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BA: biliary atresia

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BASM: biliary atresia splenic malformation

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CXR: chest X-ray

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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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US: ultrasound

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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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FIGURE 23–1
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Schematic of BA. As the disease progresses there is obliteration of the extrahepatic biliary tree, leading to obstruction of bile flow. www.barcnetwork.org.

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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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FIGURE 23–2
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Three major types of BA, based on anatomic findings.1 This image was adapted from an image available at www.mayoclinicproceedings.com. The original image is found in reference #1. Type I (about 10%): atresia of the common bile duct. Type II (about 2%): atresia of the hepatic duct. Type III (over 88%): atresia at the porta hepatis.

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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, ...

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