日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Introduction: Chylothorax/abdomen can lead to prolonged hospitalization, cause developmental delays, and sometimes become fatal. Therefore, the development of a new therapy has been investigated. Owing to the rapidly expanding knowledge about central lymphatic disease, the etiology of chylothorax/abdomen has been found to be recurrent and to leak due to lymphatic stenosis or obstruction. These lymphatic problems are similar to those in peripheral lymphatic disease, for which we analyzed the lymphatic flow and treated it with direct maneuver. Based on this, we have introduced minimally invasive procedures to correct the central lymphatic system.

Material and Methods: We included 12 pediatric patients aged 30 days to 2 years. Five patients were diagnosed with hereditary diseases other than cardiac anomalies. All patients were followed for ＞6 months after the lymphatic procedures were performed. Medical treatment was preoperatively given for ≥4 weeks and with diet/milk restrictions.

Results: Four patients were completely cured from lymphatic leakage, three patients required further treatment, and five patients died during intensive care, mainly because of respiratory distress.

Conclusion: Lymphangiography and lymphatic venous anastomosis are the most commonly performed procedures that are effective in some patients. This novel treatment remains limited to patients with complications. However, the new therapy that is based on the lymphatic flow analysis may become a novel approach for refractory chylothorax/abdomen. Therefore, studies on lymphatic disease are ongoing and further improvements are expected in the future.