Intestinal Lymphangiectasia Workup

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

Laboratory Studies

  • Hypoproteinemia: The most common laboratory finding is hypoproteinemia. Hypoalbuminemia is most prominent, and lymphocytopenia and hypogammaglobulinemia (eg, immunoglobulin A [IgA], immunoglobulin G [IgG], immunoglobulin M [IgM]) are also prominent. Cholesterol levels are not usually elevated.
  • Alpha1-antitrypsin
    • In random dry stools, alpha1-antitrypsin has been used to indirectly measure protein leakage in the GI tract. Alpha1-antitrypsin is negligibly broken down by intestinal proteases and, thus, is excreted in the stool intact. While measurement of stool alpha1-antitrypsin may serve as a good screening examination for protein loss, several studies show poor correlation between the value of alpha1-antitrypsin in the stool and its clearance measurement. In part, this is because of increased degradation of alpha1-antitrypsin in different milieus. For example, the breakdown of alpha1-antitrypsin is higher in environments where the pH level is less than 3, as in the stomach or small bowel in hyperacidity states.
    • The most specific test for protein loss in the GI tract is direct measurement of alpha1-antitrypsin clearance from plasma. Values greater than 24 cc/d in patients without diarrhea (diarrhea increases alpha1-antitrypsin clearance) and greater than 56 cc/d in those with diarrhea indicate protein loss in the GI tract. GI bleeding has also been shown to increase alpha1-antitrypsin clearance as a result of whole blood loss.
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Imaging Studies

  • Double-contrast radiographs of the small bowel may be helpful because they may show thickened folds due to intestinal edema from hypoproteinemia, nodular protrusions, and absence of mucosal ulcerations.
  • Ultrasound and CT scans are also useful in identifying dilated intestinal loops, regular and diffuse thickening of the intestinal walls, plical hypertrophy, and mesenteric edema. CT scans may help show circumferential thickening of the small bowel wall with low attenuation (< 30 H).
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Procedures

  • Endoscopy
    • Repeatedly, the role of endoscopy has been proven useful. Small bowel enteroscopy not only helps detect mucosal changes suggestive of the disease but also allows acquisition of histologic samples to establish a diagnosis.[4] }[5, 6]
    • White villi and/or spots (dilated lacteals), white nodules, and submucosal elevations are observed.
    • Xanthomatous plaques are often visualized.
  • Capsule endoscopy has also been used to help identify the characteristic changes not reachable with standard endoscopy.
  • Jejunal biopsy
    • This procedure establishes a definitive diagnosis and shows dilation of mucosal and submucosal lymphatic channels.
    • To increase the diagnostic yield, large biopsy forceps should be used when available. In addition, because of the patchy involvement of the small bowel, obtaining multiple biopsy samples from different areas is recommended.
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Histologic Findings

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

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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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