
) أ.م.د. احمد عبداالمير دفار ( اختصاصي جراحة الصدر و القلب و االوعية الدموية
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Disorders of the lymphatic system
Objective : To show the definition, etiology and management
of disorders of the
lymphatic system.
Lymphedema
Classification
-
Primary lymphedema
- Lymphedema congenita
The onset is before the first year of life.
-
Lymphedema praecox
The onset is between the ages of 1 and 35 years. It is the most common form of primary
lymphedema, accounting for more than 80% of the cases.
-
Lymphedema tarda
The onset is after the age of 35 years. Approximately half of these are associated with an inciting
event, such as infection and injury.
-
Secondary Lymphedema
- Infiltration of regional nodes by tumor
- Surgical excision of regional nodes in treatment for malignancy
- Fibrosis after infectious or inflammatory processes or radiation
- Infestation by filaria (the most common being
Wuchereria bancrofti
) is the most frequent
cause of secondary lymphedema in tropical countries.
- Tuberculous lymphangitis.
Pathogenesis
Clinical features:-
Patients commonly complain of heaviness and fatigue in the affected extremity. The limb size
increases throughout the day and decreases over the course of the night when the patient is in
bed. In the lower extremity, the swelling involves the dorsum of the foot, and the toes have a
squared-off appearance. Initially, this swelling pits easily with pressure. This early edema is often
responsive to elevation and may decrease or disappear entirely with elevation of the limb.
Later
,
the edema becomes nonpitting & limb elevation and compression with elastic garments then are
much less successful at reducing the extremity volume. The skin becomes thickened, hypertrophic,
and hyperkeratotic. Recurrent spontaneous attacks of cellulitis & lymphangitis are common and
may be due to loss of local immune defenses because of diminished lymphatic function.

) أ.م.د. احمد عبداالمير دفار ( اختصاصي جراحة الصدر و القلب و االوعية الدموية
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Investigations:-
- Duplex Ultrasound
When a patient is evaluated for edema, it is often difficult to distinguish the early stages of
lymphedema from venous insufficiency. Duplex ultrasound of the venous system can determine if
there is concomitant venous thrombosis or venous reflux.
- Lymphoscintigraphy
It requires only subcutaneous injection of
technetium labeled colloid
and serial gamma camera
imaging of the extremity.
- Lymphangiography
Contrast medium
is slowly infused, and the transit of dye is observed by serial roentgenograms.
- MRI
TREATMENT OF LYMPHEDEMA
Nonoperative Therapy
- Meticulous skin hygiene
- Bed rest and Leg elevation
- External compression with elastic support garments.
- Lymphatic massage:- The strategy is to massage the excess interstitial fluid from areas with
deficient lymphatic drainage to areas with more normal lymphatic function.
- Avoidance of local injury as well as walking barefoot.
- Care should be taken to keep the interdigital spaces dry, and frequent use of an antifungal powder
may be helpful.
- Aggressive antibiotic therapy is recommended at the earliest signs or symptoms of cellulitis &
lymphangitis.
- Use of
benzopyrones
to reduce the formation of high-protein edema.
- Treatment of the underlying disorder in the secondary lymphedemas, such as using
diethylcarbamazine
for filariasis or appropriate antibiotics for tuberculosis or lymphogranuloma
venereum.
Surgical Therapy
It's rarely indicated.