LYMPHOEDEMA - PATHO-PHYSIOLOGY AND CLASSIFICATION

  • 1 January 1985
    • journal article
    • research article
    • Vol. 26  (2) , 91-106
Abstract
Current knowledge and hypotheses about the physiology of lymph production and lymph flow and the etiology and classification of lymphedema were reviewed. One of the earlier contributions in the 1930''s by Allen suggested that primary lymphedema was caused by congenital underdevelopment of lymph vessels. At that time 2 clinical varieties (congential and praecox) were described but later a further subdivision into inflammatory and noninflammatory types of lymphedema were described. Kinmonth in 1957 produced the 1st clinical classification dividing all cases into primary or secondray lymphedema but at the time gave no indication as to the cause of the primary variety. In the 1950''s Kinmonth also developed a radiological classification which contributed considerably to the knowledge of the anatomical abnormalities of the lymphatic system in primary lymphedema. Despite the widespread use of Kinmonth''s classification the lymphographic appearances in primary lymphedema gave little or no clue to the etiology of the disease process. The etiology of primary lymphedema was not known. There were various descriptive classifications based on age of onset and radiological findings for instance, but no attempts to explain the pathophysiology of the disease. Therefore, current knowledge of the known physiology and the potential abnormalities of the collection and passage of lymph from the interstitial space to the blood system were described. A simple classification of the etiology of lymphedema was presented. Primary lymphedema may be defined as lymphedema caused by a primary abnormality or disease of the lymph conducting elements of the lymphs vessels or lymph nodes. Secondary lymphedema is edema caused by disease in the nodes or vessels that began elsewhere (e.g., neoplasia or filariasis), or lymphocytic proliferative disorders such as Hodgkin''s disease or following surgical extirpation of lymph nodes or vessels. There are 3 groups of primary lymphedema in which the functional abnormality and its cause are known: large vessel abnormalities such as congenital aplasia of the thoracic duct or cysterna chyli, congenital lymphatic valvular incompetence or congenital aplasia and lymph node fibrosis. The remainder were characterized by a reduced number of lymphatics on lymphography. Such patients had obliterated lymphatics. If they presented within a few years of birth they were probably born with too few lymphatics. However, those who present later in life may have acquired obliterative disease, the cause of which is still obscure. This last group constitutes the majority of the primary lymphedemas.

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