eMedicine Specialties > Radiology > Pediatrics

Biliary Atresia

Katherine Zukotynski, MD, Resident Physician, Joint Program in Nuclear Medicine, Harvard Medical School
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

Updated: Oct 28, 2009

Introduction

Background

Biliary atresia is a condition in which the normal extrahepatic biliary system is disrupted. Progressive damage of extrahepatic and intrahepatic bile ducts secondary to inflammation may occur, leading to fibrosis, biliary cirrhosis, and eventual liver failure. Although various treatment options are under investigation, the primary therapy is surgical.^1,2,3,4

Biliary atresia affects approximately 1 in 10,000-15,000 births and occurs in 2 distinct clinical forms: fetal-embryonic (or syndromic) and perinatal (or acquired).

The fetal-embryonic form is characterized by early cholestasis, appears in the first 2 weeks of life, and accounts for 10-35% of all cases. In this form, the bile ducts are discontinuous at birth, and 10-20% of affected neonates have associated congenital defects, including situs inversus, polysplenia, malrotation, intestinal atresia, and cardiac anomalies, among others. The perinatal form of biliary atresia accounts for the remaining 65-90% cases. This form is typically found in neonates and infants aged 2-8 weeks. Progressive inflammation and obliteration of the extrahepatic bile ducts occurs after birth. This form is not associated with congenital anomalies, and infants may have a short jaundice -free interval.

Biliary atresia may be classified according to whether the disease can or cannot be corrected. As Image 1 shows, in the correctable group (10-15% of cases), the proximal common hepatic duct is patent, allowing for primary anastomosis of the extrahepatic bile duct to the bowel. Resection of the fibrous bile duct remnant may be done, followed by a Roux-en-Y anastomosis of the bowel to the bed of the porta hepatis, according to the Kasai portoenterostomy procedure. In the uncorrectable group, the extrahepatic bile ducts do not have the same patency as in the correctable group.

Types of biliary atresia. A, Operable, or correctable, biliary atresia, a major portion of the extrahepatic bile ducts are patent. B, The inoperable group does not have the patency shown in A. Reproduced with permission from BC Decker, 2000.

• In type I, the common bile duct is obliterated while the proximal bile ducts are patent.
• In type II, atresia of the hepatic duct is seen, with cystic bile ducts found at the porta hepatis. In type IIa, the cystic and common bile ducts are patent, whereas in type IIb, the cystic, common bile duct, and hepatic ducts are all obliterated.
• Type III atresia refers to discontinuity of both the right and the left hepatic ducts to the level of the porta hepatis. Unfortunately, type III biliary atresia is common, accounting for more than 90% of cases.

Classification of biliary atresia according to the location of involvement (gray areas). Type I is obliteration of the common bile duct while the proximal bile ducts are patent. Type IIa is atresia of the hepatic duct with cystic bile ducts found at the porta hepatis. Type IIb is atresia of the cystic duct, common bile duct, and hepatic ducts. Type III is involvement of the extrahepatic biliary tree and intrahepatic ducts of the porta hepatis.

Recent studies

In a study by Petersen et al, endoscopic retrograde cholangiopancreatography (ERCP) was performed in cholestatic patients younger than 6 months suspected of having an extrahepatic cause of cholestasis, particularly biliary atresia. In this series, the sensitivity of ERCP for diagnosing biliary atresia was 92% and specificity was 73%. The authors noted that in preselected patients, ERCP is not an alternative to noninvasive imaging but can avoid unnecessary surgical procedures in approximately 25% of cases. They therefore recommended that ERCP be performed before explorative laparotomy in all patients suspected of having biliary atresia.⁵

In a retrospective analysis by Shanmugam et al, ERCP had both a high positive and a high negative predictive value for biliary atresia in cholestatic infants younger than 100 days. Of 48 patients, ERCP demonstrated a patent biliary tree in 20 patients, and in 3 infants in whom cannulation failed, biliary atresia was confirmed at exploratory laparotomy. The remaining 25 infants, who were diagnosed with biliary atresia by ERCP, also proceeded to exploratory laparotomy, in which biliary atresia was confirmed in 22.⁶

Pathophysiology

The pathogenesis of biliary atresia is poorly understood. Although several mechanisms are implicated, no single etiologic factor is identified. Association with congenital anomalies in some infants suggests genetic factors. Infection with cytomegalovirus, group C rotavirus, and reovirus type 3 has been implicated in certain cases. Immune-mediated ductal injury is another consideration. Cholestasis almost certainly contributes to ongoing hepatocellular and biliary damage in infants with the disorder.⁷

Histologic findings on liver biopsy typically include acute on chronic inflammatory change with obstruction, fibrosis, and the proliferation of ductal and glandular elements. Findings may not be definitive early in the course of the disease, and repeat biopsy may be required to reach a definitive diagnosis. Image 3 depicts the histologic findings associated with biliary atresia.

Histologic findings associated with biliary atresia. Image shows end-stage biliary cirrhosis with micronodules (open arrow). Extensive perivascular fibrosis (arrowhead) and dilated inspissated cystic areas (closed arrow) are seen at the hepatic hilum about 5-10 mm from remnants of the obstructed bile duct. Image courtesy of James Phillips, MD, Department of Pathology, Hospital for Sick Children.

Frequency

United States

The overall incidence is 1 in 10,000-15,000 live births.

International

The incidence is higher in the Asian population than in other groups.

Mortality/Morbidity

Although the exact numbers may vary, if done in the first 2-3 months of life, a hepatoportoenterostomy can restore bile flow from the liver to the bowel in 60-80% of cases. The highest success rates are seen when surgery is performed earlier, before 2 months of age. It is thought that 20-30% of patients who undergo the Kasai procedure demonstrate long-term stability of their disease, although ongoing aggressive nutritional support may be required. Alternatively, liver transplantation may be lifesaving. The overall survival rate for patients after the Kasai procedure, including those who require subsequent liver transplantation, is thought to be more than 80%.

Race

In the United States, African-American infants are more commonly affected than white infants.

Sex

A slight female predominance is noted.

Presentation

Clinical manifestations of biliary atresia are nonspecific. Infants are often born at full term, have a normal gestational history with appropriate initial weight gain, and appear healthy despite having jaundice. Stools tend to become progressively acholic during the first weeks of life. Although hepatosplenomegaly may appear early, chronic hepatic failure is rare at the time of diagnosis. Associated congenital anomalies may or may not be present. Some cases present early with bleeding due to malabsorption and resulting vitamin K deficiency.^8,9,10

No single biochemical marker can be used to distinguish biliary atresia from other causes of neonatal cholestasis. Laboratory values often show a moderate conjugated hyperbilirubinemia, a mild to moderate elevation of serum transaminases, and a striking elevation of gamma-glutamyl transferase. The patient's prothrombin time and alkaline phosphatase may be elevated. Conjugated (direct) hyperbilirubinemia is defined by a serum conjugated bilirubin concentration greater than 2 mg/dL (34.2 mmol/mL) or 15% of the total bilirubin level. At the time of diagnosis, the total serum bilirubin level is typically 6-12 mg/dL with 50-80% conjugated.

Neonatal jaundice may be due to a variety of causes, including hemolysis, sepsis, and metabolic disease. When jaundice persists longer than 14 days, biliary atresia and idiopathic neonatal hepatitis must be considered. Together, these conditions cause more than 90% of all cases of neonatal obstructive cholestasis.

Early differentiation of extrahepatic biliary atresia from medical causes of hepatic dysfunction is important because earlier surgical intervention is directly associated with better outcomes. The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition guidelines suggest that 2-week old infants with jaundice should be evaluated for cholestasis. If the child is breast-fed, this may be delayed until 3 weeks if the physical examination is normal, there is no evidence of dark urine or light stools, and the child will be carefully monitored.

Evaluation should include a complete history and physical examination, as well as laboratory assessment. In some countries, stool color cards are used to screen infants for biliary atresia; however, the efficacy of this screening tool is still being reviewed, and the specificity is thought to be low. Radiologic studies, including abdominal ultrasound, hepatobiliary scintigraphy, magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiopancreatography (ERCP), may also be helpful.

Preferred Examination

Several imaging modalities have been used in the diagnosis of biliary atresia. Although some findings are highly suggestive of the disease, none is pathognomonic, and reliance on more than one test is common.

Ultrasonography is often the initial investigation in patients with suspected biliary atresia, followed by hepatobiliary scintigraphy, a study that has been used effectively for many years. If the diagnosis remains elusive after ultrasonography and hepatobiliary scintigraphy, magnetic resonance cholangiopancreatography (MRCP) may be helpful. Liver biopsy is often used to confirm the diagnosis of biliary atresia and may be done at the same time as surgical or percutaneous cholangiography. Alternative procedures include duodenal intubation and endoscopic retrograde cholangiopancreatography, which are generally not considered in infants.

Differential Diagnoses

Other Problems to Be Considered

Extrahepatic causes of neonatal cholestasis: eg, biliary atresia, bile duct stenosis/ stricture/ or cholelithiasis, tumors or masses, choledochal cyst.

Intrahepatic causes of neonatal cholestasis: infection (eg, CMV, rubella), metabolic (cystic fibrosis), genetic (eg, Alagille syndrome), toxicity (eg, aluminum, TPN associated), other (eg, idiopathic neonatal hepatitis).

Alagille syndrome
Turner syndrome
Trisomy 21
Caroli's disease
Progressive familial intrahepatic cholestasis
Cystic fibrosis
Alpha-1-anti-trypsin deficiency
Galactose-1-phosphate uridyltransferase deficiency (galactosemia)
Gaucher's disease
Wolman's disease
Neonatal Dubin-Johnson syndrome
Hypothyroidism
Panhypopituitarism
Inborn errors of bile acid synthesis
Cytomegalovirus infection 
Herpes simplex virus infection
Rubella
Syphilis
Toxoplasmosis
Bacterial sepsis
Erythroblastosis fetalis
Choledochal cyst
Idiopathic neonatal hepatitis
Nonsyndromic intrahepatic bile duct hypoplasia
Total parenteral nutrition (TPN)–associated cholestasis...

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 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 (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.

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.

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

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.

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.

Multimedia

Media file 1: Types of biliary atresia. A, Operable, or correctable, biliary atresia, a major portion of the extrahepatic bile ducts are patent. B, The inoperable group does not have the patency shown in A. Reproduced with permission from BC Decker, 2000.

Media file 2: Classification of biliary atresia according to the location of involvement (gray areas). Type I is obliteration of the common bile duct while the proximal bile ducts are patent. Type IIa is atresia of the hepatic duct with cystic bile ducts found at the porta hepatis. Type IIb is atresia of the cystic duct, common bile duct, and hepatic ducts. Type III is involvement of the extrahepatic biliary tree and intrahepatic ducts of the porta hepatis.

Media file 3: Histologic findings associated with biliary atresia. Image shows end-stage biliary cirrhosis with micronodules (open arrow). Extensive perivascular fibrosis (arrowhead) and dilated inspissated cystic areas (closed arrow) are seen at the hepatic hilum about 5-10 mm from remnants of the obstructed bile duct. Image courtesy of James Phillips, MD, Department of Pathology, Hospital for Sick Children.

Media file 4: 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.

Media file 5: 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.

Media file 6: 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.

Media file 7: 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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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

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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