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Abstract Introduction Superior vena cava syndrome (SVCS) is caused by an obstruction of the thoracic central veins. SVCS is a rare but potentially life-threatening condition with malignancy as the most common cause. In children, SVCS is most commonly related to the use of a central venous catheter (CVC) or treatment of congenital heart defects. SVCS usually presents with swelling of the face, tounge, neck and hand, distended chest and neck veins, cyanosis, respiratory distress and watering eyes. Method One girl with duodenal atresia, gestational week (GW) 34+0, weight 2005g, and one boy with necrotizing enterocolitis, GW 32+ 5, weight 1720g developed catheter-related SVCS at two and four weeks of age. Both had been subjected to major intraabdominal surgery and received a CVC in the left subclavian vein, for parenteral nutrition. Presenting symptoms were, elevated body temperature, decreasing platelets, CRP elevation, discoloration of the upper body, swelling of the face and desaturation. The bloodcultures were positive. Ultrasound and contrast-enhanced CT for detection of catheter-related thrombosis were performed. Result CT scan, but not ultrasound, confirmed a SVC obstruction. The children were referred to the ICU for immediate treatment with heparin and the girl also received Actilyse. She developed chylothorax and required thoracic drainage for four months with additional somatostatin treatment, loop diuretics and anticoagulation with low molecular weight heparin and Rivaroxaban. Discussion SVCS is associated with potentially severe complications. The presenting symptoms are general and easily mistaken for other more common causes behind deranged vital signs. Early detection and intervention is important to minimize morbidity and mortality.
Borg et al. (Thu,) studied this question.