Intestinal Lymphangiectasia Clinical Presentation

Updated: Dec 19, 2014
  • Author: Hisham Nazer, MB, BCh, FRCP, , DTM&H; Chief Editor: Julian Katz, MD  more...
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Presentation

History

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  • Patients usually present in childhood with edema and nonbloody diarrhea. Edema may be unilateral or bilateral, depending on the site of the lesion. Edema in primary intestinal lymphangiectasia is usually bilateral, while the secondary type often manifests as unilateral edema and is caused by various neoplastic, infiltrative, and inflammatory lesions affecting one side of the body.
  • Frequently, steatorrhea, malabsorption, lymphocytopenia, and hypogammaglobulinemia are present.
  • Ascites (often chylous ascites) and chylous pleural effusions are also reported in patients with long-standing lymphangiectasia.
  • If the onset of disease occurs during the early part of the first decade of life, growth retardation usually ensues.
  • Despite hypogammaglobulinemia, opportunistic infections rarely occur, although lymphocytopenia predisposes patients to abnormal cellular immunities, including homograft rejection and cutaneous anergy.
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Physical

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  • Primary intestinal lymphangiectasia [2, 4, 5]
    • Peripheral edema is noted on physical examination in patients with primary intestinal lymphangiectasia.
    • Macular edema on funduscopic examination has been reported and is a cause of reversible blindness. Secondary lymphangiectasia may involve multiple physical findings, depending on the etiology.
  • Pachydermoperiostosis has been associated with protein-losing enteropathy due to intestinal lymphangiectasia. Pachydermoperiostosis is a rare hereditary disease characterized by clubbing of the fingers, periostosis, and skin changes.
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Causes

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