eMedicine Specialties > Gastroenterology > Intestine

Intestinal Lymphangiectasia: Follow-up

Author: Anthony Martin, MD, Assistant Professor, Department of Medicine, Division of Gastroenterology, University of Louisville School of Medicine
Coauthor(s): Richard Wright, MD, Professor and Chief, Department of Medicine, Division of Gastroenterology/Hepatology, University of Louisville School of Medicine
Contributor Information and Disclosures

Updated: Nov 9, 2009

Follow-up

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.

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.

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.

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%.

Miscellaneous

Medicolegal Pitfalls

  • Establishing the diagnosis as early as possible helps the clinician to offer the best treatment possible and to avoid serious consequences.
    • While treatment of primary intestinal lymphangiectasia is limited, especially in young children (growth retardation inevitably occurs), reaching an early diagnosis maximizes the chance of avoiding or minimizing the degree of growth retardation.
    • Identifying the causes of secondary lymphangiectasia is of paramount importance not only to eliminate the symptoms but also to stop, delay, or even reverse the progression of the primary disease and its deleterious sequelae.
 
Acknowledgments

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



More on Intestinal Lymphangiectasia

Overview: Intestinal Lymphangiectasia
Differential Diagnoses & Workup: Intestinal Lymphangiectasia
Treatment & Medication: Intestinal Lymphangiectasia
Follow-up: Intestinal Lymphangiectasia
References
Further Reading
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References

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Further Reading

Related eMedicine topics
Hypogammaglobulinemia
Infections in the Immunocompromised Host
Malabsorption
Protein-Losing Enteropathy (Gastroenterology)
Protein-Losing Enteropathy (Pediatrics: General Medicine)

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Keywords

intestinal lymphangiectasia, lymphangiectasia, protein-losing enteropathy, hypoproteinemia, medium-chain triglycerides, medium-chain fatty acids, medium-chain triglyceride, primary intestinal lymphangiectasia, congenital intestinal lymphangiectasia, Milroy disease, protein-losing gastroenteropathies, lymphocytopenia, hypogammaglobulinemia

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Contributor Information and Disclosures

Author

Anthony Martin, MD, Assistant Professor, Department of Medicine, Division of Gastroenterology, University of Louisville School of Medicine
Anthony Martin, MD is a member of the following medical societies: 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.

Medical Editor

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

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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.

CME Editor

Alex J Mechaber, MD, FACP, 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; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
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 and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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