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 WHO
Lymphatic Filariasis is the 2nd
leading cause of permanent &
long term disability in the world
after leprosy

Basics of Lymphatic System

Micronatomy of lymphatic system

Lymphatic capillaries
Blind ended
Large intercellular & intracellular
fenestrations
Allowing macromolecular influx (1000 kDa)
Collagen fibers attachment on outer surface
Dermal papillae

Microanatomy 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

Capillaries
Pre-collectors
Collectors

Deep lymphatic trunk

Anatomy

Pathophysiology

90%

10%

Pathophysiology

Pathophysiology

Collagen
deposition

lymphostasis

Sub dermal
fibrosis

LYMPHEDEMA

Dermal
thickening

Valvular
incompetence

Hypocontractility

Obstruction
Aplasia
hypoplasia

Etiology of lymphedema

LYMPHEDEMA

Primary lymphedema

Congenital

Praecox

Secondary lymphedema

Tarda

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

Lymphedema Precox
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 Tarda (Meige disease )

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

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
Stemmers sign

Skin changes
Chronic eczema
Dermatophytosis
Fissuring
Verrucae
Ulcerations
Stewart Treves syndrome

Brunner Classification
0

Histological abnormalities
Not clinical evident

Pitting edema,
Subsides with elevation

II

Non pitting edema

Not 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

MRI Scan

An indication for CT scan

or MRI
is suspicion of
malignancy,
for which these tests offer
the most information

Blood slide (Microfilaria)

Adult worms in lymph nodes

Differential diagnosis
Congestive heart failure
Liver and renal failure
Deep vein thrombosis
Venous insufficiency
Lipedema (usually sparing the feet)
Idiopathic edema
Hypoalbuminemia
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
(Dont 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