Intestinal Lymphangiectasia

Michael P. Federle, MD, FACR

Key Facts

Terminology

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

Imaging

Diffuse small bowel 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 cardiac failure, retroperitoneal fibrosis or tumor, tuberculosis, etc.

Top Differential Diagnoses

Whipple disease
Lymphoma
Intestinal opportunistic infections

Pathology

Primary form
- Congenital abnormality of lymphatic development
Secondary form
- Lymphatic obstruction of lacteals draining the small intestine
Both forms result in abnormal (deficient) absorption of chylomicrons and fat-soluble vitamins, excessive leakage of lymph into bowel lumen, and excessive loss of protein
Diagnosis is made by intestinal biopsy

(Left) 15-year-old girl with chronic diarrhea. Films from a small bowel series show diffuse nodular fold thickening of the transverse folds

of the entire small bowel, a nonspecific pattern, but one typical of SB lymphangiectasia. (Right) 36-year-old woman with diarrhea and leg edema. CT shows submucosal edema throughout much of the small intestine and hypodense, enlarged lymph nodes