2000
DOI: 10.1111/j.1460-9592.2000.00579.x
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Anaesthesia, perioperative management and outcome of correction of extrahepatic biliary atresia in the infant: a review of 50 cases in the King's College Hospital series

Abstract: Extrahepatic biliary atresia (EHBA) is an uncommon condition presenting in the first few weeks of life. It has an incidence of 0. 5-1 per 10 000 live births and is the end result of a destructive inflammatory process involving the extrahepatic biliary system of unknown aetiology occurring in utero. The net result is neonatal jaundice due to bile stasis, with subsequent hepatocellular damage and cirrhosis. In the untreated, patient death is inevitable within 2 years. Precise diagnosis (or exclusion) of EHBA in … Show more

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Cited by 92 publications
(19 citation statements)
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References 52 publications
(54 reference statements)
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“…When the gallbladder is not visualized on prenatal US, differential diagnosis between gallbladder agenesis and congenital BA should be done [5][6][7][8]17] . Clinical management includes karyotyping, genetic testing for cystic fibrosis, and digestive enzyme analysis in amniotic fluid before 22 weeks [6,11,13,14,18] .…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…When the gallbladder is not visualized on prenatal US, differential diagnosis between gallbladder agenesis and congenital BA should be done [5][6][7][8]17] . Clinical management includes karyotyping, genetic testing for cystic fibrosis, and digestive enzyme analysis in amniotic fluid before 22 weeks [6,11,13,14,18] .…”
Section: Discussionmentioning
confidence: 99%
“…Differential diagnosis is crucial due to the poor prognosis of some of the entities. Gallbladder agenesis is a benign condition with an incidence of 1/6,300 [5,6] , while BA is a severe disease, associated with liver transplantation and death, that can benefit from early management [7][8][9][10] , raising the issue of pregnancy termination.…”
Section: Introductionmentioning
confidence: 99%
“…At the end of surgery, the tracheas of most neonates can be extubated. These neonates are best managed in a high-dependency unit with morphine analgesia [ 236 ].…”
Section: Anesthetic Considerations [ 230 ]mentioning
confidence: 99%
“…If left untreated it leads to worsening cholestasis, hepatic fibrosis, cirrhosis, portal hypertension and liver failure. [12]…”
Section: Biliary Atresiamentioning
confidence: 99%