Lymphedema
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Editors-in-Chief: Benoit Blondeau, M.D. [1]; C. Michael Gibson, M.S., M.D. [2]
Synonyms and keywords: Lymphoedema, lymphatic obstruction
Overview
Editors-in-Chief: Benoit Blondeau, M.D. [1]; C. Michael Gibson, M.S., M.D. [2]
Overview
Lymphedema is a condition of localized fluid retention caused by a compromised lymphatic system. The lymphatic system (often referred to as the body’s “second” circulatory system) collects and filters the interstitial fluid of the body. Lymphedema has been barely recognized as being a serious health problem; however, this is slowly changing due to education and awareness. The danger with lymphedema comes from the constant risk of developing an uncontrolled infection in the affected limb. Still, physicians and medical staff who practice in fields where this disease is uncommon may fail to correctly diagnose the condition due to the apparent lack of information regarding this disease.
Reference
Historical Perspective
Classification
Editors-in-Chief: Benoit Blondeau, M.D. [1]; C. Michael Gibson, M.S., M.D. [2]
Overview
Classification
Lymphedema may be classified as primary or secondary.[1]
Staging and severity
Stages
Whether primary or secondary, lymphedema develops in a number of stages, from mild to severe. The stages were defined in the following manner by the Fifth WHO Expert Committee on Filariasis:[2][3]
- Stage 0 (latent): The lymphatic vessels have sustained some damage which is not yet apparent. Transport capacity is still sufficient for the amount of lymph being removed. Lymphedema is not present.
- Stage 1 (spontaneously reversible): Tissue is still at the “pitting” stage — when pressed by the fingertips, the affected area indents and holds the indentation. Usually upon waking in the morning, the limb or affected area is normal or almost normal in size.
- Stage 2 (spontaneously irreversible): The tissue now has a spongy consistency and is “non pitting” — when pressed by the fingertips, the tissue bounces back without any indentation. Fibrosis found in Stage 2 Lymphedema marks the beginning of the hardening of the limbs and increasing size.
- Stage 3 (lymphostatic elephantiasis): At this stage, the swelling is irreversible and usually the limb(s) or affected area is very large. The tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery called “debulking”. This remains controversial, however, since the risks may outweigh the benefits, and the further damage done to the lymphatic system may in fact make the lymphedema worse.
Grades
Lymphedema can also be categorized by its severity (usually referenced to a healthy extremity):
- Grade 1 (mild edema): Lymphedema involves the distal parts such as a forearm and hand or a lower leg and foot. The difference in circumference is less than 4 centimeters, and other tissue changes are not yet present.
- Grade 2 (moderate edema): Lymphedema involves an entire limb or corresponding quadrant of the trunk. Difference in circumference is more than 4 but less than 6 centimeters. Tissue changes, such as pitting, are apparent. The patient may experience erysipelas.
- Grade 3a (severe edema): Lymphedema is present in one limb and its associated trunk quadrant. The difference in circumference is greater than 6 centimeters. Significant skin alterations, such as cornification or keratosis, cysts and/or fistulae, are present. Additionally the patient may experience repeated attacks of erysipelas.
- Grade 3b (massive edema): The same symptoms as Stage 3a except that two or more extremities are affected.
- Grade 4 (gigantic edema): Also known as elephantiasis. In this stage of lymphedema, the affected extremities are huge due to almost complete blockage of the lymph channels. Elephantiasis may also affect the head and face.
Reference
- ↑ Browse NL, Stewart G (1985). “Lymphoedema: pathophysiology and classification”. J Cardiovasc Surg (Torino). 26 (2): 91–106. PMID 3884629.
- ↑ “Lymphatic filariasis: the disease and its control. Fifth report of the WHO Expert Committee on Filariasis”. World Health Organization technical report series. 821: 1–71. 1992. PMID 1441569.
- ↑ http://whqlibdoc.who.int/hq/2001/WHO_CDS_CPE_CEE_2001.26a.pdf.
Pathophysiology
Editors-in-Chief: Benoit Blondeau, M.D. [1]; C. Michael Gibson, M.S., M.D. [2]
Overview
Pathophysiology
Lymphedema may be due to:[1]
- Obliterative disease of lymph nodes
- Hyperplastic disease of lymph nodes
Lymphedema (also see Elephantiasis) 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 cancer patients this condition does not develop until months or even years after their 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.
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. In the lower extremity it will be unilateral or bilateral. If it is bilateral, one leg may be worse than the other.
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.
Reference
Causes
Editors-in-Chief: Benoit Blondeau, M.D. [1]; C. Michael Gibson, M.S., M.D. [2]
Overview
Lymphedema (also see Elephantiasis) may be inherited (primary) or caused by injury to the lymphatic vessels (secondary).
Causes
- Congential
- Recurrent infections by parasites (Wuchereria bancrifti)
- Drug induced – Pramipexole
Reference
Differentiating Lymphedema from other Diseases
Overview
Differential Diagnosis
Reference
Epidemiology and Demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Epidemiology and Demographics
- One million cases in USA and more than 100 million cases world wide have been reported by WHO.
Reference
Risk Factors
Screening
Natural History, Complications and Prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Complications
When the lymphatic impairment becomes so great that the lymph fluid exceeds the lymphatic system’s ability to transport it, an abnormal amount of protein-rich fluid collects in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid causes tissue channels to increase in size and number, reducing the availability of oxygen. This interferes with wound healing and provides a rich culture medium for bacterial growth that can result in infections: cellulitis, lymphangitis, lymphadenitis, and in severe cases, skin ulcers. It is vital for lymphedema patients to be aware of the symptoms of infection and to seek treatment at the first signs, since recurrent infections, in addition to their inherent danger, further damage the lymphatic system and set up a vicious circle.
Very rarely, in certain exceptionally severe cases, lymphedema untreated over many years can lead to a form of cancer known as Lymphangiosarcoma.
Since lymphedema is disfiguring, causes difficulties in daily living and can lead to lifestyle becoming severely limited, it may also result in psychological distress.
Reference
Diagnosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | MRI | Ultrasound | Other imaging findings | Other diagnostic studies
Treatment
Treatment
Medical therapy | Surgery | Primary prevention | Secondary prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
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