The prominent white speckles seen in the enterocytes lining the duodenum represent lymph in dilated lymphatics. Lymphatic congestion can be seen normally to a mild degree postprandially and in a variety of conditions that increase central venous pressure such as congestive heart failure. When seen to this degree, however, the diagnosis of Chronic Intestinal Lymphangiectasia should be suspected. The diagnosis can be made on biopsy, and when the tissue is sampled, the biopsy site can be seen to ooze this milky white substance which is in fact chyle. This observation is indicative of increased lymphatic pressure and this diagnosis of chronic intestinal lymphangiectasia. The pathology reveals multiple dilated lymphatics consistent with the diagnosis of Chronic Intestinal lymphangiectasia.
Patient's with Chronic Intestinal Lymphangiectasia often develop a constellation of symptoms and findings that include edema, lymphopenia, hypoproteinemia, and diarrhea associated with a protein losing enteropathy. Diagnosis can be made on biopsy, and a stool alpha 1 anti-trypsin antibody level should be checked to confirm the diagnosis of a protein losing enteropathy.
The prominence of this lymphatic pattern can vary from patient to patient as seen in this second example. Note the decreased density of the lymph containing enterocytes.
Treatment includes a fat restricted diet, enriched in medium chain triglycerides that don?t require lymphatics for absorption.
Peter B. Kelsey, M.D., Harvard Medical School, Massachusetts General Hospital
Robert H. Schapiro, M.D., Harvard Medical School, Massachusetts General Hospital