Intestinal Lymphangiectasia Clinical Presentation

Updated: Jan 19, 2018
  • Author: Hisham Nazer, MB, BCh, FRCP, , DTM&H; Chief Editor: Burt Cagir, MD, FACS  more...
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Presentation

History

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, whereas the secondary type often manifests as unilateral edema and is caused by various neoplastic, infiltrative, and inflammatory lesions affecting one side of the body.

If the onset of disease occurs during the early part of the first decade of life, growth retardation usually ensues.

Frequently, steatorrhea, malabsorption, lymphocytopenia, and hypogammaglobulinemia are present.

Despite hypogammaglobulinemia, opportunistic infections rarely occur, although lymphocytopenia predisposes patients to abnormal cellular immunities, including homograft rejection and cutaneous anergy.

Ascites (often chylous ascites) and chylous pleural effusions are also reported in patients with long-standing lymphangiectasia.

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Physical Examination

Primary intestinal lymphangiectasia

Peripheral edema is noted on physical examination in patients with primary intestinal lymphangiectasia. [6]

Macular edema on funduscopic examination has been reported and is a cause of reversible blindness. [7]

Pachydermoperiostosis has been associated with protein-losing enteropathy due to intestinal lymphangiectasia. [8] Pachydermoperiostosis is a rare hereditary disease characterized by clubbing of the fingers, periostosis, and skin changes.

Secondary lymphangiectasia

Secondary lymphangiectasia may involve multiple physical findings, depending on the etiology.

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