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LYMPHATIC SYSTEM AND ITS
DISEASES

Dept. of Surgery

ANATOMY
A 1-way system that returns lymph via

vessels to the CVS.

Consists of:
Fluid, known as lymph
Vessels that transport lymph
Organs that contain lymphoid tissue (eg,

lymph nodes, spleen, and thymus)

Organ

Function

Lymph

Contains nutrients, oxygen, hormones, and fatty acids, as

well as toxins and cellular waste products, that are

transported to and from cellular tissues

Lymphatic vessels

Transport lymph from peripheral tissues to the veins of the

cardiovascular system

Lymph nodes

Monitors the composition of lymph, the location of pathogen

engulfment and eradication, the immunologic response, and

the regulation site

Spleen

Monitors the composition of blood components, the location

of pathogen engulfment and eradication, the immunologic

response, and the regulation site

Thymus

Serves as the site of T-lymphocyte maturation, development,

and control
Functions are :

Restoration of excess interstitial fluid and

proteins to the blood

Absorption of fats and fat-soluble vitamins from

the digestive system

Defense against invading organisms

LYMPH

Fluid derived from plasma.

Contains nutrients, oxygen, and hormones, as

well as toxins and cellular waste.

This fluid is removed by lymphatic vessels that

pass through lymph nodes.

As the lymph passes through the lymph nodes,

lymphocytes and monocytes enter it.
LYMPHATIC VESSELS

Are blind-ended tubes with thin endothelial

walls.

Coalesce to form larger meshlike vessels.
Eventually form 2 lymphatic ducts: the right

lymphatic duct and the thoracic duct.

Have 1-way valves to prevent any backflow.
LYMPH NODES

Bean-shaped structures

Filter the lymph before it rejoins the blood

stream.

Approximately 600-700, predominantly in the

neck, axillae, groin, mediastinum, and

mesenteries of the GI tract.

Constitute a main line of defense by hosting 2

types of immune protective cell lines, T and B

lymphocytes.

2 distinct regions, the cortex and the medulla.

The cortex contains follicles, which are collections

of lymphocytes.

Center of the follicles is called germinal centers

that has B-lymphocytes while the remaining cells

of the cortex are T-lymphocytes.

Vessels entering the lymph nodes are called

afferent lymphatic vessels and those exiting are

called efferent lymphatic vessels
LYMPHADENOPATHY

ETIOLOGY

Five broad etiologic categories :

An immune response to infective agents (eg,

bacteria, virus, fungus)

Inflammatory cells in infections involving the

lymph node

Metastasis

Malignancy of lymphocytes or macrophages (eg,

leukemia, lymphoma)

Storage disorders
PRESENTATION

Duration

Associated symptoms

Past illnesses, infections, local trauma, or bites.

Constitutional symptoms.

If recurrent infections, HIV must be considered.

Family and social history

PHYSICAL EXAMINATION

Complete general examination.

The skin and the soft tissue drained by the

enlarge node should be carefully examined.

The character of the lymph node should be noted.

Whether the lymphadenopathy is a local or a

general phenomenon.
GENERALIZED LYMPHADENOPATHY:

Upper respiratory tract infections (rhinovirus,

adenovirus, influenzavirus, parainfluenza virus,

respiratory syncytial virus)

Epstein-Barr virus (EBV)
Cytomegalovirus (CMV)
Varicella-zoster virus
Herpes simplex virus
Paramyxovirus
Coxsackieviruses A and B
Echovirus
Enterovirus
Human herpesvirus-6
Human immunodeficiency virus

WORK UP
LABORATORY STUDIES

Perform the least invasive test that provides the

most information.

CBC .

Serum LDH- to determine the turnover rate of

cells in the case of leukemia or lymphoma.

Tuberculin skin test.

Monospot and titers for EBV, CMV, catscratch

disease, or toxoplasmosis

IMAGING STUDIES

Chest radiography to assess for bacterial

pneumonias or tuberculosis, and hilar

adenopathy in the case of malignancy.

Ultrasonography.

CT scan.

18F-FDG PET.

FNAC

BIOPSY


LYMPHEDEMA

?Lymphatic obstruction

?Dec. lymphatic transport

?Stagnation of HMW proteins
? High protein edema

? High oncotic pressure in the interstitium

favors the accumulation of additional water.

? Massive dilatation of lymphatic vessels

? Inflammation

? Shrunken and hard lymph nodes

DERMATOLOGIC PATHOLOGY

The overlying skin becomes thickened and

displays the peau d'orange appearance.

Chronic lymphedema causes fissuring and

impairment of the epidermis, allowing bacteria to

enter and grow, and leading to lymphorrhea, the

leakage of lymph.

With chronic lymphedema, the development of

verrucous, cobblestone plaques, a condition

known as elephantiasis nostra verrucosa (ENV),

can occur.
ETIOLOGY

In primary lymphedema, there is congenital

hypoplasia or aplasia of the peripheral

lymphatics or valvular incompetence.

In secondary lymphedema, the lymphatic

drainage is blocked due to:

Recurrent attacks of lymphangitis

Malignancy

Obesity

Surgery
PRIMARY LYMPHEDEMA

A developmental abnormality of the lymphatic

system.

3 main types, distinguished by their age of onset:
1. Congenital lymphedema (Milroy disease)
2. Lymphedema praecox (Meige disease)
3. Lymphedema tarda
Involve the lower extremities almost exclusively.
CONGENITAL LYMPHEDEMA

10-25% of all primary lymphedema cases.

Autosomal-dominant.

Anaplastic lymphatic channels.

Manifests at birth or later, up to age 1 year.

F:M= 2:1.

LL:UL= 3:1

The edema is most commonly pitting and non

painful.

2/3 rd of patients have bilateral lymphedema.

May improve spontaneously with increasing age.

LYMPHEDEMA PRAECOX

Most common form of primary lymphedema (65-

80%).

Becomes clinically evident after birth and before

age 35 years.

F:M= 4:1

About 70% of cases are U\L.

A hypoplastic pattern, with the lymphatics

reduced in caliber and number.
LYMPHEDEMA TARDA

Manifests later in life, usually in persons older

than 35 years.

Caused by a defect in the lymphatic valves,

resulting in incompetent valve function.

Accounts for only 10% of cases.

ASSOCIATED CONDITIONS

Distichiasis lymphedema syndrome is

lymphedema associated with distichiasis (double

row of eyelashes).

Vertebral abnormalities, spinal arachnoid cysts,

hemangiomas, cleft palate, ptosis, short stature,

webbed neck, strabismus, thoracic duct

abnormalities, and microphthalmia.

Associated with yellow nail syndrome.
ASSOCIATED CONDITIONS

Other genetic syndromes and cutaneous

conditions associated with primary lymphedema

include the following:

Turner syndrome

Noonan syndrome

Klinefelter syndrome

Neurofibromatosis type 1

Hemangiomas

Xanthomatosis

Congenital absence of nails

SECONDARY LYMPHEDEMA
SECONDARY LYMPHEDEMA

Caused by an acquired defect in the lymphatic

system.

Associated with obesity, infection, neoplasm,

trauma, and therapeutic modalities.

SECONDARY LYMPHEDEMA

1.Filariasis
The most common cause of secondary

lymphedema.

Caused by a mosquito-borne nematode infection

with the parasite Wucheria bancrofti.

Commonly occurring in developing countries

around the world.

Results in permanent lymphedema of the limb.
SECONDARY LYMPHEDEMA

2. Malignancy and cancer treatment
Obstruction from metastatic cancer or primary

lymphoma or secondary to radical lymph node

dissection and excision.

The most commonly affected area is the axillary

region after mastectomy

Lymphedema can also be seen after regional

dissection of pelvic, para-aortic, and neck lymph

nodes.

SECONDARY LYMPHEDEMA

Other causes
Trauma
Varicose vein surgery
Congestive heart failure
Portal hypertension
Peripheral vascular surgery
Lipectomy
Burns
Burn scar excision
Insect bites
Extrinsic pressure
CLINICAL PRESENTATION

HISTORY

Chronic swelling of an extremity.
First noticed by the patient as an asymmetry or

increased circumference of an extremity.

As swelling slowly progresses, patients may have

difficulty fitting into clothing.

May cause fatigue due to the size and weight of the

extremity, embarrassment in public, and severe

impairment of daily activities.

Recurrent bacterial or fungal infections.
Fevers, chills, and generalized weakness.
History of recurrent episodes of cellulitis,

lymphangitis, fissuring, ulcerations, and/or verrucous

changes.
HISTORY

In primary lymphedema, the history of onset is more

typical. Associated with other anomalies and genetic

disorders.

In secondary lymphedema, the associated history is

based on the primary etiology.

If due to filariasis, the history should include travel or

habitation in an endemic area.

Other patients should have a clear history of a

neoplasm obstructing the lymphatic system, recurrent

episodes of lymphangitis and/or cellulitis, obesity,

trauma, or lymphedema resulting after surgery

and/or radiation therapy.

A recent history of varicose vein surgery also is

reported.

PHYSICAL EXAMINATION

The earliest symptom is nontender, pitting edema

progressing to a nonpitting one

Radial enlargement of the area.

Involvement of the distal extremities is followed

by proximal advancement.

Erythema and peau d'orange of skin.


Grade (Brunner)

Clinical features

Subclinical (latent)

There is excess interstitial fluid

and

histological abnormalities in

lymphatics

and lymph nodes, but no clinically

apparent lymphoedema

I

Oedema pits on pressure and

swelling

largely or completely disappears

on

elevation and bed rest

II

Oedema does not pit and does not

significantly reduce upon

elevation

III

Oedema is associated with

irreversible skin

changes, i.e. fibrosis, papillae
ELEPHANTIASIS NOSTRA VERRUCOSA

ENV is an area of cobble-stoned, hyperkeratotic,

papillomatous plaques most commonly seen on

the shins.

The plaques can be weepy or oozing a clear or

yellow fluid.

Fissuring, ulcerations, skin breakdown.

Superinfection is common and can manifest as

impetigo with yellow crusts.
EXAMINATION

A positive Stemmer sign (inability to pinch the

dorsal aspect of skin between the first and second

toes)

Patients with congenital lymphedema may also

present with recurrent cellulitis, papillomatosis,

large caliber leg veins, and upsloping "ski-jump"

toenails.

DIFFERENTIALS

Deep vein thrombosis
Hypoalbuminemia
Renal failure
Lipedema
Postoperative complications
Baker cyst
Idiopathic edema
Congestive heart failure
Idiopathic edema
Neurofibromatosis
Sclerema neonatorum
Features of lipoedema that help differentiate it from

lymphoedema

Occurs almost exclusively in women

Onset nearly always coincides with puberty

Nearly always bilateral and symmetrical

Involvement of trunk

The feet are not involved, leading to an inverse

shouldering effect at the malleoli

No pitting

No response to elevation or compression

No skin changes of lymphoedema (negative Stemmer's

sign)

MRI shows subcutanteous fat but no fluid

accumulation

WORKUP OF LYMPHEDEMA
Liver function, blood urea nitrogen

(BUN)/creatinine levels, and urinalysis results

should be checked if a renal or hepatic etiology is

suspected.

Specific markers should be checked if a neoplasm

is suspected.

CBC with differential should be checked if an

infectious etiology is being considered.

An indication for CT scanning or MRI is suspicion

of malignancy.

MRI is useful to show lymph trunk anatomy and

causes of obstructive secondary lymphedema.

Ultrasonography to evaluate the lymphatic and

venous systems.

Lymphangiography is now rarely used because of

the potential adverse effects.

Fluorescence microlymphography demonstrates a

lack of microlymphatics.

Lymphoscintigraphy.
TREATMENT OF LYMPHEDEMA

The goal is to restore function, reduce physical

and psychologic suffering, and prevent the

development of infection.

Initiate therapy as early as possible before

extensive, irreversible fibrosclerotic changes

occur in the interstitium.

Strict compliance is essential.

The majority of compliant patients can be treated

successfully with conservative measures
1.HYGIENE AND SKIN CARE

Appropriate skin care to prevent recurrent

cellulitis or lymphangitis.

Meticulous hygiene to remove keratinous debris

and bacteria.

Cleanse the skin regularly and dry thoroughly.

Regular inspection is necessary to identify any

open wounds or developing cellulitis.

2.PHYSICAL THERAPY AND COMPRESSION

The first-line treatment.

Aimed at improving lymphedema with manual

lymphatic drainage, massage, and exercise.

It advocates the use of compression stockings (at

a minimum of 40 mm Hg), multilayer bandaging,

or pneumatic pumps.

Encourage patients to lose weight, avoid minor

trauma, and avoid constrictive clothing that

might have a tourniquet effect.

Encourage elevation of the affected extremity

whenever possible, particularly at night.
3. SURGERY

Surgical treatment is palliative, not curative,

and it does not obviate the need for continued

medical therapy.

Surgical treatment is reserved for patients who

do not improve with conservative measures or for

cases in which the extremity is so large that it

impairs daily activities and prevents successful

conservative management.

Surgical procedures are classified as physiologic

or excisional.

PHYSIOLOGIC SURGERY

Physiologic procedures attempt to improve

lymphatic drainage. Multiple techniques have

been described, including omental transposition,

buried dermal flaps, enteromesenteric bridging,

lymphangioplasty, and microvascular

lympholymphatic anastomosis.
EXCISIONAL SURGERY

Excisional techniques remove the affected tissues,

thus reducing the lymphedema-related load.

The Charles procedure is a radical excisional

technique. This procedure involves the total excision

of all skin and subcutaneous tissue from the affected

extremity. The underlying fascia is then grafted,

using the skin that has been excised. This technique

is extreme and is reserved for only the most severe

cases.

Complications include ulceration, hyperkeratosis,

keloid formation, hyperpigmentation, weeping

dermatitis, and severe cosmetic deformity.

A variant of the Charles procedure, total superficial

lymphangiectomy, involves debulking of the entire

limb.

COMPRESSION THERAPY

Patients should use compression garments

continuously during the day.

They should also have graduated compression that

increases from distal to proximal on the affected

extremity.

Intermittent pneumatic pump compression therapy

provides sequential, active compression from distal to

proximal, effectively milking the lymph from the

extremity.

This treatment is most appropriately used prior to

fibrosclerotic evolution, which it assists in preventing.

Contraindications to intermittent pneumatic pump

compression therapy include congestive heart failure,

deep vein thrombosis, and active infection.
MLD

Manual lymphatic drainage according to the

Vodder and/or Leduc techniques.

Compression garments are essential between

treatments.

Manual massage of the affected extremity; this

recruits collateral vessels, allowing the

accumulated lymph to be drained into

neighboring regions with normally functioning

lymphatics.

PHARMACOLOGIC THERAPY

Cellulitis
At the earliest signs of infection, institute topical or

systemic antifungal or antimicrobial therapy to prevent the

development of sepsis.

Filariasis
Filariasis has been treated with DEC and albendazole
Benzopyrones
These drugs bind to accumulated interstitial proteins,

inducing macrophage phagocytosis and proteolysis. The

resulting protein fragments pass more readily into the

venous capillaries and are removed by the vascular system.

The benzopyrones aid in decreasing excess edematous fluid,

softening the limb, decreasing skin temperature, and

reducing the number of secondary infections. Of note,

however, is that hepatotoxicity has been associated with

coumarin therapy.
PHARMACOLOGIC THERAPY

Retinoids

Help normalize keratinization and decrease

inflammatory and fibrotic changes.

Topical agents

Topical emollients and keratolytics have been

recommended to improve secondary epidermal

changes.

What every patient with 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

Anti-fungal 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