LYMPHOEDEMA FAISAL GHANI SIDDIQUI MBBS; FCPS; PGDIP-BIOMEDICAL ETHICS; MCPS-HPE

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1 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

2 PREAMBLE What is lymphatic system and what does it do? Lymphedema Definition Causes Clinical features Investigations

3 THE LYMPHATIC SYSTEM

4 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)

5 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)

6 LYMPHEDEMA -DEFINITION

7 LYMPHEDEMA -DEFINITION abnormal limb swelling caused by the accumulation of high protein-containing ISF secondary to defective lymphatic drainage

8 CAUSES OF LYMPHEDEMA

9 CAUSES OF LYMPHEDEMA Primary lymphedema Secondary lymphedema

10 PRIMARY LYMPHEDEMA Cause is unknown Proposed to be due to inherent congenital lymphatic dysplasia Aplasia / hypoplasia Hyperplasia

11 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

12 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, MEP Ltd 2006.

13 Lymphatic Edema secondary to Filariasis

14 CLINICAL SIGNS & SYMPTOMS

15 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

16 CLINICAL FEATURES...cont. Late changes include: Fibrosis, dermal thickening, hyperkeratosis Chronic eczema Fungal infection of skin (dermatophytosis) & nails (onychomycosis) Fissuring Verrucae Papillae

17 INVESTIGATIONS

18 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

19 Lymphatic hypoplasia Normal

20 Lymphatic hypoplasia of the right leg

21 CT SCAN Single slice through mid-calf is taken Useful test for confirming lymphedema ( honeycomb pattern in an enlarged subcutaneous tissue)

22 OTHER INVESTIGATIONS Isotope lymphoscintigraphy MRI Ultrasound Lymph node biopsy

23 MANAGEMENT OF LYMPHEDEMA

24 MANAGEMENT OF LYMPHEDEMA 1. Control of swelling 2. Skin care 3. Surgery

25 1 MANAGEMENT -CONTROL OF SWELLING

26 Massage Elevation of legs Compression garments Bandaging Pneumatic Compression Devices

27 2 MANAGEMENT -SKIN CARE

28 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

29 3 MANAGEMENT -SURGERY

30 LYMPHOEDEMA SURGICAL CHOICES Limb reduction procedures Liposuction Bypass procedures

31 SURGERY LIMB REDUCTION PROCEDURES Sistrunk Homans Thompson Charles

32 SISTRUNK PROCEDURE A wedge of skin / subcutaneous tissue is excised Wound is closed primarily Commonly done to reduce girth of the thigh

33 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

34 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

35 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

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37 LIPOSUCTION Fat is removed via a cannula (a hollow tube) and aspirator (a suction device)