1. LYMPHEDEMA AND TREATMENT Dr. Hakan Arslan

2. “ Accumulation of abnormal amount of protein rich fluid in the interstitium due to compromised lymphatic system with (near) normal net capillary filtration ”

3. In United States Highest incidence is observed following breast cancer surgery with radiotherapy (10 – 40%).

4. Worldwide 140-250 million cases of lymphedema are estimated to exist with filariasis as the most common cause

5. Lymphatic filariasis affects more than 90 million people in the world

6. According to WHO Lymphatic Filariasis is the 2 nd leading cause of permanent & long term disability in the world after leprosy

7. Basics of Lymphatic System

8.  Lymphatic capillaries  Blind ended  Large intercellular & intracellular fenestrations  Allowing macromolecular influx (1000 kDa)  Collagen fibers attachment on outer surface  Dermal papillae Micronatomy of lymphatic system

9.  Sub papillary pre-collectors  Sub-dermal collector lymphatics  Epifacial, valved, muscular lymphatics with lymphangions  Subfascial lymphatics  Interconnections at inguinal, anticubital, axillary levels Microanatomy of lymphatic system

10. Capillaries Pre-collectors Collectors Deep lymphatic trunk

11. Anatomy

12. Pathophysiology

14. 90% 10% Pathophysiology

15. Collagen deposition lymphostasis Obstruction Aplasia hypoplasia Hypocon- tractility Valvular incompetence Dermal thickening Sub dermal fibrosis LYMPH- EDEMA Pathophysiology

16. LYMPHEDEMA Primary lymphedemaSecondary lymphedema CongenitalPraecoxTarda Etiology of lymphedema

18. Congenital lymphedema < 1year of age 10-25% of all primary lymphedema Sporadic or familial (Milroy's disease) More common in males Lower extremity is involved 3 times more frequently than the upper extremity 2/3 patients have bilateral lymphedema Aplasia pattern without subcutaneous lymphatic trunks involvement

19. Evident after birth and before age 35 years Most often arises during puberty 65-80% of all primary lymphedema cases Females are affected 4 times 70% of cases are unilateral, with the left lower extremity being involved Hypoplastic pattern, with the lymphatics reduced in caliber and number Lymphedema Precox

20. Clinically not evident until 35 years or older Rarest form of primary lymphedema Only 10% of cases Hyperplasic pattern, with tortuous lymphatics increased in caliber and number Absent or incompetent valves Lymphedema Tarda (Meige disease )

21. Secondary Lymphedema Most common lymphedema having well recognized causes

22. Filariasis Commonest cause worldwide Endemic in 72 countries Affecting 5-10% population Africa, India, South America

23. Endemic areas of Filariasis

24. Filariasis Wuchereria Bancrofti (90%) Brugia malayi Brugia timori

27. Other causes of Secondary Lymphedema Breast surgery with radiotherapy Primary malignancy Prostate, cervical cancer, malignant melanoma Trauma to lymphatics Surgical excision of lymph nodes

28. Presentation of lymphedema Age of onset Painless swelling Presence or absence of family history Coexistent pathology

29. Presentation of lymphedema Characteristically foot involvement Ankle contours are lost with infilling of the submalleolar depressions Buffalo hump on foot dorsum Square shaped toes Stemmer’s sign

30. Skin changes Chronic eczema Dermatophytosis Fissuring Verrucae Ulcerations Stewart Treves syndrome

32. Brunner Classification 0Histological abnormalities Not clinical evident IPitting edema, Subsides with elevation IINon pitting edema Not relieved with elevation IIIIrreversible skin changes, fibrosis, papillae

33. Investigations Infrequently required to establish the diagnosis To determine residual lymphatic function To establish treatment preferences To evaluate therapy

34. Contrast Lymphangiography Was gold standard for mapping Damages the normal lymphatic channels due to inflammation Very painful procedure and needs GA

35. Isotope Lymphoscintigraphy Replaced the earlier Technetium labeled antimony sulphide

36. Dye needs to be injected in toe web through a 27 G needle

37. Lymphoscintigram

38. An indication for CT scan or MRI is suspicion of malignancy, for which these tests offer the most information MRI Scan

39. Blood slide (Microfilaria)

40. Adult worms in lymph nodes

41. Differential diagnosis Congestive heart failure Liver and renal failure Deep vein thrombosis Venous insufficiency Lipedema (usually sparing the feet) Idiopathic edema Hypoalbuminemia Vascular malformations

42. Treatment

43. TREATMENT ConservativeSurgical

44. Conservative PhysicalMedication

45.  Complex Lymphedema Therapy (CLT) Manual lymphatic drainage (MLD)* (massage to make the flow to normal lymphatics) Low stretch bandaging (to prevent re-accumulation) *Vodder and/or Leduc techniques

46. CLT

47. Intermittent pneumatic pump compression therapy Effectively milking the lymph from the extremity Compression garment To help prevent return of fluid

48. Skin care (Examine, dry, moisturizers) Exercises

49. Psychological support & occupational therapy

50. Antiparasitic agents Diethylecarbimazole 6mg/kg single dose or 1-3wk (Don’t use in pregnancy, infants, elderly) Ivermectin (400mcg/kg/d) Tetracycline Doxycycline (100mg/day for 6-8 wks)

51. Antibiotic For skin infections Penicillin V 500mg tds for streptococcal Flucloxacilline 250mg qid for staphylococcal Infections Miconazole 1% skin ointment Or systemic antifungal

52. Hydroxyrutosides/ coumadins Binds wit proteins, engulfed by macrophages leading to proteolysis

53. Surgical Procedures for Lymphedema

54. Surgical Ablative/reductionBypass surgeries

55. Ablative surgeries Sistrunk procedure Homan procedure Thompson procedure Charles procedure

56. Sistrunk Procedure (1918) Wedge of skin & subcutaneous tissue excised & wound closed primarily Most commonly used to reduce girth of thigh

57. Homan Procedure Skin flaps are elevated Subcutaneous tissue excised Skin flap trimmed & closed Usually staged procedure with lateral & medial separated by 3-6 months to avoid necrosis Mostly for calf

58. Thompson Procedure (1962) Denuded skin flaps sutured to deep fascia & buried (buried dermal flap) To establish connection b/w superficial and deep systems Formation of pilonidal sinus

59. Charles Procedure (1912) Excision of all skin/subcutaneous tissue down to deep fascia Covering by split thickness skin grafts from the excised skin Girth can be greatly reduced Unsatisfactory cosmetic results

60. Bypass surgeries Lymph node anastamosis with veins Lymphovenous anastamosis

63. Thanks