Intestinal lymphangiectasia is a condition characterised by dilated intestinal lacteals causing loss of lymph into the lumen of the small intestine. This can result in hypoproteinemia, hypogammaglobulinemia, hypoalbuminemia and reduced number of circulating lymphocytes or lymphopaenia.
It may present in either children or adults.
The condition may be localised of diffuse. It can occur from a number aetiologies which include.
- primary/congenital form: primary idiopathic intestinal lymphangiectasia / Waldmann disease 5
- secondary form: resulting from a tumour, fibrosis, or another cause of lymphatic obstruction.
The definitive diagnosis is made by endoscopic evaluation and biopsy of duodenum and/or jejunum or alternatively from surgically resected small intestine.
- faecal 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 a 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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