Purpose or Case Report: Intestinal lymphangiectasia is a rare disease characterised by dilated intestinal lacteals causing loss of lymph into the small bowel lumen and resultant hypoproteinemia, hypogammaglobulinemia, hypoalbuminemia and lymphopenia. The disease may occur as a primary / congenital form (primary idiopathic intestinal lymphangectasia / Waldmann disease) or as a secondary form resulting from causes of lymphatic obstruction, such as tumor or fibrosis.

Our case report describes the workup of an adolescent girl with known Primary Intestinal Lymphangiectasia (Waldmann disease) presenting with an acute history of abdominal pain and vomiting. We present her prior imaging leading to her initial diagnosis of Waldmann disease, as well as at the time of acute presentation, with imaging features raising suspicion for, and eventually leading to histo-pathologic confirmation of small bowel B-cell lymphoma.

Via this case report poster, we hope to create awareness of this rare disease, as well as its feared association of lymphoma. Typical imaging features are presented and discussed.

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