Hanna Lampela1, Maria Hukkinen2, Silja Kosola3, Timo Jahnukainen4, Mikko P Pakarinen2. 1. Gastrointestinal surgery, University of Helsinki and Helsinki University Hospital, Espoo, Finland. Electronic address: hanna.lampela@hus.fi. 2. Department of Pediatric Surgery, Pediatric Liver and Gut Research Group, Pediatric Research Center Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland. 3. Pediatric Research Center, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland. 4. Department of Pediatric Nephrology and Transplantation, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Abstract
OBJECTIVE: Our objective was to analyze performance of noninvasive markers for significant esophageal varices in relation to outcomes of endoscopic surveillance and primary prophylaxis in biliary atresia (BA). METHODS: This was a prospective follow-up study of a national cohort of BA patients born between 1989 and 2017, including 72 consecutive patients who underwent variceal surveillance endoscopies. The risk for developing significant varices (grade ≥ 2) and variceal bleeding was compared between successful (postoperative total bilirubin ≤34 μmol/L) and failed portoenterostomy (PE) patients. AUROC analyses and Wilcoxon signed ranks test were used to assess accuracy of noninvasive measures to predict the presence of significant varices after successful PE. RESULTS: In total, 72 patients underwent 471 endoscopies during 427 follow-up years. Among 45 successful PE patients (63%), varices appeared later [at median age 1.6 (0.7-14) vs. 0.8 (0.4-1.9) years] and bled less often [7% vs. 41%, p < 0.001 for both] than after failed PE. Liver biochemistry, stiffness, and predictive scores showed poor accuracy for the presence of significant varices. After failed PE, lowered plasma albumin concentration predicted varices with an AUROC of 0.69 (95% CI 0.52-0.85, p = 0.030). After successful PE the varices prediction rule with AUROC 0.72 (95% CI 0.64-0.79) was the most accurate predictor. Individual predictors showed no meaningful changes between the two consecutive endoscopies leading to discovery of varices. CONCLUSION: Accurate targeting of endoscopies based on noninvasive predictors remains difficult during primary variceal prophylaxis protocol in BA. The differing prognoses after successful and failed PE should be considered in variceal surveillance and future studies. TYPE OF STUDY: Diagnostic/prognosis study. LEVEL OF EVIDENCE: Level II.
OBJECTIVE: Our objective was to analyze performance of noninvasive markers for significant esophageal varices in relation to outcomes of endoscopic surveillance and primary prophylaxis in biliary atresia (BA). METHODS: This was a prospective follow-up study of a national cohort of BA patients born between 1989 and 2017, including 72 consecutive patients who underwent variceal surveillance endoscopies. The risk for developing significant varices (grade ≥ 2) and variceal bleeding was compared between successful (postoperative total bilirubin ≤34 μmol/L) and failed portoenterostomy (PE) patients. AUROC analyses and Wilcoxon signed ranks test were used to assess accuracy of noninvasive measures to predict the presence of significant varices after successful PE. RESULTS: In total, 72 patients underwent 471 endoscopies during 427 follow-up years. Among 45 successful PE patients (63%), varices appeared later [at median age 1.6 (0.7-14) vs. 0.8 (0.4-1.9) years] and bled less often [7% vs. 41%, p < 0.001 for both] than after failed PE. Liver biochemistry, stiffness, and predictive scores showed poor accuracy for the presence of significant varices. After failed PE, lowered plasma albumin concentration predicted varices with an AUROC of 0.69 (95% CI 0.52-0.85, p = 0.030). After successful PE the varices prediction rule with AUROC 0.72 (95% CI 0.64-0.79) was the most accurate predictor. Individual predictors showed no meaningful changes between the two consecutive endoscopies leading to discovery of varices. CONCLUSION: Accurate targeting of endoscopies based on noninvasive predictors remains difficult during primary variceal prophylaxis protocol in BA. The differing prognoses after successful and failed PE should be considered in variceal surveillance and future studies. TYPE OF STUDY: Diagnostic/prognosis study. LEVEL OF EVIDENCE: Level II.