Superior vena cava syndrome is rare and challenging clinical entity in neonates. Medical treatment options are usually effective. However, when failed, surgery is warranted. Herein, we present a preterm neonate with SVC syndrome and associated chylothorax. When 2 weeks old, he underwent successful open thrombectomy and SVC reconstruction under cardiopulmonary bypass. Immediately after the operation findings of SVC syndrome and chylotorax were completely resolved. To our knowledge, this patient is the smallest baby underwent open SVC reconstruction with cardiopulmonary bypass.
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