LYMPHATIC SYSTEM

Function
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.
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.
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 bacteremia or septicemia. 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. The
part should be rested to reduce lymphatic drainage and elevated to
reduce swelling, and the patient should be treated with intravenous
antibiotics based upon actual or suspected sensitivities.
 Failure to improve within 48 hours suggests inappropriate antibiotic
therapy, the presence of undrained pus or the presence of an
underlying systemic disorder (malignancy, immunodeficiency). The
lymphatic damage caused by acute lymphangitis may lead to recurrent
attacks of infection and lymphoedema; patients with lymphoedema are
prone to so-called acute inflammatory episodes
Lymphedema

is a condition
characterized by
swelling of one or
more extremities
because of lymphatic
insufficiency.
 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
 Lymphedema may be idiopathic (primary lymphedema) or may be caused by acquired
insufficiency due to infections, obstructions, or surgical destruction of the lymphatics
(secondary lymphedema).
Primary lymphedema. Three types of primary lymphedema are distinguished by age of
onset.
(1) Congenital lymphedema is present at birth or occurs early in infancy.
(a) It accounts for fewer than 10% of primary lymphedema cases.
(b) Lymphedema that is both congenital and hereditary is known as Milroy's disease.
(2) Lymphedema praecox occurs at any time from puberty until the end of the third
decade.
(a) Most cases of primary lymphedema are of this type.
(b) It is three times more common in women than in men.
(3) Lymphedema tarda occurs after age 30.
Secondary lymphedema is due to obstruction from a variety of causes,
including infection, parasites, mechanical injury (including surgery),
postphlebitic syndrome, and neoplasms.
(1) In developed countries, the most common causes are obstruction
by malignancies, postsurgical lymphedema (e.g., after mastectomy),
and lymphatic destruction from therapeutic radiation.
 Filariasis
This is the most common cause of lymphedema worldwide, affecting
up to 100 million individuals. It is particularly prevalent in Africa, India
and South America where 5–10% 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. 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. Proximal lymphatics become grossly
dilated with adult parasites.
The degree of oedema is often massive, in which case it is termed
elephantiasis Immature parasites (microfilariae) enter the blood at night
and can be identified on a blood smear, in a centrifuged specimen of urine
or in lymph itself. A complement fixation test is also available and is
positive in present or past infection. Eosinophilia is usually present.
 Diethylcarbamazine destroys the parasites but does not reverse the
lymphatic changes, although there may be some regression over time.
Once the infection has been cleared, treatment is as for primary
lymphedema. Public health measures to reduce mosquito breeding,
protective clothing and mosquito netting may be usefully employed to
combat the condition.
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.
Plantar edema develops in childhood and rapidly spreads proximally.
The condition is prevented and its progression is slowed by the wearing
of shoes.
Bacterial infection

Lymphangitis and lymphadenitis can cause lymphatic destruction

that predisposes to lymphedema complicated by further
acute inflammatory episodes. Interestingly, in such patients,
lymphangiography has revealed abnormalities in the contralateral,
unaffected limb, suggesting an underlying, possibly
inherited, susceptibility. Lymphatic and lymph node destruction
by tuberculosis is also a well-recognised cause of lymphedema,
especially in resource-poor countries.
 Malignancy and its treatment
Treatment (surgery, radiotherapy) for
breast carcinoma is the most common cause
of lymphedema in resource-rich countries,
but is decreasing in incidence as surgery
becomes more Conservative. Lymphoma may
present with lymphedema, as may
malignancy of the pelvic organs and external
genitalia. Kaposi’s sarcoma, developing in the
course of human immunodeficiency virus
(HIV)-related illness, may cause lymphatic
obstruction and is a growing cause of
lymphedema in certain parts of the world.
Malignancies associated with lymphedema

●● Lymphangiosarcoma (Stewart–Treves’ syndrome)

●● Kaposi’s sarcoma (human immunodeficiency virus)
●● Squamous cell carcinoma
●● Liposarcoma
●● Malignant melanoma
●● Malignant fibrous histiocytoma
●● Basal cell carcinoma
●● Lymphoma
Trauma

It is not unusual for patients to develop chronic localised or

generalised swelling following trauma. The etiology is often
multifactorial and includes disuse, venous thrombosis and lymphatic
injury or destruction. Degloving injuries and burns are
particularly likely to disrupt dermal lymphatics. Tenosynovitis
can also be associated with localised subcutaneous lymphedema,
which can be a cause of troublesome persistent swelling
following ankle and wrist ‘sprains’ and repetitive strain injury.
 Age of onset
Lymphedema congenita (onset at or within 2 years of birth) is more
common in males and is more likely to be bilateral and involve the whole
leg. Lymphedema praecox (onset from 2 to 35 years) is three times more
common in females, has a peak incidence shortly after menarche, is three
times more likely to be unilateral than bilateral and usually only extends to
the knee. Lymphedema tarda develops, by definition, after the age of 35
years and is often associated with obesity, with lymph nodes being
replaced with fibrofatty tissue. The cause is unknown. Lymphedema
developing for the first time after 50 years should prompt a thorough
search for underlying (pelvic, genitalia) malignancy. It is worth noting that,
in such patients, lymphedema often commences proximally in the thigh
rather than distally
 Lymphedema and chronic venous
insufficiency
It is important to appreciate the relationship between lymphedema and
CVI as both conditions are relatively common and so often coexist in the
same patient, and it can be difficult to unravel which components of the
patient’s symptom complex are caused by each. There is no doubt that
superficial venous thrombophlebitis (SVT) and DVT can both lead to
lymphatic destruction and secondary lymphedema, especially if recurrent.
Lymphedema is an important contributor to the swelling of the post-
thrombotic syndrome. It has also been suggested that lymphedema can
predispose to DVT, and possible SVT, through immobility and acute
inflammatory episodes. Certainly, tests of venous function (duplex
ultrasonography, plethysmography) are frequently abnormal in patients with
lymphedema.
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
lymphedema. Pretibial myxedema is due to the obliteration of initial
lymphatics by mucin
Diagnosis of lymphedema is usually made clinically.
a. The history characteristically includes edema, which begins at the
foot and ankle and progresses proximally. Progression is slow, usually
over the course of several months.
b. Physical examination. Lymphedema has no dark brawny edema or
ulceration of the skin. Whereas the swelling secondary to venous
disease usually starts at the ankle, lymphedema usually involves the
dorsum of the foot.
c. Laboratory tests. The diagnosis can be proven by lymphangiography,
but this test is not done routinely because it is difficult and hazardous.
Isotope lymphoscintigraphy
Isotope lymphoscintigraphy 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. 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.
Computed tomography
A single, axial computed tomography (CT) slice through
the mid-calf has been proposed as a useful diagnostic test for
lymphedema (coarse, non-enhancing, reticular ‘honeycomb’
pattern in an enlarged subcutaneous compartment), venous
edema (increased volume of the muscular compartment)
and lipedema (increased subcutaneous fat). CT can also be
used to exclude pelvic or abdominal mass lesions. It can also
be used to monitor response to treatment through serial measurements
of cross-sectional area and tissue density.
Magnetic resonance imaging

Non-contrast magnetic resonance imaging (MRI) is also

useful in identifying the classical circumferential reticular
pattern (honeycomb) within the epifascial compartment

Ultrasound can provide useful information about venous

function including DVT and venous abnormalities.
MANAGEMENT OF LYMPHOEDEMA
Relief of pain.
50% of patients with lymphedema complain of significant pain. The
pain is usually multifactorial and its severity and underlying cause(s)
will vary depending on the etiology of the lymphedema. Treatment
involves the considered use of non-opioid and opioid analgesics,
corticosteroids, tricyclic antidepressants, muscle relaxants,
antiepileptics, nerve blocks, physiotherapy and adjuvant anticancer
therapies (chemo-, radio- and hormonal therapy), as well as measures
to reduce swelling, if possible. In patients with noncancer- Related
lymphedema, the best way to reduce pain is to control swelling and
prevent the development of complications.
MANAGEMENT OF LYMPHOEDEMA
Control of swelling
Physical therapy for lymphoedema, comprising bed rest, elevation,
bandaging, compression garments, massage and exercises.
The current preferred term is decongestive lymphoedema therapy
(DLT), which comprises two phases. The first is a short intensive period
of therapist-led care and the second is a maintenance phase in which
the patient uses a self-care regime with occasional professional
intervention. The intensive phase comprises skin care, manual
lymphatic drainage (MLD) and multilayer lymphoedema bandaging
(MLLB), and exercises.
Multilayer lymphedema bandaging
and compression garments
Elastic bandages provide compression, produce a sustained high
resting pressure and ‘follow in’ as limb swelling reduces. However, the
sub-bandage pressure does not alter greatly in response to changes in
limb circumference consequent upon muscular activity and posture. By
contrast, short-stretch bandages exert support through the production
of a semi-rigid casing where the resting pressure is low, but changes
quite markedly in response to movement and posture. This pressure
variation produces a massaging effect within the limb and stimulates
lymph flow. Whether the aim is to provide support or compression, the
pressure exerted must be graduated (100% ankle/foot, 70% knee, 50%
mid-thigh, 40% groin).
Effects of multilayer lymphedema
bandaging
●● Reduces edema
●● Restores shape to the affected area
●● Reduces skin changes (hyperkeratosis, papillomatosis)
●● Eliminates lymphorrhea
●● Supports inelastic skin
●● Softens subcutaneous tissues
 Exercise
Lymph formation is directly proportional to arterial inflow
and 40% of lymph is formed within skeletal muscle. Vigorous
exercise, especially if it is anaerobic and isometric, will tend
to exacerbate lymphedema and patients should be advised to
avoid prolonged static activities, for example carrying heavy
shopping bags or prolonged standing. In contrast, slow, rhythmic
isotonic movements (e.g. swimming) and massage will
increase venous and lymphatic return through the production
of movement between skin and underlying tissues (essential
to the filling of initial lymphatics) and augmentation of the
muscle pumps. Exercise also helps to maintain joint mobility.
Skin care
The patient must be carefully educated in the principles and
practice of skin care. The patient should inspect the affected
skin daily, with special attention paid to skinfolds, where
maceration may occur.
 Apart from lymphangiosarcoma, acute inflammatory episodes are
probably the most serious complications of lymphedema and frequently
lead to emergency hospital admission. About 25% of primary and 5% of
secondary lymphedema patients are affected. Acute inflammatory
episodes start rapidly, often without warning or a precipitating event, with
tingling, pain and redness of the limb. Patients feel ‘viral’ and severe
attacks can lead to the rapid onset of fever, rigors, headache, vomiting and
delirium. Patients who have suffered previous attacks can usually predict
the onset and many learn to carry antibiotics with them and self-medicate
at the first hint of trouble. This may stave off a full-blown attack and
prevent the further lymphatic injury that each acute inflammatory
episode causes. It is rarely possible to isolate a responsible bacterium, but
the majority are presumed to be caused by group A β-hemolytic
streptococci and/or staphylococci.
 The diagnosis is usually obvious but dermatitis, thrombophlebitis and
DVT are in the differential diagnosis. Oral amoxicillin is the treatment of
choice with erythromycin or clarithromycin in those with penicillin allergy.
Flucloxacillin should be added for those with evidence of S. aureus
infection (folliculitis, crusted dermatitis). Oral clindamycin is a second-line
agent for those with failure to respond to initial therapy. Hospital
admission is required for patients with: signs of septicemia; continuing or
deteriorating systemic signs after 48 hours of antibiotic treament;
unresolving or deterioratin local signs despite trials of first- and second-
line antibiotics. Intravenous amoxycillin or benzyl penicillin with
clindamycin in penicillin-allergic patients or as second-line therapy is most
commonly recommended. Bed rest will reduce lymphatic drainage and
the spread of infection, elevation will reduce the edema and heparin
prophylaxis will reduce the risk of DVT
Surgery

Only a small minority of patients with lymphoedema benefit

from surgery. Operations fall into three categories: bypass procedures,
liposuction and reduction procedures.
 Bypass procedures
The rare patient with proximal ilioinguinal lymphatic obstruction
and normal distal lymphatic channels might benefit, at least
in theory, from lymphatic bypass. More recently, direct
lymphaticovenular anastomosis (LVA) has been carried out
on vessels of 0.3–0.8 mm diameter using super-microsurgical
techniques. The procedures are technically demanding and
not without morbidity. They are more often attempted in the
upper limb following lymph node resection or radiotherapy for
breast cancer. The outcomes are best in patients with earlier
stages of lymphedema
Liposuction

Liposuction has been used in the treatment of chronic lymphedema.

It is usually reserved for patients who have progressed
to non-pitting edema. Case series reported thus far have
shown promising results with more than 100% reduction in
limb edema volume, which can be maintained by ongoing
use of compression hosiery for at least 1 year. While liposuction
appears to be safe, results of long-term efficacy and effects
on the incidence of future lymphedema complications (e.g.
infection) are awaited.
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: SISTRUNK; HOMANS; THOMPSON;
CHARLES
 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.

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. The main complication is skin flap necrosis. There
must be at least 6 months between operations on the medial and lateral
sides of the limb and the flaps must not pass the midline.
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’)

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.