Pediatric Liver Disease Alyssa Brzenski Pediatric Liver Disease Keely DA, Hartley. End Stage Liver Disease. Chronic Liver Disease Acute Liver Disease Biliary Atresia Biliary Atresia. The Lancet. The Statistics Most common in southeast Asia 1: 15,000 born in the US 2.5 times more common in african american mothers Most common cause of pediatric liver transplant 10-20% associated with congenital anomalies Biliary atresia splenic malformation Polysplenia (90%) Situs invertus (50%) Vascular anomalies (70%) Intestinal malrotation (60%) Cardiac anomalies- VSD, ASD, HLHS (50%) Presentation Jaundice Pale Stools Dark Urine Failure to Thrive Coagulopathy Work up Kasai Procedure Anesthesia for a Kasai Routine Monitors +/- A-line 2 PIV Balanced Anesthetic +/- Epidural Prognosis Previously unpublished data, based on UK Biliary Atresia Registry (1999–2008).93 BASM=biliary atresia splenic malformation syndrome. Liver Transplants Statistics 16,414- currently on the Liver Transplant waitlist (as of March 9, 2013) 475 of which are children 3 children listed at Radys In 2011, there were 536 pediatric recipients in US Within 2 years of being listed for a liver transplant, 2030% of patients had not been transplanted LI 8.1 Pediatric patients waiting for a liver transplant LI 8.2 Distribution of pediatric patients waiting for a liver transplant LI 8.7 Pre-transplant mortality rates among pediatric patients wait-listed for a liver transplant, by age LI 8.8 Pediatric liver transplants, by donor type LI 8.12 Use of DCD donors in pediatric liver transplant recipients LI 8.18 Survival among pediatric liver transplant recipients, 2002–2006, by age Split Liver Transplant Anesthesia Considerations Full stomach- RSI Large bore Upper extremity IV access 1 central line Large bore PIV Arterial Line Surgical Stages Yudkowitz FS, Chietero M. Anesthesia Issues in Pediatric Liver Transplants. Pediatric Transplant. 2005; 9; 666-72. Hepatic Artery Thrombosis Hematocrit less than 30 Attempt to avoid platelet transfusion Avoid hypercoagulable state Dextran used by some centers Sources Hartley JL, Davenport M, Kelly DA. Biliary Atresia. Lancet 2009; 374:1704-13. Scientific Registry of Transplant Recipients. 2011 Liver OPTN/SRTR annual report. http://www.ustransplant.org/ Hendrickson R et al. Pediatric Liver transplantation. Current Opin Pediatr 2004; 16: 309-13. Hartley J and Kelly D. End stage liver failure. Paediatrics and Child Health 2009; 20: 30-5.