Management of Intestinal Lymphangiectasia
The cornerstone of treatment is a strict low-fat diet with long-chain triglycerides restricted to <5% of total energy intake, combined with medium-chain triglyceride (MCT) supplementation to >20% of total energy intake, along with high protein intake of at least 1.2 g/kg/day and energy intake of at least 30 kcal/kg/day. 1, 2
Dietary Management (First-Line Therapy)
Restrict long-chain triglycerides to <5% of total energy intake to prevent chyle engorgement of intestinal lymphatic vessels and their subsequent rupture with lymph loss 1, 2, 3
Supplement with medium-chain triglycerides (MCT) to >20% of total energy intake because MCTs bypass intestinal lymphatics and are absorbed directly into the portal venous circulation, avoiding lacteal overloading 1, 2, 3
Ensure protein intake of at least 1.2 g/kg actual body weight/day to compensate for ongoing protein losses through the enteropathy 1, 2
Maintain energy intake of at least 30 kcal/kg actual body weight/day to address the frequent malnutrition in these patients 1, 2
Provide supplementation with essential fatty acids and fat-soluble vitamins (A, D, E, K) due to malabsorption from the fat-restricted diet 4
This dietary control must be permanent and lifelong, as clinical and biochemical findings reappear after low-fat diet withdrawal 3, 4
Assessment of Disease Extent
Perform magnetic resonance lymphangiography to assess the extent and location of abnormal lymphatic lesions, as this determines treatment options beyond dietary therapy 5
Consider videocapsule endoscopy to evaluate the extent of disease throughout the small bowel when standard endoscopy is not contributory 3, 4
Treatment Based on Disease Extent
Focal/Segmental Disease
Surgical intestinal resection is indicated for rare cases with focal, short-segment intestinal lymphangiectasia that fail dietary therapy 5, 3, 4
Radiologic lymphatic embolization is an alternative for patients with focal abnormal lymphatic lesions who are not surgical candidates 5, 6
Diffuse/Extensive Disease
Sirolimus (mTOR inhibitor) is the first-line pharmacological choice for patients with extensive lymphangiectasis, showing clinical improvement within 1-4 months of treatment 6, 4
Octreotide (somatostatin analogue) has inconsistent efficacy and is not an optimal choice for extensive lymphangiectasis, with recurrence often occurring after 3 months of use 6, 4
Budesonide may be helpful for patients with hypoalbuminemia poorly responsive to other therapies, though close monitoring for hypercortisolism is necessary 7, 2
Antiplasmin and corticosteroids have been proposed but show inconsistent effectiveness 3
Supportive Management
Diuretics and albumin infusions may be useful adjuncts to dietary therapy for managing edema and serous effusions 4
Parenteral nutrition support should be considered during acute exacerbations or severe malnutrition 8
Monitoring and Follow-Up
Measure serum albumin levels regularly to evaluate treatment efficacy 1, 2
Monitor total protein, immunoglobulin concentrations, and lymphocyte counts as these remain at risk of being below normal even with treatment 8
Perform anthropometric measurements (weight, height, BMI) at regular intervals to ensure no growth retardation in children 8
Conduct lifelong clinical and biological monitoring due to the chronic nature of the disease and risk of complications 4
Critical Pitfalls to Avoid
Do not delay dietary modification while awaiting definitive diagnosis—the low long-chain triglyceride/high MCT diet is safe and beneficial across all etiologies 2
Do not rely solely on dietary management for all cases—extensive disease requires pharmacological intervention with sirolimus 6
Do not discontinue the fat-free diet once symptoms improve—this is a lifelong requirement as symptoms recur upon diet withdrawal 3, 4
Do not overlook surveillance for B-cell lymphoma, which can occur as a complication in adults with intestinal lymphangiectasia, affecting the gastrointestinal tract or extra-intestinal sites 3, 4