الفهرس 
Importance of ductal plate
Patthogenesiis and EttiiollogyOnly 14 pages are availabe for public view
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Abstract
Biliary Atresia (BA) is a rare condition that occurs in about 1:15,000 live births (1.5 times more common in females) and represents the most common surgical cause of neonatal jaundice and the main indication for Liver Transplantation (LT) in children. BA is a progressive, fibro-obliterative cholangiopathy that affects both the extra- and intrahepatic biliary tree to various degrees, resulting in obstructive bile flow, cholestasis and jaundice in neonates. If this condition is left untreated, progressive liver cirrhosis leads to death by the age of two years. Biliary atresia is classified based on the level of extrahepatic obstruction of the biliary tree. In type I BA (about 5%) the biliary tract is obstructed at the level of the common bile duct. In type II (about 2%), obstruction occurs at the level of the common hepatic duct. In type III (> 90%) BA, the most proximal part of the extrahepatic biliary tract in the porta hepatis is obstructed and fibrotic, without any macroscopic remnants of the hepatic ducts. Even in the case of type III BA, microscopic residual bile ductules of variable size at the level of the porta hepatis remain in continuity with the intrahepatic biliary tree. Kasai portoenterostomy, initially described in 1959, is a palliative operation performed to restore bile drainage from these microscopic residual ductules. Since introduction of Kasai procedure, a dramatically improved survival rate in patients suffering from BA over the short term has been observed. While long-term survival into adulthood continues to be a challenge. The advent of the liver transplant era has brought about further improvement in long-term outcomes; as most patients with BA ultimately require liver transplantation (LT) even after a successful Kasai procedure. Liver transplantation is indicated in the case of failure to establish biliary flow, liver cirrhosis, liver failure, gastrointestinal bleeding due to portal hypertension, growth retardation, pruritus, progressive intrapulmonary shunting, hepatopulmonary syndrome and repeated cholangitis, either alone or in combination. Biliary atresia remains the most common indication for liver transplantation in children, accounting for about 75% of transplantations in those younger than two years old. The clinical outcome following Kasai portoenterostomy has many determinants; some of them are uncorrectable like type of biliary atresia, preoperative degree of pathological affection of the liver, association with polysplenia syndrome and portal venous pressure at time of surgery. The other determinants are correctable factors and include the age of the patient at Kasai operation, surgical experience and accessibility of liver transplantation. The aim of our work was to evaluate the clinical outcome of both techniques of treatment options for cases with biliary atresia either Kasai portoenterostomy or Liver transplantation & to confirm that these two approaches are not necessarily mutually exclusive, but rather that they may complement each other. Sixty six patients with biliary atresia were included in our study during the period from April 2015 to April 2017. They were divided into three groups: group A: those 62 patients were subjected to Kasai portoenterostomy. The operation was done in the National Liver Institute, Menoufia University and Pediatric Surgery Department, Tanta University Hospital. group B: Liver transplantation was done for three patients after Kasai portoenterostomy. Left lateral segment of living related donor was transplanted with bilioenteric anastomosis. The operation was done in the National Liver Institute, Menoufia University. group C: one patient was diagnosed at 2 years old & was submitted for primary liver transplantation in the National Liver Institute, Menoufia University. Left lateral segment of living related donor was transplanted with bilioenteric anastomosis