eMedicine Specialties > Radiology > Pediatrics

Biliary Atresia: Follow-up

Author: Katherine Zukotynski, MD, Resident Physician, Joint Program in Nuclear Medicine, Harvard Medical School
Coauthor(s): Paul S Babyn, MD, Associate Professor, Department of Medical Imaging, University of Toronto; Radiologist-in-Chief, Department of Diagnostic Imaging, The Hospital for Sick Children
Contributor Information and Disclosures

Updated: Oct 28, 2009

Intervention

Biliary atresia is a serious condition. If untreated, it can lead to liver cirrhosis, portal hypertension, hepatocellular cancer, and death before 2 years of age. Therefore, early diagnosis and treatment are essential.

Cholangiography

In general, if clinical concern persists after ultrasonography and hepatobiliary scintigraphy are performed, exploratory laparotomy with surgical cholangiography may be recommended. This is typically done by injecting contrast material through the gallbladder. If no communication is seen between the biliary tree and the gastrointestinal tract, biliary atresia is diagnosed.

Percutaneous transhepatic cholangiography can be used to diagnose biliary atresia, especially when combined with sonography. It may be technically challenging, and the results are definitive only if a normal intrahepatic and extrahepatic biliary system is seen. Sonography-guided percutaneous cholecystocholangiography is a relatively new technique in which radiographic contrast material is injected into the gallbladder under sonographic guidance and the extrahepatic biliary system is viewed with fluoroscopy. Although invasive, the technique has distinct advantages in that it is easier to perform and does not require general anesthesia.

Endoscopic retrograde cholangiopancreatography allows direct visualization of the extrahepatic biliary tree with the injection of radiologic contrast agent into the extrahepatic biliary system through the papilla of Vater. It is a rarely used invasive technique, and it requires a general anesthetic, substantial expertise, and the availability of sufficiently small endoscopes. This technique can show obstruction in the common bile duct and enables visualization of the extrahepatic biliary system distal to the common hepatic duct and the extrahepatic biliary system with bile lakes at the porta hepatis.

Duodenal intubation

Duodenal intubation is not commonly used in diagnosing biliary atresia because it is cumbersome and because strict criteria for the technique are not defined. To perform this study, a nasogastric tube is placed in the distal duodenum. The absence of bilirubin or bile acids in aspirated fluid suggests obstruction. Positive predictive values are as high as 92%, with sensitivity and specificity higher than 90%.

The detection of radioactive tracer in aspirated duodenal fluid after hepatobiliary scintigraphy was studied. The utility of the test is not well defined.

Liver biopsy

Percutaneous liver biopsy is useful in evaluating neonatal cholestasis. Histologic findings, including bile-duct proliferation and obstruction, may not be definitive in neonates younger than 2 weeks. Results of repeat biopsy at 2-week intervals confirm the diagnosis in as many as 95% of patients.

Surgery
The current treatment of biliary atresia is surgical and typically involves the creation of a hepatoportoenterostomy by means of surgical anastomosis of the bowel with bile-duct remnants at the porta hepatis. The Kasai procedure consists of mobilizing the extrahepatic ducts and anastomosing a jejunal Roux en-Y loop to the liver hilum. This procedure seems to increase survival dramatically, especially if it is performed within the first 2 months of life. Complications include progressive biliary cirrhosis, ascending cholangitis, and portal hypertension.

Liver transplantation is indicated in cases of failed portoenterostomy, progressive fibrosis, or biliary cirrhosis. In fact, biliary atresia is the most common cause of end-stage liver disease in infants and a leading indication for liver transplantation.

 


More on Biliary Atresia

Overview: Biliary Atresia
Imaging: Biliary Atresia
Follow-up: Biliary Atresia
Multimedia: Biliary Atresia
References
Further Reading
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Keywords

biliary atresia, extrahepatic biliary system, extrahepatic bile ducts, intrahepatic bile ducts, fetal biliary atresia, embryonic biliary atresia, postnatal biliary atresia, neonatal obstructive cholestasis, Kasai portoenterostomy procedure, Kasai classification, type I biliary atresia, type II biliary atresia, type III biliary atresia, gallbladder ghost triad, triangular cord sign

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Contributor Information and Disclosures

Author

Katherine Zukotynski, MD, Resident Physician, Joint Program in Nuclear Medicine, Harvard Medical School
Katherine Zukotynski, MD is a member of the following medical societies: Radiological Society of North America
Disclosure: Nothing to disclose.

Coauthor(s)

Paul S Babyn, MD, Associate Professor, Department of Medical Imaging, University of Toronto; Radiologist-in-Chief, Department of Diagnostic Imaging, The Hospital for Sick Children
Paul S Babyn, MD is a member of the following medical societies: Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

Henrique M Lederman, MD, PhD, Consulting Staff, Department of Radiology, LeBonheur Children's Medical Center and St Jude Children's Research Hospital; Professor of Radiology and Pediatric Radiology, Chief, Division of Diagnostic Imaging in Pediatrics, Federal University of Sao Paulo, Brazil
Henrique M Lederman, MD, PhD is a member of the following medical societies: Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD, Consulting Radiologist, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.

 
 
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