Int J Palliat Nurs. 2001 Aug;7(8): 381-388
  4. Pereira de Godoy JM, Azoubel LM, de Fatima Guerreiro de Godoy M. Intensive treatment of leg lymphedema. Indian J Dermatol. 2010 Apr-Jun;55(2):144-7
  5. Elgendy IY, Lo MC. Unilateral lower extremity swelling as a rare presentation of non-Hodgkin's lymphoma.BMJ Case Rep. 2014 Jan 15; 2014. pii: bcr2013202424.
  6. Fife C. Massive localized lymphedema, a disease unique to the morbidly obese: a case study. Ostomy Wound Manage. 2014 Jan;60(1):30-5.
  7. Prakash J, Kumar M, Singh V, Sankhwar S. Giant penile elephantiasis after circumcision: a devastating complication. BMJ Case Rep. 2013 Sep 16; 2013 pii:bcr2013200780.
  8. Namnyak S, Adhami Z, Toms G, Jenks P. Pasteurella multocida septicaemia in Milroy's disease. J Infect. 1995 Sep;31(2):175-6.
  9. Zuther JE. Lymphedema Management: The Comprehensive Guide for Practitioners. 2nd ed. New York, NY: Thieme; 2009.
  10. Connell F, Brice G, Jeffery S, Keeley V, Mortimer P, Mansour S. A new classification system for primary lymphatic dysplasias based on phenotype. Clin Genet. 2010 May;77(5):438-52.
  11. Connell F, Brice G, Mortimer P. Phenotypic characterization of primary lymphedema. Ann N Y Acad Sci. 2008;1131:140-6.

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Last updated: 2019-07-11 22:37