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
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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
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Polysplenia (90%)
Situs invertus (50%)
Vascular anomalies (70%)
Intestinal malrotation (60%)
Cardiac anomalies- VSD, ASD, HLHS (50%)
Presentation
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Jaundice
Pale Stools
Dark Urine
Failure to Thrive
Coagulopathy
Work up
Kasai Procedure
Anesthesia for a Kasai
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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Previously unpublished data, based on UK Biliary Atresia Registry (1999–2008).93 BASM=biliary atresia splenic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
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Hematocrit less than 30
Attempt to avoid platelet transfusion
Avoid hypercoagulable state
Dextran used by some centers
Sources
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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.