Biliary atresia choledochal malformation

Biliary atresia/choledochal malformation

Congenital or acquired (e.g. cytomegalovirus) extrahepatic biliary atresia is a progressive obliterative cholangiopathy with absent or narrow bile ducts. Type I involves the common bile duct, type II the common hepatic duct, and 80% have the most common type III, involving the proximal bile ducts. Biliary atresia presents with conjugated hyperbilirubinaemia, pale stools and dark urine in the first few weeks of life. If presenting Morio Kasai , 1922–2008, pioneering Japanese surgeon, trained by C Everett Koop. César Roux , 1857–1934, Swiss surgeon, assistant to Theodor Kocher. - ) - - late, there may be malabsorption, growth failure and coagulop - athy . Some associations appear in Table 18.1 . The diagnosis is confirmed with a radionucleotide hepatobiliary iminodia - cetic acid (HIDA) scan. Early diagnosis and avoiding sepsis ma y prevent irreversible liver fibrosis and death. The Kasai he patico-portoenterostomy using a jejunal Roux-en-Y loop anastomosed to the portal plate gives drainage for some years,

Figure 18.10 A ventilated neonate with an acute abdomen awaiting laparotomy. Figure 18.11 Abdominal radiograph showing pneumatosis.

but many need liver transplantation. Congenital choledochal malformations manifest as cystic dilatations of the biliary tree and are also managed with resection and portoenterostomy .

Figure 18.12 Operative appearance of neonatal necrotising entero colitis.

Biliary atresia/choledochal malformation

Congenital or acquired (e.g. cytomegalovirus) extrahepatic biliary atresia is a progressive obliterative cholangiopathy with absent or narrow bile ducts. Type I involves the common bile duct, type II the common hepatic duct, and 80% have the most common type III, involving the proximal bile ducts. Biliary atresia presents with conjugated hyperbilirubinaemia, pale stools and dark urine in the first few weeks of life. If presenting Morio Kasai , 1922–2008, pioneering Japanese surgeon, trained by C Everett Koop. César Roux , 1857–1934, Swiss surgeon, assistant to Theodor Kocher. - ) - - late, there may be malabsorption, growth failure and coagulop - athy . Some associations appear in Table 18.1 . The diagnosis is confirmed with a radionucleotide hepatobiliary iminodia - cetic acid (HIDA) scan. Early diagnosis and avoiding sepsis ma y prevent irreversible liver fibrosis and death. The Kasai he patico-portoenterostomy using a jejunal Roux-en-Y loop anastomosed to the portal plate gives drainage for some years,

Figure 18.10 A ventilated neonate with an acute abdomen awaiting laparotomy. Figure 18.11 Abdominal radiograph showing pneumatosis.

but many need liver transplantation. Congenital choledochal malformations manifest as cystic dilatations of the biliary tree and are also managed with resection and portoenterostomy .

Figure 18.12 Operative appearance of neonatal necrotising entero colitis.

Biliary atresia/choledochal malformation

Congenital or acquired (e.g. cytomegalovirus) extrahepatic biliary atresia is a progressive obliterative cholangiopathy with absent or narrow bile ducts. Type I involves the common bile duct, type II the common hepatic duct, and 80% have the most common type III, involving the proximal bile ducts. Biliary atresia presents with conjugated hyperbilirubinaemia, pale stools and dark urine in the first few weeks of life. If presenting Morio Kasai , 1922–2008, pioneering Japanese surgeon, trained by C Everett Koop. César Roux , 1857–1934, Swiss surgeon, assistant to Theodor Kocher. - ) - - late, there may be malabsorption, growth failure and coagulop - athy . Some associations appear in Table 18.1 . The diagnosis is confirmed with a radionucleotide hepatobiliary iminodia - cetic acid (HIDA) scan. Early diagnosis and avoiding sepsis ma y prevent irreversible liver fibrosis and death. The Kasai he patico-portoenterostomy using a jejunal Roux-en-Y loop anastomosed to the portal plate gives drainage for some years,

Figure 18.10 A ventilated neonate with an acute abdomen awaiting laparotomy. Figure 18.11 Abdominal radiograph showing pneumatosis.

but many need liver transplantation. Congenital choledochal malformations manifest as cystic dilatations of the biliary tree and are also managed with resection and portoenterostomy .

Figure 18.12 Operative appearance of neonatal necrotising entero colitis.