INTESTINAL LYMPHANGIECTASIA

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Abstract
The roentgenographic features of 20 patients with intestinal lymphangiectasia are presented. Five had normal small bowel studies. Of the 15 patients with an abnormal small bowel study at initial evaluation 9 had a characteristic appearance consisting of enlargement of the valvulae conniventes of both jejunum and ileum, increased secretions, and minimal or absent dilatation of the bowel. Four patients had nodular mucosal defects. Two patients had a striking, irregularly serrated margin to the ileum. One patient developed an internal hernia secondary to dense adhesions found after a partial jejunal resection. A roentgenographic differential diagnosis of the characteristic appearance of the disease is discussed. Pedal lymphangiographic findings of 9 patients were abnormal in 7, supporting the concept that this disease is a systemic lymphatic dysplasia. Lower extremity abnormalities were similar to those associated with primary lymphedema. In the abdomen 1 patient had hypoplasia of lymph nodes, another showed moderate contrast material reflux into mesenteric lymphatics associated with possible obstruction of the cisterna chyli and enlarged lymph nodes. Two patients had enlarged, tortuous thoracic ducts, and 2 patients had probable obstruction or absence of this structure. Recently, a patient followed for 15 years with intestinal lymphangiectasia has developed disseminated lymphosarcoma.

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