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Intestinal lymphangiectasia — RACP Paediatrics MCQ

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HardGIIntestinal lymphangiectasiaRACP Paediatrics

A 3-year-old presents with diarrhoea, peripheral oedema, and hypoalbuminaemia. Small bowel biopsy shows dilated lymphatic channels in the intestinal villi. Lymphocyte count is low. Immunoglobulins are all low. Stool alpha-1 antitrypsin clearance is markedly elevated. What is the most likely diagnosis?

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Correct answer: C — Intestinal lymphangiectasia

Dilated mucosal lymphatic channels on biopsy with protein-losing enteropathy (hypoalbuminaemia, lymphopenia, hypogammaglobulinaemia) is primary intestinal lymphangiectasia (Waldmann disease). Protein and lymphocytes are lost through leaking lymphatics. Treatment includes a high-protein, low-fat diet supplemented with medium-chain triglycerides (MCTs) which are absorbed directly into the portal circulation, bypassing lymphatics.

Reference: RACP Paediatric Curriculum – GI; RCH Melbourne – 2023