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PubMed · 15001126

Biliary atresia.

Hiroyuki Kobayashi·Mark D Stringer
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Abstract

Biliary atresia (BA) is a congenital obliterative cholangiopathy of unknown aetiology, affecting both the intra- and extrahepatic bile ducts. Although relatively rare, BA must be excluded in any infant with conjugated hyperbilirubinaemia since the prognosis is improved by early diagnosis and prompt surgery. At least two phenotypes of BA are currently recognized; the syndromic variety is associated with other congenital anomalies and a poorer outcome. The results of treatment have steadily improved and, with a combination of timely expert surgery (Kasai portoenterostomy) and liver transplantation in specialist centres, good quality long-term survival is now possible in more than 90% of affected patients. A better understanding of the aetiology of BA and the pathogenesis of hepatic fibrosis is needed in order to develop new therapeutic strategies.

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Biliary AtresiaHumansInfant, NewbornJournal ArticleReview

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BibTeXRIS

Hiroyuki Kobayashi, Mark D Stringer. 2003. Biliary atresia.. https://doi.org/10.1016/s1084-2756(03)00065-4

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PubMed2007

Hepatobiliary scintigraphy with SPECT in infancy.

UNLABELLED: Hepatobiliary scintigraphy (HBS) is an important investigation for the diagnosis of biliary atresia (BA) and its differentiation from causes of conjugated hyperbilirubinemia that do not require surgical intervention. Delayed imaging at 24 hours and phenobarbitone augmentation for 5 days has been required to achieve high sensitivity and specificity with current techniques. This study explores whether adding single photon emission computed tomography (SPECT) performs as well as existing methods without requiring delayed 24-hour imaging and whether the phenobarbitone premedication is necessary in all cases. METHODS: A retrospective analysis of 105 HBS studies on 94 patients was performed. HBS included SPECT at 4 to 6 hours postinjection when no tracer was seen in the gastrointestinal tract in the first 60 minutes. This was done in 80 patients. RESULTS: Gastrointestinal activity was seen in 14 patients within 60 minutes. For 4- to 6-hour studies, standard HBS and HBS with SPECT data showed a sensitivity of 100% for the diagnosis of BA. The specificity, accuracy, and positive likelihood ratios (PLR) were 67%, 75%, and 3 (confidence interval [CI]=2.03-4.16) for planar imaging at 4 to 6 hours and 90%, 93%, and 10 (CI=4.42-19) for 4- to 6-hour planar and SPECT imaging. When the 11 patients who had phenobarbitone stimulation were included, the results improved to 97%, 98%, and 30 (CI=7.06-80). CONCLUSION: The addition of SPECT 4 to 6 hours postinjection of tracer significantly improves the diagnostic accuracy of hepatobiliary scintigraphy compared with planar imaging alone. This accuracy is as good as HBS performed after phenobarbitone stimulation. The combined technique of HBS with SPECT and phenobarbitone has the highest accuracy. Delayed imaging at 24 hours is usually not necessary.

Biliary Atresia↗
PubMed2006

Biliary atresia with choledochal cyst: implications for classification.

BACKGROUND & AIMS: To illustrate the limitations of the embryonic and perinatal classification system of biliary atresia (BA), we present a child with an antenatal diagnosis of choledochal cyst (CC) associated with BA and review the published literature. METHODS: Medline and Pubmed were searched for "BA and CC," "cystic biliary atresia," "BA and cysts," and "biliary cystic malformations." RESULTS: A 7-week-old with an antenatal diagnosis of CC was found to have BA associated with CC. The literature search identified 88 cases of BA with CC. Sixty-seven cases had type 1 BA (atresia of the common bile duct), 2 had type 2 BA (atresia of the common hepatic duct), and 19 had type 3 BA (atresia of the porta hepatis). Of the 27 cases of antenatal diagnosis of BA with CC only 1 had associated congenital anomalies. Outcome analysis showed poor outcomes were significantly more common (P = .009) and occurred earlier (P = .0249) in patients with type 3 BA. Children with type 3 BA were 5.4 times more likely to develop poor outcomes compared with type 1 (hazard ratio, 5.4; 95% confidence interval, 1.03-27.8). CONCLUSIONS: BA associated with CC forms a distinct subtype of BA, characterized by a preponderance of type 1 BA, a relatively good clinical outcome after surgery, and an absence of associated congenital anomalies. Antenatal diagnosis of many affected infants supports their inclusion within the embryonic BA group and suggests that a broader interpretation of the embryonic phenotype and further classification of BA based on genetic susceptibility may be required.

Biliary Atresia↗
PubMed2006

Usefulness of magnetic resonance cholangiopancreatography in biliary structures in infants: a four-case report.

In this paper, we report the usefulness of magnetic resonance cholangiopancreatography (MRCP) in excluding biliary atresia (BA) as the cause of neonatal cholestasis. MRCP with a 1.5-T magnetic resonance (MR) imaging unit was performed on four jaundiced neonates and infants aged from 38 days to 106 days. The diagnosis of BA (n=2) was confirmed with surgery, liver biopsy and surgical cholangiography. Diagnosis of neonatal hepatitis (NH, n=2) was confirmed with clinical follow-up until jaundice resolved, while one of them was diagnosed with surgical cholangiography. In all discoloured acholic stools, increased direct bilirubin (4.4-11.3 mg/dl) with positive lipoprotein X prompted technetium 99mTc disofenin scanning, which showed no excretion. Computed tomography (CT) showed a gallbladder in one with hepatitis but no intrahepatic bile duct in two with BA. The Kasai operation was performed in two patients with BA. In two patients with BA, neither the common bile duct nor the common hepatic ducts were visible at MRCP. In two patients with NH, MRCP clearly depicted both the common hepatic and the common bile ducts. MRCP was accurate in excluding BA as the cause of neonatal cholestasis, while 99mTc disofenin cholescintigraphic findings were false-positive in two patients with non-obstructive cholestasis. We conclude that MRCP can be used to depict the major biliary structures of neonates and small infants and to exclude BA as the cause of neonatal cholestasis by allowing visualisation of the biliary tract.

Biliary Atresia↗