1. Pediatric Liver Disease Alyssa Brzenski

2. Pediatric Liver Disease Keely DA, Hartley. End Stage Liver Disease.

3. Chronic Liver Disease

4. Acute Liver Disease

5. Biliary Atresia Biliary Atresia. The Lancet.

6.  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%) The Statistics

7.  Jaundice  Pale Stools  Dark Urine  Failure to Thrive  Coagulopathy Presentation

8. Work up

9. Kasai Procedure

10.  Routine Monitors +/- A-line  2 PIV  Balanced Anesthetic  +/- Epidural Anesthesia for a Kasai

11. Prognosis Previously unpublished data, based on UK Biliary Atresia Registry (1999–2008).93 BASM=biliary atresia splenic malformation syndrome.

12. Liver Transplants

13. 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, 20- 30% of patients had not been transplanted

14. LI 8.1 Pediatric patients waiting for a liver transplant

15. LI 8.2 Distribution of pediatric patients waiting for a liver transplant

16. LI 8.7 Pre-transplant mortality rates among pediatric patients wait-listed for a liver transplant, by age

17. LI 8.8 Pediatric liver transplants, by donor type

18. LI 8.12 Use of DCD donors in pediatric liver transplant recipients

19. LI 8.18 Survival among pediatric liver transplant recipients, 2002–2006, by age

20. Split Liver Transplant

21.  Full stomach- RSI  Large bore Upper extremity IV access  1 central line  Large bore PIV  Arterial Line Anesthesia Considerations

22. Surgical Stages Yudkowitz FS, Chietero M. Anesthesia Issues in Pediatric Liver Transplants. Pediatric Transplant. 2005; 9; 666-72.

23.  Hematocrit less than 30  Attempt to avoid platelet transfusion  Avoid hypercoagulable state  Dextran used by some centers Hepatic Artery Thrombosis

24.  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/ 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. Sources