Introduction: Patients with end-stage intestinal failure require long-term total parenteral nutrition (TPN) via central venous access; however, recurrent episodes of line sepsis and multiple replacements of central venous catheters may cause venous thrombosis, resulting in loss of all central venous access. Herein, we report our experience in a patient with end-stage central venous access failure 10 years after intestinal transplantation.

Case report: A 12-year-old girl with intestinal failure due to allied disorders of Hirschsprung’s disease underwent deceased intestinal transplantation. The proximal end of the intestinal graft measuring 320 cm in length was anastomosed in an end-to-end fashion to the recipient’s duodenum and the distal end was brought through the abdominal wall as a single-barrel ileostomy (cold ischemia time, 7 h 18 min; warm ischemia time, 43 min). Venous outflow from the intestinal graft was conveyed into portal venous circulation. At the time of transplant, left internal jugular vein was the only patent central venous access and all other central veins were occluded by frequent episodes of catheter-related infection resulting in venous thrombosis. After the transplantation, she suffered multiple bouts of enteritis both from cytomegalovirus and bacterial infection and acute cellular rejection, which eventually lead to intestinal graft failure. She was forced to go back to long-term TPN. Thereafter, her left internal jugular vein got occluded and a computed tomography scan revealed her left hepatic vein to be the only patent central vein that was accessible percutaneously. We placed a Broviac catheter in the left hepatic vein via percutaneous and transhepatic route, 9 years after transplantation. The catheter was displaced accidentally 8 months later, and we safely exchanged the catheter under the same approach. At the time of this report, her oral intake is limited to fat-free liquid diet. She now weighs 27 kg and is on tacrolimus (target trough level, 2-3 ng/ml), prednisolone 5 mg QD, and mycophenolate mofetil 1000 mg QD. She is currently under evaluation for intestinal retransplantation.

Conclusion: Transhepatic central venous catheter placement is a viable option in a patient with end-stage central venous access failure; however, its long-term management to prevent accidental removal has yet to be defined.