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 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

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

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