106: All is not lost: a new route for central venous access

Introduction: We present the case of a 6 months old boy admitted to our intestinal rehabilitation center with short bowel syndrome, severe malnutrition and loss of central venous access. Methods: The child was born in Romania. He underwent short bowel resection following midgut volvulus at 2 days of life. The remaining intestine was estimated at 55 cm, with no ileo-caecal valve and the entire colon. He received intermittent parenteral nutrition (PN) and underwent numerous central catheter placements with either thrombosis or vein ligature after insertions which left the local team in the impossibility to insert further central catheters and to administer PN. His parents asked for the transfer to our unit. Because Romania is part of the European Union, the child could benefit from social security in our country. He was admitted at 6 months of life in a cachectic pre-mortem state. A central catheter was inserted with difficulties in the superior vena cava through the right internal jugular vein. He was then slowly re-fed with PN to avoid refeeding syndrome. He received anti-coagulant prophylaxis and was discharged on home PN two months later. He progressively developed superior vena cava syndrome with increased head circumference and mild cerebral oedema. The CT-scan showed that the catheter was placed through a narrow varicose network and was obstructing the blood flow to the superior vena cava. All other central venous access were thrombosed including the inferior vena cava. At that stage, the child was still highly dependent on PN. Results: After several multidisciplinary discussions (pediatric gastro-enterologist, cardiac surgeons, anesthetists, cardiolgists and neuro-surgeons), the decision was made to perform a superior vena cava plasty and to insert a cuffed central venous catheter in the right atrium directly through the myocardium with an abdominal exit site (figure 1 and 2). The procedure was performed at the age of 13 months. The surgery was successful but was complicated with a PN pericardial effusion which was firstly mistaken for a chylo-pericardium. A second procedure was undertaken 5 days later to reposition the tip of the catheter which had migrated from the atrium cavity to the pericardium. From then, the PN was administered safely through this catheter for 15 months until the child was totally weaned off PN. Superior vena cava syndrome completely lifted. Anti-coagulant treatment was stopped 6 months after the procedure. Four months after weaning PN, he underwent another cardiac surgery to remove the central line. Conclusion: Cardiac surgery with insertion of a central line directly through the myocardium into the right atrium can be a life-saving procedure in infants and children with total loss of central venous access.