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Developmental arrest or
abnormalities can result in primary hypoplasia or absence of ducts and lymph nodes sacs can produce unilocular or multilocular lymph cysts termed cystic hygromas
the axilla, mediastinum, retroperitoneum, or intestinal mesentery
cannot be reabsorbed by the venules, red blood cells, bacteria, and other larger particles can be evacuated only through the lymphatics.
found in the intermuscular fascia, they are absent in muscles, tendon, cartilage, brain, and cornea.
classied as: mild: < 20% excess limb volume; moderate: 2040% excess limb volume; severe: > 40% excess limb volume.
percent of cases
In proximal hypoplasia, the lymphedema is more
extensive, involving the entire extremity, and it occurs equally among males and females.
The combination of proximal and distal hypoplasia
uncommon (8 percent), and those with bilateral hyperplasia usually can be recognized by diffuse capillary angiomata on the lateral sides of the feet.
Grade (Brunner)
Subclinical (latent) :There is excess interstitial uid and
histological abnormalities in lymphatics and lymph nodes, but no clinically apparent lymphoedema
I Oedema pits on pressure and swelling largely or
upon elevation
III Oedema is associated with irreversible skin changes,
Clinical Manifestations
Lymphedema is a clinical diagnosis and should be restricted to
situations where other causes of edema have been excluded or a specific lymphatic abnormality has been demonstrated.
The presence of bilateral dependent pitting edema usually
family history of primary lymphedema and whether the patient has visited any countries where filariasis is endemic suggests small bowel lymphangiectasia.
Complications of lymphedema
It is important to document
limb size to identify isolated limb gigantism and the KlippelTrnaunay syndrome that may have hypoplastic lymphatics in addition to venous abnormalities, capillary nevus, and limb elongation.
Diagnostic Studies
Dye Injection- patent blue& sky
blue dye If the collecting vessels are obstructed or inadequate, the dye diffuses through the dermal lymphatics to produce a marbled appearance called dermal backflow.
Lymphography- Lipiodol CT MRI USG
human serum albumin labeled with radioactive iodine or technetium 99m colloid has been used to monitor lymphatic clearance by serial scanning.
1.5 g/dL The ratio of albumin to globulin also is higher in lymphedema fluid than in plasma
Management
Supportive Treatment
primary objectives remain for control of edema,
maintenance of healthy skin, and avoidance of the complications of cellulitis and lymphangitis. Elevation Exercise sequential pneumatic compression boots to massage the leg
and swelling usually signifies early cellulitis or lymphangitis, which can be recognized by red streaking up the leg. The usual causative organism is staphylococcus or beta-hemolytic streptococcus, which must be treated vigorously, usually with intravenous antibiotics
lymphatic ducts, short stretch compression bandaging, therapeutic exercise, and skin care.
Emil Vodder
usually will respond to hydrocortisone cream. Antifungal agents may be necessary, topically and systemically, for chronic infections, particularly between the toes. In contrast to the stasis edema of venous insufficiency, ulceration is unusual, although fissures and lymph fistulas can develop and require surgical excision.
surgery
Homans procedure
Operative Treatment Sistrunk Charles Kinmonth's modification of
Homans's procedure
Thompsons reduc-
tion operation
Bypass procedures
Charles procedure
removed the pulp under magnification, and sutured the node capsule with its afferent lymphatics into a vein. This procedure is more suitable for secondary lymphedema than primary, in which the disorder lies in the lymphatic channels themselves connection was developed by Cordeiro and modified by Degni, who used a special needle for insertion of lymphatic vessels directly into veins and fixed them there by a single suture vessels of 0.50.8 mm diameter using supermicrosurgical techniques, with promising results.
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