eMedicine Specialties > Gastroenterology > Intestine

Intestinal Lymphangiectasia

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

Introduction

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

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. In a series from Japan, the average age at onset was 22.9 years.

Clinical

History

  • Patients usually present in childhood with edema and nonbloody diarrhea. Edema may be unilateral or bilateral, depending on the site of the lesion. Edema in primary intestinal lymphangiectasia is usually bilateral, while the secondary type often manifests as unilateral edema and is caused by various neoplastic, infiltrative, and inflammatory lesions affecting one side of the body.
  • Frequently, steatorrhea, malabsorption, lymphocytopenia, and hypogammaglobulinemia are present.
  • Ascites (often chylous ascites) and chylous pleural effusions are also reported in patients with long-standing lymphangiectasia.
  • If the onset of disease occurs during the early part of the first decade of life, growth retardation usually ensues.
  • Despite hypogammaglobulinemia, opportunistic infections rarely occur, although lymphocytopenia predisposes patients to abnormal cellular immunities, including homograft rejection and cutaneous anergy.

Physical

  • Primary intestinal lymphangiectasia2
    • Peripheral edema is noted on physical examination in patients with primary intestinal lymphangiectasia.
    • Macular edema on funduscopic examination has been reported and is a cause of reversible blindness. Secondary lymphangiectasia may involve multiple physical findings, depending on the etiology.
  • Pachydermoperiostosis has been associated with protein-losing enteropathy due to intestinal lymphangiectasia. Pachydermoperiostosis is a rare hereditary disease characterized by clubbing of the fingers, periostosis, and skin changes.

Causes

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

  1. Vignes S, Carcelain G. Increased surface receptor Fas (CD95) levels on CD4+ lymphocytes in patients with primary intestinal lymphangiectasia. Scand J Gastroenterol. 2009;44(2):252-6. [Medline].

  2. Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis. Feb 22 2008;3:5. [Medline].

  3. Safatle-Ribeiro AV, Iriya K, Couto DS, et al. Secondary lymphangiectasia of the small bowel: utility of double balloon enteroscopy for diagnosis and management. Dig Dis. 2008;26(4):383-6. [Medline].

  4. Desai AP, Guvenc BH, Carachi R. Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. Eur J Pediatr Surg. Aug 2009;19(4):241-5. [Medline].

  5. Kim NR, Lee SK, Suh YL. Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. J Pediatr Surg. Oct 2009;44(10):e13-7. [Medline].

  6. Aoyagi K, Iida M, Yao T, Matsui T, Okada M, Fujishima M. Intestinal lymphangiectasia: value of double-contrast radiographic study. Clin Radiol. Nov 1994;49(11):814-9. [Medline].

  7. Aoyagi K, Iida M, Yao T, Matsui T, Okada M, Oh K. Characteristic endoscopic features of intestinal lymphangiectasia: correlation with histological findings. Hepatogastroenterology. Jan-Feb 1997;44(13):133-8. [Medline].

  8. Ballinger AB, Farthing MJ. Octreotide in the treatment of intestinal lymphangiectasia. Eur J Gastroenterol Hepatol. Aug 1998;10(8):699-702. [Medline].

  9. Bouhnik Y, Etienney I, Nemeth J, Thevenot T, Lavergne-Slove A, Matuchansky C. Very late onset small intestinal B cell lymphoma associated with primary intestinal lymphangiectasia and diffuse cutaneous warts. Gut. Aug 2000;47(2):296-300. [Medline].

  10. Filik L, Oguz P, Koksal A, Koklu S, Sahin B. A case with intestinal lymphangiectasia successfully treated with slow-release octreotide. Dig Liver Dis. Oct 2004;36(10):687-90. [Medline].

  11. Fuss IJ, Strober W, Cuccherini BA, Pearlstein GR, Bossuyt X, Brown M, et al. Intestinal lymphangiectasia, a disease characterized by selective loss of naive CD45RA+ lymphocytes into the gastrointestinal tract. Eur J Immunol. Dec 1998;28(12):4275-85. [Medline].

  12. Kuroiwa G, Takayama T, Sato Y, Takahashi Y, Fujita T, Nobuoka A, et al. Primary intestinal lymphangiectasia successfully treated with octreotide. J Gastroenterol. Feb 2001;36(2):129-32. [Medline].

  13. MacLean JE, Cohen E, Weinstein M. Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy. Pediatrics. Jun 2002;109(6):1177-80. [Medline]. [Full Text].

  14. Maconi G, Molteni P, Manzionna G, Parente F, Bianchi Porro G. Ultrasonographic features of long-standing primary intestinal lymphangiectasia. Eur J Ultrasound. Aug 1998;7(3):195-8. [Medline].

  15. Medical Economics Staff. Physicians' Desk Reference. 56th ed. Montvale, NJ: Medical Economics Co; 2001.

  16. Ralph PM, Troutman KC. The oral manifestations of intestinal lymphangiectasia: case report. Pediatr Dent. Nov-Dec 1996;18(7):461-4. [Medline].

  17. Rust C, Pratschke E, Hartl W, Kessler M, Weibecke B, Sauerbruch T. Fibrotic entrapment of the small bowel in congenital intestinal lymphangiectasia. Am J Gastroenterol. Oct 1998;93(10):1980-3. [Medline].

  18. Salomons HA, Kramer P, Nikulasson S, Schroy PC. Endoscopic features of long-standing primary intestinal lymphangiectasia. Gastrointest Endosc. May 1995;41(5):516-8. [Medline].

  19. Sethuraman G, Malhotra AK, Khaitan BK, Sharma VK, Kumar R, Makharia GK, et al. Familial pachydermoperiostosis in association with protein-losing enteropathy. Clin Exp Dermatol. Jul 2006;31(4):531-4. [Medline].

  20. Sleisenger MV. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, and Management. 6th ed. Philadelphia: WB Saunders Co; 1998:369-75.

  21. Takahashi H, Imai K. What are the objectives of treatment for intestinal lymphangiectasia?. J Gastroenterol. Feb 2001;36(2):137-8. [Medline].

  22. Yamada T, Alpers DH, Laine L, et al. Textbook of Gastroenterology: Self-Assessment Review. Philadelphia: Lippincott Williams & Wilkins; 1999.

  23. Yang DM, Jung DH. Localized intestinal lymphangiectasia: CT findings. AJR Am J Roentgenol. Jan 2003;180(1):213-4. [Medline].

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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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