Intestinal lymphangiectasias
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Synopsis

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.
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.
Codes
ICD10CM:
I89.0 – Lymphedema, not elsewhere classified
SNOMEDCT:
788291006 – Acquired lymphangiectasia
788292004 – Congenital lymphangiectasia
I89.0 – Lymphedema, not elsewhere classified
SNOMEDCT:
788291006 – Acquired lymphangiectasia
788292004 – Congenital lymphangiectasia
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Differential Diagnosis & Pitfalls
- Celiac disease
- Allergic gastroenteropathy
- Eosinophilic gastritis
- Collagenous colitis
- Systemic lupus erythematosus
- Carbohydrate-deficient glycoprotein syndrome
- Lactose intolerance
- Congenital lymphatic malformation
- Tropical sprue
- Ménétrier disease
- Helicobacter pylori
- 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 abdominal carcinoma
- Lymphoma
- Sarcoidosis
- AIDS-associated Kaposi sarcoma
- Drug-induced enteropathy
- Mesenteric tuberculosis
- Mesenteric or lymphatic malignancy
- Whipple disease
- Graft-versus-host disease (acute, chronic)
- Postoperative complication (particularly Fontan procedure)
- Infectious colitis (Clostridioides difficile, Giardia, parasitic infection [eg, amebic colitis], H pylori)
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Last Updated:11/01/2022