Download MBBS Surgery Presentations 36 Lymphatic System And Its Diseases Lecture Notes

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ANATOMY
A 1-way system that returns lymph via

vessels to the CVS.

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

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lymph nodes, spleen, and thymus)

Organ

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Function

Lymph

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

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well as toxins and cellular waste products, that are

transported to and from cellular tissues

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Lymphatic vessels

Transport lymph from peripheral tissues to the veins of the

cardiovascular system

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Lymph nodes

Monitors the composition of lymph, the location of pathogen

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engulfment and eradication, the immunologic response, and

the regulation site

Spleen

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Monitors the composition of blood components, the location

of pathogen engulfment and eradication, the immunologic

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response, and the regulation site

Thymus

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

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and control
Functions are :

Restoration of excess interstitial fluid and

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proteins to the blood

Absorption of fats and fat-soluble vitamins from

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the digestive system

Defense against invading organisms

LYMPH

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Fluid derived from plasma.

Contains nutrients, oxygen, and hormones, as

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well as toxins and cellular waste.

This fluid is removed by lymphatic vessels that

pass through lymph nodes.

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As the lymph passes through the lymph nodes,

lymphocytes and monocytes enter it.
LYMPHATIC VESSELS

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Are blind-ended tubes with thin endothelial

walls.

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

lymphatic duct and the thoracic duct.

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

Bean-shaped structures

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Filter the lymph before it rejoins the blood

stream.

Approximately 600-700, predominantly in the

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neck, axillae, groin, mediastinum, and

mesenteries of the GI tract.

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Constitute a main line of defense by hosting 2

types of immune protective cell lines, T and B

lymphocytes.

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2 distinct regions, the cortex and the medulla.

The cortex contains follicles, which are collections

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of lymphocytes.

Center of the follicles is called germinal centers

that has B-lymphocytes while the remaining cells

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of the cortex are T-lymphocytes.

Vessels entering the lymph nodes are called

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afferent lymphatic vessels and those exiting are

called efferent lymphatic vessels
LYMPHADENOPATHY

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ETIOLOGY

Five broad etiologic categories :

An immune response to infective agents (eg,

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bacteria, virus, fungus)

Inflammatory cells in infections involving the

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lymph node

Metastasis

Malignancy of lymphocytes or macrophages (eg,

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leukemia, lymphoma)

Storage disorders
PRESENTATION

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Duration

Associated symptoms

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Past illnesses, infections, local trauma, or bites.

Constitutional symptoms.

If recurrent infections, HIV must be considered.

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Family and social history

PHYSICAL EXAMINATION

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Complete general examination.

The skin and the soft tissue drained by the

enlarge node should be carefully examined.

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The character of the lymph node should be noted.

Whether the lymphadenopathy is a local or a

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general phenomenon.
GENERALIZED LYMPHADENOPATHY:

Upper respiratory tract infections (rhinovirus,

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adenovirus, influenzavirus, parainfluenza virus,

respiratory syncytial virus)

Epstein-Barr virus (EBV)

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Cytomegalovirus (CMV)
Varicella-zoster virus
Herpes simplex virus
Paramyxovirus
Coxsackieviruses A and B

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Echovirus
Enterovirus
Human herpesvirus-6
Human immunodeficiency virus

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WORK UP
LABORATORY STUDIES

Perform the least invasive test that provides the

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most information.

CBC .

Serum LDH- to determine the turnover rate of

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cells in the case of leukemia or lymphoma.

Tuberculin skin test.

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Monospot and titers for EBV, CMV, catscratch

disease, or toxoplasmosis

IMAGING STUDIES

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Chest radiography to assess for bacterial

pneumonias or tuberculosis, and hilar

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adenopathy in the case of malignancy.

Ultrasonography.

CT scan.

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18F-FDG PET.

FNAC

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BIOPSY


LYMPHEDEMA

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?Lymphatic obstruction

?Dec. lymphatic transport

?Stagnation of HMW proteins

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? High protein edema

? High oncotic pressure in the interstitium

favors the accumulation of additional water.

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? Massive dilatation of lymphatic vessels

? Inflammation

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? Shrunken and hard lymph nodes

DERMATOLOGIC PATHOLOGY

The overlying skin becomes thickened and

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displays the peau d'orange appearance.

Chronic lymphedema causes fissuring and

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impairment of the epidermis, allowing bacteria to

enter and grow, and leading to lymphorrhea, the

leakage of lymph.

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With chronic lymphedema, the development of

verrucous, cobblestone plaques, a condition

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known as elephantiasis nostra verrucosa (ENV),

can occur.
ETIOLOGY

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In primary lymphedema, there is congenital

hypoplasia or aplasia of the peripheral

lymphatics or valvular incompetence.

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In secondary lymphedema, the lymphatic

drainage is blocked due to:

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Recurrent attacks of lymphangitis

Malignancy

Obesity

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Surgery
PRIMARY LYMPHEDEMA

A developmental abnormality of the lymphatic

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system.

3 main types, distinguished by their age of onset:
1. Congenital lymphedema (Milroy disease)

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2. Lymphedema praecox (Meige disease)
3. Lymphedema tarda
Involve the lower extremities almost exclusively.
CONGENITAL LYMPHEDEMA

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10-25% of all primary lymphedema cases.

Autosomal-dominant.

Anaplastic lymphatic channels.

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

F:M= 2:1.

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LL:UL= 3:1

The edema is most commonly pitting and non

painful.

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2/3 rd of patients have bilateral lymphedema.

May improve spontaneously with increasing age.

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LYMPHEDEMA PRAECOX

Most common form of primary lymphedema (65-

80%).

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Becomes clinically evident after birth and before

age 35 years.

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F:M= 4:1

About 70% of cases are U\L.

A hypoplastic pattern, with the lymphatics

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reduced in caliber and number.
LYMPHEDEMA TARDA

Manifests later in life, usually in persons older

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than 35 years.

Caused by a defect in the lymphatic valves,

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resulting in incompetent valve function.

Accounts for only 10% of cases.

ASSOCIATED CONDITIONS

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Distichiasis lymphedema syndrome is

lymphedema associated with distichiasis (double

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row of eyelashes).

Vertebral abnormalities, spinal arachnoid cysts,

hemangiomas, cleft palate, ptosis, short stature,

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webbed neck, strabismus, thoracic duct

abnormalities, and microphthalmia.

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Associated with yellow nail syndrome.
ASSOCIATED CONDITIONS

Other genetic syndromes and cutaneous

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conditions associated with primary lymphedema

include the following:

Turner syndrome

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Noonan syndrome

Klinefelter syndrome

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Neurofibromatosis type 1

Hemangiomas

Xanthomatosis

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Congenital absence of nails

SECONDARY LYMPHEDEMA
SECONDARY LYMPHEDEMA

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Caused by an acquired defect in the lymphatic

system.

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Associated with obesity, infection, neoplasm,

trauma, and therapeutic modalities.

SECONDARY LYMPHEDEMA

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1.Filariasis
The most common cause of secondary

lymphedema.

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Caused by a mosquito-borne nematode infection

with the parasite Wucheria bancrofti.

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Commonly occurring in developing countries

around the world.

Results in permanent lymphedema of the limb.

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SECONDARY LYMPHEDEMA

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

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lymphoma or secondary to radical lymph node

dissection and excision.

The most commonly affected area is the axillary

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region after mastectomy

Lymphedema can also be seen after regional

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dissection of pelvic, para-aortic, and neck lymph

nodes.

SECONDARY LYMPHEDEMA

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Other causes
Trauma
Varicose vein surgery
Congestive heart failure

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Portal hypertension
Peripheral vascular surgery
Lipectomy
Burns
Burn scar excision

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Insect bites
Extrinsic pressure
CLINICAL PRESENTATION

HISTORY

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

increased circumference of an extremity.

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As swelling slowly progresses, patients may have

difficulty fitting into clothing.

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May cause fatigue due to the size and weight of the

extremity, embarrassment in public, and severe

impairment of daily activities.

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

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lymphangitis, fissuring, ulcerations, and/or verrucous

changes.
HISTORY

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In primary lymphedema, the history of onset is more

typical. Associated with other anomalies and genetic

disorders.

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In secondary lymphedema, the associated history is

based on the primary etiology.

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If due to filariasis, the history should include travel or

habitation in an endemic area.

Other patients should have a clear history of a

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neoplasm obstructing the lymphatic system, recurrent

episodes of lymphangitis and/or cellulitis, obesity,

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trauma, or lymphedema resulting after surgery

and/or radiation therapy.

A recent history of varicose vein surgery also is

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reported.

PHYSICAL EXAMINATION

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The earliest symptom is nontender, pitting edema

progressing to a nonpitting one

Radial enlargement of the area.

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Involvement of the distal extremities is followed

by proximal advancement.

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Erythema and peau d'orange of skin.


Grade (Brunner)

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Clinical features

Subclinical (latent)

There is excess interstitial fluid

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and

histological abnormalities in

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lymphatics

and lymph nodes, but no clinically

apparent lymphoedema

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I

Oedema pits on pressure and

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swelling

largely or completely disappears

on

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elevation and bed rest

II

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Oedema does not pit and does not

significantly reduce upon

elevation

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III

Oedema is associated with

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irreversible skin

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

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ENV is an area of cobble-stoned, hyperkeratotic,

papillomatous plaques most commonly seen on

the shins.

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The plaques can be weepy or oozing a clear or

yellow fluid.

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Fissuring, ulcerations, skin breakdown.

Superinfection is common and can manifest as

impetigo with yellow crusts.

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EXAMINATION

A positive Stemmer sign (inability to pinch the

dorsal aspect of skin between the first and second

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toes)

Patients with congenital lymphedema may also

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present with recurrent cellulitis, papillomatosis,

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

toenails.

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DIFFERENTIALS

Deep vein thrombosis
Hypoalbuminemia

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Renal failure
Lipedema
Postoperative complications
Baker cyst
Idiopathic edema

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Congestive heart failure
Idiopathic edema
Neurofibromatosis
Sclerema neonatorum
Features of lipoedema that help differentiate it from

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lymphoedema

Occurs almost exclusively in women

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Onset nearly always coincides with puberty

Nearly always bilateral and symmetrical

Involvement of trunk

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The feet are not involved, leading to an inverse

shouldering effect at the malleoli

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No pitting

No response to elevation or compression

No skin changes of lymphoedema (negative Stemmer's

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sign)

MRI shows subcutanteous fat but no fluid

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accumulation

WORKUP OF LYMPHEDEMA
Liver function, blood urea nitrogen

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(BUN)/creatinine levels, and urinalysis results

should be checked if a renal or hepatic etiology is

suspected.

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Specific markers should be checked if a neoplasm

is suspected.

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CBC with differential should be checked if an

infectious etiology is being considered.

An indication for CT scanning or MRI is suspicion

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of malignancy.

MRI is useful to show lymph trunk anatomy and

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causes of obstructive secondary lymphedema.

Ultrasonography to evaluate the lymphatic and

venous systems.

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Lymphangiography is now rarely used because of

the potential adverse effects.

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Fluorescence microlymphography demonstrates a

lack of microlymphatics.

Lymphoscintigraphy.

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TREATMENT OF LYMPHEDEMA

The goal is to restore function, reduce physical

and psychologic suffering, and prevent the

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development of infection.

Initiate therapy as early as possible before

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extensive, irreversible fibrosclerotic changes

occur in the interstitium.

Strict compliance is essential.

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The majority of compliant patients can be treated

successfully with conservative measures
1.HYGIENE AND SKIN CARE

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Appropriate skin care to prevent recurrent

cellulitis or lymphangitis.

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Meticulous hygiene to remove keratinous debris

and bacteria.

Cleanse the skin regularly and dry thoroughly.

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Regular inspection is necessary to identify any

open wounds or developing cellulitis.

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2.PHYSICAL THERAPY AND COMPRESSION

The first-line treatment.

Aimed at improving lymphedema with manual

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lymphatic drainage, massage, and exercise.

It advocates the use of compression stockings (at

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a minimum of 40 mm Hg), multilayer bandaging,

or pneumatic pumps.

Encourage patients to lose weight, avoid minor

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trauma, and avoid constrictive clothing that

might have a tourniquet effect.

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Encourage elevation of the affected extremity

whenever possible, particularly at night.
3. SURGERY

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Surgical treatment is palliative, not curative,

and it does not obviate the need for continued

medical therapy.

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Surgical treatment is reserved for patients who

do not improve with conservative measures or for

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cases in which the extremity is so large that it

impairs daily activities and prevents successful

conservative management.

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Surgical procedures are classified as physiologic

or excisional.

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PHYSIOLOGIC SURGERY

Physiologic procedures attempt to improve

lymphatic drainage. Multiple techniques have

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been described, including omental transposition,

buried dermal flaps, enteromesenteric bridging,

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lymphangioplasty, and microvascular

lympholymphatic anastomosis.
EXCISIONAL SURGERY

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Excisional techniques remove the affected tissues,

thus reducing the lymphedema-related load.

The Charles procedure is a radical excisional

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technique. This procedure involves the total excision

of all skin and subcutaneous tissue from the affected

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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

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cases.

Complications include ulceration, hyperkeratosis,

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keloid formation, hyperpigmentation, weeping

dermatitis, and severe cosmetic deformity.

A variant of the Charles procedure, total superficial

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lymphangiectomy, involves debulking of the entire

limb.

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COMPRESSION THERAPY

Patients should use compression garments

continuously during the day.

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They should also have graduated compression that

increases from distal to proximal on the affected

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extremity.

Intermittent pneumatic pump compression therapy

provides sequential, active compression from distal to

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proximal, effectively milking the lymph from the

extremity.

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This treatment is most appropriately used prior to

fibrosclerotic evolution, which it assists in preventing.

Contraindications to intermittent pneumatic pump

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compression therapy include congestive heart failure,

deep vein thrombosis, and active infection.
MLD

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Manual lymphatic drainage according to the

Vodder and/or Leduc techniques.

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Compression garments are essential between

treatments.

Manual massage of the affected extremity; this

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recruits collateral vessels, allowing the

accumulated lymph to be drained into

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neighboring regions with normally functioning

lymphatics.

PHARMACOLOGIC THERAPY

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Cellulitis
At the earliest signs of infection, institute topical or

systemic antifungal or antimicrobial therapy to prevent the

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development of sepsis.

Filariasis
Filariasis has been treated with DEC and albendazole

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Benzopyrones
These drugs bind to accumulated interstitial proteins,

inducing macrophage phagocytosis and proteolysis. The

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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,

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softening the limb, decreasing skin temperature, and

reducing the number of secondary infections. Of note,

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however, is that hepatotoxicity has been associated with

coumarin therapy.
PHARMACOLOGIC THERAPY

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Retinoids

Help normalize keratinization and decrease

inflammatory and fibrotic changes.

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Topical agents

Topical emollients and keratolytics have been

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recommended to improve secondary epidermal

changes.

What every patient with lymphoedema should receive

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An explanation of why the limb is swollen and the

underlying cause

Guidance on skin hygiene and care and the avoidance of

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acute infective episodes

Anti-fungal prophylactic therapy to prevent athlete's foot
Rapid access to antibiotic therapy if necessary, hospital

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admission for acute infective episodes

Appropriate instructions regarding exercise therapy
Manual lymphatic drainage (MLD)

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Multilayer lymphoedema bandaging (MLLB)
Compression garments and, if appropriate, specialised

footwear

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Advice on diet
Access to support services and networks