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.
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Epidemiology
It may present in either children or adults.
Pathology
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.
Biochemical tests
- fecal alpha-1-antitrypsin is often raised
Radiographic features
General
Non-specific and may show bowel wall thickening to varying degrees.
Complications
- 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.
Differential diagnosis
As a broad differential of bowel thickening, consider forms of enteritis.
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