DISEASE OF LYMPH VESSELS

Signs and symptoms of lymphoedema

Typical signs and symptoms of lymphedema include:

• The onset of lymphedema is usually insidious. Affected patients may initially experience aching pain at the affected area and a sense of heaviness or fullness of the limb. Over time, the skin becomes dry and firm with less pitting and is fibrous to palpation.
• Two-thirds of cases of lymphedema are unilateral, although the laterality depends on the precipitating event. For example, an axillary node dissection will increase the risk of lymphedema in the ipsilateral arm while a pelvic node dissection increases the risk of bilateral lower extremity edema.
• At onset, swelling in the affected limb is typically characterized as “soft” and “pitting”. Pitting is variable in patients with lymphedema and reflects movement of the excess interstitial water in response to pressure. It is generally absent with progressive lymphedema, reflecting the evolution of fibrosis and adipose tissue deposition.

  For patients who had previously undergone a lymph node dissection and radiation, lymphedema is typically characterized by slowly progressive ipsilateral swelling of an arm following axillary node dissection or a leg following inguinal node dissection. The swelling may first be apparent only in the proximal portion of the limb, or it can affect only a portion of the distal limb including the digits.

  Among patients with breast cancer, there may also be swelling over the ipsilateral breast and/or upper chest wall. Other manifestations include a feeling of heaviness, tightness, aching or discomfort in the limb, and restricted range of motion.

• Skin changes — With worsening lymphedema, dermal thickening becomes clinically apparent, which is manifested by cutaneous fibrosis. The overlying skin of the affected limb also becomes hyperkeratotic, which can lead to verrucous and vesicular skin lesions.
• Discomfort — A feeling of heaviness, tightness, aching or discomfort in the affected limb commonly accompanies swelling.
• Restricted range of motion — With later stages of lymphedema, patients may develop restricted range of motion in the affected limb as a result of the increased weight, which may limit their ability to perform activities of daily living (ADLs).

Summary of Clinical Features

• Swelling in arms and legs
• Tissues of the neck and head may be affected also.
• Reduced mobility due to swelling
• Heaviness of the affected area
• Skin changes i.e discoloration
• Blister formation
• Leaking of fluid from the skin
• Infections
• Poor vision, ear pain and nasal congestion in case of head and neck lymphoedema
• Difficult in swallowing
• Difficulty in breathing and talking
• Salivating
• Lymphangitis and cellulitis occur as complications which presents as streaky red patch on area affected, fever, chills and itching

Diagnosis and Investigation of lymphoedema

1. History: Components of history that should be addressed include:

• Age of onset
• Area(s) of involvement
• Associated symptoms (e.g., pain)
• Medications — While none is directly associated with an increased risk of lymphedema, some are associated with edematous states (e.g., NSAIDs agents) or are contraindicated in the treatment of lymphedema (e.g., diuretics)
• Progression of symptoms
• Past medical history, including of medical conditions associated with lymphedema, any prior travel, infections, surgery or prior RT
• Family history

      2. Physical Examination.

• The physical exam should evaluate the vascular system, skin, and soft tissue, and palpation of the lymph nodes.

If primary lymphedema is suspected, evaluation should include documentation of any physical signs or congenital anomalies associated with an inherited condition. Examples include:

• Short stature (Turner Syndrome)
• Port wine stains or hemangiomas (Klippel-Trenaunay-Weber Syndrome)
• Shield chest (Turner Syndrome, Noonan Syndrome)
• A positive Stemmer sign is indicative of lymphedema. It is characterized by the examiner’s inability to lift the skin of the affected limb compared to the contralateral limb. It is also described as difficulty lifting the skin of the dorsum of the fingers or toes of the affected limb. A positive Stemmer sign can be found in any stage of lymphedema. While it is possible to have a false negative Stemmer sign, a false positive sign is rare.
• Opto electronic volume try  — Volume can be assessed utilizing an infrared, optoelectronic measurements. This technique utilizes infrared beams to scan the limb and calculate a volume.
• Circumferential measurements  — Circumferential measurements on the affected and contralateral arm are a simple and inexpensive method to estimate edema. Measurements can be taken at any point in the arm or leg, or circumferentially around the head, neck, or trunk, as long as the clinician is utilizing anatomic landmarks to reproduce the measurements.  Measurements in the arm are made at four points in both the affected and contralateral arm: 
• Metacarpal-phalangeal joints
• Wrists
• Ten centimeters distal to the lateral epicondyles
• Fifteen centimeters proximal to the lateral epicondyles
• Water displacement detects changes in volume of less than 1 percent. For patients with limb lymphedema, volume difference of 200 mL or more between the affected and opposite limbs is typically considered as a cutoff point to define lymphedema.
• Bioimpedance spectroscopy  — Bioimpedance spectroscopy (BIS) is a reliable and accurate tool to determine volume. Using electrical current, resistance measurements are used to compare the composition of fluid compartments.

         4. Imaging

• Computed tomography (CT) scan can demonstrate accumulation of fluid within soft tissue with good sensitivity
• MRI has also been used in the evaluation of lymphedema.
• Lymphoscintigraphy  — Lymphoscintigraphy is a technique used to image the flow of fluid from the skin to the lymph nodes, particularly in the extremities.
• X-ray of lymphatic system by introducing a dye between toes or in the groin
• Genetic testing  — For patients diagnosed with primary lymphedema or suspected of lymphedema tarda, referral to a medical geneticist or genetic counselling service is suggested for evaluation of the family history and recommendations for further work-up.
• Blood smear to isolate filarial worms