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Evaluation of the triangular cord sign in the diagnosis of biliary atresia
1Departments of Pediatrics and Radiology, Cairo University Children's Hospital, Cairo, Egypt.
Insights
The triangular cord (TC) sign aids in diagnosing biliary atresia in infants with jaundice. This ultrasound finding is reliable for early detection and management of this serious liver condition.
Area of Science:
- Pediatric Gastroenterology
- Diagnostic Imaging
- Hepatology
Background:
- Infantile cholestasis presents diagnostic challenges, necessitating timely identification of surgically correctable conditions like biliary atresia.
- Early diagnosis of biliary atresia is crucial to prevent irreversible liver damage.
Purpose of the Study:
- To evaluate the diagnostic utility of the triangular cord (TC) sign in infants with conjugated hyperbilirubinemia.
- To assess the TC sign's role in diagnosing biliary atresia and its potential in post-operative monitoring.
Main Methods:
- Prospective study of 65 infants (32-161 days) with conjugated hyperbilirubinemia.
- Ultrasonographic examination using a 7.0-MHz transducer to identify the TC sign, defined as an echogenic density cranial to the portal vein bifurcation.
- Correlation of TC sign presence with surgical and histological findings of biliary atresia.
Main Results:
- The TC sign was present in 25 infants, all confirmed to have biliary atresia.
- Among 40 infants without the TC sign, diagnoses included paucity of intrahepatic bile ducts, alpha-1-antitrypsin deficiency, and neonatal hepatitis.
- The TC sign disappeared post-Kasai procedure in 7 infants, reappearing in 3 with recurrent cholestasis.
Conclusions:
- The TC sign is a simple, reliable, and time-saving tool for diagnosing biliary atresia in infants with cholestasis.
- The TC sign can aid in monitoring patients post-hepatoportoenterostomy.
- A proposed diagnostic strategy involves intraoperative cholangiogram for TC sign-positive cases and percutaneous liver biopsy for TC sign-negative cases.
Background:
Infantile cholestasis continues to represent a diagnostic challenge. It is very important to diagnose surgically correctable disorders, such as biliary atresia, in a timely manner to prevent progressive damage to the liver. It has been recently suggested that the triangular cord (TC) sign is a simple and useful tool in the diagnosis of biliary atresia.
Methods:
We prospectively studied 65 infants presenting with conjugated hyperbilirubinemia (age range: 32-161 days). All patients underwent ultrasonographic examination with a 7.0-MHz transducer (Acuson, Mountain View, CA). The TC was defined as a triangular, or tubular, echogenic density seen immediately cranial to the portal vein bifurcation.
Results:
The TC sign was identified in 25 infants, and all of them had histologic features suggestive of biliary atresia; the diagnosis was confirmed at surgery by gross morphology of hepatobiliary system, and liver biopsy, with or without intraoperative cholangiogram. Among the 40 patients who did not have the TC sign, 6 had paucity of the intrahepatic bile ducts. Three had alph-1-antitrypsin deficiency, and 31 had neonatal hepatitis. None of the 40 patients who did not have the TC sign developed acholic stools. Seven patients with biliary atresia were followed by ultrasonographic examination for 6 months after the Kasai procedure. The TC sign disappeared in all patients after the surgery; however, the TC sign reappeared in 3 patients who developed progressive cholestasis after the procedure.
Conclusion:
The TC sign is a simple, timesaving, and reliable diagnostic tool in the evaluation of infants with infantile cholestasis. The TC sign may also prove to be helpful in following patients after hepatoportoenterostomy. We suggest a new diagnostic strategy for patients suspected to have biliary atresia. When the TC sign is visualized, the patient should undergo intraoperative cholangiogram to confirm the diagnosis of biliary atresia, reserving percutaneous liver biopsy for those patients in whom the TC sign could not be detected.
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