Trends in the management of Lymphedema


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Trends in the management of Lymphedema

  1. 1. TRENDS in Lmphedema Management Dr. Mansoor Khan MBBS, FCPS-I Surgical “D” unit, Khyber Teaching Hospital, Peshawar
  2. 4. “ Accumulation of abnormal amount of protein rich fluid in the interstitium due to compromised lymphatic system with (near) normal net capillary filtration ”
  3. 5. In United States Highest incidence is observed following breast cancer surgery with radiotherapy (10 – 40%).
  4. 6. Worldwide 140-250 million cases of lymphedema are estimated to exist with filariasis as the most common cause
  5. 7. Lymphatic filariasis affects more than 90 million people in the world
  6. 8. According to WHO Lymphatic Filariasis is the 2 nd leading cause of permanent & long term disability in the world after leprosy
  7. 9. Basics of Lymphatic System
  8. 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
  9. 11. Embryology
  10. 12. <ul><li>Lymphatic capillaries </li></ul><ul><ul><li>Blind ended </li></ul></ul><ul><ul><li>Large intercellular & intracellular </li></ul></ul><ul><ul><li>fenestrations </li></ul></ul><ul><ul><li>Allowing macromolecular influx (1000 kDa) </li></ul></ul><ul><ul><li>Collagen fibers attachment on outer surface </li></ul></ul><ul><ul><li>Dermal papillae </li></ul></ul>Micronatomy of lymphatic system
  11. 13. <ul><li>Sub papillary pre-collectors </li></ul><ul><li>Sub-dermal collector lymphatics </li></ul><ul><li>Epifacial, valved, muscular lymphatics </li></ul><ul><li>with lymphangions </li></ul><ul><li>Subfascial lymphatics </li></ul><ul><li>Interconnections at inguinal, anticubital, </li></ul><ul><li>axillary levels </li></ul>Microanatomy of lymphatic system
  12. 14. Capillaries Pre-collectors Collectors Deep lymphatic trunk
  13. 15. Anatomy
  14. 16. Pathophysiology
  15. 18. 90% 10% Pathophysiology
  16. 19. Pathophysiology Collagen deposition lymphostasis Obstruction Aplasia hypoplasia Hypocon- tractility Valvular incompetence Dermal thickening Sub dermal fibrosis LYMPH- EDEMA
  17. 20. Etiology of lymphedema LYMPHEDEMA Primary lymphedema Secondary lymphedema Congenital Praecox Tarda
  18. 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
  19. 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
  20. 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 )
  21. 25. Secondary Lymphedema Most common lymphedema having well recognized causes
  22. 26. Filariasis Commonest cause worldwide Endemic in 72 countries Affecting 5-10% population Africa, India, South America
  23. 27. Endemic areas of Filariasis
  24. 28. Filariasis Wuchereria Bancrofti (90%) Brugia malayi Brugia timori
  25. 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
  26. 33. Presentation of lymphedema Age of onset Painless swelling Presence or absence of family history Coexistent pathology
  27. 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
  28. 35. Skin changes Chronic eczema Dermatophytosis Fissuring Verrucae Ulcerations Stewart Treves syndrome
  29. 36. Presentation of lymphedema Chyluria, chylous ascites, chylothorax, Lymphorrhoea MEGALYMPHATICS
  30. 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
  31. 39. Investigations
  32. 40. Investigations Infrequently required to establish the diagnosis To determine residual lymphatic function To establish treatment preferences To evaluate therapy
  33. 41. Contrast Lymphangiography Was gold standard for mapping Damages the normal lymphatic channels due to inflammation Very painful procedure and needs GA
  34. 42. Lymphangiogram
  35. 43. Lymphangiogram
  36. 44. Isotope Lymphoscintigraphy Replaced the earlier Technetium labeled antimony sulphide
  37. 45. Dye needs to be injected in toe web through a 27 G needle
  38. 46. Lymphoscintigram
  39. 47. An indication for CT scan or MRI is suspicion of malignancy, for which these tests offer the most information MRI Scan
  40. 48. Blood slide (Microfilaria)
  41. 49. Blood slide
  42. 50. Adult worms in lymph nodes
  43. 51. Others Eosinophilia Increased IgE levels Compliment fixation test Antigens of filaria
  44. 52. Treatment
  45. 53. TREATMENT Conservative Surgical
  46. 54. Conservative Physical Medication
  47. 55. <ul><li>Complex Lymphedema Therapy (CLT) </li></ul><ul><ul><li>Manual lymphatic drainage (MLD) * </li></ul></ul><ul><ul><li>(massage to make the flow to normal lymphatics) </li></ul></ul><ul><ul><li>Low stretch bandaging </li></ul></ul><ul><ul><li> (to prevent re-accumulation) </li></ul></ul>*Vodder and/or Leduc techniques
  48. 56. CLT
  49. 57. Intermittent pneumatic pump compression therapy Effectively milking the lymph from the extremity Compression garment To help prevent return of fluid
  50. 58. Skin care (Examine, dry, moisturizers) Exercises
  51. 59. Psychological support & occupational therapy
  52. 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)
  53. 61. Antibiotic For skin infections Penicillin V 500mg tds for streptococcal Flucloxacilline 250mg qid for staphylococcal Infections Miconazole 1% skin ointment Or systemic antifungal
  54. 62. Hydroxyrutosides/ coumadins Binds wit proteins, engulfed by macrophages leading to proteolysis
  55. 63. Surgical Procedures for Lymphedema
  56. 64. Surgical Ablative/reduction Bypass surgeries
  57. 65. Ablative surgeries Sistrunk procedure Homan procedure Thompson procedure Charles procedure
  58. 66. Sistrunk Procedure (1918) Wedge of skin & subcutaneous tissue excised & wound closed primarily Most commonly used to reduce girth of thigh
  59. 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
  60. 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
  61. 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
  62. 70. Bypass surgeries Lymph node anastamosis with veins Lymphovenous anastamosis
  63. 73. Lymphedema
  64. 74. Thanks