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Introduction: Congenital chylothorax is a rare condition that occurs in 1/15,000 of newborns. Its main manifestation is respiratory distress, associated with tachypnea, subcostal draft and cyanosis. The definitive diagnosis is made by analyzing the pleural fluid obtained by thoracentesis. Treatment involves continuous drainage in a water seal, ventilatory and nutritional support. Objective: To report a case of idiopathic pleural effusion in a newborn. Methodology: This is a descriptive observational study based on the analysis of the patient's medical record and literature review. Results: Full-term newborn, with adequate prenatal care and appropriate weight for gestational age. Born by elective caesarean section without complications, with an APGAR of 2 in the first minute, 3 in the second minute, 6 in the third minute and 7 in the tenth minute. He developed respiratory distress and cyanosis, requiring hospitalization in an intensive care bed and orotracheal intubation, which lasted 13 days. A chest X-ray showed total veiling of the left hemithorax and obliteration of the bilateral costophrenic sinuses, corroborating a diagnosis of pleural effusion. Echocardiograms ruled out cardiac causes, and ultrasound showed no alterations in the lungs or kidneys. Normal serial blood tests ruled out an infectious cause. Due to albumin depletion as a result of the pleural effusion, the patient was given. The patient underwent four chest drainages, resulting in a total volume of 930 mL. With the progression of the oral diet, the pleural effusion evolved to a milky appearance, which was treated with a fat-free diet and octreotide. The patient progressed well in the face of the proposed treatment, maintaining continuous drainage and ventilatory support through oxygen therapy as well as respiratory physiotherapy, removing the drain after 31 days, and being discharged in good general condition at 43 days of life. Conclusion: This is a rare case of congenital pleural effusion, of idiopathic cause, with no family history, which evolved into chylothorax with the progression of the diet, and showed a good evolution of clinical parameters in the face of the established treatment, with no further recurrences.
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