eMedicine Specialties > Radiology > Pediatrics

Biliary Atresia: Imaging

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

Radiography

Findings

Radiography is generally not the study of choice for the evaluation of children with suspected biliary atresia.

Computed Tomography

Findings

CT is generally not the study of choice for the evaluation of children with suspected biliary atresia.

Magnetic Resonance Imaging

Findings

MR cholangiography is a relatively new technique for neonatal imaging.

Findings in infants with biliary atresia include incomplete visualization of the extrahepatic biliary system and periportal high signal intensity on T2-weighted MRIs, which may represent cystic dilatation of fetal bile ducts with surrounding fibrosis.

Degree of Confidence

Complete visualization of the extrahepatic biliary system excludes biliary atresia, whereas nonvisualization of the common or hepatic bile ducts suggests the disease. Preliminary studies suggest sensitivity and specificity of 90% and 77%, respectively, for the technique.

Ultrasonography

Findings

Ultrasonography is generally the initial investigation in patients with suspected biliary atresia. It can be used to assess the neonatal hepatobiliary system and may exclude other anatomic anomalies.11,12

Findings in infants with biliary atresia typically include an atretic gallbladder and a thin, indistinct gallbladder wall with an irregular or lobulated contour.

Although a normal (1.5 cm) or long (>4 cm) gallbladder may be seen in up to 10% of patients with biliary atresia, a length of less than 1.9 cm is most common. The constellation of findings constituting the gallbladder ghost triad are a gallbladder length less than 1.9 cm, a thin or indistinct gallbladder wall, and an irregular and lobular contour. Image 4 illustrates the gallbladder ghost triad, as seen in babies with biliary atresia.

Gallbladder ghost triad in babies with biliary at...

Gallbladder ghost triad in babies with biliary atresia. Longitudinal scans of the gallbladder in a 3-week-old girl (A) and a 5-week-old boy (B) demonstrate a short gallbladder, an irregular or lobulated contour, and a relatively indistinct lining and wall. Reproduced with permission from Tan Kendrick et al, 2003.

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Gallbladder ghost triad in babies with biliary at...

Gallbladder ghost triad in babies with biliary atresia. Longitudinal scans of the gallbladder in a 3-week-old girl (A) and a 5-week-old boy (B) demonstrate a short gallbladder, an irregular or lobulated contour, and a relatively indistinct lining and wall. Reproduced with permission from Tan Kendrick et al, 2003.


Ultrasonography can also be used to evaluate the hepatic parenchyma. In biliary atresia, the hepatic parenchyma is often inhomogeneous, with a marked increase in periportal echoes due to fibrosis. Sonograms in infants with biliary atresia often show a circumscribed, focal, triangular or tubular echogenic density more than 3 mm thick located cranial to the portal vein bifurcation. This is the triangular cord sign, as illustrated in Image 5, and corresponds to fibrosis of the extrahepatic biliary system.

Triangular cord. Transverse (a) and longitudinal...

Triangular cord. Transverse (a) and longitudinal (b) scans of the triangular cord in a baby with biliary atresia, which appears as a focal echogenic triangular or ovoid density just cranial to the bifurcation of the portal vein. Reproduced with permission from Tan Kendrick AP et al, 2003.

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Triangular cord. Transverse (a) and longitudinal...

Triangular cord. Transverse (a) and longitudinal (b) scans of the triangular cord in a baby with biliary atresia, which appears as a focal echogenic triangular or ovoid density just cranial to the bifurcation of the portal vein. Reproduced with permission from Tan Kendrick AP et al, 2003.


Although dilatation of the intrahepatic bile duct occurs infrequently, it suggests biliary atresia when present. Central biliary cysts or choledochal cysts may be associated with biliary atresia and are well depicted on sonograms, as illustrated in Image 6.

Biliary atresia and central cyst. A, Oblique son...

Biliary atresia and central cyst. A, Oblique sonogram demonstrates a large cystic structure in the porta hepatis. B, Intraoperative cholangiogram demonstrates filling of the cyst and mildly dilated intrahepatic ducts but no communication with the duodenum. Reproduced with permission from BC Decker, 2000.

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Biliary atresia and central cyst. A, Oblique son...

Biliary atresia and central cyst. A, Oblique sonogram demonstrates a large cystic structure in the porta hepatis. B, Intraoperative cholangiogram demonstrates filling of the cyst and mildly dilated intrahepatic ducts but no communication with the duodenum. Reproduced with permission from BC Decker, 2000.


A prominent hepatic artery is often seen in children with cirrhotic changes.

The absence of gallbladder contraction is only suggestive of biliary atresia. As many as 20% of children with biliary atresia have normal gallbladder contraction. Furthermore, the absence of gallbladder contraction is seen in children with cholestasis due to other causes.

Congenital anomalies may be present in children with biliary atresia. In particular, situs inversus and polysplenia are among the associated congenital anomalies that may be seen on sonograms.

Degree of Confidence

The presence of the gallbladder ghost triad is up to 97% sensitive and 100% specific for biliary atresia.

An absent common bile duct is thought to be 93% sensitive and 92% specific for the diagnosis of biliary atresia.

Reports suggest that the sensitivity of the triangular cord sign for the diagnosis of extrahepatic biliary atresia is greater than 72%, the specificity is greater than 97%, and the positive predictive value is 95%. The sensitivity may be decreased if diffusely increased periportal echogenicity from inflammation or cirrhosis obscures visualization.

It has been suggested that ultrasound may distinguish biliary atresia from other causes of conjugated hyperbilirubinemia in over 90% of infants if multiple ultrasound features are carefully evaluated.

Nuclear Imaging

Findings

Hepatobiliary scintigraphy has been used in the diagnosis of biliary atresia for many years.13

A technetium-labeled iminodiacetic acid (IDA) analogue is typically used. For example, radiopharamceuticals used include Tc-99m DISIDA (diisopropyl-iminodiacetic acid) or Tc-99m mebrofenin (trimethylbromo-iminodiacetic acid). Infants with biliary atresia usually have normal hepatocyte uptake of the radiotracer if they are younger than 2 months of age.

Improved sensitivity and specificity has been reported with delayed imaging, and following tracer administration, images are often acquired at 4-6 hours and 24 hours. The administration of phenobarbital 5 mg/kg/day in 2 equal doses for 3-5 days before the study may increase diagnostic accuracy. The addition of single photon emission computed tomography (SPECT) may increase specificity.

If excretion of radiotracer into the bowel is seen, biliary atresia is virtually excluded. If radiotracer excretion is absent after 24 hours, biliary atresia is suspected (see Image 7).

Anterior technetium-labeled diisopropyl iminodiac...

Anterior technetium-labeled diisopropyl iminodiacetic acid (IDA) scan in a patient with biliary atresia shows no excretion of the radiopharmaceutical into the bowel at 24 hours.

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Anterior technetium-labeled diisopropyl iminodiac...

Anterior technetium-labeled diisopropyl iminodiacetic acid (IDA) scan in a patient with biliary atresia shows no excretion of the radiopharmaceutical into the bowel at 24 hours.


Hepatobiliary scintigraphy may also be useful for the assessment of biliary excretion following surgical correction for biliary atresia.

Degree of Confidence

The reported sensitivity of hepatobiliary scintigraphy is high (~100%). The specificity is variable.

Several factors may limit the study. For example, severe neonatal hepatitis may result in decreased hepatic radiotracer uptake and therefore decreased excretion into the bowel. Also, because biliary atresia may be an evolving process, excretion of radiotracer into the gastrointestinal tract may be seen in children with biliary atresia in the early stages of the disease. Furthermore, reliability of the test diminishes with serum bilirubin levels greater than 10 mg/dL.

Patients should not have had barium studies within the 48 hours preceding hepatobiliary scintigraphy. If a barium study has been performed in this time frame, an abdominal radiograph may be indicated to make sure the bowel is clear of barium, a high density material that can result in artifacts.

Angiography

Findings

Angiography is generally not the study of choice for the evaluation of children with suspected biliary atresia.

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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