Intestinal Lymphangiectasia

Primary intestinal lymphangiectasia (Waldmann disease)

• Rare congenital disorder characterized by hypoproteinemia, peripheral edema, and lymphocytopenia resulting from loss of lymphatic fluid into intestine

• Important cause of protein-losing enteropathy

Secondary: Much more common

• Causes include mesenteric or retroperitoneal node dissection (obliterates lymphatics)

• Right heart failure; fibrosing mesenteritis; retroperitoneal fibrosis are also implicated

Contrast-enhanced, multiplanar CT is 1st-line imaging test

Diffuse small bowel (SB) wall thickening with submucosal edema

Infiltration of small bowel mesentery

± mesenteric and retroperitoneal lymphadenopathy

± short segment, nonobstructing SB intussusceptions

For secondary form of lymphangiectasia

• May see signs of surgery, cardiac failure, retroperitoneal fibrosis or tumor, tuberculosis, etc.

Whipple disease, lymphoma, intestinal opportunistic infections

Both primary & secondary forms result in decreased absorption of chylomicrons and fat-soluble vitamins, excessive leakage of lymph into bowel lumen, and excessive loss of protein

Laboratory findings: Low serum albumen, gamma globulins, cholesterol, fat-soluble vitamins

Diagnosis is made by intestinal biopsy

Primary intestinal lymphangiectasia (Waldmann disease)

Rare disease or syndrome characterized by hypoproteinemia, peripheral edema, and lymphocytopenia resulting from loss of lymphatic fluid into intestine

Important cause of protein-losing enteropathy