The intraoperative hepatic subcapsular spider-like telangiectasia (HSST) sign may help in differentiating biliary atresia (BA) from other causes of hepatic cholestasis, including Alagille syndrome (ALGS), according to a study published in BMC Pediatrics.
“We initially thought that HSST sign might be related to liver fibrosis, but later observed that young patients (younger than 20 days) without liver fibrosis also have HSST sign,” the study authors said.
They observed the HSST sign in all patients with BA on diagnostic laparoscopy but not in patients with other cholestatic diseases. The diagnostic performance of the HSST was remarkable, showing a sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of 100% for diagnosing BA. The area under the receiver operating characteristic curve was 1.
In addition, the BA group had a higher incidence of abnormal gallbladder and liver stiffness values, as well as increased γ-gamma-glutamyl transferase (γ-GGT) levels compared to the non-BA group. The assessment of the prognostic value of γ-GGT >182 U/L revealed a sensitivity, specificity, PPV, and NPV of 75.5%, 85.0%, 92.5%, and 58.6%, respectively. Eight patients in the non-BA group had an abnormal gallbladder and 2 patients had an irregular gallbladder, including 1 patient with ALGS.
Read more about ALGS differential diagnosis
On the other hand, Zhang et al did not find any significant differences in the levels of total bilirubin, direct bilirubin, alkaline phosphatase (ALP), alanine aminotransferase (ALT), and aspartate aminotransferase (AST) between the BA group and the non-BA group.
The retrospective study enrolled 69 participants with jaundice who were divided into 2 groups: the BA group (n=49) and the non-BA group (n=20). The non-BA group included patients with cytomegalovirus hepatitis (n=6), idiopathic neonatal hepatitis (n=10), progressive familial intrahepatic cholestasis (n=3), and ALGS (n=1).
Zhang K, Tang Y, Liu R, et al. Intraoperative hepatic subcapsular spider-like telangiectasia sign for the definitive diagnosis of biliary atresia. BMC Pediatr. 2023;23(1):63. doi:10.1186/s12887-022-03831-z