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Kasai Procedure: Difference between revisions
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→Biliary atresia
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== Biliary atresia == | == Biliary atresia == | ||
Biliary atresia is a congenital malformation of the biliary tree, causing the occlusion of the extra hepatic bile duct. It is marked by an increase of conjugated bilirubin, resulting in jaundice, pale stools, dark urine. Coagulopathy, failure to thrive, hepatosplenomegaly and ascites are also common presentations. In the first few months of life, liver function is generally preserved, mantaining normal levels of albumin and prothrombin.<ref>[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3531003/ Anaesthesia for biliary atresia and hepatectomy in paediatrics]</ref> | Biliary atresia is a congenital malformation of the biliary tree, causing the occlusion of the extra hepatic bile duct. It is marked by an increase of conjugated bilirubin, resulting in jaundice, pale stools, dark urine. Coagulopathy, failure to thrive, hepatosplenomegaly and ascites are also common presentations. In the first few months of life, liver function is generally preserved, mantaining normal levels of albumin and prothrombin.<ref name=":1">[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3531003/ Anaesthesia for biliary atresia and hepatectomy in paediatrics]</ref> | ||
As the disease progresses if left untreated, cholestasis, hepatic fibrosis, cirrhosis, portal hypertension and liver failure can develop. | As the disease progresses if left untreated, cholestasis, hepatic fibrosis, cirrhosis, portal hypertension and liver failure can develop. | ||
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Medical management consists of antibiotic therapy (prevention of cholangitis), nutritional support (fat soluble vitamin supplementation), and improvement of bile flow (ursodeoxycholic acid and steroids - controversial). Surgical management includes portoenterostomy and liver transplantation. | Medical management consists of antibiotic therapy (prevention of cholangitis), nutritional support (fat soluble vitamin supplementation), and improvement of bile flow (ursodeoxycholic acid and steroids - controversial). Surgical management includes portoenterostomy and liver transplantation. | ||
Over 80% of the patients with a sucessful Kasai procedure are likely to survive more than 10 years with their native liver. However, all children will develop portal fibrosis, cirrhosis and portal hypertension to a greater or lesser extent, even if surgery is successful. Recurrent cholangitis is a risk factor for progression of disease. In these patients, combined management with liver transplantation can be beneficial. | Over 80% of the patients with a sucessful Kasai procedure are likely to survive more than 10 years with their native liver<ref name=":1" />. However, all children will develop portal fibrosis, cirrhosis and portal hypertension to a greater or lesser extent, even if surgery is successful. Recurrent cholangitis is a risk factor for progression of disease. In these patients, combined management with liver transplantation can be beneficial. | ||
== Anesthesia considerations for Kasai procedure == | == Anesthesia considerations for Kasai procedure == | ||