Surgery of Biliary Atresia

Abstract

biliary atresia (ba) is a progressive fibro-obliterative cholangiopathy of unclear etiology affecting varying degrees of both extra-and intrahepatic biliary tree resulting in obstructive bile flow and cholestasis in neonates. the diagnostic work-up is designed to diagnose or rule out ba without any unnecessary delay. Kasai portoenterostomy is a palliative operation performed to establish bile drainage from microscopic bile ductules that remain in the porta hepatis. it is advantageous to perform portoenterostomy as early after birth as possible for better chances of success. actuarial native liver 5-year survival rates range from 30% to 60% after portoenterostomy, and about 20% of all patients undergoing Kasai portoenterostomy during infancy survive into adulthood with their native liver. Portoenterostomy remains as the first line operative treatment in ba while liver transplantation serves as a salvage treatment when portoenterostomy fails or liver function gradually deteriorates after initially successful establishment of bile flow.

BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service

“…Hepatobiliary scintigraphy has a limited value because it presents with high sensitivity, often at 100%, and has disagreeing values about its specificity, varying from 40% to 100% (2) . Although duodenal tubing has been reported as a useful method for BA diagnosis, with a specificity of 93.7% and a sensitivity varying from 97.3% to 100% (24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34) , it was responsible for the delay in propaedeutics at this institution (37) . It is mandatory to improve the agility in diagnosis, increase the efficiency of the diagnosis process, and decrease the time gap between referral and portoenterostomy.…”

“…It is a disease of both the intra-and extrahepatic bile ducts resulting from an inflammatory process which leads to the absence or progressive obliteration of ducts (26,37) . Etiopathogenic factors are unknown, however, multifactorial events may play a role (4) , in which genetic and environmental factors interact to promote the obliteration of the biliary tree (16,32) . BA should always be recalled as a probable diagnosis when jaundice with cholestatic characteristics lasts beyond the 14th day of life (30) .…”

National centralization of biliary atresia care to an assigned multidisciplinary team provides high-quality outcomes

“…Written treatment and follow-up protocols were implemented to ascertain care uniformity. Three experienced pediatric surgeons performed the PE [23] on all patients with two surgeons present in each operation in order to increase caseload per surgeon, and thereby ascertain reproducible surgical quality. All patients received uniform protocol driven adjuvant medical and nutritional therapy, and participated in a fixed surveillance protocol.…”

Extrahepatic biliary atresia with choledochal cyst: Prenatal MRI predicted and post natally confirmed: A case report

“…[12] The present case also underwent Kasai's portoenterostomy after the excision of fibrotic extrahepatic biliary ducts. Early surgery, as evident in this case that was operated <8 weeks of life, has yielded satisfactory initial biliary excretion leading to normal serum bilirubin levels.…”