eMedicine Specialties > Gastroenterology > Intestine

Intestinal Lymphangiectasia: Treatment & Medication

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

Treatment

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:

  • Dietary modifications include a low-fat diet and substitution of long-chain fatty acids with medium-chain fatty acids.4 A logical step might be to decrease the amount of salt intake, although this has not been proven to decrease edema.
  • Medications that may be used include over-the-counter remedies (eg, bulking agents, drugs to control diarrhea). Treatment of secondary causes of lymphangiectasia target the underlying disease. In several reports, octreotide has demonstrated efficacy in refractory cases. A case refractory to octreotide and nutritional manipulations has been successfully treated with tranexamic acid. (This patient presented with refractory anemia due to continued GI blood loss.)
  • Treatment of patients with secondary causes of intestinal lymphangiectasia involves management of the underlying disease.

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:

  • A gastrectomy improves protein loss caused by giant hypertrophic gastritis (ie, Ménétrier disease).
  • Correction of a lymphenteric fistula should eliminate protein loss.
  • A pericardiectomy for severe symptomatic constrictive pericarditis should decrease marked protein loss through the GI tract.
  • Localized intestinal lymphangiectasia may be treated with surgical resection.5

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:

  • First, long-chain fatty acids lead to chylomicrons, obstructing lymphatics and increasing lymphatic pressure and lymphocyte loss.
  • Second, medium-chain fatty acids are thought to be more water-soluble and, thus, absorbed through portal venous channels rather than through lymphatics.
  • In a literature review, Desai et al investigated the efficacy of a medium-chain fatty acid diet in the treatment of primary intestinal lymphangiectasia.4 The authors compared the outcomes from 27 patients who were treated with medium-chain fatty acids with those from 28 patients who were not. In the fatty acid group, complete symptom resolution occurred in 17 patients (63%), compared with 10 patients (35.7%) in the other group. In addition, there was 1 death (3.7%) in the fatty acid group, while the second group experienced 5 (17.8%) deaths. The authors concluded that a medium-chain fatty acid diet is a valid option for the treatment of pediatric patients.

Activity

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

Medication

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).

Somatostatin analogs

Used to inhibit effects of GI hormones.


Octreotide (Sandostatin)

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.

Adult

50 mcg bid/tid usually administered SC (IV use also acceptable); titration to higher doses is frequently required

Pediatric

Not established; 1-10 mcg/kg SC recommended and usually tolerated well

May reduce effects of cyclosporine; patients on insulin, oral hypoglycemics, beta-blockers, and calcium channel blockers may need dosage adjustments

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Adverse effects are primarily related to altered GI motility and include nausea, abdominal pain, diarrhea, and increased incidence of gallstones and biliary sludge; because of alteration in counter-regulatory hormones (ie, insulin, glucagon, GH), hypo- or hyperglycemia may be observed; bradycardia, cardiac conduction abnormalities, and arrhythmias have been reported; because of inhibition of TSH secretion, hypothyroidism may also occur; exercise caution in patients with renal impairment; cholelithiasis may occur

Antihemophilic agents

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


Tranexamic acid (Cyklokapron)

Inhibits plasminogen activators, interfering with fibrinolysis.

Adult

25 mg/kg PO tid/qid (dosage recommended for tooth extraction); dose used in case reports for lymphangiectasia 1-2 g PO tid

Pediatric

Administer as in adults

Concomitant administration with thrombolytics may reduce efficacy of both drugs

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Caution in renal impairment, history of thromboembolic disease, or if DIC

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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