Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis

Fujishiro, Jun; Suzuki, Kan; Watanabe, Miho; Uotani, Chizue; Takezoe, Toshiko; Takamoto, Naohiro; Hayashi, Kentarō

doi:10.1007/s00383-018-4316-3

Cited by 27 publications

(10 citation statements)

References 16 publications

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“…Intraoperative cholangiography (which can be undertaken laparoscopically) was considered a definitive and gold standard for diagnosing BA until the twenty-first century ( 7 – 9 ). This technique, however, from the beginning has been reported to lead to misdiagnoses in 1967 ( 10 ), and this situation is now revealed by an increasing number of studies ( 11 – 17 ). Furthermore, the Cholestasis Guideline Committee stated that intraoperative cholangiography could be misleading in up to 20% of the cases ( 18 ).…”

Section: Introductionmentioning

confidence: 99%

Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants

Rong

Tang

et al. 2022

Front. Pediatr.

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ObjectiveOperative cholangiography, the gold standard for the diagnosis of biliary atresia (BA), is being challenged due to an increase in the studies of misdiagnosis. A previous study has shown that the laparoscopic hepatic subcapsular spider-like telangiectasis (HSST) sign was accurate for diagnosing BA. This study aims to compare the performance of the HSST sign with cholangiography in the identification of BA.MethodsWe prospectively screened consecutive infants with cholestasis who underwent laparoscopic exploration in this multicenter study. Demographics, intraoperative findings (videos and images), and outcomes were retrospectively analyzed. The data of the HSST sign and cholangiography were compared according to the final diagnosis. Then, the diagnostic accuracy of the BA using the HSST sign and cholangiography was validated in other independent cohorts.ResultsA total of 2,216 patients were enrolled in this study. The sensitivity and negative predictive values were both 100% for diagnosing BA based on the HSST sign and cholangiography. The specificity, negative predictive value, and accuracy of the HSST sign (97.2, 99.2, 99.3%) in discriminating BA were significantly higher than operative cholangiography (81.6, 94.9, 95.8; p < 0.001). Moreover, to realize the early diagnosis of BA, the accuracy of the HSST sign in identifying BA was better than cholangiography in the subgroup of neonates (98.7% vs. 95.0%; p = 0.032). Interestingly, 92 non-BA patients without the HSST sign had positive cholangiography. Among them, 28 infants had negative cholangiography when the common bile duct was compressed and 39 patients displayed visible bile ducts due to repeated postoperative biliary irrigation. The other 25 patients (18 with the Alagille syndrome, 5 with progressive familial intrahepatic cholestasis, and 2 with the neonatal hepatitis syndrome) had consistently positive cholangiography. In the independent validation cohort, the diagnostic accuracy of the HSST sign (99.2%) was higher than cholangiography (95.0%, p = 0.012).ConclusionThe laparoscopic HSST sign is superior to cholangiography in the diagnosis of BA in the infants with cholestasis and has advantages in early diagnosis. This method is expected to become a novel shift for diagnosing BA during ongoing laparoscopy.

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Section: Introductionmentioning

confidence: 99%

Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants

Rong

Tang

et al. 2022

Front. Pediatr.

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“…Fujishiro et al carried out a systematic review [6 studies (394 AGS patients)] and found that liver transplantation (odds ratio:6.46) and mortality (odds ratio:15.05) were significantly higher in AGS patients who underwent a Kasai than in those who did not. (2) An intra-operative cholangiogram may help in solving this diagnostic dilemma however in AGS there may be non-visualisation of the common hepatic duct or confluence masquerading as BA. (4) Eventually it is testing for JAG1 (or rarely NOTCH2) mutation that gives one a confirmatory diagnosis as seen in our patient.…”

Section: Discussionmentioning

confidence: 99%

“…(1) There have been reports in the past where children with AGS have been initially diagnosed as BA and have even inadvertently undergone a Kasai procedure, which has led to poor outcomes. (2) We present a 2.5-month old female in whom the diagnostic dilemma between AGS and BA was eventually sorted by genetic analysis. We highlight the clinical, biochemical and radiological features that helps one in differentiating between these two conditions.…”

Section: Introductionmentioning

confidence: 99%

Alagille Syndrome Mimicking Biliary Atresia

Chacham¹,

Bolia²,

Bhakat³

et al. 2022

Annals of Pediatric Gastroenterology &Amp; Hepatology

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The clinical presentation of jaundice, dark urine, persistently acholic stools, hepatosplenomegaly and a rudimentary, non-contractile gall bladder on ultrasound in an infant raises a suspicion of biliary atresia (BA). However, Alagille syndrome (AGS) may clinically, radiologically and histopathologically mimic BA and should be suspected in infants with other extrahepatic features.We report a 2.5-month old girl with an initial suspicion of BA who was eventually found to have AGS. We highlight the clinical, biochemical and radiological features that helps one in differentiating between these two conditions.

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“…16 In addition, a recent systematic review indicated that patients who underwent KP could have a poor prognosis. 17 Therefore, the possibility of other BA cases of subgroup ο overlapping Alagille syndrome in the JBAR cannot be excluded. Therefore, BA with subgroup ο should be managed with careful attention to the association with Alagille syndrome.…”

Section: Discussionmentioning

confidence: 99%

Anatomical patterns of biliary atresia including hepatic radicles at the porta hepatis influence short‐ and long‐term prognoses

Sasaki

Nio

Ando

et al. 2021

J Hepato Biliary Pancreat

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Background/Purpose: The biliary atresia (BA) inflammatory process leads to various obstructive patterns of extrahepatic biliary trees. The significance of the various BA obstructive patterns is unclear. This study aimed to determine the relationship between the anatomical patterns of the biliary tract and short-and long-term prognoses in BA. Methods: Between 1989 and 2018, 3483 patients were registered in the Japanese Biliary Atresia Registry. For this study, we selected 2649 patients who underwent Kasai portoenterostomy (KP) between the ages of 31 and 90 days to eliminate the influence of age at KP as much as possible. Results: Regarding the main type, there were significant differences in the jaundice clearance rate (JCR; Type I: 67.9%, Type I-cyst: 79.4%, Type II: 74.5%, Type III: 60.9%; P < .0001) and the native liver survival rate (NLSR; P < .0001). In subgroups with hepatic radicles in Types I, II, and I-cyst, there was a significant difference in JCR (P = .0004) and NLSR (P = .0026). In subgroups with hepatic radicles in Type III, there was a significant difference in JCR (P = .0148) and NLSR (P = .0421). Conclusions: Anatomical patterns of obstruction influenced short-and long-term prognoses in BA. These patterns were suggested to be prognostic factors following KP.

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Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis

Abstract: The Kasai operation was associated with poor outcomes in AGS patients. It remains unclear if the Kasai operation directly deteriorates liver and patient outcomes in AGS patients.

Cited by 27 publications

References 16 publications

Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants

Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants

Alagille Syndrome Mimicking Biliary Atresia

Anatomical patterns of biliary atresia including hepatic radicles at the porta hepatis influence short‐ and long‐term prognoses

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