Idiopathic Congenital Lymphedema – A Case Report

Introduction:
Lymphoedema is defined as regional accumulation of
excessive interstitial protein-rich fluid caused primarily
by an imbalance between the inflow and the removal
of interstitial fluid and protein, and secondarily by a
malformation or malfunction of the lymphatic system1.
There are two major categories of lymphoedema:
primary and secondary2. Primary lymphedema is a
rare disease that results from impaired lymphatic
drainage. It is estimated to occur in about 1.15 in
100,000 persons who are less than 20 years old with
a sex ratio of about one male to three females3.
Causes of primary lymphedema are due to congenital
absence, hypoplasia or underdevelopment of the
superficial lymphatic channel. The disease is limited
to the leg but arms, hand and feet can also be
affected4. If lymphatics are damaged or blocked by
any pathologic process like infection, surgical
excision, neoplasm, irradiation, or trauma, the
interstitial fluid builds up in the tissues causing
secondary lymphedema5.

 

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