Technical Considerations for Functional Vascular Access for Hemodialysis Therapy in the Inborn Error of Metabolism Before Liver Transplantation.

Abstract

Purpose: Neonatal hyperammonemia may be caused by primary defects of one of the enzymes in the metabolic pathway and dialysis therapy must be initiated in patients who do not respond to medical treatment within 4 to 6 hours. A functional vascular access is the most important technical component determining successful provision of continuous renal replacement therapy (CRRT). Herein, the study addresses the surgical techniques to achieve reliable vascular access in the infantile patients with hyperammonemia received CRRT. Materials and Methods: From 2008 to 2011 in Taipei Veterans General Hospital, the medical records of the infants with inborn metabolic error and treated with CRRT were retrospectively reviewed. Vascular access to right internal jugular vein through semipermanant tunneled double-lumen catheters was performed in emergency before CRRT. Results: Totally 11 infants (4 MMA, 2 MSUD, 3 homocystinuria, 1 congested heart failure and 1 respiratory failure) were treated CRRT. Three of them were treated at newborn stage with low body weight and eight were treated at infantile stage. Small caliber double-lumen catheters (7Fr.=1, 8Fr.=8, 9Fr.=2) were inserted for CVVH(n=4) and HD (n=7). The function of catheters was excellent to remove toxins. Conclusion: CRRT is an efficient therapeutic strategy for newborns/ infants with metabolic inborn error to protect brain and serve as bridge to liver transplantation. Catheters in right internal jugular vein through a long subcutaneous tunnel can provide a secure route and locking solution replacement with heparin is mandatory after every CRRT.