Lymphedema
Definition
Lymphedema is a chronic disorder characterized by an abnormal accumulation of lymph fluid in the tissue of one or more body regions.
Accumulation of fluid is due to mechanical insufficiency of the lymphatic system.
Types of lymphedema
- Primary lymphedema
- Caused by a condition that is congenital or hereditary.
- The formation of lymph nodes and lymph vessels are abnormal.
- The most common abnormality is hypoplasia, a condition in which there are fewer lymphatic vessels and they are smaller than normal.
- Primary lymphedema appears in Milroy's disease.
- Secondary lymphedema
- Caused by injury to the lymphatic system, or some part of the lymphatic system has been blocked, dissected, fibrosis, or altered.
- The common cause of secondary lymphedema is surgery, radiation therapy, and treatment of cancer of the prostate bladder uterus ovaries skin, and breast.
- Secondary lymphedema is more prevalent or widespread than primary lymphedema.
- Secondary lymphedema commonly appears in filariasis.
Clinical presentation:
Swelling distal to the area where the lymph system has been impaired.
Swelling usually not relieved by elevation
Pitting edema in the early stages of the disease and non-pitting edema in later stages
the feeling of fatigue, heaviness, pressure, or tightness in the affected region
Numbness and tingling (sensory disturbance)
Increased girth and weight.
Grading of Lymphedema
according to the international society of lymphology staging system:
Preclinical: patient begins to feel "heaviness" in the limb, fluid accumulation occurs before visible swelling on pitting.
Stage 1: reversible lymphedema: accumulation of protein-rich fluid; elevation reduces swelling; pits on pressure.
Stage 2: spontaneously reversible lymphedema: proteins stimulate fibroblast formation; connective and scar tissue proliferates; minimal pitting even with moderate swelling.
Stage 3: lymphatic elephantiasis: hardening of dermal tissues, papillomas of the skin, the appearance of the skin is elephant-like.
Stages of Lymphedema
Prevention of Lymphedema
Avoid static, dependent positioning of the lower limb such as prolonged sitting or standing.
Avoid sitting with legs crossed.
When traveling long distances by car, stop at intervals and walk around, or support an involved upper extremity on the car's window ledge or seatback.
Elevated involved limbs and perform repetitive pumping exercises during the day.
Avoid vigrous, repetitive activities with the involved limb.
Avoid carrying heavyweight such as suitcase, backpack, shoulder bag.
Wear compression garments during exercise.
Avoid wearing tight clothing or jewelry that restricts circulation.
Minimize sodium intake and control body weight.
Avoid a hot environment or use of local heat.
Avoid having BP taken on an involved upper extremity.
Diagnosis:
A patient history consistent with lymph system damage or deformity, a system review, differential diagnosis, inspection, palpation, and growth measurements is adequate for accurate diagnosis in most cases.
Findings:
Stemmer's sign
Girth measurements
Skin texture changes
Skin folds
Fibrosis
Increase in girth
Papules
Lymph leakage
Elephantiasis
Special test:
Lymphoscintigraphy
Uses a radioactive tracer and Gama camera to provide images for the lymphatic system.
This test is useful for differential diagnosis.
Intervention
Two-phase program of complete decongestive therapy (CDT)
Phase 1 (intensive)
Skincare,
Manual lymphatic drainage (MLD),
Lymphedema bandaging,
Exercise,
Compression garments.
Phase 2 (Self-Management)
Skincare,
Compression garments during day,
Exercise,
Lymphedema bandaging at night,
Manual lymphatic drainage.
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