Medication Summary
No maintenance medications for primary intestinal lymphangiectasia are indicated, other than the use of octreotide.
Patients with secondary intestinal lymphangiectasia should continue the maintenance medications of their primary underlying disease.
Two case reports documented 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. [19] The second report showed that octreotide at 200 mcg twice daily 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. [20]
Additional cases have been reported with the successful use of octreotide, including the long-acting formulation (LAR). [14, 21]
Somatostatin analogs
Class Summary
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.
Antihemophilic agents
Class Summary
Agents like tranexamic acid can competitively inhibit activation of plasminogen to plasmin, diminishing bleeding.
Tranexamic acid (Cyklokapron)
Inhibits plasminogen activators, interfering with fibrinolysis.
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Intestinal villi of normal height with dilated lymphatics as usually seen on histology of villi in intestinal lymphagiectasia.
