OCTREOTIDE IN THE TREATMENT OF INTESTINAL LYMPHANGIECTASIA

Primary intestinal lymphangiectasia is characterized by dilated small bowel lymphatics and loss of lymph into the bowel lumen resulting in h ypoproteinaemia and oedema, Some patients have a more generalized lymp hatic abnormality associated with lymphoedema of the limbs and chylous pleural effusions. There is no specific treatment although enteric pr otein loss may decrease with a low-fat diet This report describes a pa tient with severe primary intestinal lymphangiectasia, associated with limb oedema and recurrent pleural effusions, who responded to treatme nt with octreotide, Before starting octreotide she required weekly int ravenous albumin infusions to maintain the serum albumin above 20 g/l, Bilateral pleural effusions repeatedly reaccumulated despite pleurect omy and subsequently tetracycline pleurodesis. Treatment with octreoti de, 200 mu g twice daily, resulted in a reduction in enteric protein l oss from 16 to 4.1% in 5 days (normal less than 1%) and the serum albu min was maintained between 22 and 26 g/l without the need for albumin infusion. Oedema in the arms resolved completely and the pleural effus ions did not reaccumulate. The mechanism of action of octreotide in th is condition appears to be due to a reduction in gut protein loss and another, as yet unidentified, action. (C) 1998 Lippincott Williams & W ilkins.