Primary Intestinal Lymphangiectasia (Waldmann’s Disease) Presenting with Chylous Effusions in a 15-Year-Old

Abstract

Primary Intestinal Lymphangiectasia (PIL) is a rare disease of unknown aetiology which presents in the paediatric age group with anasarca, diarrhoea, hypoproteinaemia, lymphoedema and chylous effusions. Tuberculosis, filariasis, chest trauma, malignancies and haematological disorders usually contribute to most cases of secondary lymphangiectasia and chylous effusions. We hereby describe a case of PIL presenting with chylous effusions which remained undiagnosed for eight years.

Keywords

• fluid accumulation
• hypoproteinaemia
• idiopathic
• lymphatic dilatation
• milky lymph