Abstract
Primary intestinal lymphangiectasia is an uncommon condition that usually presents early in childhood. This incurable condition is consequent to underlying lymphatic abnormalities that lead to loss of lymphatic contents into the intestinal lumen. This article outlines an approach to the assessment of children presenting with characteristic features and consideration of other conditions that could lead to enteric protein loss. An overview of the management of primary intestinal lymphangiectasia is outlined.
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