Intestinal Lymphangiectasia Follow-up

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

Further Outpatient Care

  • Although patients are encouraged to maintain a physically active lifestyle, adjustments must be made to minimize peripheral edema.
    • For most patients, postural drainage by elevating the affected extremities above the level of the heart is easy to promote compliance.
    • Suggestions to increase compliance may include the use of recliners in the evenings and the use of elastic support stockings to decrease the potential for cellulitis and lymphangitis.
  • Theoretically, limiting the patient's salt intake could decrease edema, although no reports on this subject are known. In addition, the effects are probably not significant because diuretics do not have an important role in controlling edema in patients with primary intestinal lymphangiectasia.
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Inpatient & Outpatient Medications

  • No maintenance medications for primary intestinal lymphangiectasia are indicated, other than the use of octreotide.
  • Patients with secondary intestinal lymphangiectasia should continue the maintenance medications of their primary underlying disease.
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Complications

  • Primary intestinal lymphangiectasia is associated with an increased risk of lymphoma.
  • Fibrotic entrapment of the small bowel is reported in patients with congenital intestinal lymphangiectasia.
  • Oral manifestations include gingivitis caused by poor lymphocytic function and enamel defects caused by poor calcium absorption.
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Prognosis

  • For patients with primary intestinal lymphangiectasia with an onset early in life (usually during the first decade), growth retardation usually occurs.
  • The prognosis of patients with secondary intestinal lymphangiectasia depends on the extent and severity of the underlying disease.
  • The clinical course is highly variable with about 23% of patients showing improvement and 64% remaining unchanged; the mortality rate is 13%.
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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.

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