DISORDERS OF THE INTESTINAL MESENTERIC LYMPHATIC-SYSTEM

Disorders of the intestinal lymphatic transport system are rare and ty pically associated with protein losing enteropathy (PLE). Hypoproteine mia caused by intestinal lymphangiectasia is often associated with lym phedema of the legs and occasionally with chyluria, chylometrorrhea an d chylous ascites. This article examines the varied presentations of l ymphangiectasia syndromes including its pathophysiology. Diagnosis is based on signs and symptoms, specific laboratory findings, and confirm ed by contrast small bowel series, lymphography and best of all laparo scopy. We describe 12 patients with PLE secondary to primary intestina l lymphangiectasia (1980-1991). Treatment was non-operative (dietary) in 8 patients and surgical in 4 including segmental resections of the jejunum in two, lymphatic-mesenteric venous anastomosis in one, and pe ritoneal-venous (Le Veen) shunt in one with overall satisfactory resul ts.