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Purpose: To evaluate clinical characteristics of pediatric nontraumaitc chylothorax and to suggest appropriate therapeutic managements. Methods: We retrospectively reviewed medical records of 22 patients with nontraumatic chylothorax from January 2005 to December 2018 in the Children’s Hospital of Seoul National University. We analyze their etiology, treatment, complications and outcome. Results: Of the 22 patients, 16 were diagnosed before 1 year old and 6 after 1 year old. The causes of chylothorax under 1-year-old children were related to congenital factors (n=9), unknown causes (n=5), and high central venous pressure (n=2). The causes of chylothorax over 1-year-old children were related to congenital factors (n=3), unknown causes (n=1), high venous pressure (n=1), and lymphoma (n=1). All patients had dietary modification. Eight of them were cured by dietary modification, but there was no improvement in over 1-year-old children. Medication was added to patients refractory to dietary modification. Four patients with medication were improved and 5 were improved by surgical management. Nutritional, immunological and other complications occurred in many patients. Five death cases were reported. Four patients were under 1 year old and 1 was over 1 year old. The causes of nontraumatic chylothorax in dead patients were high central venous pressure (n=3), congenital (n=1), and unknown (n=1). Conclusion: Nontraumatic chylothorax more frequently occurs in under 1-year-old children. The most common cause is congenital factors. Stepwise management is effective in many patients, but specific treatment is needed in some cases. The prognosis is related to the onset of age and underlying diseases.

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