Causes and manifestations of chylothorax in children in China: Experience from a children's medical center, 2007–2017

IMPORTANCE: Chylothorax is the most common cause of pleural effusion in neonates and relatively rare in children. It can cause significant respiratory morbidity. Many clinical entities may contribute to chylothorax. OBJECTIVE: To investigate the causes and manifestations of chylothorax in infants and children in China. METHODS: Case records of 107 cases with chylothorax seen in Beijing Children's Hospital from 2007 to 2017 were retrieved and analyzed; follow-up was carried out by telephone. RESULTS: Of 107 cases, 58.9% (63/107) were primary chylothorax (PC) and 41.1% (44/107) were secondary chylothorax (SC). Also, 36.4% (39/107) were neonatal chylothorax (NC) and 35.5% (38/107) were postoperative chylothorax. In PC with a verified lymphatic anomaly, there was one case of diffuse pulmonary lymphangiomatosis (DPL) and six cases of generalized lymphatic anomaly (GLA), which accounted for 6.5% (7/107) of cases. In most patients, chylothorax was alleviated by conservative treatment based on total parenteral nutrition (TPN); 13.1% (14/107) of cases needed further surgery. In NC, the median duration of TPN was 9 days, but 10 of 20 cases who improved had recurrence upon re-introduction of a fat-free diet, which was alleviated by further TPN. The duration of hospitalization was (23 ± 14) days for congenital chylothorax. Upon long- term follow-up, except for GLA and DPL, most patients were doing well without recurrence. INTERPRETATION: NC and postoperative chylothorax are the common subtypes. TPN is effective for most patients. Despite a prolonged and fluctuating clinical course, most patients had a good long-term prognosis.