Intestinal lymphangiectasia is characterized by protein- losing enteropathy, and is diagnosed by a small bowel biopsy demonstrating dilated lymphatics in the mucosa, submucosa and serosa in the absence of coexistent inflammation. We report a case of primary intestinal lymphangiectasia that occurred in a 2-year-6-month-old girl who was treated successfully with antiplasmin and octreotide. Initially, the patient was treated with a lipid restriction diet with medium chain triglyceride oil, but her symptoms were not relieved. This case shows that antiplasmin and octreotide therapy might be useful for treating refractory primary intestinal lymphangiectasia.
Biopsy ; Diet ; Female ; Humans ; Inflammation ; Mucous Membrane ; Octreotide* ; Serous Membrane ; Triglycerides