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Pathology of Intestinal Lymphangiectasia
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lymphangiectasia (IL) is a rare disease characterized by severe
edema, thickening of the small-bowel wall, protein-losing
enteropathy, ascites, and pleural effusion.
Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture.
Intestinal lymphangiectasia may be generalized or localized, depending on the site of blockage of mesenteric lymphatic drainage.
Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy.
Intestinal lymphangiectasia may vary widely in its manifestations and severity.
The intestinal tract should be always examined for enteric protein losses if other causes (i.e. , malnutrition and protein loss from other sites) are excluded.
The patient may be asymptomatic or present as vague abdominal pain, chronic diarrhea, steatorrhea, edema, chylous pleural effusion, ascites, hypoproteinemia, lymphocytopenia or protein-losing enteropathy .
Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage.
(i) IL usually occurs in children or young adults, and is suspected to be caused by a congenital abnormality in the lymphatic system.
(ii) It can be seen in the aged people, which may be secondary to disorders causing lymphatic obstruction, such as lymphoma, carcinoma, tuberculosis, constrictive pericarditis, retroperitoneal fibrosis, post-radiation effects, and repeated parasite infestation.
Endoscopy shows white opaque spots on the small intestinal mucosa, which indicates dilated lacteal vessels.
There may be great variation , ranging from a normal appearance to severe changes.
Findings at pathology include a dilatation of the lymphatics in the mucosa and submucosa of the small bowel with resultant bowel wall thickening due to edema and congestion.
Lymphatic block may occur at different sites -in the lamina propria only, generalized (lamina propria, submucosa, serosa, and mesentery), or conversely in the mesentery alone with minimal changes in the lamina propria.
The diagnosis of IL is confirmed on small intestinal (jejunal, duodenal) biopsy demonstrating dilated lymphatic lacteal vessels.
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