Pediatric Liver DiseaseAlyssa Brzenski

Pediatric Liver Disease

Keely DA, Hartley. End Stage Liver Disease.

Chronic Liver Disease

Acute Liver Disease

Biliary Atresia

Biliary Atresia. The Lancet.

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

Jaundice Pale Stools Dark Urine Failure to Thrive Coagulopathy

Presentation

Work up

Kasai Procedure

Routine Monitors +/- A-line 2 PIV Balanced Anesthetic +/- Epidural

Anesthesia for a Kasai

Prognosis

Previously unpublished data, based on UK Biliary Atresia Registry (1999–2008).93 BASM=biliary atresia splenicmalformation syndrome.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, 20-30% 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

Full stomach- RSI Large bore Upper extremity IV access

1 central line Large bore PIV

Arterial Line

Anesthesia Considerations

Surgical Stages

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

Hematocrit less than 30 Attempt to avoid platelet transfusion Avoid hypercoagulable state Dextran used by some centers

Hepatic Artery Thrombosis

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.

Sources