LYMPHEDEMA AND TREATMENT

Dr. Hakan Arslan

“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 WHO

Lymphatic 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 papillae

Micronatomy of lymphatic system

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

Capillaries Pre-collectors

Collectors

Deep lymphatic trunk

Anatomy

Pathophysiology

90% 10%

Pathophysiology

Collagen deposition

lymphostasis

Obstruction

Aplasiahypoplasia

Hypocon-tractility

Valvular incompetence

Dermal thickening

Sub dermalfibrosis

LYMPH-EDEMA

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LYMPHEDEMA

Primary lymphedema Secondary lymphedema

Congenital Praecox Tard

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Etiology of lymphedema

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

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

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 worldwide

Endemic in 72 countries

Affecting 5-10% population Africa, India, South America

Endemic areas of Filariasis

Filariasis

Wuchereria Bancrofti (90%)

Brugia malayi

Brugia timori

Other causes of Secondary Lymphedema

Breast surgery with radiotherapy

Primary malignancy

Prostate, cervical cancer, malignant melanoma

Trauma to lymphatics

Surgical excision of lymph nodes

Presentation of lymphedema

Age of onset

Painless swelling

Presence or absence of family history

Coexistent pathology

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

Skin changes

Chronic eczema

Dermatophytosis

Fissuring

Verrucae

Ulcerations

Stewart Treves syndrome

Brunner Classification

0 Histological abnormalitiesNot clinical evident

I Pitting edema, Subsides with elevation

II Non pitting edemaNot relieved with elevation

III Irreversible skin changes,fibrosis, papillae

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 failure

Liver and renal failureDeep vein thrombosis

Venous insufficiencyLipedema (usually sparing the feet)

Idiopathic edemaHypoalbuminemia

Vascular malformations

Treatment

TREATMENT

Conservative Surgical

Conservative

Physical Medication

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

(Don’t use in pregnancy, infants, elderly)

Ivermectin (400mcg/kg/d)

Tetracycline

Doxycycline (100mg/day for 6-8 wks)

Antibiotic

For skin infections

Penicillin V 500mg tds for streptococcal

Flucloxacilline 250mg qid for staphylococcal

Infections

Miconazole 1% skin ointment

Or systemic antifungal

Hydroxyrutosides/ coumadins

Binds wit proteins, engulfed by macrophages leading to proteolysis

Surgical Procedures for Lymphedema

Surgical

Ablative/reduction Bypass surgeries

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 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

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

skin

Girth can be greatly reduced

Unsatisfactory cosmetic results

Bypass surgeries

Lymph node anastamosis with veins

Lymphovenous anastamosis

Thanks