Primary intestinal lymphangiectasia

Abstract

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterised by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminaemia and hypogammaglobulinaemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb oedema. Oedema may be moderate-to-severe with anasarca and includes pleural effusion, pericarditis, or chylous ascites. This case of primary intestinal lymphangectasia was diagnosed of Osmania General Hospital, Afzalgunj, Hyderabad.