Intestinal lymphangiectasia

Other small bowel enteropathies (e.g., Whipple s disease, tropical sprue, amyloid, intestinal lymphangiectasia)... 

Primary intestinal lymphangiectasia is a rare disorder characterised by a protein-losing gastroentero-pathy leading to secondary hypoproteinaemia due to lymphatic vessel obstruction and the loss of lymphatic fluid in the gastrointestinal tract. It is believed to be a congenital disorder, although the time of its clinical onset can vary widely. 

A strict dietary regimen including only medium-chain triglycerides (MCT) is recommended as the initial treatment. It has been reported that tissue fibrinolytic activity in the mucosa of intestinal lymphangiectasia is increased, and there are some reports indicating that antiplasmin therapy can dramatically reduce protein loss by inhibiting lymphatic permeability some authors have recently observed that octreotide, a somatostatin analogue, may play a therapeutic role. 

Frequent US findings of primary intestinal lymphangiectasia are a markedly dilated small intestinal loop with increased (mixed but mainly echogenic) fluid content (Fig. 10.1), and small bowel tract with regular and diffuse bowel wall thickening and mesenteric oedema (Fig. 10.2). 

Loreti L, Masselli G, Melisi MT et al (2003) Intestinal lymphangiectasia. Rays 28 409-416... 

Pancreas Pancreatitis has previously been associated with use of a somatostatin analogue, and another case has been reported in 1 of 13 children who were given subcutaneous octreotide 15-20 micrograms/kg bd for 3-37 months for refractory primary intestinal lymphangiectasia [70 ]. 

Sari S, Baris Z, Dalgic B. Primary intestinal lymphangiectasia in children is octreotide an effective and safe option in the treatment J Pediatr Gastroenterol Nutr 2010 51(4) 454-7. 

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