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STAGES OF LYMPHEDEMA ACCORDING TO CORRELATION AMONG PATHOPHYSIOLOGY, CLINICAL FEATURES, IMAGING AND MORPHOLOGY OF THE AFFECTED LIMBS

AUTHOR: Salvador Nieto, M.D, FICA.
SALVADOR NIETO FOUNDATION “for the development of lymphedematology”. 
Av. Santa Fe 2679, 2º “D”; (1425) Buenos Aires. Argentina.

PAPER PRESENTED IN THE 19º INTERNATIONAL CONGRESS OF LYMPHOLOGY - SEPTEMBER 4-6, 2003 - FREIBURG, GERMANY.

INTRODUCTION
The presence of lymphedema means that lymphatic system and every protective mechanism against high protein edemas have been overwhelmed. From this moment, pathological changes in tissues follow the development of this illness. We refer to this sequence of changes produced by lymphedema as the “Chain of events” that mark the progression of the lymphatic disease.

LYMPHEDEMA - CHAIN OF EVENTS
LYMPHANGIOPATHY
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Lymphatic Transport Impairment
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Stagnation of Plasmatic Proteins in the Interstice
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High Protein Edema
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Fibrosis – Fibroesclerosis
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ç Infections

Elephantiasis
It enables to establish an accurate correlation among pathophysiology, clinical features, computerized tomography imaging and limb morphology. Thus, in a horizontal cut we are able to establish stages. Each stage is formed by a corresponding pathophysiological grade, clinical aspects, CT image and characteristic morphology of the affected limb. Similarly, a vertical cut draws a distinction into five stages of lymphedema which can be assessed according to that association.
 
EDEMATOUS PHASE
Corresponds with stage I of the Histological-clinical-densimetric classification (HCDC) of extremity lymphedema.
High protein edema.
Tissue consistence: soft.
Pitting edema.
Improvement by elevation of limbs.
CAT Scan. Subcutis: small quantity of fibrotic circular bands, sometimes hardly perceptible. Its density fluctuates between -150 and -110 HU (Hounsfield Units) (normal: =/~ -150 HU).
Limb morphology: slight difference to normal (comparing affected and not affected limbs).
 
Stage I.
CAT Scan. Subcutis: Small quantity of fibrotic circular bands
Stage I.
Slight difference between limbs.
 
FIBROEDEMATOUS PHASE
Corresponds with stage IIA of the HCDC.
Tissue consistence: elastic (like "rubber").
Pitting edema.
Slight improvement by elevation of limbs.
CAT Scan. Subcutis characteristics: regular amount of fibrotic circular bands. Its density fluctuates between -110 and -60 HU.
Limb morphology: difference between limbs is more evident than previous stage.
Stage II.
CAT Scan: moderate fibrosis.
Stage II.
Moderate difference between limbs
 
FIBROSCLEROTIC PHASE
Corresponds with stage IIB of the HCDC.
Tissue consistence: elastic (like "rubber").
Skin maximum distention ("almost bursting").
Edema without fovea.
No improvement by elevation of limbs.
Mobility of the affected limb: mild limitation.
CAT Scan: Subcutis plenty of fibrotic bands outlining a "honeycomb" pattern. Its density fluctuates between -110 and -60 HU.
Limb morphology: marked difference between affected and not affected limbs.
 
Stage III.
CAT Scan: rich in fibrosis.
Stage III.
Marked difference between limbs
 
SCLEROTIC PHASE
Corresponds with stage III of the HCDC.
Tissue consistence: very much increased (like "wood").
Edema without fovea.
No improvement by elevation of limbs.
Mobility of affected limbs: severe limitation.
CAT Scan. Subcutis: very irregular aspect owing to exuberant fibrosclerotic tissue drawing a typical "honeycomb" pattern. Its density fluctuates between -60 and 0 HU.
Limb morphology: strongly marked difference between affected and not affected limbs.
 
Stage IV.
CAT Scan: Honeycomb pattern.
Stage IV.
Difference strongly marked between limbs.
 
ELEPHANTIASIS

Corresponds with stage IV of the HCDC.
Limbs with severe skin disease.
Limb morphology: monstrous characteristics.
This is the most advanced stage. It is so peculiar that clinical examination makes other studies unnecessary.
   
(From Földi)
CONCLUSION
The explanation and interpretation of phenomenon caused by lymphedema allow us to have an accurate view of the illness and to place each patient in the correct point of the disease in order to apply the form of treatment that will best favour him. In this sense, we also emphasize the importance of lymphoscintigraphy.

REFERENCES
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