Primary intestinal lymphangiectasia in children: Twelve years of experience in the diagnosis and management.

BACKGROUND AND OBJECTIVES: Primary Intestinal Lymphangiectasia (PIL) is a rare congenital and digestive disease, which could present through a broad spectrum of clinical manifestations, diagnostic and treatment management. The aim of this study was to introduce the diagnosis and nutrition treatment of children with PIL through the twelve years of experience.

METHODS AND STUDY DESIGN: The patients diagnosed with PIL admitted to the Department of Gastroenterology and Nutrition in Xinhua Hospital from June 2006 to September 2017 were included in the study.

RESULTS: Ten patients were found to have PIL, and 5 of them were male. The mean age was 66 months at the time of diagnosis and 11 months at onset. The main clinical manifestations were diarrhea, edemas and abdominal distention. Marked dilatation of the intestinal lymphatic vessels was the characteristic of the endoscopic. All the patients presented with hypoproteinemia and hypoimmunoglobulinia. Six of them were treated with parenteral nutrition, and 9 of them were treated with a low-long-chain triglycerides (LCT), high-protein diet supplemented with medium-chain triglycerides (MCT). The clinical symptoms of the patients have improved after the MCT diet therapy.

CONCLUSIONS: PIL should be considered first when there are clinical manifestations of chronic diarrhea, edema and abdominal distention, and biochemical results indicated the hypoproteinemia and hypoimmunoglobulinia, and the general treatment is invalid. Gastroscopy and E-colonoscopy with biopsies are the preferred method of diagnosis. Diet intervention (MCT diet) is the cornerstone and longtime medical treatment, which can improve the nutritional status and promote the survival quality of patients with PIL.