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. The pattern of fold enlargement is uniform, orderly and symmetrical in a single loop. A stacked coin appearance is common and the folds may have a regular biconvex appearance.
Dilatation is minimal or absent, which is important in differentiating from other disorders.
Nodular filling defects corresponding to dilated lymph vessels can also be seen. Excessive intestinal secretions result in dilution of the barium column at times in association with fragmentation and segmentation of the contrast material. Diseases causing thickening of the folds and nodular filling defects include such entities as giardiasis, cystic fibrosis, strongyloidosis in the differential diagnosis. Treatment is directed towards decreasing lymph production by administering medium chain triglycerides. In secondary forms, treatment of underlying disorders results in resolution of the intestinal lymphangiectasia.