Intestinal Lymphangiectasia

Updated: Nov 21, 2023
  • Author: John W Birk, MD, FACG; Chief Editor: Burt Cagir, MD, FACS  more...
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Overview

Background

Intestinal lymphangiectasia is a rare protein-losing gastroenteropathy characterized by impaired drainage of lymph from the small intestine, associated with dilatation of the intestinal lymphatic channels. This leads to inappropriate loss of lymph into the gastrointestinal (GI) tract, causing hypoproteinemia, edema, lymphocytopenia, hypogammaglobinemia, and immunologic dysfunction. [1]

Protein-losing gastroenteropathies have been classified into three groups (depending on the mechanism of their etiology) that include the following [2] : (1) inflammatory-induced mucosal erosions and/or ulcerations (eg, Crohn disease), (2) mucosal damage or abnormalities resulting in increased protein permeability without mucosal erosions or ulcerations (eg, celiac disease), and (3) protein loss stemming from mechanical lymphatic obstruction (eg, intestinal lymphangiectasia).

See protein-losing enteropathy for a more comprehensive discussion on this topic.

Pathophysiology

Protein-losing gastroenteropathy occurs when protein loss into the gastrointestinal (GI) tract exceeds the liver’s production capacity. Intestinal lymphangiectasia is defined by compromised enteric lymph drainage and the presence of dilated lymphatic channels. [3, 4] This condition is categorized into two types: primary and secondary intestinal lymphangiectasia.

Primary intestinal lymphangiectasis (PIL), also known as Waldmann disease, pertains to congenital anomalies. These malformations manifest as extensive or localized dilatations (ectasia) of enteric lymphatics, situated within the mucosa, submucosa, and/or subserosa layers of the intestine. Several genes associated with the development of the lymphatic system have been studied, but none have been conclusively identified as clinically significant contributors to PIL.

 
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