Intestinal Lymphangiectasia

Published on 19/07/2015 by admin

Last modified 22/04/2025

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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 (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 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 SB

• Both forms result in decreased 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

CLINICAL ISSUES

• Diarrhea, steatorrhea, peripheral edema, growth retardation

• Ascites, pleural effusion, lower extremity edema

• Primary lymphangiectasia is usually diagnosed in children or in young adults

(Left) This young man had lower extremity edema, hypoproteinemia, and diarrhea. A film from a small bowel follow-through shows diffuse, nodular, thickened folds throughout the entire SB, proved to be due to lymphangiectasia.

(Right) This 36-year-old woman has chronic diarrhea and lower extremity edema. CT shows fluid-distended SB with submucosal edema . More striking is the marked edema within the mesentery .

(Left) CT in the same patient shows more of the SB wall thickening , mesenteric edema , as well as ascites and enlarged, low density lymph nodes .

(Right) CT in the same patient shows more of the fluid-distended SB and colon and additional hypodense nodes or infiltration at the root of the SB mesentery . Intestinal lymphangiectasia was the final diagnosis.

TERMINOLOGY

Synonyms

• Primary intestinal lymphangiectasia (Waldmann disease)

Definitions

• 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

Radiographic Findings

• Barium small bowel (SB) follow-through

Diffuse fold thickening of jejunum and ileum

CT Findings

• Diffuse SB wall thickening with submucosal edema

May have “halo” sign of edema between enhanced mucosa and serosa

• Infiltration of small bowel mesentery

• ± mesenteric and retroperitoneal lymphadenopathy

May be soft tissue or lower density

• ± short-segment, nonobstructing SB intussusceptions

• ± ascites, usually small amount

• For secondary form of lymphangiectasia

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

DIFFERENTIAL DIAGNOSIS

Whipple Disease

• Probably indistinguishable from lymphangiectasia by imaging

• Clinical findings (arthralgias, pigmentation, anemia, CNS symptoms) are different

• Diagnosis by SB biopsy with PAS(+) material and bacilli

Lymphoma

• May cause or simulate lymphangiectasia

• Lymphadenopathy usually more prominent

• Less diarrhea and protein-losing enteropathy

• Diagnosis by biopsy

Intestinal Opportunistic Infections

• May cause or simulate lymphangiectasia

• Giardiasis, cryptosporidiosis, etc.

• Mycobacterial disease especially

Can cause small bowel fold thickening, mesenteric, and retroperitoneal lymphadenopathy

PATHOLOGY

General Features

• Etiology

Primary form

– Congenital abnormality of lymphatic development

Secondary form

– Lymphatic obstruction of lacteals draining SB (by tumor, infection, etc.)

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

Chylous ascites results from serosal and mesenteric lymphatic obstruction

Blockage of thoracic duct leads to chylous pleural effusions

Continual loss of protein (especially albumin) allows fluid to leak out of vessels and results in edema

• Genetics

Rare familial pattern of inheritance

• Associated abnormalities

Loss of proteins (antibodies, gamma globulins) and lymphocytes leads to impaired immune system

– But opportunistic infections are uncommon

Loss of fat-soluble vitamins, iron, and lipids can lead to nutritional deficiencies and anemia

Can be caused by any retroperitoneal or abdominal tumor, retroperitoneal fibrosis, sclerosing mesenteritis, celiac disease, scleroderma, heart failure, sarcoidosis