Intestinal Lymphangiectasia

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Background

Traditionally, protein-losing gastroenteropathies have been classified into 3 groups (depending on the mechanism of their etiology) that include the following: (1) those causing mucosal damage leading to increased permeability to protein (usually not involving mucosal ulcerations), (2) those with mucosal erosions and/or ulcerations, and (3) those in which protein loss is secondary to mechanical lymphatic obstruction.

While a more detailed discussion on Protein-Losing Enteropathy is presented in another article, this article specifically addresses intestinal lymphangiectasia.

Pathophysiology

Intestinal lymphangiectasia is a disease characterized by hypoproteinemia, edema, and lymphocytopenia, resulting from dilatation of intestinal lymphatics and loss of lymph fluid into the gastrointestinal (GI) tract.[1] This leads to immunologic abnormalities, including hypogammaglobulinemia, anergy, and impaired allograft rejection. In addition to the loss of other serum components (eg, lipids), iron and certain trace metals may also be affected.[2]

Epidemiology

Frequency

United States

Frequency in the United States and internationally is unknown.

Mortality/Morbidity

Morbidity is related to the pathophysiology of this disease. Edema and diarrhea are predominant clinical features; however, the following negative sequelae are also observed:

Race

No racial predilection exists.

Sex

The male-to-female ratio is 3:2.

Age

Intestinal lymphangiectasia can be primary (ie, congenital), in which case it affects children and young adults (mean age of onset, 11 y). The diagnosis in these cases often occurs during the first decade of life, with the first manifestations being persistent diarrhea and peripheral edema. This condition can also be secondary to other disease states, thus affecting older adults.[3] In a series from Japan, the average age at onset was 22.9 years.

History

Physical

Causes

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Intestinal biopsy results reveal the characteristic dilatation of the lymph vessels of the mucosa and submucosa without any evidence for inflammation.

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:

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:

Consultations

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

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:

Activity

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

Medication Summary

Two case reports document the use of octreotide to control symptoms in refractory cases. In the first report, octreotide improved symptoms, findings on scintigraphy and endoscopy, and histology of the duodenum in a patient with intestinal lymphangiectasia. The second report showed that octreotide at 200 mcg bid resulted in reduction in enteric protein loss from 16% to 4.1% in 5 days, and albumin infusions, which were necessary to maintain an acceptable level, were eliminated in a single patient with intestinal lymphangiectasia. Additional cases have been reported with the successful use of octreotide, including the long-acting formulation (LAR).

Octreotide (Sandostatin)

Clinical Context:  Acts in a similar fashion to the hormone somatostatin. Very potent inhibitor of growth hormone, glucagon, and insulin. Markedly decreases splanchnic blood flow and suppresses LH response to GnRH. Has a strong suppressive effect of GI hormones, including gastrin, motilin, secretin, and pancreatic polypeptide. Because of its suppressive effects on GI tract, octreotide is used in a variety of GI diseases, such as VIPoma and carcinoid tumors.

Class Summary

Used to inhibit effects of GI hormones.

Tranexamic acid (Cyklokapron)

Clinical Context:  Inhibits plasminogen activators, interfering with fibrinolysis.

Class Summary

Agents like tranexamic acid can competitively inhibit activation of plasminogen to plasmin, diminishing bleeding.

Further Outpatient Care

Inpatient & Outpatient Medications

Complications

Prognosis

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

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

Disclosure: Nothing to disclose.

Specialty Editors

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

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

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

Disclosure: Nothing to disclose.

Chief Editor

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

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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