This document discusses lymphatic disorders and lymphoedema. It begins with an introduction to the lymphatic system and its functions. It then discusses causes of lymphatic swelling including acute lymphangitis, filariasis, and elephantiasis. It defines primary and secondary lymphoedema and covers the clinical features, investigations and treatments for lymphoedema including manual lymphatic drainage, compression garments, and surgery. Surgical procedures for lymphoedema including bypass operations, liposuction, and limb reduction techniques are described. The document concludes by discussing chylous ascites, chylothorax, and chyluria.

1. LYMPHATIC DISORDERS
DR.SUNIL KUMAR
ASSISTANT PROFESSOR
DEPT.OF GEN.SURGERY
MNR MEDICAL COLLEGE SANGAREDDY
25-03-2019

2. • The main functions of the lymphatic system
• The 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.

4. • 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.

5. ANATOMY AND PHYSIOLOGY OF THE
LYMPHATIC SYSTEM
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.

6. • 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.

7. 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.

9. 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.

10. The severity of unilateral limb lymphoedema
can be classified as:
• mild: <20 per cent excess limb volume;
• moderate: 20–40 per cent excess limb volume;
• severe: >40 per cent excess limb volume.

13. 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 (Tables 58.1
and 58.2).

22. SECONDARY LYMPHOEDEMA
• This is the most common form of lymphoedema.
• There are several well-recognised causes including
infection, inflammation, neoplasia and trauma (Table
58.6).

26. 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 nematode Wucheria bancrofti, whose
only host is man, is responsible for 90 % of cases and
is spread by the mosquito.

27. • 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.

29. 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.

31. Miscellaneous conditions
• Rheumatoid and psoriatic arthritis (chronic
inflammation and lymph node fibrosis), contact
dermatitis, snake and insect bites, and
retroperitoneal fibrosis are all rare but well-
documented causes of lymphoedema.
• Pretibial myxoedema is due to the obliteration of
initial lymphatics by mucin.

32. CONDITIONS MIMICKING LYMPHOEDEMA
Factitious lymphoedema
• This is caused by application of a tourniquet (a ‘rut’
and sharp cut-off is seen on examination) or ‘hysterical’
disuse in patients with psychological and psychiatric
problems.
Immobility
• Generalised or localised immobility of any cause leads
to chronic limb swelling that can be misdiagnosed as
lymphoedema, for example the elderly person who
spends all day (and sometimes all night) sitting in a
chair (armchair legs), the hemiplegic stroke patient and
the young patient with multiple sclerosis.

33. LIPOEDEMA
• This presents almost exclusively in women and
comprises bilateral, usually symmetrical,
enlargement of the legs and, sometimes, the lower
half of the body because of the abnormal deposition
of fat.

35. INVESTIGATION OF LYMPHOEDEMA
Lymphangiography
• Direct lymphangiography involves the injection of
contrast medium into a peripheral lymphatic vessel
and subsequent radiographic visualisation of the
vessels and nodes.
• It remains the ‘gold standard’ for showing structural
abnormalities of larger lymphatics and nodes (Figure
58.9).

40. ISOTOPE LYMPHOSCINTIGRAPHY
• This has largely replaced lymphangiography as the
primary diagnostic technique in cases of clinical
uncertainty.
• Radioactive technetium-labelled protein or colloid
particles are injected into an interdigital web space
and specifically taken up by lymphatics, and serial
radiographs are taken with a gamma camera.

41. • The technique provides a qualitative measure of
lymphatic function rather than quantitative function
or anatomical detail.
• Quantitative lymphoscintigraphy is performed using
a dynamic (exercise) component in addition to the
static test and provides information on lymphatic
transport.

42. Computed tomography
• A single, axial computed tomography (CT) slice
through the midcalf has been proposed as a useful
diagnostic test for 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).
• CT can also be used to exclude pelvic or abdominal
mass lesions.

43. Magnetic resonance imaging
• Magnetic resonance imaging (MRI) can provide clear
images of lymphatic channels and lymph nodes, and
can be useful in the assessment of patients with
lymphatic hyperplasia.
• MRI can also distinguish venous and lymphatic
causes of a swollen limb, and detect tumours that
may be causing lymphatic obstruction.
• Ultrasound

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

49. 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.

50. • 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.

51. • 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.

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

53. 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).

54. • More recently, direct lymphaticovenular anastomosis
(LVA) has been carried out on vessels of 0.3–0.8 mm
diameter using supermicrosurgical techniques.

55. 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.

56. • 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.

57. 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.

58. 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.

59. Homans
• 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
(Figure 58.11).
• The main complication is skin flap necrosis.

61. • 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.

62. 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’)
(Figure 58.12).

64. • 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.

65. 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 (Figure
58.13).
• 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.

67. Chylous ascites and chylothorax
• These are associated with megalymphatics.
• The diagnosis may be obvious if accompanied by
lymphoedema and lymphangioma.
• However, some patients develop chylous ascites
and/or chylothorax in isolation, in which case the
diagnosis can be confirmed by aspiration and the
identification of chylomicrons in the aspirate.
• Cytology for malignant cells should also be carried
out.

68. • A CT scan may show enlarged lymph nodes and CT
with guided biopsy, laparoscopy or even laparotomy
and biopsy may be necessary to exclude lymphoma
or other malignancy.
• Lymphangiography may indicate the site of a
lymphatic fistula that can be surgically ligated.
• Even if no localised lesion is identified, it may be
possible to control leakage at laparotomy or even
remove a segment of affected bowel.

69. Chyluria
• Filariasis is the most common cause, with chyluria
occurring in 1–2 per cent of cases 10–20 years after
initial infestation.
• It usually presents as painless passage of milky white
urine, particularly after a fatty meal.
• The chyle may clot, leading to renal colic, and
hypoproteinaemia may result.

70. • Chyluria may also be caused by ascariasis, malaria,
tumour and tuberculosis.
• Intravenous urography and/or lymphangiography will
often demonstrate the lymphourinary fistula.
• Treatment includes a low-fat and highprotein diet,
increased oral fluids to prevent clot colic, and
laparotomy and ligation of the dilated lymphatics.

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