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Alagille Syndrome

Laparoscopic HSST better than cholangiography at diagnosing biliary atresia

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Laparoscopic hepatic subcapsular spider-like telangiectasis (HSST) sign is more accurate for diagnosing biliary atresia (BA) in infants with cholestasis than cholangiography, the current gold standard for the diagnosis of BA, according to a study.

Researchers prospectively screened 2216 consecutive infants with cholestasis who underwent laparoscopic exploration.

The predictive sensitivity and negative values were 100% each for diagnosing BA based on the HSST sign and cholangiography. The HSST sign was 97.2% in discriminating BA compared with 81.6% with cholangiography; negative predictive values were 99.2% and 94.9% and accuracy was 99.3% and 95.8%, respectively.

In a subgroup of neonates to determine the early diagnosis of BA, the accuracy of the HSST sign was 98.7% compared with 95.0% with cholangiography.

There were 92 patients without BA without the HSST sign that he positive cholangiography, of which 28 had negative cholangiography when the common bile duct was compressed and 39 displayed visible bile ducts due to repeated postoperative biliary irrigation. There was consistent positive cholangiography in the remaining 25 patients 18 with the Alagille syndrome, 5 with progressive familial intrahepatic cholestasis, and 2 with the neonatal hepatitis syndrome). In the independent validation cohort, the diagnostic accuracy of the HSST sign was 99.2% compared with cholangiography (95.0%, p = 0.012).

Reference
Li Y, Rong L, Tang J, et al. Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants. Front Pediatr. 2022;10:850449. doi: 10.3389/fped.2022.850449. PMID: 35547547; PMCID: PMC9081763.

 

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