What is Lymphoedema?

What is Lymphoedema?

Lymphedema is a chronic disease marked by the increased collection of lymphatic fluid in the body, causing swelling, which can lead to skin and tissue changes. The chronic, progressive accumulation of protein-rich fluid within the interstitium (a contiguous fluid-filled space existing between a structural barrier, such as a cell wall or the skin, and internal structures, such as organs, including muscles and the circulatory system) and the fibro-adipose tissue exceeds the capacity of the lymphatic system to transport the fluid.

 

Swelling associated with lymphedema can occur anywhere in the body, including the arms, legs, genitals, face, neck, chest wall, and oral cavity.

 

There are many psychological, physical, and social sequelae related to a diagnosis of lymphedema.

 

Lymphedema is classified as either (genetic) primary lymphedema or (acquired) secondary lymphedema. Signs and symptoms of lymphedema include

 

  • Distal swelling in the extremities including the arms, hands, legs, feet
  • Swelling proximally in the breast, chest, shoulder, pelvis, groin, genitals, face/intraoral tissues
  • Restricted range of motion in the joints because of swelling and tissue changes
  • Skin discoloration
  • Pain and altered sensation
  • Limb heaviness;
  • Difficulty fitting into clothing

 

Pathophysiology

 

Primary lymphedema

  • Associated with dysplasia of the lymphatic system and can also develop with conditions of other vascular abnormalities, including Klippel-Trenaunay-Weber syndrome, and Turner syndrome.
  • Primary lymphedema is marked by hyperplasia, hypoplasia, or aplasia of the lymphatic vessels.

 

Secondary lymphedema

 

  • Develops due to damage or dysfunction of the normally functioning lymphatic system.
  • Although cancer treatments, including oncologic surgical procedures such as axillary lymph node dissection and excision in breast cancer and radiation treatment, are the most common cause of lymphedema in the United States, filariasis is the most common cause of secondary lymphedema globally.
  • Filariasis is the direct infestation of lymph nodes by the parasite, Wuchereria bancrofti. The spread of the parasite by mosquitos affects millions of people in the tropic and subtropic regions of Asia, Africa, the Westen Pacific, and Central and South America.
  • Oncologic surgical procedures such as sentinel lymph node biopsy and radical dissection that require excision of regional lymph nodes or vessels can lead to the development of secondary lymphedema.
  • Other surgical procedures linked to secondary lymphedema development include peripheral vascular surgery, burn scar excision, vein stripping, and lipectomy.

 

Nonsurgical causes of lymphedema include

 

  • Recurrent tumors or malignancy that have metastasized to the lymph nodes
  • Obstructive lesions within the lymphatic system
  • Infected and/or traumatized lymphatic vessels
  • Scar tissue obliterating the lumen of the lymphatic vessels.
  • Edema from deep venous thrombosis (DVT) or nonobstructive causes of chronic venous insufficiency at the extremities may lead to secondary lymphedema.

 

Although there is no definitive cure for lymphedema, with proper diagnosis and management, its progression and potential complications can successfully be managed

 

Stages of Lymphoedema

 

Stage 0 (Latency stage)

 

  • The patient is considered “at-risk” for lymphedema development due to injury to the lymphatic vessels but does not present with outward signs of edema.
  • Includes patients with breast cancer who have undergone sentinel lymph node biopsy and or radiation but have not yet developed swelling.
  • Lymphatic transport capacity has been reduced, which predisposes the patient to lymphatic overload and resultant edema.

 

Stage 1 (Spontaneous)

 

  • Reversible
  • Has pitting edema
  • Swelling at this stage is soft, and may respond to elevation

 

Stage 2 (Spontaneously irreversible)

 

  • Has tissue fibrosis/induration
  • Swelling does not respond to elevation
  • Skin and tissue thickening occurs as the limb volume increases
  • Pitting may be present, but may be difficult to assess due to tissue and or skin fibrosis

 

Stage 3 (Lymphostatic elephantiasis)

 

  • Show the following:
  • Pitting edema
  • Fibrosis
  • Skin changes
  • During this stage papillomas may form, infections/cellulitis may occur, and the skin becomes dry

 

Evaluation

 

Lymphedema is often confused with other causes of extremity edema and enlargement.

 

  • Understanding the risk factors and physical examination signs of lymphedema can accurately diagnose patients about 90% of the time.
  • Correct diagnosis is imperative so patients can be managed appropriately.
  • Diagnosis is suspected by evaluating the history and physical examination.
  • Lymphoscintigraphy confirms the diagnosis.
  • Imaging is unnecessary to make the diagnosis but can be used as confirmation, assessment of the extent of involvement, and help to determine therapeutic intervention.

 

Newer technologies include 3-dimensional magnetic resonance imaging (MRI), computerized tomography (CT), ultrasound, and bioelectrical impedance analysis. Ultrasound is useful to exclude other etiologies like DVT, venous insufficiency and can also help in identifying tissue changes and masses that might be the cause of lymphatic compression. CT and MRI can investigate soft tissue edema with good sensitivity and specificity, but they are relatively expensive.

 

Treatment/Management

 

  • Lymphedema is a progressive disease, and early diagnosis and treatment are paramount.
  • Critical to diagnose and treat both mild and early onset cases to halt the progression of this lifelong and often debilitating condition.
  • For patients to improve their knowledge base and learn helpful evidence-based management and coping strategies, patients must be referred to a specialist holding certification in lymphedema treatment and management eg. a physician, an occupational therapist, or physical therapist.

 

Therapy

 

  • Decongestive lymphedema therapy (DLT): Is the primary treatment for moderate-to-severe lymphedema and mobilizes lymph and dissipate fibrosclerotic tissue.
  • Manual lymph drainage (MLD): Light lymph massage designed to increase lymph flow
  • Compression: Helps with drainage but can increase the risk of infection
  • Skincare: Fastidious skincare is essential to prevent secondary skin infections
  • Exercise: Light exercise promotes lymph drainage and protein absorption via muscle contraction.

 

Drug therapy: Adjunctive only for pain control or secondary infection

 

Surgery

 

  • Debulking is often ineffective

 

Microsurgical techniques

 

  • Vascularized Lymph Node Transfer (VLNT)
  • Lymphaticovenous Anastomoses (LVA): VLNT and LVA are microsurgical procedures that can improve the patient's physiologic drainage of the lymphatic fluid and eliminate the need for compression garments in some patients. These procedures have better results when performed when a patient's lymphatic system has less damage.
  • Suction-Assisted Protein Lipectomy (SAPL): Is more effective in later stages of lymphedema and allows removal of lymphatic solids and fatty deposits that are poor candidates for conservative lymphedema therapy, or VLNT or LVA surgeries.

 

Prognosis

 

  • A cure is rarely achieved once lymphedema occurs.
  • Meticulous treatment and preventive measures can help lessen symptoms, slow or stop disease progression, and prevent complications.
  • A systematic review and meta-analyses suggest the need for complete awareness of the factors contributing to the wide variability in lymphedema in order to improve QOL people living with cancer-related lymphedema in low and middle-income countries.
  • Patients with chronic lymphedema for ten years have a 10% risk of developing lymphangiosarcoma. This tumor is highly aggressive, requires radical amputation of the involved extremity, and has a very poor prognosis. Five-year survival is less than 10%.

 

Complications

 

Complications of lymphedema also include:

 

  • Cellulitis: often recurrent
  • Lymphangitis
  • Superficial bacterial and fungal infections
  • Lymphangio-adenitis
  • Deep vein thrombosis (DVT)
  • Severe functional impairment
  • Psychosocial dysfunction
  • Cosmetic embarrassment
  • Amputation
  • Complications following surgery are common and include:
    • Partial wound separation
    • Seroma
    • Hematoma
    • Skin necrosis

 

Physical Therapy Management

 

Education regarding the following points are paramount

 

  • Self MLD
  • Infection prevention
  • Exercise
  • Instruction in proper diet to decrease fluid retention and how to avoid injury and infection, anatomy
  • Self bandaging and use of compression garments. Garment fitting.
  • Weight control
  • Avoid venipuncture in the affected extremity
  • Avoidance of other constricting items; do not take BP measurements on the affected extremity
  • Life-long education regarding eg. Rigid adherence to compression stockings is mandatory to obtain relief from the pain and swelling. In addition, skin dryness and pruritus also need to be addressed. All patients should be seen by a wound care nurse if there is tissue breakdown. At this point, the chances of healing are small, and daily wound dressings are necessary.

 

Interventions include:

 

  • Manual lymph drainage (to help improve the flow of lymph from the affected arm or leg from proximal to distal).
  • Short/low stretch Compression garment wear following lymphatic drainage.
  • Skin Hygiene and care (such as cleaning the skin of the arm or leg daily and moisten with lotion).
  • Exercise to improve cardiovascular health and help decrease swelling in some cases.
  • Compression pumps
  • Psychological and emotional support

 

Complex Decongestive Therapy: the primary treatment for moderate-to-severe lymphedema and mobilizes lymph and dissipate fibrosclerotic tissue

  • Phase 1:
    • Skin care
    • Light manual massage (manual lymph drainage)ROMCompression (multi-layered bandage wrapping, highest level tolerated 20-60 mm Hg)Phase 2:
      • Compression by low-stretch elastic stocking or sleeve
      • Skin care
      • Exercise
      • Light massage as needed

 

Contraindications for compression include arterial disease, painful postphlebitic syndrome, and occult visceral neoplasia.

 

 

 

Reference: https://www.physio-pedia.com/Lymphoedema

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