LYMPHOEDEMA FAISAL GHANI SIDDIQUI MBBS; FCPS; PGDIP-BIOMEDICAL ETHICS; MCPS-HPE PROFESSOR OF SURGERY & DIRECTOR, PROFESSIONAL DEVELOPMENT CENTRE J I N N A H S I N D H M E D I C A L U N I V E R S I T Y faisal.siddiqui@jsmu.edu.pk
PREAMBLE What is lymphatic system and what does it do? Lymphedema Definition Causes Clinical features Investigations
THE LYMPHATIC SYSTEM
COMPONENTS OF THE LYMPHATIC SYSTEM Lymphatic channels Lymphoid organs (lymph nodes, spleen, Peyer s patches, thymus, tonsils) Circulating elements (lymphocytes and other mononuclear immune cells)
FUNCTION OF THE LYMPHATIC SYSTEM Returns the following from the interstitial fluid (ISF) to the circulation via lymphatics; Water Electrolytes Low molecular weight peptides Macromolecules (fibrinogen, albumin, globulins)
LYMPHEDEMA -DEFINITION
LYMPHEDEMA -DEFINITION abnormal limb swelling caused by the accumulation of high protein-containing ISF secondary to defective lymphatic drainage
CAUSES OF LYMPHEDEMA
CAUSES OF LYMPHEDEMA Primary lymphedema Secondary lymphedema
PRIMARY LYMPHEDEMA Cause is unknown Proposed to be due to inherent congenital lymphatic dysplasia Aplasia / hypoplasia Hyperplasia
PRIMARY LYMPHEDEMA -TYPES Lymphedema congenita Onset at or within 2 years of birth Lymphedema praecox Onset from 2 to 35 years Lymphedema tarda Onset after the age of 35 years
SECONDARY LYMPHOEDEMA - CAUSES Trauma Malignant disease Infection Inflammatory disorders Endocrine disorders Immobility / dependency Lymph node excision Radiotherapy Lymph node metastases Lymphoma Filariasis Tuberculosis Lymphadenitis Rheumatoid arthritis Dermatitis Psoriasis Pretibial myxedema Reproduced from: Lymphoedema Framework. Best practice management of lymphoedema. International Consensus. London: MEP Ltd, 2006. MEP Ltd 2006.
Lymphatic Edema secondary to Filariasis
CLINICAL SIGNS & SYMPTOMS
CLINICAL FEATURES Unlike other types of edema, lymphedema involves foot Ankle contour lost due to infilling of submelleolar depressions Buffalo hump on the dorsum of the foot Toes appear square Skin on the dorsum cannot be pinched (Stemmer s sign) Swelling pits in the early stages
CLINICAL FEATURES...cont. Late changes include: Fibrosis, dermal thickening, hyperkeratosis Chronic eczema Fungal infection of skin (dermatophytosis) & nails (onychomycosis) Fissuring Verrucae Papillae
INVESTIGATIONS
LYMPHANGIOGRAPHY Injection of contrast medium into; Peripheral lymphatic vessel (direct lymphangiography) Intra-dermally in a web space (indirect lymphangiography) Radiographs taken to visualize lymphatics and nodes Shows structural abnormalities
Lymphatic hypoplasia Normal
Lymphatic hypoplasia of the right leg
CT SCAN Single slice through mid-calf is taken Useful test for confirming lymphedema ( honeycomb pattern in an enlarged subcutaneous tissue)
OTHER INVESTIGATIONS Isotope lymphoscintigraphy MRI Ultrasound Lymph node biopsy
MANAGEMENT OF LYMPHEDEMA
MANAGEMENT OF LYMPHEDEMA 1. Control of swelling 2. Skin care 3. Surgery
1 MANAGEMENT -CONTROL OF SWELLING
Massage Elevation of legs Compression garments Bandaging Pneumatic Compression Devices
2 MANAGEMENT -SKIN CARE
SKIN CARE Limb washed daily Dried with hair dryer on low heat Paraffin cream for dryness Keratolytic agent e.g. 5% salicylic acid for hyperkeratosis Avoid lotions/soaps containing sensitizers
3 MANAGEMENT -SURGERY
LYMPHOEDEMA SURGICAL CHOICES Limb reduction procedures Liposuction Bypass procedures
SURGERY LIMB REDUCTION PROCEDURES Sistrunk Homans Thompson Charles
SISTRUNK PROCEDURE A wedge of skin / subcutaneous tissue is excised Wound is closed primarily Commonly done to reduce girth of the thigh
HOMANS PROCEDURE Skin flaps are elevated Subcutaneous tissues are excised beneath the flaps Flaps are trimmed to accommodate reduced girth of the limb and closed primarily
THOMPSON PROCEDURE One skin flap is denuded (shaved of epidermis) Sutured to the deep fascia and buried beneath the second skin flap Helps create new lymphatic connections between the superficial and deep systems
CHARLES PROCEDURE Involves excision of all skin and subcutaneous tissue down to the deep fascia Split-skin graft then used to cover the denuded area Help greatly reduce the girth of the limb
LIPOSUCTION Fat is removed via a cannula (a hollow tube) and aspirator (a suction device)