Intestinal Lymphangiectasia Treatment & Management

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

Medical Care

Treatment of patients with primary intestinal lymphangiectasia involves control of symptoms with the use of dietary, pharmaceutical, and behavioral modifications. These include the following:

  • Dietary modifications include a low-fat diet and substitution of long-chain fatty acids with medium-chain fatty acids. [9] A logical step might be to decrease the amount of salt intake, although this has not been proven to decrease edema.
  • Medications that may be used include over-the-counter remedies (eg, bulking agents, drugs to control diarrhea). Treatment of secondary causes of lymphangiectasia target the underlying disease. In several reports, octreotide has demonstrated efficacy in refractory cases. [10] A case refractory to octreotide and nutritional manipulations has been successfully treated with tranexamic acid. (This patient presented with refractory anemia due to continued GI blood loss.)
  • Treatment of patients with secondary causes of intestinal lymphangiectasia involves management of the underlying disease.
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Surgical Care

No role for surgery is evident for patients with primary intestinal lymphangiectasia; however, multiple causes of secondary intestinal lymphangiectasia can be addressed surgically, as follows:

  • A gastrectomy improves protein loss caused by giant hypertrophic gastritis (ie, Ménétrier disease).
  • Correction of a lymphenteric fistula should eliminate protein loss.
  • A pericardiectomy for severe symptomatic constrictive pericarditis should decrease marked protein loss through the GI tract.
  • Localized intestinal lymphangiectasia may be treated with surgical resection. [11]
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Consultations

Whenever suspicion for protein-losing gastroenteropathy develops, refer the patient to a gastroenterologist.

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Diet

Modify the patient's diet to reduce intake of long-chain fatty acids, substituting short-chain and medium-chain fatty acids. [2] The rationale for this is based on the following 2 principles:

  • First, long-chain fatty acids lead to chylomicrons, obstructing lymphatics and increasing lymphatic pressure and lymphocyte loss.
  • Second, medium-chain fatty acids are thought to be more water-soluble and, thus, absorbed through portal venous channels rather than through lymphatics.
  • In a literature review, Desai et al investigated the efficacy of a medium-chain fatty acid diet in the treatment of primary intestinal lymphangiectasia. [9] The authors compared the outcomes from 27 patients who were treated with medium-chain fatty acids with those from 28 patients who were not. In the fatty acid group, complete symptom resolution occurred in 17 patients (63%), compared with 10 patients (35.7%) in the other group. In addition, there was 1 death (3.7%) in the fatty acid group, while the second group experienced 5 (17.8%) deaths. The authors concluded that a medium-chain fatty acid diet is a valid option for the treatment of pediatric patients.
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Activity

No activity restrictions are suggested. Encourage patients to maintain an active lifestyle as much as their disease allows.

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