Lymphatic disorders

1. LYMPHATIC
DISORDERS
DR MOHAMMAD ASAD JUNEJA
ASSISTANT PROFESSOR
DEPARTMENT OF GENERAL SURGERY
GMERS MEDICAL COLLEGE, GOTRI, VADODARA

2. The main functions of the lymphatic system
The various causes of limb swelling
• The aetiology, clinical features, investigations and treatment of lymphoedema of the
lymphatic system

3. INTRODUCTION
The lymphatic system was first described by Erasistratus in Alexandria more than 2000 years ago.
William Hunter, in the late eighteenth century, was the first to describe the function of the lymphatic
system.
• Starling’s pioneering work on the hydrostatic and haemodynamic forces controlling the movement
of fluid across the capillary provided further insights into the function of the lymphatics.
• However, there is much about the lymphatic system that is not understood and debate continues
over the precise aetiology of the most common abnormality of the system, namely lymphoedema.

4. ANATOMY AND PHYSIOLOGY OF THE LYMPHATIC
SYSTEM

7. FUNCTIONS
The principal function of the lymphatics is the return of protein rich fluid to the circulation
through the lymphaticovenous junctions in the jugular area.
• Thus, water, electrolytes, low molecular weight moieties (polypeptides, cytokines, growth
factors) and macromolecules (fibrinogen, albumin, globulins, coagulation and fibrinolytic
factors) from the interstitial fluid (ISF) return to the circulation via the lymphatics.

8. Intestinal lymph (chyle) transports cholesterol, long- chain fatty acids, triglycerides and the
fat-soluble vitamins (A, D, E and K) directly to the circulation, bypassing the liver.
• Lymphocytes and other immune cells also circulate within the lymphatic system.

9. ACUTE INFLAMMATION OF THE LYMPHATICS
Acute lymphangitis is an infection, often caused by Streptococcus pyogenes or
Staphylococcus aureus, which spreads to the draining lymphatics and lymph nodes
(lymphadenitis) where an abscess may form.
Eventually this may progress to bacteraemia or septicaemia.
The normal signs of infection (rubor, calor, dolor) are present and a red streak is seen in the
skin along the line of the inflamed lymphatic (Figure 58.1).
• The part should be rested to reduce lymphatic drainage and elevated to reduce swelling,
and the patient should be treated with intravenous antibiotics.

11. LYMPHOEDEMA
Definition
• Lymphoedema may be defined as abnormal limb swelling caused by the accumulation
of increased amounts of high protein ISF (interstitial fluid) secondary to
defective lymphatic drainage in the presence of (near) normal net capillary filtration.

12. •The severity of unilateral limb lymphoedema can be classified as:
1. mild: <20 per cent excess limb volume;
2. moderate: 20-40 per cent excess limb volume
3. severe: >40 per cent excess limb volume.

14. SYMPTOMS FREQUENTLY EXPERIENCED BY
PATIENTS WITH LYMPHOEDEMA
Swelling, clothing or jewellery becoming tighter
Constant dull ache, even severe pain
Burning and bursting sensations
General tiredness and debility
Sensitivity to heat
‘Pins and needles’
Cramp
Skin problems, including flakiness, weeping, excoriation and breakdown
Immobility, leading to obesity and muscle wasting
Backache and joint problems
Athlete’s foot
• Acute infective episodes

15. WHAT EVERY PATIENTWITH LYMPHOEDEMA
SHOULD RECEIVE
An explanation of why the limb is swollen and the underlying cause
Guidance on skin hygiene and care and the avoidance of acute infective episodes
Antifungal prophylactic therapy to prevent athlete’s foot
Rapid access to antibiotic therapy if necessary, hospital admission for acute infective episodes
Appropriate instructions regarding exercise therapy
Manual lymphatic drainage (MLD)
Multilayer lymphoedema bandaging (MLLB)
Compression garments and, if appropriate, specialised footwear
Advice on diet
• Access to support services and networks

16. CLASSIFICATION
1. Primary lymphoedema, in which the cause is unknown (or at least uncertain and
unproven); it is thought to be caused by ‘congenital lymphatic dysplasia’.
2. Secondary or acquired lymphoedema, in which there is a clear underlying cause.
• Primary lymphoedema is usually further subdivided on the basis of the presence of family
history, age of onset and lymphangiographic findings

17. AETIOLOGICAL CLASSIFICATION

18. PRIMARY LYMPHOEDEMA
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)

20. SECONDARY LYMPHOEDEMA
•This is the most common form of lymphoedema.
• There are several well-recognised causes including infection, inflammation, neoplasia and
trauma.

21. CLASSIFICATION OF CAUSES OF 2° LYMPHOEDEMA

23. FILARIASIS
This is the most common cause of lymphoedema worldwide, affecting up to 100 million individuals.
It is particularly prevalent in Africa, India and South America where 5-10 per cent of the population may
be affected.
• The viviparous nematodeWucheria bancrofti, whose only host is man, is responsible for 90% of cases
and is spread by the mosquito.
• The disease is associated with poor sanitation.
• The parasite enters lymphatics from the blood and lodges in lymph nodes, where it causes fibrosis and
obstruction, due partly to direct physical damage and partly to the immune response of the
host.

25. ENDEMIC ELEPHANTIASIS (PODOCONIOSIS)
This is common in the tropics and affects more than 500 000 people in Africa.
• The barefoot cultivation of soil composed of alkaline volcanic rocks leads to
destruction of the peripheral lymphatics by particles of silica, which can be seen
in macrophages in draining lymph nodes.

27. INVESTIGATIONS
• Lymphangiograpghy(gold standard)
• Isotope Lymphoscintigraphy
• CommutedTomography –honeycomb pattern
• Magnetic resonance imaging
• Ultrasound

28. MANAGEMENT OF LYMPHOEDEMA
Relief of pain
• Control of swelling
• Skin care
• Manual lymphatic drainage
• Multilayer lymphoedema bandaging and compression garments
• Exercise

29. DRUGS
There are considerable, and scientifically inexplicable differences in the use of specific drugs
for venous disease and lymphoedema between different countries.
The benzpyrones are a group of several thousand naturally occurring substances, of
which the flavonoids have received the most attention.
• Diethylcarbamazapine 100mg tid for 12 days every 6 month.

30. Enthusiasts will argue that a number of clinical trials have shown benefit from these
compounds, which are purported to reduce capillary permeability, improve
microcirculatory perfusion, stimulate interstitial macrophage proteolysis, reduce
erythrocyte and platelet aggregation, scavenge free radicals and exert an anti-inflammatory
effect.
• In the UK, oxerutins (Paroven®) are the only such drugs licensed for venous disease and
none has a license for lymphoedema.

31. • Diuretics are of no value in pure lymphoedema.
• Their chronic use is associated with side effects, including electrolyte disturbance, and
should be avoided.
• • With increasing understanding of lymphangiogenesis pathways there is hope that
specific pharmacological targets or gene therapy may become available in the future but
this remains in the very early stages at present.

32. SURGERIES
• Only a small minority of patients with lymphoedema benefit from surgery.
• Operations fall into three categories:
bypass procedures
1. liposuction and
2. reduction procedures.

33. BYPASS PROCEDURES
The rare patient with proximal ilioinguinal lymphatic obstruction and normal distal
lymphatic channels might benefit, at least in theory, from lymphatic bypass.
• A number of methods have been described including the omental pedicle, the skin
bridge (Gillies), anastomosing lymph nodes to veins (Neibulowitz) and the ileal
mucosal patch (Kinmonth).
• More recently, direct lymphaticovenular anastomosis (LVA) has been carried out on
vessels of 0.3-0.8 mm diameter using supermicrosurgical techniques.

34. LIPOSUCTION
Liposuction has been used in the treament of chronic lymphoedema.
It is usually reserved for patients who have progressed to non-pitting oedema.
• Case series reported thus far have shown promising results with more than
100% reduction in limb oedema volume which can be maintained by ongoing
use of compression hosiery for at least one year.
• While liposuction appears to be safe, results of long- term efficacy and effects on the
incidence of future lymphoedema complications (e.g. Infection) are awaited.

35. LIMB REDUCTION PROCEDURES
These are indicated when a limb is so swollen that it interferes with mobility and
livelihood.
These operations are not ‘cosmetic’ in the sense that they do not create a normally
shaped leg and are usually associated with significant scarring.
• Four operations have been described.

36. SISTRUNK
• A wedge of skin and subcutaneous tissue is excised and the
wound closed primarily.
• • This is most commonly carried out to reduce the girth
of the thigh.
HOMAS
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.
This is the most satisfactory operation for the calf .
• The main complication is skin flap necrosis.
• There must be at least six months between operations
on the medial and lateral sides of the limb and the flaps
must not pass the midline.
• This procedure has also been used on the upper limb, but
is contraindicated in the presence of venous
obstruction or active malignancy.

38. THOMPSON
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’)
• This procedure has become less popular as
pilonidal sinus formation is common.
• The cosmetic result is no better than that
obtained with the Homans’ procedure and there
is no evidence that the buried flap establishes
any new lymphatic connections.
CHARLES
• 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
This leaves a very unsatisfactory cosmetic
result and graft failure is not uncommon.
• However, it does enable the surgeon to
reduce greatly the girth of a massively
swollen limb.

39. THOMAS CHARLES