Lymphedema is swelling caused by impaired lymphatic drainage. It is classified as primary or secondary and staged clinically. Treatment involves complex decongestive therapy with skin care, manual lymphatic drainage, compression bandaging, and exercise in two phases. For severe cases, surgery such as lymphovenous anastomosis or tissue resection may be considered after conservative therapy. Proper management can control swelling and prevent complications like infection or lymphangiosarcoma.

1. Lymphedema
MODERATOR: Dr Shekappa C M
PRESENTER: Dr Vinayaka S B

2. Headings
 Anatomy and
Physiology
 Definition
 Pathophysiology
 Classification
 Clinical Staging
 Approach to The
Patients
• Signs and Symptoms
• Investigations
• Differential Diagnosis
 Management
• Preventive
• Non-operative
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3.  Lymphatic capillaries
• Blind ended
• Larger sized (50 µm)
• Fenestrated (1000 kDa )
• Collapsed (at rest)
 Precollector lymphatics
 Collecting lymphatics
Anatomy & Physiology1

4.  Lymphatic Trunks
ANALOGOUS TO VENOUS ANATOMY
• Structure of vessel wall
• Superficial and Deep system
• Unidirectional flow
UNLIKE VENOUS SYSTEM
• Flow: superficial > deep vessel
(10%)

5. 1
 Absorption
• Starling’s forces and net capillary
filtration
• Inter and intra endothelial pores
• Active phagocytosis
 Transportation
• Intrinsic lymphatic contractility
• Prevention of reflux because of
valves
• Augmented by exercise, limb
return of protein-rich fluid to the circulation

6. • The ISF compartment (10–12 litres in a 70-kg man)
• About 8 litres (protein concentration approximately 20–30 g/L, similar to
ISF) of lymph is produced each day and travels in afferent lymphatics to
lymph nodes.
• There, the volume is halved and the protein concentration doubled,
resulting in 4 litres of lymph re-entering the venous circulation each day via
efferent lymphatics.
Some facts

7. Lymphedema is the term used to
 Swelling of one or more limbs and occasionally the trunk and genitalia.
 Caused by progressive accumulation of protein-enriched interstitial fluid
 As a consequence of relative impairment of lymphatic vascular function
 At birth, 1 in 6000 people will develop lymphoedema
 With an overall prevalence of 0.13–2%.
 Significant impact on quality of life (QOL)
2 Definition

8. 3 Pathophysiology
 Lymph Production Lymph Absorption
Accumulation of protein and cellular metabolites in the interstitial space
 Tissue colloid osmotic pressure
 Water accumulation in interstitial space
Secondary inflammatory and immune responses characterized by
infiltration of mononuclear cells, fibroblasts, and adipocytes
Adipose and collagen deposition in the skin and
subcutaneous tissues
Progressive subcutaneous fibrosis

9. 4 Classification
It is based on etiology;
But doesn’t help in
management

10.  Congenital
• (onset <2 years old): sporadic; familial (Nonne–Milroy’s disease)
 Praecox
• (onset 2–35 years old): sporadic; familial (Letessier–Meige’s disease)
• most commonly appears at the onset of puberty
• accounting for up to 94% of cases
• 10: 1 female-to male prevalence
• usually unilateral
 Tarda
• (onset after 35 years old)
Primary Lymphedema

11. Trauma and tissue damage
o Lymph node excision
o Radiotherapy
o Burns
o Variscose vein surgery/harvesting
o Large/circumferential wounds Scarring
Infection
o Cellulitis/erysipelas
o Lymphadenitis
o Tuberculosis
o Filariasis
Malignant disease
o Lymph node metastases
o Infiltrative carcinoma
o Lymphoma
o Pressure from large tumours
Inflammation
o Rheumatoid arthritis
o Dermatitis
o Psoriasis
o Sarcoidosis
o Dermatosis with epidermal involvement
Venous disease
o Chronic venous insufficiency
o Venous ulceration
o Post-thrombotic syndrome
o Intravenous drug use
Miscellaneous
o Pretibial myxoedema
o Immobility and dependency
o Dependency oedema
o Paralysis
Secondary Lymphedema

12. Grade (Brunner) Latent I. II. III.
CLINICAL FEATURES
 Edema Not apparent Pits on pressure Not pits on
pressure
Irreversible
 Upon Limb Elevation -- Largely or
completely
disappears
Not significantly
reduce
Irreversible
 Fibrosis No clinical evidence Moderate to severe Severe
fibrosclerosis
Excess interstitial
fluid and
histological
abnormalities in
lymphatics and
lymph nodes
Positive
stemmer’s sign
Lymphostatic
elephantiasis
5 Clinical Staging

13. • ‘Buffalo hump’ on
the dorsum of the
foot
• Loss of the
normal
perimalleolar
shape, resulting in
a “tree trunk”
pattern
6 Clinical Approach
Signs and Symptoms
• Characteristically
involves the foot
• Initially pitting
edema and  on
limb elevation
• ‘Square toes’ • ‘Stemmer’s sign’
skin on the
dorsum of the
toes cannot be
pinched because
of subcutaneous
fibrosis.

14. • Skin changes
• Hyperkeratosis, lichenification, and
development of peau d’orange. ‘Pigskin’
• Chronic eczema, fungal infection of the skin
(dermatophytosis) and nails (onychomycosis),
fissuring, verrucae and papillae (warts) are
frequently seen in advanced disease.
• Ulceration is unusual<< higher degree of
hydration and elasticity
• Lymphorrhea
• Other symptoms
• Pain per se rare but mainly heaviness of limb,
causing immobility and debility

15. 1. Infections: proceeds and precipitates the lymphedema
• Recurrent cellulitis: good substrate and less host immune response
2. Malnutrition and immunodeficiency:
• Especially in lymphangiectasia with protein-losing enteropathy or
chylous ascites or chylothorax
3. Malignancies:
• That appear with increased frequency in lymphedematous limbs
include
 Lymphangiosarcoma
 Kaposi’s sarcoma,
 Squamous cell carcinoma,
 Malignant lymphoma, and
 Melanoma.
Complications

16. • Lymphangiosarcoma
 After long-standing secondary lymphedema,
 Is a rare malignant disease that frequently results in limb loss or even death.
 Is manifested as multicentric lesions with bluish nodules, sclerotic plaques, or bullous
changes.
 Originally described in postmastectomy oedema (stewart–treves’ syndrome) and
affects around 0.5% of patients at a mean onset of 10 years.
 Confirmed by skin biopsy.
 Amputation offers the best chance of survival

17. Differential Diagnosis of The Swollen Limb
Lymphatic Venous Arterial Non-lymphatic
Non-Vascular
Systemic
Local
Cardiac /Hepatic /Renal
Hypoproteinemia
Drugs /Allergic
Obesity
Trauma/ Hematoma
Tumors- bony/soft tissue
Ruptured baker cyst
Myositis ossificans
AV malformations
Aneurysms
DVT
CVI
Lymphedema

18. NOTE: First rule out systemic and ominous local causes
-- if there is a severe, atypical, complicated swollen limb, then only investigations are done
• ‘Routine’ tests:
CBC,
RFT, LFT, Urine analysis
TFT,
ECG, 2D Echo,
Blood smear for microfilariae,
Chest radiograph and Ultrasound/Doppler.
• Specific Investigations
Lymphoscintigraphy
Lymphangiography
MRI/ CT
Investigations

19. Lymphoscintigraphy
Radioactive technetium-labelled albumin or antimony trisulfide are injected into an
interdigital web space and specifically taken up by lymphatics, and serial radiographs are
taken with a gamma camera.

20. Interpretation Of Lymphoscintigraphy
It is functional assessment
A. Normal study: uptake at groin
nodes (1hr), bladder, liver (3hr)
B. Primary disease: none
C. Lymphedema: dermal backflow,
no node uptake

21. • Anatomical assessment:
used primarily before
reconstructive lymphatic
surgery.
• Done by the direct
injection of iodine-based,
lipid-soluble agents into
the subcutaneous
lymphatics,
• Has technically difficult;
unpleasant for the patient;
may cause further
lymphatic injury---->
largely, it has become
obsolete
Lymphangiography

22. • A single, axial slice through the mid-calf
Lymphoedema (coarse, non-enhancing, reticular ‘honeycomb’ pattern
in an enlarged subcutaneous compartment),
Venous oedema (increased volume of the muscular compartment) and
Lipoedema (increased subcutaneous fat).
• Also be used to exclude pelvic or abdominal mass lesions.
• Also be used to monitor response to treatment through serial
measurements
• MRI corroborated with scintigraphy for surgery
CT & MRI

23. • The goals of treatment are to
1. Reduce swelling
2. Prevent the development of complications.
• The standard of care for lymphedema is commonly referred as complex
decongestive therapy (CDT).
• It is the combination of four components and two phases.
The components are:
1. Skin care,
2. Manual lymphatic drainage (MLD) and
3. Multilayer lymphedema bandaging (MLLB), and
4. Exercises.
Phases are
1. First, short intensive period: therapist-led care (weeks)
2. The second is a maintenance phase: self-care regime with occasional professional
intervention. (lifelong)
Management: Non Operative7

24. Skin Care
Thorough limb wash Careful Skin drying Use of Moisturizer
 For marked hyperkeratosis: keratolytics (5% salicylic acid)
 Fungal infections : 3% benzoic acid oint, powders
 At risk sites of maceration : an antiseptic agent such as eosin
 Lymphorrhoea: emollients, elevation, compression and sometimes cautery
under anaesthetic

25. • .
Manual Lymphatic Drainage
apply gentle pressure to the skin
stretch the superficial
lymphatics
contraction and sequestration of
lymphatic vessels
Evacuation of fluid and protein
from the interstitial space
Segmental + sequential manner: distal to
proximal
https://www.youtube.com/watch?v=X7oM_vi5Umw

26. • The goal is to create an Internal Pump-like Action.
• Two different types of pressures are produced by the bandaging:
(1) the low resting pressure (20 to 30 mm Hg), which is the result of short-stretch bandages
on the patient during resting; and
(2) the high working pressure, which is the result of short stretch bandages on the patient
during muscle contraction.
Multilayer Lymphedema Bandaging
(1) tubular bandage
lining
(2) under the cast padding
(3) multiple layers of short
stretch bandages
https://www.youtube.com/watch?v=7aQnRujbEZo

27. • The pressure exerted must be graduated (100% ankle/foot, 70% knee, 50% mid-thigh,
40% groin).
• Provided there is no underlying peripheral arterial disease, sustained pressure of 60 to 70
mm Hg has been suggested as a maximum upper limit to treat lymphedema.
• The patient should put the stocking on first thing in the morning before rising
• Use of Multichember pneumatic compression – new thing
Compression Garments and Their Associated Pressure
Type Garment Pressure
Over the counter 7-15 mm Hg. Not graduated
Anti-embolism 15-20 mm Hg. Graduated
Chronic venous insufficiency or
lymphedema
20-30, 30-40, 40-50, and 50-60 mm
Hg
Patients with comorbidities 20-30 mm Hg or less
Upper extremity 20-30 mm Hg
Lower limbs with recalcitrant chronic
lymphedema
30–40 mm Hg

28. • Lymph formation  directly proportional to arterial
inflow and 40% of lymph is formed within skeletal
muscle.
• Exercise routines should include combinations of
lymphedema remedial exercises (e.g., Active,
Repetitive, Nonresistive Motion of the involved
body part) along with three main types of exercise
(i.e., AEROBIC, STRENGTH, and FLEXIBILITY).
• How it works….
Pumping action of muscles
Intrathoracic negative pressure
Weight reduction
Exercise

29.  Operations fall into three categories:
1. Reconstructive or Bypass Procedures,
2. Liposuction
3. Resection or Reduction Procedures.
If reconstruction possible … can be offered early in the course
If planned for resection… should be last option
Surgical intervention should be considered only after a trial of
conservative therapy for at least 6 months
Management: Operative

30. (I) Autologous Lymphatic Grafting
Bypass Surgeries
(1) Free Transfer (2) Transposition

31. (II) Lymphovenous Anastomosis
Techniques of lymphatic reconstruction
with interposition vein graft (A) or
lymphovenous anastomosis (B).
Technique of invagination of multiple
lymphatics into an interposition vein
graft: lymphatic-venous-lymphatic
anastomosis (C).

32. • The rationale: chronic lymphedema causes hypertrophy of the subcutaneous fat.
• Removal of the hypertrophied and edematous adipose tissue is performed through 3-mm-
long incisions (20 to 30 incisions) with vacuum aspiration.
• Liposuction can be useful in patients with no functioning lymphatics, but in others, the
destruction of functioning lymphatics and worsening of the edema are possible
Liposuction
https://www.plasticsurgery.org/cosmetic-procedures/liposuction/animation

33. Reduction Surgeries
Sistrunk
A wedge of skin and subcutaneous tissue is excised and
the wound closed primarily.

34. Homan’s
First, skin flaps are elevated, and then subcutaneous tissue is
excised from beneath the flaps, which are then trimmed to size to
accommodate the reduced girth of the limb and closed primarily.

35. Thompson’s
This is a modification of the Homans’ procedure aimed to create new lymphatic
connections between the superficial and deep systems. One skin flap is
denuded (shaved of epidermis), sutured to the deep fascia and buried beneath
the second skin flap (the so-called ‘buried dermal flap’)

36. Charles Procedure
This operation was initially designed for filariasis and involved excision
of all of the skin and subcutaneous tissues down to the deep fascia,
with coverage using split-skin grafts

37. Therapeutic Approach Based on Clinical Stages
Stage I Stage II Stage III
DURATION 2-3 weeks 3-4 weeks 4-6 weeks
Phase I
(Decongestion)
 MLD 1-2 × day
 Short-stretch bandages
 Skin care
 Remedial exercises
 MLD 2 × day
 Short-stretch bandages
 Skin care
 Remedial exercises
 MLD 2-3× day
 Short-stretch bandages
 Skin care
 Remedial exercises
Phase II
(Maintenance)
 MLD as needed
 Compression garments
 Skin care
 Remedial exercises
 MLD as needed 1-2×
per week
 Compression garments
 Skin care
 Remedial exercises
 MLD as needed 1-2×
per week
 Compression garments
 Skin care
 Remedial exercises
Repeating phase I (1-2×) Repeating phase I (3-4×)
Surgery may be indicated
Summary