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Biliary Atresia - USG Reporting & Findings
Note: The query appears to be "biliary atresia" (not "biliary ascleresis" - there is no recognized condition by that name). If you meant a different condition, please clarify.
Background
Biliary atresia (BA) is a neonatal fibro-obliterative disease of the extrahepatic and/or intrahepatic bile ducts, occurring in ~1:8,000-18,000 live births. It is the most common cause of childhood liver transplant. Jaundice persisting beyond 2 weeks of life should raise suspicion.
Fasting ultrasound is the gold-standard first-line investigation when biliary atresia is suspected.
Sonographic Signs of Biliary Atresia
1. Triangular Cord Sign (Most Specific)
The most important and specific USG finding.
- Defined as a triangular or tubular echogenic density (fibrous ductal remnant) located cranial to the portal vein bifurcation, at the anterior aspect of the right portal vein at its bifurcation
- Represents the fibrotic obliterated remnant of the extrahepatic bile ducts
- Positive criterion: thickness of echogenic anterior wall of right portal vein (EARPV) >3-4 mm on longitudinal scan
Diagnostic performance (meta-analysis of 20 studies):
- Sensitivity: 74% (95% CI: 0.61-0.84)
- Specificity: 97% (95% CI: 0.95-0.99) - highly specific
- Accuracy: ~93-94%
- PPV: ~94%, NPV: ~94%
Fig: (A) Fibrous atretic plate (arrowheads) at porta hepatis - Triangular cord sign. No biliary dilatation is present. (B) Small (~10 mm) gallbladder with abnormal wall (arrowheads). - Grainger & Allison's Diagnostic Radiology
2. Gallbladder Abnormalities
Diagnostic performance (meta-analysis of 19 studies):
- Sensitivity: 85% (95% CI: 0.76-0.91)
- Specificity: 92% (95% CI: 0.81-0.97)
Abnormalities include:
| Finding | Detail |
|---|
| Absent gallbladder | Specificity ~99%; highly diagnostic |
| Small gallbladder | Length <1.5 cm (fasting), or <1.9 cm |
| Gallbladder "Ghost" Triad | (a) Length <1.9 cm, (b) lack of smooth/complete echogenic mucosal lining with indistinct wall, (c) irregular or lobular contour |
| Abnormal wall | Irregular, non-smooth, poorly defined wall |
| Dysmotility | No contraction after feeding |
Note: In ~10% of BA patients, the distal biliary tract is patent and a gallbladder may be seen - so presence of gallbladder does NOT exclude BA.
3. Absence of Common Bile Duct
- Non-visualization of the CBD is a supporting finding
- Less valuable than triangular cord sign and gallbladder findings
4. No Biliary Dilatation
- Critical negative finding - intrahepatic bile ducts are NEVER dilated in BA
- This differentiates BA from choledochal malformation and other obstructive causes
- Absence of biliary dilatation or sludge in a jaundiced infant should prompt suspicion for BA
5. Hepatic Artery Enlargement
- Hepatic artery diameter >2 mm at the porta hepatis is a supporting sign
- Less specific but useful adjunct
6. Liver Parenchyma Changes
- Often inhomogeneous with markedly increased periportal echoes due to fibrosis
- Progressive liver enlargement
- Signs of portal hypertension in advanced/late cases: splenomegaly, ascites, reversal of portal venous flow
Combined Diagnostic Performance
The combination of triangular cord sign + gallbladder abnormalities is superior to either alone:
- Sensitivity: 95% (95% CI: 0.70-0.99)
- Specificity: 89% (95% CI: 0.79-0.94)
A negative triangular cord sign combined with normal gallbladder morphology has a high negative predictive value (>90%) for extrahepatic BA in infants up to 3 months with conjugated hyperbilirubinemia - Grainger & Allison's Diagnostic Radiology.
Standard USG Reporting Checklist for Suspected Biliary Atresia
When reporting a fasting USG in a neonate with conjugated jaundice, document all of the following:
- Liver - size, echogenicity, parenchymal texture, periportal echogenicity
- Porta hepatis / Triangular cord sign - presence/absence, thickness of EARPV (measure in mm)
- Gallbladder - present/absent; if present: length, wall characteristics (smooth vs. irregular), mucosal lining, response to feeding (motility)
- Common bile duct - visualized/not visualized; if seen, measure diameter
- Intrahepatic bile ducts - dilated or not (dilatation argues against BA)
- Hepatic artery - diameter at porta hepatis (>2 mm supports BA)
- Portal vein - direction of flow, any thrombosis
- Spleen - size (splenomegaly suggests portal hypertension or polysplenia syndrome)
- Associated anomalies - polysplenia, preduodenal portal vein, situs, IVC
Differential Diagnosis on USG
| Condition | Gallbladder | Bile ducts | Triangular cord |
|---|
| Biliary atresia | Absent/small/abnormal | NOT dilated | Present |
| Choledochal cyst | Normal | Dilated (cystic) | Absent |
| Neonatal hepatitis | Normal | Not dilated | Absent |
| Inspissated bile | May contain sludge/echogenic bile | May be mildly dilated | Absent |
Additional Investigations After USG
- Hepatobiliary scintigraphy (99mTc-HIDA/DISIDA): High sensitivity but false-positive rate >20%; non-excretion of radionuclide into bowel supports BA but not specific
- MRCP: 82% accurate, 90% sensitive, 77% specific; useful for anatomy
- Liver biopsy: Inflammatory cells + fibrotic parenchyma + bile neoductal structures = definitive diagnosis
- Intraoperative cholangiography: Definitive diagnosis; required to define surgical anatomy for Kasai portoenterostomy
Key clinical point: Kasai portoenterostomy performed before 8 weeks of age gives the best outcomes - approximately half of patients reach adolescence without needing transplant. Early USG diagnosis is therefore time-critical.
Sources: Grainger & Allison's Diagnostic Radiology | Sleisenger & Fordtran's Gastrointestinal and Liver Disease | Schwartz's Principles of Surgery, 11th ed. | Bailey & Love's Short Practice of Surgery, 28th ed. |
AJR meta-analysis on triangular cord sign |
Medscape Biliary Atresia Imaging