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Trends in the management of Lymphedema

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  • 1. TRENDS in Lmphedema Management Dr. Mansoor Khan MBBS, FCPS-I Surgical “D” unit, Khyber Teaching Hospital, Peshawar
  • 2.  
  • 3.  
  • 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
  • 17.  
  • 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
  • 21.  
  • 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
  • 29.  
  • 30.  
  • 31.  
  • 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
  • 37.  
  • 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
  • 71.  
  • 72.  
  • 73. Lymphedema
  • 74. Thanks