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Intestinal lymphangiectasias
Other Resources UpToDate PubMed

Intestinal lymphangiectasias

Contributors: Michael W. Winter MD, Desiree Rivera-Nieves MD, Khaled Bittar MD, Nishant H. Patel MD, Paritosh Prasad MD
Other Resources UpToDate PubMed


Intestinal lymphangiectasia is a disease characterized by a protein-losing enteropathy (hypoproteinemia and edema) and lymphocytopenia due to intestinal lymphatic dilation and subsequent loss of protein-rich lymphatic fluid via the gastrointestinal tract.

The symptoms and laboratory findings are reflective of this protein loss: anasarca and bowel edema due to hypoalbuminemia, immunocompromise due to hypogammaglobulinemia, hypolipidemia, and malabsorptive diarrhea with subsequent nutritional deficiencies due to bowel edema.
Primary intestinal lymphangiectasia typically presents as a watery diarrhea, edema, and failure to thrive in the first decade of life. In adults, intestinal lymphangiectasia can be caused by many conditions (see differential diagnosis) including autoimmune, malignant, inflammatory, and vascular disease processes.


I89.0 – Lymphedema, not elsewhere classified

788291006 – Acquired lymphangiectasia
788292004 – Congenital lymphangiectasia

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Differential Diagnosis & Pitfalls

  • Celiac disease
  • Allergic gastroenteropathy
  • Eosinophilic gastroenteritis
  • Collagenous Colitis
  • Systemic lupus erythematosus
  • Congenital disorder of glycosylation
  • Lactose intolerance
  • Congenital lymphatic malformation
  • Tropical sprue
  • Ménétrier disease
  • Helicobacter pylori infection
  • Amyloidosis (AL amyloidosis, AA amyloidosis)
  • Chronic pancreatitis
  • Retroperitoneal fibrosis
  • Mixed connective tissue disease
  • Scleroderma
  • Congestive heart failure
  • Portal hypertensive enteropathy (see Portal hypertension)
  • Inflammatory bowel disease (Crohn disease, Ulcerative colitis)
  • Retroperitoneal or Gastric cancer
  • Lymphoma
  • Sarcoidosis
  • AIDS-associated Kaposi sarcoma
  • Drug-induced enteropathy
  • Tuberculous enteritis
  • Mesenteric or lymphatic malignancy
  • Whipple disease
  • Graft-versus-host disease (Acute graft-versus-host disease, Chronic graft-versus-host disease)
  • Postoperative complication (particularly Fontan procedure)
  • Infectious Colitis (Clostridioides difficile colitis, Giardiasis, parasitic infection [eg, Amebic colitis], pylori)

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Last Updated:11/01/2022
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Intestinal lymphangiectasias
A medical illustration showing key findings of Intestinal lymphangiectasias (Common Findings) : Diarrhea, Fatigue, Nausea, Vomiting, Failure to thrive, Hypoalbuminemia, Peripheral leg edema, Asthenia
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