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 hypoproteinaemia, hypogammaglobulinemia, hypoalbuminaemia and lymphopenia.
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Epidemiology
It may present in either children or adults.
Associations
yellow nail syndrome may associated with the primary form 6
Pathology
The condition may be localised or diffuse. It can occur from a number of 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 the duodenum and/or jejunum or alternatively from surgically resected small intestine.
Biochemical tests
faecal alpha-1-antitrypsin is often raised
Radiographic features
Imaging (e.g. CT) may be non-specific, such as showing bowel wall thickening to varying degrees.
Treatment and prognosis
Treatment has usually comprised of a low fat diet along with medium-chain triglyceride oral supplements.
Complications
malignant transformation into lymphoma: may occur in around 5% of cases 1
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.