Intestinal Lymphangiectasia Treatment & Management

  • Author: Anthony E Martin, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 5, 2012
 

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.[7] 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. 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.[8]
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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.[7] 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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Contributor Information and Disclosures
Author

Anthony E Martin, MD  Associate Professor of Medicine, Division of Gastroenterology, Hepatology, and Nutrition, Fellowship Training Program Director, University of Louisville School of Medicine

Anthony E Martin, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Physicians, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, Association of Military Surgeons of the US, Kentucky Medical Association, and Special Operations Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Richard Wright, MD  Professor and Chief, Department of Medicine, Division of Gastroenterology/Hepatology, University of Louisville School of Medicine

Richard Wright, MD is a member of the following medical societies: American College of Physician Executives, American College of Physicians, American Gastroenterological Association, American Medical Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Specialty Editor Board

Rajeev Vasudeva, MD, FACG  Clinical Professor of Medicine, Consultants in Gastroenterology, University of South Carolina School of Medicine

Rajeev Vasudeva, MD, FACG is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, Columbia Medical Society, South Carolina Gastroenterology Association, and South Carolina Medical Association

Disclosure: Pricara Honoraria Speaking and teaching; UCB Consulting fee Consulting

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Oscar S Brann, MD, FACP  Associate Clinical Professor, Department of Medicine, University of California at San Diego; Consulting Staff, Mecklenburg Medical Group

Oscar S Brann, MD, FACP is a member of the following medical societies: American Gastroenterological Association

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Additional Contributors

We wish to thank Raoul Joubran, MD, for his previous contributions to this article.

References

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