Scoring system in diagnosing biliary atresia
Authors: Rui Dong, Shan Zheng
Journal: Journal of Hepatology (2014)

Design and validation of a diagnostic score for biliary atresiaJournal of HepatologyVol. 61Issue 1PreviewThe dilemma of early diagnosis of biliary Atresia (BA), particularly distinguishing it from other causes of neonatal cholestasis is challenging. The aim was to design and validate a scoring system for early discrimination of BA from other causes of neonatal cholestasis. Full-Text PDF Reply to: “Scoring system in diagnosing biliary atresia”Journal of HepatologyVol. 61Issue 6PreviewWe appreciate the interest of Dong and Zheng in our recent study [1]. Although hepatobiliary scintigraphy (HBS) is one of the most sensitive methods for the diagnosis of biliary atresia (BA), it has a low specificity (70.4%) as reported in a meta-analysis of 81 studies [2]. Sun et al. [3] reported a 13.3% (66/498) false positive rate of HBS. They concluded that the excessive dependence on HBS may contribute to the misdiagnosis of BA. Although HBS, showing biliary excretion, excludes BA, non-excretion neither confirms the diagnosis of BA nor rules out the diagnosis of non-BA aetiologies [4]. Full-Text PDF Open Access We read with great interest the article by El-Guindi et al. [[1]El-Guindi M.A. Sira M.M. Sira A.M. Salem T.A. El-Abd O.L. Konsowa H.A. et al.Design and validation of a diagnostic score for biliary atresia.J Hepatol. 2014; 61: 116-123Abstract Full Text Full Text PDF PubMed Scopus (80) Google Scholar] who propose a diagnostic scoring system for biliary atresia (BA), including clinical, laboratory, ultrasonographic, and histopathological parameters. They conclude that the scoring system could discriminate BA from other causes of neonatal cholestasis with a sensitivity, specificity and accuracy of 100%, 97.67%, and 98.83%, respectively, in the validation set. Thus, unnecessary intraoperative cholangiography can be avoided in non-BA patients. Although we appreciate their impressive findings, we would raise a few issues.First, it is known that hepatobiliary scintigraphy is an important diagnostic modality for neonatal cholestasis and phenobarbital-enhanced hepatobiliary scintigraphy (PEHS) is considered to be the most sensitive, non-invasive diagnostic method for BA [2Kianifar H.R. Tehranian S. Shojaei P. Adinehpoor Z. Sadeghi R. Kakhki V.R. et al.Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature.Pediatr Radiol. 2013; 43: 905-919Crossref PubMed Scopus (61) Google Scholar, 3Kwatra N. Shalaby-Rana E. Narayanan S. Mohan P. Ghelani S. Majd M. Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center.Pediatr Radiol. 2013; 43: 1365-1375Crossref PubMed Scopus (24) Google Scholar]. Two decades of experience at one tertiary centre demonstrated that PEHS was 100% sensitive, 93.0% specific, and 94.6% accurate in diagnosing BA [[3]Kwatra N. Shalaby-Rana E. Narayanan S. Mohan P. Ghelani S. Majd M. Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center.Pediatr Radiol. 2013; 43: 1365-1375Crossref PubMed Scopus (24) Google Scholar]. Experience at another centre demonstrated that 99mTc-EHIDA hepatobiliary scintigraphy in combination with duodenal fluid examination had both a sensitivity and specificity of 100% [[4]Liu S.X. Huang Z.H. The value of radionuclide hepatobiliary scintigraphy in combination with determination of bilirubin from duodenal drainage in differential diagnosis of infantile persistent jaundice.Front Med China. 2010; 4: 342-345Crossref PubMed Scopus (7) Google Scholar]. Thus, hepatobiliary scintigraphy is a non-invasive, safe, and accurate method for differential diagnosis of BA and other causes of neonatal cholestasis [3Kwatra N. Shalaby-Rana E. Narayanan S. Mohan P. Ghelani S. Majd M. Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center.Pediatr Radiol. 2013; 43: 1365-1375Crossref PubMed Scopus (24) Google Scholar, 5Lin W.Y. Lin C.C. Changlai S.P. Shen Y.Y. Wang S.J. Comparison technetium of Tc-99m disofenin cholescintigraphy with ultrasonography in the differentiation of biliary atresia from other forms of neonatal jaundice.Pediatr Surg Int. 1997; 12: 30-33Crossref PubMed Scopus (35) Google Scholar]. Unfortunately, the present study did not incorporate hepatobiliary scintigraphy in obtaining a diagnostic score for BA. Thus, the lack of this important imaging parameter might be responsible for the false positivity in one of the patients who was wrongly diagnosed with BA by the scoring system.Second, although liver biopsy appears to be an important diagnostic steps in the evaluation of BA, which may be safely performed in small infants, it is undeniably an invasive procedure associated with several adverse events and complications, such as pain, bleeding, and even death [[6]Rockey D.C. Caldwell S.H. Goodman Z.D. Nelson R.C. Smith A.D. Liver biopsy.Hepatology. 2009; 49: 1017-1044Crossref PubMed Scopus (1464) Google Scholar]. Moreover, liver biopsy is limited by sampling errors and observer variability, with possibility of omissions and false-positive or false-negative results. Thus, liver biopsy should be avoided whenever possible, and hepatobiliary scintigraphy, especially PEHS, may take the place of liver biopsy in the diagnosis of BA [3Kwatra N. Shalaby-Rana E. Narayanan S. Mohan P. Ghelani S. Majd M. Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center.Pediatr Radiol. 2013; 43: 1365-1375Crossref PubMed Scopus (24) Google Scholar, 5Lin W.Y. Lin C.C. Changlai S.P. Shen Y.Y. Wang S.J. Comparison technetium of Tc-99m disofenin cholescintigraphy with ultrasonography in the differentiation of biliary atresia from other forms of neonatal jaundice.Pediatr Surg Int. 1997; 12: 30-33Crossref PubMed Scopus (35) Google Scholar].Finally, the sample size was relatively small in the present study, and thus, validation of the scoring system in a large cohort of patients is essential before the scoring system can be used in clinical practice. We recommend that the scoring system be reassessed with or without incorporation of PEHS and with or without removal of liver biopsy in a large study.In addition, there seems an error in the manuscript; the cut-off value may be “31.164”, not “311.644” in Fig. 1C and D.Conflict of interestThe authors declared that they do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript. We read with great interest the article by El-Guindi et al. [[1]El-Guindi M.A. Sira M.M. Sira A.M. Salem T.A. El-Abd O.L. Konsowa H.A. et al.Design and validation of a diagnostic score for biliary atresia.J Hepatol. 2014; 61: 116-123Abstract Full Text Full Text PDF PubMed Scopus (80) Google Scholar] who propose a diagnostic scoring system for biliary atresia (BA), including clinical, laboratory, ultrasonographic, and histopathological parameters. They conclude that the scoring system could discriminate BA from other causes of neonatal cholestasis with a sensitivity, specificity and accuracy of 100%, 97.67%, and 98.83%, respectively, in the validation set. Thus, unnecessary intraoperative cholangiography can be avoided in non-BA patients. Although we appreciate their impressive findings, we would raise a few issues. First, it is known that hepatobiliary scintigraphy is an important diagnostic modality for neonatal cholestasis and phenobarbital-enhanced hepatobiliary scintigraphy (PEHS) is considered to be the most sensitive, non-invasive diagnostic method for BA [2Kianifar H.R. Tehranian S. Shojaei P. Adinehpoor Z. Sadeghi R. Kakhki V.R. et al.Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature.Pediatr Radiol. 2013; 43: 905-919Crossref PubMed Scopus (61) Google Scholar, 3Kwatra N. Shalaby-Rana E. Narayanan S. Mohan P. Ghelani S. Majd M. Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center.Pediatr Radiol. 2013; 43: 1365-1375Crossref PubMed Scopus (24) Google Scholar]. Two decades of experience at one tertiary centre demonstrated that PEHS was 100% sensitive, 93.0% specific, and 94.6% accurate in diagnosing BA [[3]Kwatra N. Shalaby-Rana E. Narayanan S. Mohan P. Ghelani S. Majd M. Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center.Pediatr Radiol. 2013; 43: 1365-1375Crossref PubMed Scopus (24) Google Scholar]. Experience at another centre demonstrated that 99mTc-EHIDA hepatobiliary scintigraphy in combination with duodenal fluid examination had both a sensitivity and specificity of 100% [[4]Liu S.X. Huang Z.H. The value of radionuclide hepatobiliary scintigraphy in combination with determination of bilirubin from duodenal drainage in differential diagnosis of infantile persistent jaundice.Front Med China. 2010; 4: 342-345Crossref PubMed Scopus (7) Google Scholar]. Thus, hepatobiliary scintigraphy is a non-invasive, safe, and accurate method for differential diagnosis of BA and other causes of neonatal cholestasis [3Kwatra N. Shalaby-Rana E. Narayanan S. Mohan P. Ghelani S. Majd M. Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center.Pediatr Radiol. 2013; 43: 1365-1375Crossref PubMed Scopus (24) Google Scholar, 5Lin W.Y. Lin C.C. Changlai S.P. Shen Y.Y. Wang S.J. Comparison technetium of Tc-99m disofenin cholescintigraphy with ultrasonography in the differentiation of biliary atresia from other forms of neonatal jaundice.Pediatr Surg Int. 1997; 12: 30-33Crossref PubMed Scopus (35) Google Scholar]. Unfortunately, the present study did not incorporate hepatobiliary scintigraphy in obtaining a diagnostic score for BA. Thus, the lack of this important imaging parameter might be responsible for the false positivity in one of the patients who was wrongly diagnosed with BA by the scoring system. Second, although liver biopsy appears to be an important diagnostic steps in the evaluation of BA, which may be safely performed in small infants, it is undeniably an invasive procedure associated with several adverse events and complications, such as pain, bleeding, and even death [[6]Rockey D.C. Caldwell S.H. Goodman Z.D. Nelson R.C. Smith A.D. Liver biopsy.Hepatology. 2009; 49: 1017-1044Crossref PubMed Scopus (1464) Google Scholar]. Moreover, liver biopsy is limited by sampling errors and observer variability, with possibility of omissions and false-positive or false-negative results. Thus, liver biopsy should be avoided whenever possible, and hepatobiliary scintigraphy, especially PEHS, may take the place of liver biopsy in the diagnosis of BA [3Kwatra N. Shalaby-Rana E. Narayanan S. Mohan P. Ghelani S. Majd M. Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center.Pediatr Radiol. 2013; 43: 1365-1375Crossref PubMed Scopus (24) Google Scholar, 5Lin W.Y. Lin C.C. Changlai S.P. Shen Y.Y. Wang S.J. Comparison technetium of Tc-99m disofenin cholescintigraphy with ultrasonography in the differentiation of biliary atresia from other forms of neonatal jaundice.Pediatr Surg Int. 1997; 12: 30-33Crossref PubMed Scopus (35) Google Scholar]. Finally, the sample size was relatively small in the present study, and thus, validation of the scoring system in a large cohort of patients is essential before the scoring system can be used in clinical practice. We recommend that the scoring system be reassessed with or without incorporation of PEHS and with or without removal of liver biopsy in a large study. In addition, there seems an error in the manuscript; the cut-off value may be “31.164”, not “311.644” in Fig. 1C and D. Conflict of interestThe authors declared that they do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript. The authors declared that they do not have anything to disclose regarding funding or conflict of interest with respect to this manuscript.