Dr  Sampurna Roy  MD

Intestinal 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.

Visit: Malabsorption syndrome ; Coeliac Disease ;An approach to evaluation of small intestinal biopsy ; Enteropathy-associated T-cell lymphoma

Clinical presentation:  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.

Endoscopic findings:  Endoscopy shows white opaque spots on the small intestinal mucosa, which indicates dilated lacteal vessels.

Pathological findings: 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.

- Normal Histology of the Large Intestine - Interpretation of Large Intestinal Biopsies - Assessment of abnormalities -1 (lumen, surface epithelium, subepithelial zone) - Assessment of abnormalities - 2  (crypt density , architecture and epithelium) - Assessment of abnormalities - 3 (changes in the lamina propria,muscularis mucosae and submucosa) - Microscopic/ collagenous colitis ;   - Pseudomembranous colitis; - Pathology of Amebic Colitis ;  - Drug related lesions of the Gastrointestinal Tract - Gross examination of colorectal resection specimens in  non-neoplastic diseases

Normal histology of the small intestine for anatomic pathologists An approach to evaluation of small intestinal biopsy. Malabsorption syndrome (causes  and clinical investigations) Tropical Sprue Coeliac Disease Enteropathy-associated T-cell lymphoma Intestinal lymphan-giectasia Lesions causing small bowel obstruction and bleeding - Intussusception : Adhesions : Volvulus Meckel's diverticulum Ischemic bowel disease  Brunner's Gland Adenoma Duodenal  Gangliocytic Paraganglioma Lymphoma of the small intestine