Secondary lymphangiectasia can be caused by any process obstructing the lymphatic system as seen in tuberculosis, Waldenstrom's macroglobulinemia, systemic lupus erythematosis, Whipple's disease, radiation and malignancy. The presenting symptom in intestinal lymphangiectasia is usually generalized edema. Most cases are diagnosed during early childhood. However, there are cases in the literature presenting later in life as primary intestinal lymphangiectasia. Patients may have no symptoms, they may present with diarrhea, vomiting, abdominal pain, steatorrhea, and protein losing enteropathy.
Laboratory studies show a decrease in albumin and total protein level.
Immunological abnormalities can be detected because of leakage of lymphocytess and immunoglobulins into the intestine. Recently, primary intestinal lymphangiectasia has been considered as a part of a generalized dysplasia of the lymphatic system which varies widely in clinical outcome and severity depending upon the anatomic location and extent of the lymphatic abnormality. The diagnosis is made by a small intestinal biopsy which shows marked dilatation of the lymph vessels of the mucosa and submucosa without associated imflammation. The radiological features reflect the underlyling histopathology with enlargement of mucosal folds by dilated lymphatics usually affecting both jejunum and ileum. Edema resulting from hypoproteinemia may also contribute to enlargement of folds especially in cases with diffuse involvement.