[02] lymphedema
DESCRIPTION
kedokteranTRANSCRIPT
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LYMPHEDEMA AND TREATMENT
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Accumulation of abnormal amount of protein rich fluid in the interstitium due to compromised lymphatic system with (near) normal net capillary filtration
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In United States Highest incidence is observed following breast cancer surgery with radiotherapy (10 40%).
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Worldwide 140-250 million cases of lymphedema are estimated to exist with filariasis as the most common cause
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Lymphatic filariasis affects more than 90 million people in the world
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According to WHOLymphatic Filariasis is the 2nd leading cause of permanent & long term disability in the world after leprosy
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Basics of Lymphatic System
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Lymphatic capillaries Blind ended Large intercellular & intracellular fenestrations Allowing macromolecular influx (1000 kDa) Collagen fibers attachment on outer surface Dermal papillaeMicronatomy of lymphatic system
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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
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Capillaries Pre-collectors Collectors Deep lymphatic trunk
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Anatomy
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Pathophysiology
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90%10%Pathophysiology
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Pathophysiology
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Etiology of lymphedema
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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
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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
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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 )
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Secondary Lymphedema Most common lymphedema having well recognized causes
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Filariasis Commonest cause worldwideEndemic in 72 countries Affecting 5-10% population Africa, India, South America
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Endemic areas of Filariasis
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Other causes of Secondary Lymphedema
Breast surgery with radiotherapyPrimary malignancyProstate, cervical cancer, malignant melanoma Trauma to lymphatics Surgical excision of lymph nodes
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Presentation of lymphedemaAge of onsetPainless swellingPresence or absence of family historyCoexistent pathology
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Presentation of lymphedemaCharacteristically foot involvement Ankle contours are lost with infilling of the submalleolar depressionsBuffalo hump on foot dorsumSquare shaped toes Stemmers sign
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Skin changesChronic eczemaDermatophytosisFissuringVerrucaeUlcerationsStewart Treves syndrome
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Brunner Classification
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Investigations
Infrequently required to establish the diagnosis
To determine residual lymphatic function
To establish treatment preferences
To evaluate therapy
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Contrast Lymphangiography
Was gold standard for mapping
Damages the normal lymphatic channels due to inflammation
Very painful procedure and needs GA
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Isotope Lymphoscintigraphy
Replaced the earlier
Technetium labeled antimony sulphide
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Dye needs to be injected in toe web through a 27 G needle
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Lymphoscintigram
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An indication for CT scan or MRI is suspicion of malignancy, for which these tests offer the most information MRI Scan
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Blood slide (Microfilaria)
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Adult worms in lymph nodes
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Differential diagnosisCongestive heart failureLiver and renal failureDeep vein thrombosisVenous insufficiencyLipedema (usually sparing the feet)Idiopathic edemaHypoalbuminemiaVascular malformations
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Treatment
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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
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CLT
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Intermittent pneumatic pump compression therapy Effectively milking the lymph from the extremity
Compression garment To help prevent return of fluid
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Skin care (Examine, dry, moisturizers) Exercises
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Psychological support & occupational therapy
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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)
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Antibiotic
For skin infectionsPenicillin V 500mg tds for streptococcalFlucloxacilline 250mg qid for staphylococcalInfectionsMiconazole 1% skin ointmentOr systemic antifungal
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Hydroxyrutosides/ coumadins
Binds wit proteins, engulfed by macrophages leading to proteolysis
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Surgical Procedures for Lymphedema
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Ablative surgeries Sistrunk procedure
Homan procedure
Thompson procedure
Charles procedure
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Sistrunk Procedure (1918)
Wedge of skin & subcutaneous tissue excised & wound closed primarily
Most commonly used to reduce girth of thigh
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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
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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
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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
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Bypass surgeries Lymph node anastamosis with veinsLymphovenous anastamosis
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