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Supplement Nr. 10 | 2019: Biliary atresia

Supplement Nr. 10 | 2019: Biliary atresia

Schlüsselwörter ICD 10: Q44.2, Extrahepatic biliary atresia, familial extrahepatic biliary atresia, idiopathic extrahepatic biliary atresia
Keywords ICD 10: Q44.2, Extrahepatic biliary atresia, familial extrahepatic biliary atresia, idiopathic extrahepatic biliary atresia
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Summary

Biliary atresia (BA) is a rare and fatal progressive inflammatory disease of infancy affecting the intra and extra hepatic bile ducts leading to cholestasis, fibrosis and cirrhosis. It has a varying incidence ranging from 1:10,000 to 1:20,000 live births. Persistent jaundice for more than 2 weeks in a term infant mandates evaluation for BA. Without medical intervention, BA leads to liver failure and ultimately death within the first two years of life. 

Kasai’s portenterostomy (KPE) is the first line of treatment which aims at restoring the forward flow of bile from the liver into the intestines using a jejunal Roux-en-Y limb, which is anastomosed to the porta hepatis after resection of biliary remnants. Orthotopic liver trans­plantation (OLT) is reserved for children with a failed Kasai’s procedure. Kasai’s portoente­rostomy procedure and liver transplantation along with adjuvant medical therapy and nutritional support have improved the prognosis of infants with BA and survival up to adult­hood has been documented. The main anaesthetic concerns during portoenterostomy are conduct of safe anesthesia in an infant with mild to moderate derangement of liver function scheduled for a lengthy upper abdominal wall laparotomy. This necessitates meticulous attention to patient’s intraoperative fluid status, temperature regulation, glucose metabolism and provision of adequate perioperative analgesia in the presence of a compromised and dysfunctional liver. Surgical manoeuvres causing transient obstruction to inferior vena caval blood flow and hypotension need to be anticipated and managed appropriately.

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