LYMPHOEDEMA

DR KRANTHI M
CONTENTS

•Introduction
•pathophysiology
•Aetiology
•Clinical presesntation
•Investigations
•Treatment
•Recent advances
•conclusion
 INTRODUCTION
DEFINITION
• Lymphoedema may be defined as abnormal limb swelling caused by the
accumulation of increased amounts of high protein ISF secondary to
defective lymphatic drainage in the presence of (near) normal net capillary
filtration.

• At birth, 1 in 6000 people will develop lymphoedema with an overall

prevalence of 0.13–2 per cent.
INTRODUCTION

• The condition is not only associated with significant physical

symptoms and complications but is also a frequent cause of
emotional and psychological distress, which can lead to difficulties
with relationships, education and work

• Despite this significant impact on quality of life, many sufferers

choose not to seek medical advice because of embarrassment and a
belief that nothing can be done
INTRODUCTION

• Lymphoedema is often misdiagnosed and mistreated by doctors.

• making an early diagnosis is important because relatively simple

measures can be highly effective at this stage and can prevent the
development of disabling late disease, which is often very difficult to
treat.
 PATHOPHYSIOLOGY
• Lymphedema is the result of an inability of the existing lymphatic
system to accommodate the protein and fluid entering the interstitial
compartment at the tissue level.

impaired lymphatic drainage

protein-rich fluid accumulation in the interstitial compartment

soft pitting edema

PATHOPHYSIOLOGY
accumulation of fibroblasts, adipocytes and macrophages

local inflammatory response.

deposition of connective tissue and adipose elements at the skin and

subcutaneous level

nonpitting, and has a spongy consistency

PATHOPHYSIOLOGY
local inflammatory response and recurrent infectious episodes

worsening the underlying insufficiency of the lymphatic system

excessive subcutaneous fibrosis and scarring

lymphostatic elephantiasis
PATHOPHYSIOLOGY
Hypocontra
Aplasia
ctility
hypoplasia

Valvular
Obstruction incompetence
LYMPH-
EDEMA
l

Sub dermal Dermal

lymphostasis Collagen
fibrosis thickening
deposition
PATHOPHYSIOLOGY

(1) Normal lymphatic flow in (a) deep systems and (b) superficial systems. Note the
small collateral vessels interconnecting the 2 systems. (2) Lymphedema develops
from obstruction, dilation of valves, valvular insufficiency, and subsequent reversal of
lymphatic flow.
CLASSIFICATION
Lymphoedema

Primary lymphoedema Secondary or acquired lymphoedema

Congenital Praecox Tarda

CONGENITAL LYMPHOEDEMA
• <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
LYMPHOEDEMA 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
LYMPHOEDEMA 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 LYMPHOEDEMA

• Most common lymphoedema having well recognized causes.

• Axillary node dissection leading to lymphoedema of arm is the most

common cause of secondary lymphoedema in united states

Filariasis

• Commonest cause worldwide

• Endemic in 72 countries

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

SECONDARY LYMPHOEDEMA
Filariasis

Wuchereria Brugia malayi Brugia timori

Bancrofti (90%)
 SECONDARY LYMPHOEDEMA
Other causes of Secondary Lymphedema

• Primary lymphatic malignancy

• Metastatic spread to lymph nodes

• Radiotherapy to lymph nodes

• Surgical excision of lymph nodes

• Trauma (particularly degloving injuries)

• Superficial thrombophlebitis

• Deep venous thrombosis

PRESENTATION OF LYMPHOEDEMA

• Chronic swelling

• Primarily lower extremity(80%)

• Fever ,chills and generalized weakness

• Fatigue related to size and weight of extremity

• Recurrent bacterial and fungal infections

PRESENTATION OF
LYMPHOEDEMA
• Characteristically foot involvement

• Ankle contours are lost with infilling of the submalleolar depressions

• Buffalo hump on foot dorsum

• Square shaped toes

• Stemmer’s sign
PRESENTATION OF LYMPHOEDEMA
PRESENTATION OF
LYMPHOEDEMA
Skin changes

• Chronic eczema

• Dermatophytosis

• Fissuring

• Verrucae

• Ulcerations

• Stewart Treves syndrome

CLINICAL CLASSIFICATION OF
LYMPHOEDEMA
BRUNNER CLASSIFICATION
0 Histological abnormalities
Not clinical evident
I Pitting edema,
Subsides with elevation
II Non pitting edema
Not relieved with elevation
III Irreversible skin changes,
fibrosis, papillae
DIAGNOSIS

• Detailed history and physical examination

• Investigations may help confirm the diagnosis, inform management

and provide prognostic information
INVESTIGATIONS

• Infrequently required to establish the diagnosis

• To determine residual lymphatic function

• To establish treatment preferences

• To evaluate therapy
INVESTIGATIONS

CONTRAST LYMPHANGIOGRAPHY
• Was gold standard for mapping

• Damages the normal lymphatic channels due to inflammation

• Very painful procedure and needs GA

INVESTIGATIONS

ISOTOPE LYMPHOSCINTIGRAPHY
• Replaced the earlier

• Technetium labeled antimony sulphide

• serial radiographs are taken with a gamma camera

• it has a sensitivity of 70% to 90% and a specificity of nearly 100%

INVESTIGATIONS

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

• An indication for CT scan or MRI

is suspicion of malignancy,

for which these tests offer

the most information

INVESTIGATIONS
OTHERS
• Eosinophilia

• Increased IgE levels

• Compliment fixation test

• Antigens of filaria
TREATMENT

• Ninety-five percent of patients with lymphoedema can be managed

nonoperatively

Three goals of treatment are

• to relieve pain.

• To reduce swelling .

• to prevent the development of complications.

TREATMENT
TREATMENT

Conservative Surgical
TREATMENT
Conservative

Physical Medication
TREATMENT
GENERAL THERAPEUTIC MEASURES
• skin care and avoidance of injuries.

• The patients should always be instructed to see their physicians

early for signs of infections.

• Infections should be aggressively and promptly treated with

appropriate antibiotics
TREATMENT

GENERAL THERAPEUTIC MEASURES

• Eczema at the level of the fore foot and toes requires treatment.

• basic range of motion exercises for the extremities.

• ideal body weight.

TREATMENT
COMPLEX DECONGESTIVE PHYSICALTHERAPY
• Manual lymphatic drainage (MLD) (massage to make the flow to
normal lymphatics)

• Low stretch bandaging (to prevent re-accumulation)

TREATMENT
TREATMENT

MULTILAYER LYMPHOEDEMA BANDAGING:

• pressure exerted must be graduated

• 100 per cent ankle/foot

• 70 per cent knee

• 50 per cent mid-thigh

• 40 per cent groin

TREATMENT

• Ankle–brachial pressure index (ABPI) is usually necessary prior to

commencing any form of compression therapy.

• Standard MLLB and compression is used in patients with ABPI 0.8.

• lower pressures in those with moderate arterial disease (ABPI 0.5–

0.8).

• It is contraindicated in severe arterial insufficiency (ABPI <0.5),

uncontrolled heart failure and severe peripheral neuropathy
TREATMENT

EFFECTS OF MLLB
• Reduces oedema

• Restores shape to the affected area

• Reduces skin changes (hyperkeratosis, papillomatosis)

• Eliminates lymphorrhoea.

• Supports inelastic skin

• Softens subcutaneous tissues

TREATMENT

INTERMITTENT PNEUMATIC PUMP COMPRESSION

THERAPY
• Effectively milking the lymph from the extremity

COMPRESSION GARMENT
• To help prevent return of fluid
TREATMENT
TREATMENT
EXERCISE
• Rhythmic isotonic movements (e.g. Swimming) and massage Will
increase venous and lymphatic return .

• Exercise also helps to maintain joint mobility

• When at rest, the lymphoedematous limb should be positioned with

the foot/hand above the level of the heart.
 TREATMENT
DRUGS
• Benzopyrones have potentially effective agents in the treatment of

lymphedema.

• This class of medications including coumarin (1,2-benzopyrone) is

thought to reduce lymphedema

• Through stimulation of proteolysis by tissue macrophages and

stimulation of the peristalsis and pumping action of the collecting

lymphatics.
TREATMENT

• Surgical intervention may be considered for patients with stage II and

stage III lymphedema who have severe functional impairment,
recurrent episodes of lymphangitis.

• severe pain despite optimal medical therapy.

TREATMENT
SURGERY

RECONSTRUCTIVE EXCISIONAL
TREATMENT
RECONSTRUCTIVE OPERATIONS:
• patients with proximal (either primary or secondary) obstruction of
the extremity lymphatic circulation with preserved, dilated
lymphatics peripheral to the obstruction
TREATMENT
• RECONSTRUCTIVE OPERATIONS:
• Anastomosing lymph nodes to veins (Neibulowitz).

• ileal mucosal patch (Kinmoth).

• lymphovenous bypass.

• lymphaticolymphatic bypass.
TREATMENT

EXCISIONAL SURGERY
• patients without residual lymphatics of adequate size for
reconstructive procedures
TREATMENT

• Homan procedure

• Sistrunk procedure

• Thompson procedure

• Charles procedure
 TREATMENT

HOMAN PROCEDURE
Skin flaps are elevated
Subcutaneous tissue excised
Skin flap trimmed & closed
Usually staged procedure with lateral &
medial incisions.
Separated by 3-6 months to avoid
necrosis
TREATMENT

SISTRUNK PROCEDURE
• Wedge of skin & subcutaneous tissue excised & wound closed
primarily.

• Most commonly used to reduce girth of thigh.

 TREATMENT
THOMPSON PROCEDURE
Denuded skin flaps sutured
to deep fascia & buried
(buried dermal flap)
To establish connection b/w
superficial and deep
systems
TREATMENT

CHARLES PROCEDURE
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
RECENT ADVANCES

• Lymphangiogenesis appears to be regulated by the vascular

endothelial growth factors C and D (VEGF-C, VEGF-D) their receptor,
VEGFR-3 and their binding protein,neuropilin 2 (Nrp2)
RECENT ADVANCES

• molecular treatments are based on the activation of the VEGFR-3

pathway by administration of cognate ligands VEGF-C and VEGF-D
using a variety of methods.

• At this point, these treatments have been tested only in animal

models, with promising results.

• Formal clinical trials are now needed to evaluate the therapeutic

potential and possible untoward effects of therapeutic
lymphangiogenesis
SUMMARY

• Lymphoedema is often misdiagnosed and mistreated by doctors, who

frequently have a poor understanding of the importance of the
condition, believing it to be primarily a cosmetic problem in the early
stages.

• However, making an early diagnosis is important because relatively

simple measures can be highly effective at this stage and can prevent
the development of disabling late disease, which is often very difficult
to treat.
SUMMARY

• Ninety-five percent of patients with lymphedema can be managed

nonoperatively

• Surgical intervention may be considered for patients with stage II and

stage III lymphedema who have severe functional impairment,
recurrent episodes of lymphangitis.

• severe pain despite optimal medical therapy.

THANK YOU