The document summarizes trends in lymphedema management. It discusses the basics of the lymphatic system, embryology, anatomy, pathophysiology, etiology of primary and secondary lymphedema, presentation, investigations, and treatment including conservative therapies like manual lymphatic drainage and compression garments as well as surgical procedures. Worldwide, it is estimated that 140-250 million people have lymphedema with filariasis being the most common cause.

1. TRENDS in Lmphedema Management Dr. Mansoor Khan MBBS, FCPS-I Surgical “D” unit, Khyber Teaching Hospital, Peshawar

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

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

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

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

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

9. Basics of Lymphatic System

10. Develops from 4 primitive cystic spaces, 2 in the neck and 2 in the groin Cisterns elongate & develop communications Condensations along the connections are lymph nodes * Persistence of primitive cisterns are cystic hygromas Embryology of lymphatic system

11. Embryology

12. 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

13. 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

14. Capillaries Pre-collectors Collectors Deep lymphatic trunk

15. Anatomy

16. Pathophysiology

18. 90% 10% Pathophysiology

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

20. Etiology of lymphedema LYMPHEDEMA Primary lymphedema Secondary lymphedema Congenital Praecox Tarda

22. 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

23. 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

24. 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 )

25. Secondary Lymphedema Most common lymphedema having well recognized causes

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

27. Endemic areas of Filariasis

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

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

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

34. 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

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

36. Presentation of lymphedema Chyluria, chylous ascites, chylothorax, Lymphorrhoea MEGALYMPHATICS

38. Brunner Classification 0 Histological abnormalities Not clinical evident I Pitting edema, Subsides with elevation II Non pitting edema Not relieved with elevation III Irreversible skin changes, fibrosis, papillae

39. Investigations

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

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

42. Lymphangiogram

43. Lymphangiogram

44. Isotope Lymphoscintigraphy Replaced the earlier Technetium labeled antimony sulphide

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

46. Lymphoscintigram

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

48. Blood slide (Microfilaria)

49. Blood slide

50. Adult worms in lymph nodes

51. Others Eosinophilia Increased IgE levels Compliment fixation test Antigens of filaria

52. Treatment

53. TREATMENT Conservative Surgical

54. Conservative Physical Medication

55. 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

56. CLT

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

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

59. Psychological support & occupational therapy

60. 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)

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

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

63. Surgical Procedures for Lymphedema

64. Surgical Ablative/reduction Bypass surgeries

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

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

67. 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

68. 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

69. 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

70. Bypass surgeries Lymph node anastamosis with veins Lymphovenous anastamosis

73. Lymphedema

74. Thanks