lymphedema internal medicine ppt presentation

Lymphedema

1. Lymphedema IM presentation 3C

2. Headings Anatomy and Physiology Definition Pathophysiology Classification Clinical Staging Approach to The Patients Signs and Symptoms Investigations Differential Diagnosis Management Preventive Non-operative Operative 1 2 3 4 5 6 7

3. Lymphatic capillaries Blind ended Larger sized ( 50 µm ) Fenestrated ( 1000  kDa ) Collapsed ( at rest ) Precollector lymphatics Collecting lymphatics Anatomy & Physiology 1

4. Lymphatic Trunks Analogous To Venous Anatomy Structure of vessel wall Superficial and Deep system Unidirectional flow Unlike Venous System Flow: superficial > deep vessel (10%)

5. 1 Absorption Starling’s forces and net capillary filtration Inter and intra endothelial pores Active phagocytosis Transportation Intrinsic lymphatic contractility Prevention of reflux because of valves Augmented by exercise, limb movement and external compression return of protein-rich fluid to the circulation

6. The ISF compartment ( 10–12 litres in a 70-kg man ) About 8 litres (protein concentration approximately 20–30 g/L, similar to ISF) of lymph is produced each day and travels in afferent lymphatics to lymph nodes . There, the volume is halved and the protein concentration doubled, resulting in 4 litres of lymph re-entering the venous circulation each day via efferent lymphatics. Some facts

7. Lymphedema is the term used to Swelling of one or more limbs and occasionally the trunk and genitalia. Caused by progressive accumulation of protein-enriched interstitial fluid As a consequence of relative impairment of lymphatic vascular function At birth, 1 in 6000 people will develop lymphoedema With an overall prevalence of 0.13–2%. Significant impact on quality of life (QOL) 2 Definition

8. 3 Pathophysiology  Lymph Production  Lymph Absorption Accumulation of protein and cellular metabolites in the interstitial space  Tissue colloid osmotic pressure  Water accumulation in interstitial space Secondary inflammatory and immune responses characterized by infiltration of mononuclear cells, fibroblasts, and adipocytes Adipose and collagen deposition in the skin and subcutaneous tissues Progressive subcutaneous fibrosis

9. 4 Classification It is based on etiology; But doesn’t help in management

10. Congenital (onset <2 years old): sporadic; familial ( Nonne –Milroy’s disease ) Praecox (onset 2–35 years old): sporadic; familial ( Letessier – Meige’s disease ) most commonly appears at the onset of puberty accounting for up to 94% of cases 10: 1 female-to male prevalence usually unilateral Tarda (onset after 35 years old) Primary Lymphedema

11. Trauma and tissue damage Lymph node excision Radiotherapy Burns Variscose vein surgery/harvesting Large/circumferential wounds Scarring Infection Cellulitis/erysipelas Lymphadenitis Tuberculosis Filariasis Malignant disease Lymph node metastases Infiltrative carcinoma Lymphoma Pressure from large tumours Inflammation Rheumatoid arthritis Dermatitis Psoriasis Sarcoidosis Dermatosis with epidermal involvement Venous disease Chronic venous insufficiency Venous ulceration Post-thrombotic syndrome Intravenous drug use Miscellaneous Pretibial myxoedema Immobility and dependency Dependency oedema Paralysis Secondary Lymphedema

12. Grade (Brunner) Latent I. II. III. Clinical Features Edema Not apparent Pits on pressure Not pits on pressure Irreversible Upon Limb Elevation -- Largely or completely disappears Not significantly reduce Irreversible Fibrosis No clinical evidence Moderate to severe Severe fibrosclerosis Excess interstitial fluid and histological abnormalities in lymphatics and lymph nodes Positive stemmer’s sign Lymphostatic elephantiasis 5 Clinical Staging

13. ‘ Buffalo hump ’ on the dorsum of the foot Loss of the normal perimalleolar shape, resulting in a “ tree trunk ” pattern 6 Clinical Approach Signs and Symptoms Characteristically involves the foot Initially pitting edema and  on limb elevation ‘Square toes’ ‘ Stemmer’s sign ’ skin on the dorsum of the toes cannot be pinched because of subcutaneous fibrosis.

14. Skin changes Hyperkeratosis, lichenification , and development of peau d’orange . ‘ Pigskin ’ Chronic eczema, fungal infection of the skin ( dermatophytosis ) and nails (onychomycosis), fissuring, verrucae and papillae (warts) are frequently seen in advanced disease. Ulceration is unusual << higher degree of hydration and elasticity Lymphorrhea Other symptoms Pain per se rare but mainly heaviness of limb , causing immobility and debility

15. Infections: proceeds and precipitates the lymphedema Recurrent cellulitis: good substrate and less host immune response Malnutrition and immunodeficiency: Especially in lymphangiectasia with protein-losing enteropathy or chylous ascites or chylothorax Malignancies: That appear with increased frequency in lymphedematous limbs include Lymphangiosarcoma Kaposi’s sarcoma, Squamous cell carcinoma, Malignant lymphoma, and Melanoma. Complications

16. Lymphangiosarcoma After long-standing secondary lymphedema, Is a rare malignant disease that frequently results in limb loss or even death. Is manifested as multicentric lesions with bluish nodules, sclerotic plaques, or bullous changes. Originally described in postmastectomy oedema ( stewart – treves ’ syndrome ) and affects around 0.5% of patients at a mean onset of 10 years. Confirmed by skin biopsy. Amputation offers the best chance of survival

17. Differential Diagnosis of The Swollen Limb Lymphatic Venous Arterial Non-lymphatic Non-Vascular Systemic Local Cardiac /Hepatic /Renal Hypoproteinemia Drugs /Allergic Obesity Trauma/ Hematoma Tumors- bony/soft tissue Ruptured baker cyst Myositis ossificans AV malformations Aneurysms DVT CVI Lymphedema

18. NOTE: First rule out systemic and ominous local causes -- if there is a severe, atypical, complicated swollen limb, then only investigations are done ‘Routine’ tests: CBC, RFT, LFT, Urine analysis TFT, ECG, 2D Echo, Blood smear for microfilariae, Chest radiograph and Ultrasound/Doppler. Specific Investigations Lymphoscintigraphy Lymphangiography MRI/ CT Investigations

19. Lymphoscintigraphy Radioactive technetium-labelled albumin or antimony trisulfide are injected into an interdigital web space and specifically taken up by lymphatics, and serial radiographs are taken with a gamma camera.

20. Interpretation Of Lymphoscintigraphy It is functional assessment Normal study: uptake at groin nodes (1hr), bladder, liver (3hr) Primary disease: none Lymphedema: dermal backflow, no node uptake

21. Anatomical assessment: used primarily before reconstructive lymphatic surgery. Done by the direct injection of iodine-based, lipid-soluble agents into the subcutaneous lymphatics, Has technically difficult; unpleasant for the patient; may cause further lymphatic injury----> largely, it has become obsolete Lymphangiography

22. A single, axial slice through the mid-calf Lymphoedema (coarse, non-enhancing, reticular ‘honeycomb’ pattern in an enlarged subcutaneous compartment), Venous oedema (increased volume of the muscular compartment) and Lipoedema (increased subcutaneous fat). Also be used to exclude pelvic or abdominal mass lesions. Also be used to monitor response to treatment through serial measurements MRI corroborated with scintigraphy for surgery CT & MRI

23. The goals of treatment are to Reduce swelling Prevent the development of complications. The standard of care for lymphedema is commonly referred as complex decongestive therapy (CDT) . It is the combination of four components and two phases. The components are: Skin care, Manual lymphatic drainage (MLD) and Multilayer lymphedema bandaging (MLLB), and Exercises. Phases are First, short intensive period: therapist-led care (weeks) The second is a maintenance phase: self-care regime with occasional professional intervention. (lifelong) Management: Non Operative 7

24. Skin Care Thorough limb wash Careful Skin drying Use of Moisturizer For marked hyperkeratosis: keratolytics (5% salicylic acid) Fungal infections : 3% benzoic acid oint , powders At risk sites of maceration : an antiseptic agent such as eosin Lymphorrhoea : emollients, elevation, compression and sometimes cautery under anaesthetic

25. . Manual Lymphatic Drainage apply gentle pressure to the skin stretch the superficial lymphatics contraction and sequestration of lymphatic vessels Evacuation of fluid and protein from the interstitial space Segmental + sequential manner: distal to proximal https://www.youtube.com/watch?v=X7oM_vi5Umw

26. The goal is to create an Internal Pump-like Action . Two different types of pressures are produced by the bandaging: (1) the low resting pressure (20 to 30 mm Hg), which is the result of short-stretch bandages on the patient during resting; and (2) the high working pressure , which is the result of short stretch bandages on the patient during muscle contraction. Multilayer Lymphedema Bandaging (1) tubular bandage lining (2) under the cast padding (3) multiple layers of short stretch bandages https://www.youtube.com/watch?v=7aQnRujbEZo

27. The pressure exerted must be graduated (100% ankle/foot, 70% knee, 50% mid-thigh, 40% groin). Provided there is no underlying peripheral arterial disease, sustained pressure of 60 to 70 mm Hg has been suggested as a maximum upper limit to treat lymphedema. The patient should put the stocking on first thing in the morning before rising Use of Multichember pneumatic compression – new thing Compression Garments and Their Associated Pressure Type Garment Pressure Over the counter 7-15 mm Hg. Not graduated Anti-embolism 15-20 mm Hg. Graduated Chronic venous insufficiency or lymphedema 20-30, 30-40, 40-50, and 50-60 mm Hg Patients with comorbidities 20-30 mm Hg or less Upper extremity 20-30 mm Hg Lower limbs with recalcitrant chronic lymphedema 30–40 mm Hg

28. Lymph formation  directly proportional to arterial inflow and 40% of lymph is formed within skeletal muscle. Exercise routines should include combinations of lymphedema remedial exercises (e.g., Active, Repetitive, Nonresistive Motion of the involved body part) along with three main types of exercise (i.e., AEROBIC, STRENGTH, and FLEXIBILITY ). How it works…. Pumping action of muscles Intrathoracic negative pressure Weight reduction Exercise

29. Operations fall into three categories: Reconstructive or Bypass Procedures, Liposuction Resection or Reduction Procedures. If reconstruction possible … can be offered early in the course If planned for resection… should be last option Surgical intervention should be considered only after a trial of conservative therapy for at least 6 months Management: Operative

30. (I) Autologous Lymphatic Grafting Bypass Surgeries (1) Free Transfer (2) Transposition

31. (II) Lymphovenous Anastomosis Techniques of lymphatic reconstruction with interposition vein graft (A) or lymphovenous anastomosis (B). Technique of invagination of multiple lymphatics into an interposition vein graft: lymphatic-venous-lymphatic anastomosis (C).

32. The rationale: chronic lymphedema causes hypertrophy of the subcutaneous fat. Removal of the hypertrophied and edematous adipose tissue is performed through 3-mm-long incisions (20 to 30 incisions) with vacuum aspiration. Liposuction can be useful in patients with no functioning lymphatics , but in others, the destruction of functioning lymphatics and worsening of the edema are possible Liposuction https://www.plasticsurgery.org/cosmetic-procedures/liposuction/animation

33. Reduction Surgeries Sistrunk A wedge of skin and subcutaneous tissue is excised and the wound closed primarily.

34. Homan’s First, skin flaps are elevated, and then subcutaneous tissue is excised from beneath the flaps, which are then trimmed to size to accommodate the reduced girth of the limb and closed primarily.

35. Thompson’s This is a modification of the Homans ’ procedure aimed to create new lymphatic connections between the superficial and deep systems. One skin flap is denuded (shaved of epidermis), sutured to the deep fascia and buried beneath the second skin flap (the so-called ‘ buried dermal flap ’)

36. Charles Procedure This operation was initially designed for filariasis and involved excision of all of the skin and subcutaneous tissues down to the deep fascia, with coverage using split-skin grafts

37. Therapeutic Approach Based on Clinical Stages Stage I Stage II Stage III DURATION 2-3 weeks 3-4 weeks 4-6 weeks Phase I (Decongestion ) MLD 1-2 × day Short-stretch bandages Skin care Remedial exercises MLD 2 × day Short-stretch bandages Skin care Remedial exercises MLD 2-3× day Short-stretch bandages Skin care Remedial exercises Phase II (Maintenance) MLD as needed Compression garments Skin care Remedial exercises MLD as needed 1-2× per week Compression garments Skin care Remedial exercises MLD as needed 1-2× per week Compression garments Skin care Remedial exercises Repeating phase I (1-2×) Repeating phase I (3-4×) Surgery may be indicated Summary