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BASM: biliary atresia splenic malformation
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DISIDA: diisopropyl iminodiacetic acid; hepatobiliary scintigraphy
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ERCP: endoscopic retrograde cholangiopancreatography
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HPE: hepatoportoenterostomy
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MCT: medium-chain triglycerides
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PPS: peripheral pulmonary stenosis
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PTLD: post-transplant lymphoproliferative disease
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Biliary atresia (BA) is a progressive idiopathic, necroinflammatory
process involving the extrahepatic biliary tree, which can be either
segmentally or entirely affected. As the disease progresses, there
is obliteration of the extrahepatic bile duct lumen and obstruction
to bile flow (Figure 23–1). The result is cholestasis and
chronic liver damage. With time, the intrahepatic biliary system becomes
increasingly involved. BA is the most common cause of neonatal jaundice
for which surgery is indicated and the most common indication for
liver transplantation in children.
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Many different classifications of BA have been proposed over
the years. One classification system focuses on the anatomy of the
biliary tree. The most comprehensive is that used in the Japanese
BA Registry (Figure 23–2).1 In this classification
system there are three major types of atresia: type
I, atresia of the common bile duct (10% of patients); type II, atresia of the hepatic ducts (2% of patients); and type III,
atresia at the porta hepatitis (88% of patients). Within
each type there are several subtypes, which will not be discussed
here. The first two types of BA are sometimes labeled “correctable atresia,” whereas
type III corresponds to the so-called non-correctable type of atresia,
and accounts for the majority of patients. However, with current
hepatoportoenterostomy (HPE) techniques, most patients achieve at
least some bile drainage, and thus the correctable versus non-correctable
distinction is rarely used.
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Another classification system is based on the associated malformations
and onset of jaundice. Many textbooks refer to embryonic and perinatal
BA. In the former, there is typically onset of jaundice at birth
and often multiple major malformations, the most common of which
is heterotaxy. In the latter, jaundice appears several weeks after
birth and there are usually no associated malformations. Perinatal
BA accounts for 70–80% of all cases. Because there
are so many exceptions to these patterns, new nomenclature systems
have ...