Preferred Label : Primary intestinal lymphangiectasia;
ICD-11 definition : Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated
intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible
for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia.
PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients.
The main manifestation is moderate-to-severe bilateral lower limb edema. Edema can
progress to anasarca and includes pleural effusion, pericarditis or chylous ascites.;
ICD-11 synonym : Waldmann syndrome;
Origin ID : 52162548;
Automatic exact mappings (from CISMeF team)
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated
intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible
for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia.
PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients.
The main manifestation is moderate-to-severe bilateral lower limb edema. Edema can
progress to anasarca and includes pleural effusion, pericarditis or chylous ascites.
