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Dr. Hakan Arslan. LYMPHEDEMA AND TREATMENT. “ Accumulation of abnormal amount of protein rich fluid in the interstitium due to compromised lymphatic system with (near) normal net capillary filtration ”. In United States - PowerPoint PPT Presentation



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

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

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

  • Lymphatic filariasis affects more than 90 million people in the world

  • According to WHOLymphatic Filariasis is the 2nd leading cause of permanent & long term disability in the world after leprosy

  • Basics of Lymphatic System

  • Lymphatic capillaries Blind ended Large intercellular & intracellular fenestrations Allowing macromolecular influx (1000 kDa) Collagen fibers attachment on outer surface Dermal papillaeMicronatomy of lymphatic system

  • Sub papillary pre-collectors Sub-dermal collector lymphatics Epifacial, valved, muscular lymphatics with lymphangions Subfascial lymphatics Interconnections at inguinal, anticubital, axillary levelsMicroanatomy of lymphatic system

  • Capillaries Pre-collectors Collectors Deep lymphatic trunk

  • Anatomy

  • Pathophysiology

  • 90%10%


  • Pathophysiology

  • Etiology of lymphedema

  • Congenital lymphedema

    < 1year of age10-25% of all primary lymphedema Sporadic or familial (Milroy's disease)More common in malesLower extremity is involved 3 times more frequently than the upper extremity 2/3 patients have bilateral lymphedema Aplasia pattern without subcutaneous lymphatic trunks involvement

  • Evident after birth and before age 35 yearsMost often arises during puberty 65-80% of all primary lymphedema casesFemales 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

  • 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 )

  • Secondary Lymphedema Most common lymphedema having well recognized causes

  • Filariasis Commonest cause worldwideEndemic in 72 countries Affecting 5-10% population Africa, India, South America

  • Endemic areas of Filariasis

  • Other causes of Secondary Lymphedema

    Breast surgery with radiotherapyPrimary malignancyProstate, cervical cancer, malignant melanoma Trauma to lymphatics Surgical excision of lymph nodes

  • Presentation of lymphedemaAge of onsetPainless swellingPresence or absence of family historyCoexistent pathology

  • Presentation of lymphedemaCharacteristically foot involvement Ankle contours are lost with infilling of the submalleolar depressionsBuffalo hump on foot dorsumSquare shaped toes Stemmers sign

  • Skin changesChronic eczemaDermatophytosisFissuringVerrucaeUlcerationsStewart Treves syndrome

  • Brunner Classification

  • Investigations

    Infrequently required to establish the diagnosis

    To determine residual lymphatic function

    To establish treatment preferences

    To evaluate therapy

  • Contrast Lymphangiography

    Was gold standard for mapping

    Damages the normal lymphatic channels due to inflammation

    Very painful procedure and needs GA

  • Isotope Lymphoscintigraphy

    Replaced the earlier

    Technetium labeled antimony sulphide

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

  • Lymphoscintigram

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

  • Blood slide (Microfilaria)

  • Adult worms in lymph nodes

  • Differential diagnosisCongestive heart failureLiver and renal failureDeep vein thrombosisVenous insufficiencyLipedema (usually sparing the feet)Idiopathic edemaHypoalbuminemiaVascular malformations

  • Treatment

  • 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

  • CLT

  • Intermittent pneumatic pump compression therapy Effectively milking the lymph from the extremity

    Compression garment To help prevent return of fluid

  • Skin care (Examine, dry, moisturizers) Exercises

  • Psychological support & occupational therapy

  • Antiparasitic agents

    Diethylecarbimazole 6mg/kg single dose or 1-3wk(Dont use in pregnancy, infants, elderly)Ivermectin (400mcg/kg/d)Tetracycline Doxycycline (100mg/day for 6-8 wks)

  • Antibiotic

    For skin infectionsPenicillin V 500mg tds for streptococcalFlucloxacilline 250mg qid for staphylococcalInfectionsMiconazole 1% skin ointmentOr systemic antifungal

  • Hydroxyrutosides/ coumadins

    Binds wit proteins, engulfed by macrophages leading to proteolysis

  • Surgical Procedures for Lymphedema

  • Ablative surgeries Sistrunk procedure

    Homan procedure

    Thompson procedure

    Charles procedure

  • Sistrunk Procedure (1918)

    Wedge of skin & subcutaneous tissue excised & wound closed primarily

    Most commonly used to reduce girth of thigh

  • Homan ProcedureSkin flaps are elevatedSubcutaneous tissue excisedSkin flap trimmed & closedUsually staged procedure with lateral & medial separated by 3-6 months to avoid necrosisMostly for calf

  • 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

  • Charles Procedure (1912)Excision of all skin/subcutaneous tissue down to deep fascia Covering by split thickness skin grafts from the excised skinGirth can be greatly reducedUnsatisfactory cosmetic results

  • Bypass surgeries Lymph node anastamosis with veinsLymphovenous anastamosis

  • Thanks


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