Intestinal lymphangiectasia is a condition characterized by dilated intestinal lacteals causing loss of lymph into the lumen of the small intestine. This can result in hypoproteinemia, hypogammaglobulinemia, hypoalbuminemia and lymphopenia.
It may present in either children or adults.
The condition may be localized or diffuse. It can occur from a number of etiologies, which include:
- primary/congenital form: primary idiopathic intestinal lymphangiectasia / Waldmann disease 5
- secondary form: resulting from a tumor, fibrosis, or another cause of lymphatic obstruction.
The definitive diagnosis is made by endoscopic evaluation and biopsy of the duodenum and/or jejunum or alternatively from surgically resected small intestine.
- fecal alpha-1-antitrypsin is often raised
Non-specific and may show bowel wall thickening to varying degrees.
- malignant transformation into lymphoma: may occur in around 5% of cases 1.
Treatment and prognosis
Treatment has usually comprised of a low fat diet along with medium-chain triglyceride oral supplements.
History and etymology
It was first described by TA Waldmann in 1961 1.
As a broad differential of bowel thickening, consider forms of enteritis.
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