A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl

Abstract

Key Clinical Message Primary intestinal lymphangiectasia is a rare disease that affects children and young adults, causing mainly gastrointestinal disorders that lead to edema and immunologic abnormalities. The majority of patients typically present bilateral lower limb edema, along with pericarditis, pleural effusion, or chylous ascites. The goal of the treatment is to restrict the consumption of long‐chain fats and administer a formula that includes protein and medium‐chain triglycerides. Here, we report 11‐year‐old Syrian girl presented to the hospital with 2 years history of diarrhea and generalized edema. Furthermore, investigation showed ascites and pleural effusion. The disease was detected by several biopsies from duodenum that showed dilation of the lymph vessels within the lamina propria without any evidence for inflammation. She was managed by modifying her diet and albumin transfusion, which caused significant improvement in the child's condition.

Keywords

• case report
• edema
• gastrointestinal
• primary intestinal lymphangiectasia