Medical Care
The treatment of patients with primary intestinal lymphangiectasia (PIL) includes dietary, pharmaceutical, and behavioral modifications. Note the following:
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Medications to consider include over-the-counter agents (eg, bulking agents like fiber and anti-diarrheal drugs such as loperamide).
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In several reports, octreotide has shown efficacy in refractory cases, as octreotide theoretically decrease intestinal absorption of fats. [19] Troskot et al reported a case of a 42-year-old man with primary intestinal lymphangiectasia, in which therapeutic resolution was achieved only with octreotide. A slow-release form of octreotide led to partial remission. [20] In another instance, a case of intestinal lymphangiectasia refractory to octreotide and nutritional manipulations was successfully treated with tranexamic acid. The patient had presented with refractory anemia due to ongoing gastrointestinal blood loss.
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Pollack and colleagues reported a case of PIL in a female patient with tuberous sclerosis complex (TSC) and a TSC2 mutation. A trial of the mTOR inhibitor rapamycin (sirolimus) yielded improvements in her clinical symptoms and abnormal laboratory values. [21] Tan et al also described a case in which PIL was the initial presentation of TSC. [22] Sirolimus and everolimus have proven to be effective for young pediatric patients with extensive lymphangiectasia. Sirolimus affects lymphatic endothelial cells by altering mTOR signaling, suppressing lymphatic sprouting and proliferation, and inducing apoptosis. [23]
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Tranexamic acid have been used in some cases of refractory lymphangiectasia with clinical improvement. [24] The mechanism of antiplasmin therapy involves normalizing tissue fibrinolytic activity. Elevated fibrinolytic activity, believed to contribute to intestinal protein loss, is considered a factor in this process. [23]
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Other less substantiated therapies include steroids and intravenous albumin infusions; however, there is insufficient evidence to recommend their routine use for this condition. [7]
The treatment of secondary intestinal lymphangiectasia revolves addressing and managing the underlying disease. [4]
Consultations
Whenever there is a suspicion of protein-losing gastroenteropathy, it is advisable to refer the patient to a gastroenterologist.
Surgical Care
Surgery has no established role in primary intestinal lymphangiectasia. However, surgical interventions may be considered for addressing secondary intestinal lymphangiectasia due to various causes. Note the following:
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Gastrectomy effectively alleviates protein loss resulting from giant hypertrophic gastritis (eg, Ménétrier disease).
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Correction of a lymphenteric fistula can eliminate protein loss.
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Pericardiectomy, when necessary for severe symptomatic constrictive pericarditis, can significantly reduce protein loss through the gastrointestinal tract.
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Localized intestinal lymphangiectasia may be treated with surgical resection. [25]
Diet
Dietary modifications involve adhering to a low-fat diet and replacing long-chain fatty acids with medium-chain fatty acids. [4, 26] The rationale for this dietary modification includes the following:
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Long-chain fatty acids result in chylomicron formation, which obstructs lymphatic vessels, elevates lymphatic pressure, and causes lymphocyte loss.
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Medium-chain fatty acids are considered more water-soluble and are absorbed through portal venous channels instead of lymphatics, thereby decreasing strain on the lacteals. [5]
In a literature review, Desai et al investigated the efficacy of a medium-chain fatty acid diet for treating primary intestinal lymphangiectasia in 27 patients, comparing the results to those of 28 control patients. In the fatty acid group, 17 patients (63%) experienced complete symptom resolution, while in the non-fatty acid group, this figure was 10 patients (35.7%). Moreover, the fatty acid group had 1 death (3.7%), whereas the second group had 5 deaths (17.8%). The authors concluded that a medium-chain fatty acid diet is a viable treatment option for pediatric patients. [26]
For patients who do not respond to a low-fat diet, enteral nutritional therapy (including elemental, semi-elemental, and polymeric diets) may become necessary. [5] In event of severe malnutrition, partial or total parenteral nutrition (TPN) can be considered, although enteral nutrition is usually preferred. [27]
Theoretically, limiting the patient's salt intake could decrease edema, although no known reports address this issue. Moreover, the impact of dietary salt limitation is likely insignificant since diuretics do not play a significant role in controlling edema in patients with primary intestinal lymphangiectasia.
Activity
No specific activity restrictions are recommended. Patients should be encouraged to follow an active lifestyle within the limits of their condition. For patients with peripheral edema, elevating the affected limbs above heart level can improve postural drainage of lymph. Additionally, we recommend using recliners and elastic support stockings to reduce edema and minimize the risk of cellulitis and lymphangitis.
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Intestinal villi of normal height with dilated lymphatics as usually seen on histology of villi in intestinal lymphagiectasia.
