Intestinal Lymphangiectasia Treatment & Management

Updated: Jan 19, 2018
  • Author: Hisham Nazer, MB, BCh, FRCP, , DTM&H; Chief Editor: Burt Cagir, MD, FACS  more...
  • Print
Treatment

Medical Care

Treatment of patients with primary intestinal lymphangiectasia involves control of symptoms with the use of dietary, pharmaceutical, and behavioral modifications, such as the following:

  • Dietary modifications include a low-fat diet and substitution of long-chain fatty acids with medium-chain fatty acids. [13] A logical step might be to reduce 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. [14] In a case report of a 42-year-old man with primary intestinal lymphangiectasia, Troskot et al found that only octreotide provided therapeutic resolution. [15] Following the use of a slow-release form of octreotide, the patient had a partial remission. A case of intestinal lymphangiectasia refractory to octreotide and nutritional manipulations was successfully treated with tranexamic acid. (This patient presented with refractory anemia due to continued gastrointestinal [GI] blood loss.)
  • Pollack and colleagues reported a case of primary intestinal lymphangiectasia (PIL) in a female patient with tuberous sclerosis complex (TSC) and a TSC2 mutation in whom a trial of the mTOR inhibitor rapamycin was very effective. [16] There was improvement in her clinical symptoms of PIL as well as in abnormal laboratory values. The investigators concluded that these findings suggest that PIL is a rare manifestation of TSC, thereby justifying the use of mTOR inhibitors in future studies. [16] Tan et al also reported a patient in whom PIL was the first manifestation of TSC. [17]

Treatment of patients with secondary causes of intestinal lymphangiectasia involves management of the underlying disease.

Consultations

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

Next:

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 gastrointestinal tract.
  • Localized intestinal lymphangiectasia may be treated with surgical resection. [18]
Previous
Next:

Diet

Modify the patient's diet to reduce the intake of long-chain fatty acids, substituting short-chain and medium-chain fatty acids. [4] The rationale for this is based on the following two principles:

  • Long-chain fatty acids lead to chylomicrons, obstructing lymphatics and increasing lymphatic pressure and lymphocyte loss.
  • 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 in 27 patients compared to results of 28 control patients. [13] In the fatty acid group, complete symptom resolution occurred in 17 patients (63%), compared to 10 patients (35.7%) in the non-fatty acid group. In addition, there was 1 death (3.7%) in the fatty acid group, whereas 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. [13]

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.

Previous
Next:

Activity

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

Previous