How do you treat Primary lymphangiectasia?

• A. Low fat diet; Anti-inflammatory tapering course of steroids ~1 mg/kg/d
• B. High fat diet; Antibiotics
• C. High protein diet only
• D. No treatment

Answer: (A)

Primary lymphangiectasia causes protein-losing enteropathy because dilated intestinal lymphatics leak protein into the gut. Treatment focuses on reducing lymph flow through the gut and limiting ongoing protein loss. A diet very low in long-chain fats decreases chyle production, since long-chain fats require lymphatic transport; incorporating medium-chain triglycerides provides calories and essential fats that are absorbed directly into the portal vein, bypassing the lymphatics. In some patients, a short course of corticosteroids around 1 mg/kg per day with taper can reduce intestinal inflammation and help decrease protein loss. The other options don’t address the lymphatic leak or can worsen it (high-fat diet), or are insufficient on their own (no treatment, antibiotics alone, or a high-protein diet only).