Last revised by Henry Knipe on 5 Mar 2024

Lymphangiomatosis is a rare mesenchymal disorder that is characterized by developmental "malformation" of multiple lymphatic channels (usually with dilatation).


If lymphatic channels are purely dilated and not malformed the term lymphangiectasia is usually used. If lymphangiomatosis occurs in a singular localized form it is then the term lymphangioma can be used, and if it is widespread throughout the body then generalized lymphatic anomaly can be used.


The condition can have various forms depending on organ involvement:


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