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Ye F, Ling W, Wu Q, Ma H, Huang Z, Fang Y, Lyu G, Weng Z. Porta hepatis lymph nodes on US: not only identify biliary atresia but also predict outcomes after Kasai portoenterostomy surgery. Insights Imaging 2024; 15:154. [PMID: 38900331 PMCID: PMC11189885 DOI: 10.1186/s13244-024-01735-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2024] [Accepted: 06/02/2024] [Indexed: 06/21/2024] Open
Abstract
OBJECTIVES To evaluate the usefulness of porta hepatis lymph nodes (PHLNs) on ultrasonography (US) scans in diagnosing biliary atresia (BA) and predicting the outcomes after Kasai portoenterostomy (KPE) surgery. METHODS A total of 668 patients from one hospital were enrolled in the study (542 non-BA and 126 BA). The independent and combined diagnostic efficacy of PHLNs, triangular cord (TC) thickness, and gallbladder morphology were assessed by drawing the receiver operating characteristic (ROC) curves and counting the area under the ROC curve (AUC), sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). The US features, histopathological findings of PHLNs, and serum total bilirubin (TBIL) levels 3 months post-KPE were correlated. RESULTS The AUC, sensitivity, specificity, PPV, and NPV of PHLNs with hyperechogenicity and a maximum length larger than 8.4 mm were 0.898, 81.8%, 97.8%, 89.6%, and 95.8%, respectively. The combination of PHLNs, TC thickness, and gallbladder morphology achieved the best overall diagnostic efficacy among all indicators with an AUC of 0.927 and a sensitivity of 99.2%. The germinal center number and bile particle number of PHLNs were positively correlated with pathological size and US echogenicity intensity of PHLNs, respectively (r = 0.591, 0.377, p = 0.001, 0.004). The pathological size of PHLNs in BA patients was negatively correlated with jaundice clearance status 3 months after KPE surgery (r = -0.385, p = 0.047). CONCLUSION PHLNs with hyperechogenicity and a maximum length > 8.4 mm are useful US indicators for BA diagnosis. Additionally, the enlargement of PHLNs might play a role in predicting outcomes of KPE surgery. CRITICAL RELEVANCE STATEMENT The article proposed for the first time that PHLNs with hyperechogenicity and a maximum length > 8.4 mm are a useful US indicator for diagnosing BA. KEY POINTS PHLNs may be helpful in diagnosing BA and predicting outcomes after surgery. Enlarged hyperechoic PHLNs are a useful diagnostic indicator for BA, and play a role in predicting surgical outcomes. These findings can assist clinicians in more accurately diagnosing BA, enabling more timely treatments.
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Affiliation(s)
- Fengying Ye
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Wen Ling
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Qiumei Wu
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Hong Ma
- Department of Pathology, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Zhen Huang
- Department of Pathology, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Yifan Fang
- Department of Pediatric Surgery, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Guorong Lyu
- Department of Medical Ultrasonics, the Second Affiliated Hospital of Fujian Medical University, Quanzhou, China.
- Department of Clinical Medicine, Quanzhou Medical College, Quanzhou, China.
| | - Zongjie Weng
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.
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Zhou W, Ye Z, Huang G, Zhang X, Xu M, Liu B, Zhuang B, Tang Z, Wang S, Chen D, Pan Y, Xie X, Wang R, Zhou L. Interpretable artificial intelligence-based app assists inexperienced radiologists in diagnosing biliary atresia from sonographic gallbladder images. BMC Med 2024; 22:29. [PMID: 38267950 PMCID: PMC10809457 DOI: 10.1186/s12916-024-03247-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/26/2023] [Accepted: 01/02/2024] [Indexed: 01/26/2024] Open
Abstract
BACKGROUND A previously trained deep learning-based smartphone app provides an artificial intelligence solution to help diagnose biliary atresia from sonographic gallbladder images, but it might be impractical to launch it in real clinical settings. This study aimed to redevelop a new model using original sonographic images and their derived smartphone photos and then test the new model's performance in assisting radiologists with different experiences to detect biliary atresia in real-world mimic settings. METHODS A new model was first trained retrospectively using 3659 original sonographic gallbladder images and their derived 51,226 smartphone photos and tested on 11,410 external validation smartphone photos. Afterward, the new model was tested in 333 prospectively collected sonographic gallbladder videos from 207 infants by 14 inexperienced radiologists (9 juniors and 5 seniors) and 4 experienced pediatric radiologists in real-world mimic settings. Diagnostic performance was expressed as the area under the receiver operating characteristic curve (AUC). RESULTS The new model outperformed the previously published model in diagnosing BA on the external validation set (AUC 0.924 vs 0.908, P = 0.004) with higher consistency (kappa value 0.708 vs 0.609). When tested in real-world mimic settings using 333 sonographic gallbladder videos, the new model performed comparable to experienced pediatric radiologists (average AUC 0.860 vs 0.876) and outperformed junior radiologists (average AUC 0.838 vs 0.773) and senior radiologists (average AUC 0.829 vs 0.749). Furthermore, the new model could aid both junior and senior radiologists to improve their diagnostic performances, with the average AUC increasing from 0.773 to 0.835 for junior radiologists and from 0.749 to 0.805 for senior radiologists. CONCLUSIONS The interpretable app-based model showed robust and satisfactory performance in diagnosing biliary atresia, and it could aid radiologists with limited experiences to improve their diagnostic performances in real-world mimic settings.
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Affiliation(s)
- Wenying Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Zejun Ye
- School of Computer Science and Engineering, Sun Yat-Sen University, No. 132, East Outer Ring Road, Guangzhou, 510006, People's Republic of China
| | - Guangliang Huang
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Xiaoer Zhang
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Ming Xu
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Baoxian Liu
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Bowen Zhuang
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Zijian Tang
- Department of Ultrasound, Shenzhen Children's Hospital, No. 7019, Yitian Road, Futian District, Shenzhen, 518026, People's Republic of China
| | - Shan Wang
- Department of Ultrasound, Shenzhen Children's Hospital, No. 7019, Yitian Road, Futian District, Shenzhen, 518026, People's Republic of China
| | - Dan Chen
- Department of Ultrasound, Guangdong Women and Children's Hospital, No. 521 Xingnan Avenue, Panyu District, Guangzhou, 511400, People's Republic of China
| | - Yunxiang Pan
- Department of Ultrasound, Guangdong Women and Children's Hospital, No. 521 Xingnan Avenue, Panyu District, Guangzhou, 511400, People's Republic of China
| | - Xiaoyan Xie
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China
| | - Ruixuan Wang
- School of Computer Science and Engineering, Sun Yat-Sen University, No. 132, East Outer Ring Road, Guangzhou, 510006, People's Republic of China
| | - Luyao Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan Er Road, Guangzhou, 510080, People's Republic of China.
- Department of Ultrasound, Shenzhen Children's Hospital, No. 7019, Yitian Road, Futian District, Shenzhen, 518026, People's Republic of China.
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Lee HJ, Lee MS, Kim JY. ['Triangular Cord' Sign in Biliary Atresia]. JOURNAL OF THE KOREAN SOCIETY OF RADIOLOGY 2022; 83:1003-1013. [PMID: 36276196 PMCID: PMC9574273 DOI: 10.3348/jksr.2022.0085] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/03/2022] [Revised: 08/05/2022] [Accepted: 09/11/2022] [Indexed: 12/03/2022]
Abstract
Biliary atresia is an unknown etiology of extrahepatic bile duct obstruction with a 'fibrous ductal remnant,' which represents the obliterated ductal remnant in the porta hepatis. The sonographic'triangular cord' (TC) sign has been reported to indicate a fibrous ductal remnant in the porta hepatis. In this review, we discuss the correlations among surgicopathological and sonographic findings of the porta hepatis and the definition, objective criteria, diagnostic accuracy, and differential diagnosis of the TC sign in biliary atresia.
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Obaid AM, Turki A, Bellaaj H, Ksontini M. Detection of Biliary Artesia using Sonographic Gallbladder Images with the help of Deep Learning approaches. 2022 8TH INTERNATIONAL CONFERENCE ON CONTROL, DECISION AND INFORMATION TECHNOLOGIES (CODIT) 2022:705-711. [DOI: 10.1109/codit55151.2022.9804084] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
Affiliation(s)
| | - Amina Turki
- Control and Energy Management Laboratory (CEMLab), National Engineering School of Sfax,Sfax,Tunisia,3038
| | - Hatem Bellaaj
- Research laboratory on Development and Control of Distributed Applications. (ReDCAD) IPEIS-Route Menzel Chaker Km 0,5-3018 Sfax Tunisie,Sfax,Tunisia,BP 1172-3018
| | - Mohamed Ksontini
- Control & Energy Management Laboratory (CEM-Lab), National Engineering School of Sfax, University of Sfax,Sfax,Tunisia,3038
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Brahee DD, Lampl BS. Neonatal diagnosis of biliary atresia: a practical review and update. Pediatr Radiol 2022; 52:685-692. [PMID: 34331566 DOI: 10.1007/s00247-021-05148-y] [Citation(s) in RCA: 26] [Impact Index Per Article: 8.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/01/2021] [Revised: 06/03/2021] [Accepted: 06/28/2021] [Indexed: 02/02/2023]
Abstract
Biliary atresia is challenging to diagnose because many of the clinical and imaging features of this condition overlap with those of other causes of cholestasis in newborns. When jaundice persists beyond 2 weeks of age, the neonate should be evaluated for cholestasis, and biliary atresia - the most common cause of neonatal cholestasis - should be considered. It is critical to diagnose biliary atresia early because failure to treat can result in hepatic fibrosis and death in less than 1 year. In this paper, we review the current diagnostic imaging methods, differential considerations and treatment options for biliary atresia.
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Affiliation(s)
- Deborah D Brahee
- Department of Radiology, Cleveland Clinic, 9500 Euclid Ave., Mail Code L10, Cleveland, OH, 44193, USA.
| | - Brooke S Lampl
- Department of Radiology, Cleveland Clinic, 9500 Euclid Ave., Mail Code L10, Cleveland, OH, 44193, USA
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Weng Z, Ye F, Zhou L, Chen F, Ling W, Fang Y, Liu M, Wu Q, Qiu X, Lyu G. A nomogram model based on preoperative grey-scale US features and routine serum biomarkers to predict the outcome of infants with biliary atresia after Kasai portoenterostomy. Front Pediatr 2022; 10:972855. [PMID: 36340714 PMCID: PMC9631934 DOI: 10.3389/fped.2022.972855] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/19/2022] [Accepted: 09/27/2022] [Indexed: 11/13/2022] Open
Abstract
OBJECTIVE To establish a nomogram to predict the outcome of biliary atresia (BA) infants 3-months post- Kasai portoenterostomy (KPE). METHODS BA Infants who underwent KPE from two hospitals were included in the training (n = 161) and validation cohorts (n = 64). A logistic regression equation (Equation A) for predicting the serum total bilirubin (TBIL) level 3-month post-KPE was established in the training cohort. Then, a nomogram was developed based on Equation A in the training cohort and validated in the validation cohort. Moreover, a new equation (Equation B) was generated based on the nomogram and the size of the enlarged hilar lymph nodes (LNs) in the validation cohort. The predictive performance of the nomogram was evaluated by the receiver operating characteristic (ROC) curve and by calculating the area under the ROC curve (AUC), sensitivity, specificity, and positive (PPV) and negative (NPV) prediction values. RESULTS A nomogram based on gallbladder morphology and serum levels of TBIL and total protein (TP) was established with AUC (95%CI) of 0.673 (0.595, 0.745) and 0.647 (0.518, 0.763), sensitivity (95%CI) of 71.4% (62.1%,79.6%) and 81.8% (59.7%,94.8%), specificity (95%CI) of 63.3% (48.3%,76.6%) and 47.6% (32.0%,63.6%), PPV (95%CI) of 81.6% (72.5%,88.9%) and 45.0% (29.3%,61.5%), and NPV (95%CI) 49.2% (36.4%,62.1%) and 83.3% (62.6%,95.3%), respectively, in the training and validation cohorts. Furthermore, in the validation cohort, the AUC (95%CI) of Equation B was 0.798 (95%CI: 0.679, 0.888), which was significantly higher than that of the nomogram (P = 0.042). CONCLUSION A nomogram based on the pre-KPE gallbladder morphology, TBIL, and TP to predict the outcome of BA 3-months post-KPE is established. Moreover, the addition of the size of the enlarged hilar LNs into the nomogram further improves its predictive value.
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Affiliation(s)
- Zongjie Weng
- Department of Medical Ultrasonics, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, China.,Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Fengying Ye
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Luyao Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
| | - Fa Chen
- Department of Epidemiology and Health Statistics, School of Public Health, Fujian Medical University, Fuzhou, China
| | - Wen Ling
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Yifan Fang
- Department of Pediatric Surgery, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Min Liu
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Qiumei Wu
- Department of Medical Ultrasonics, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Xiuqing Qiu
- Department of Gynecology, Fujian Maternity and Child Health Hospital, College of Clinical Medicine for Obstetrics / Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China
| | - Guorong Lyu
- Department of Medical Ultrasonics, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, China.,Department of Clinical Medicine, Quanzhou Medical College, Quanzhou, China
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Yang P, Tang Y, Wang H, Zhang X, Yang B. Latest diagnostic performance of different ultrasonic features for biliary atresia. Acta Radiol 2021; 63:1593-1602. [PMID: 34854739 DOI: 10.1177/02841851211055820] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
BACKGROUND Early diagnosis of biliary atresia (BA) is an important clinical challenge. PURPOSE To summarize the latest diagnostic performance of different ultrasonic (US) features for BA. MATERIAL AND METHODS MeSH terms "biliary atresia" and "ultrasonography" and related hyponyms were used to search PubMed, EMBASE, and the Cochrane Central Register of Controlled Trials. Eligible articles were included and data were retrieved. The methodologic quality was assessed by version 2 of the Quality Assessment of Diagnostic Accuracy Studies tool. Estimated sensitivity and specificity of each US feature were calculated by Stata 14.0. RESULTS Fifty eligible studies on 5622 patients were included. Respective summary sensitivity and specificity were 77% (95% CI=69-84) and 98% (95% CI=96-99) for triangular cord sign (TCS) in 32 studies, 86% (95% CI=78-92) and 86% (95% CI=72-94) for shear wave elastography (SWE) in seven studies, 75% (95% CI=65-83) and 92% (95% CI=86-95) for gallbladder and biliary system abnormality (GBA) in 25 studies, and 81% (95% CI=69-90) and 79% (95% CI=67-87) for hepatic artery (HA) enlargement in seven studies. The overall US features from 11 studies yielded a summary sensitivity of 84% (95% CI=72-92) and specificity of 86% (95% CI=77-92). CONCLUSION TCS and GBA were the two most widely accepted US features currently used for differential diagnosis of BA. The newly developed SWE was an objective and convenient method with good diagnostic performance. HA enlargement can be used as an auxiliary sign.
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Affiliation(s)
- Pan Yang
- Department of Medical Ultrasonics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, PR China
| | - Yajie Tang
- Department of Cardiovascular Surgery, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, PR China
| | - Hongying Wang
- Department of Medical Ultrasonics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, PR China
| | - Xiangxiang Zhang
- Department of Medical Ultrasonics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, PR China
| | - Boyang Yang
- Department of Medical Ultrasonics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, PR China
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Zhou W, Yang Y, Yu C, Liu J, Duan X, Weng Z, Chen D, Liang Q, Fang Q, Zhou J, Ju H, Luo Z, Guo W, Ma X, Xie X, Wang R, Zhou L. Ensembled deep learning model outperforms human experts in diagnosing biliary atresia from sonographic gallbladder images. Nat Commun 2021; 12:1259. [PMID: 33627641 PMCID: PMC7904842 DOI: 10.1038/s41467-021-21466-z] [Citation(s) in RCA: 50] [Impact Index Per Article: 12.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/31/2020] [Accepted: 01/27/2021] [Indexed: 02/05/2023] Open
Abstract
It is still challenging to make accurate diagnosis of biliary atresia (BA) with sonographic gallbladder images particularly in rural area without relevant expertise. To help diagnose BA based on sonographic gallbladder images, an ensembled deep learning model is developed. The model yields a patient-level sensitivity 93.1% and specificity 93.9% [with areas under the receiver operating characteristic curve of 0.956 (95% confidence interval: 0.928-0.977)] on the multi-center external validation dataset, superior to that of human experts. With the help of the model, the performances of human experts with various levels are improved. Moreover, the diagnosis based on smartphone photos of sonographic gallbladder images through a smartphone app and based on video sequences by the model still yields expert-level performances. The ensembled deep learning model in this study provides a solution to help radiologists improve the diagnosis of BA in various clinical application scenarios, particularly in rural and undeveloped regions with limited expertise.
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Affiliation(s)
- Wenying Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China
| | - Yang Yang
- School of Computer Science and Engineering, Sun Yat-sen University, Guangzhou, P. R. China
| | - Cheng Yu
- Department of Ultrasound, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, P. R. China
| | - Juxian Liu
- Department of Ultrasound, West China Hospital, Sichuan University, Chengdu, P. R. China
| | - Xingxing Duan
- Department of Ultrasound, Hunan Children's Hospital, Changsha, P. R. China
| | - Zongjie Weng
- Department of Medical Ultrasonics, Fujian Provincial Maternity and Children's Hospital, Affiliated Hospital of Fujian Medical University, Fuzhou City, P. R. China
| | - Dan Chen
- Department of Ultrasound, Guangdong Women and Children' Hospital, Guangzhou, P. R. China
| | - Qianhong Liang
- Department of Ultrasound, Hexian Memorial Affiliated Hospital of Southern Medical University, Guangzhou, P. R. China
| | - Qin Fang
- Department of Ultrasound, The First People's Hospital of Foshan, Foshan City, P. R. China
| | - Jiaojiao Zhou
- Department of Ultrasound, West China Hospital, Sichuan University, Chengdu, P. R. China
| | - Hao Ju
- Department of Ultrasound, Shengjing Hospital of China Medical University, Shenyang, P. R. China
| | - Zhenhua Luo
- Institute of Precision Medicine, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China
| | - Weihao Guo
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China
| | - Xiaoyan Ma
- Department of Ultrasound, Guangdong Women and Children' Hospital, Guangzhou, P. R. China
| | - Xiaoyan Xie
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China.
| | - Ruixuan Wang
- School of Computer Science and Engineering, Sun Yat-sen University, Guangzhou, P. R. China.
| | - Luyao Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, P. R. China.
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Kim IH, An YS, Lee SJ, Hong J, Yoon JK. Hilar Choledochal Cyst Mimicking Biliary Atresia on Hepatobiliary Scintigraphy: a Case Report. Nucl Med Mol Imaging 2021; 55:96-99. [PMID: 33968276 DOI: 10.1007/s13139-021-00688-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2020] [Revised: 01/15/2021] [Accepted: 01/18/2021] [Indexed: 11/29/2022] Open
Abstract
Hepatobiliary scintigraphy has been widely used for the differential diagnosis of neonatal cholestasis. Relatively good hepatic uptake with no evidence of excretion into the bowel for up to 24 h is a representative finding of biliary atresia. Hepatobiliary scintigraphy has very high sensitivity and moderate specificity. Here, we report a false-positive case of hepatobiliary scintigraphy in a child with a choledochal cyst, which mimicked biliary atresia.
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Affiliation(s)
- Il-Hyun Kim
- Department of Nuclear Medicine & Molecular Imaging, Ajou University School of Medicine, Worldcup-ro 164, Suwon, Kyunggi-do 16499 Republic of Korea
| | - Young-Sil An
- Department of Nuclear Medicine & Molecular Imaging, Ajou University School of Medicine, Worldcup-ro 164, Suwon, Kyunggi-do 16499 Republic of Korea
| | - Su Jin Lee
- Department of Nuclear Medicine & Molecular Imaging, Ajou University School of Medicine, Worldcup-ro 164, Suwon, Kyunggi-do 16499 Republic of Korea
| | - Jeong Hong
- Department of Surgery, Ajou University School of Medicine, Worldcup-ro 164, Suwon, Kyunggi-do 16499 Republic of Korea
| | - Joon-Kee Yoon
- Department of Nuclear Medicine & Molecular Imaging, Ajou University School of Medicine, Worldcup-ro 164, Suwon, Kyunggi-do 16499 Republic of Korea
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Zhao D, Gu S, Gong X, Li Y, Sun X, Chen Y, Deng Z, Zhang Y. Web-based calculator for biliary atresia screening in neonates and infants with cholestasis. Transl Pediatr 2021; 10:225-235. [PMID: 33708508 PMCID: PMC7944186 DOI: 10.21037/tp-20-170] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/11/2022] Open
Abstract
BACKGROUND Distinguishing biliary atresia from non-biliary atresia in patients with cholestasis is challenging, as these conditions have a similar clinical presentation. We developed and externally validated a screening model for biliary atresia and devised a web-based calculator for use in clinical settings. METHODS A screening model was developed based on data from 227 cholestatic infants (82 and 145 with and without biliary atresia, respectively) and validated in 234 infants (90 and 144 with and without biliary atresia, respectively) admitted to three hospitals. Variables were selected from routine examination results using the least absolute shrinkage and selection operator method and entered into a logistic regression model to construct a biliary-atresia-risk-predicting equation. Cutoff values for risk stratification were estimated using model sensitivity, derived from the receiver-operating characteristic curves. RESULTS The final screening model included seven variables (i.e., weight at admission, clay-colored stools, γ-glutamyl transpeptidase and albumin levels at admission, abnormal gallbladder, triangular cord sign, and change in total bilirubin levels). The model generated an area under the curve of 0.94 with a sensitivity of 91.46 and specificity of 86.62 in the derivation cohort. This was confirmed in the validation cohort, as we found an area under the curve of 0.93 with a sensitivity of 93.1 and specificity of 80.15. Patients were stratified into three risk groups (low-, moderate-, and high-risk groups). Biliary atresia was excluded in the low-risk group. The high-risk group showed a higher detection rate of biliary atresia compared to the stool color screening method alone. This model was integrated into a user-friendly web-based system. CONCLUSIONS The screening tool had a high predictive value and may help in decision-making by physicians at tertiary and community hospitals.
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Affiliation(s)
- Dongying Zhao
- Department of Neonatology, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, China
| | - Shengli Gu
- Department of Ultrasound, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, China
| | - Xiaohui Gong
- Department of Neonatology, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Yahui Li
- Department of Neonatology, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, China
| | - Xiaoang Sun
- Department of Pediatric Digestive Diseases, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Yan Chen
- Department of Neonatology, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, China
| | - Zhaohui Deng
- Department of Pediatric Digestive Diseases, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Yongjun Zhang
- Department of Neonatology, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, China
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11
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Chen Y, Zhao D, Gu S, Li Y, Pan W, Zhang Y. Three-color risk stratification for improving the diagnostic accuracy for biliary atresia. Eur Radiol 2020; 30:3852-3861. [PMID: 32162000 DOI: 10.1007/s00330-020-06751-7] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2019] [Revised: 02/10/2020] [Accepted: 02/14/2020] [Indexed: 12/14/2022]
Abstract
OBJECTIVES It is challenging to early differentiate biliary atresia from other causes of cholestasis. We aimed to develop an algorithm with risk stratification to distinguish biliary atresia from infantile cholestasis. METHODS In this study, we enrolled infants with cholestasis into 2 subgroups from January 2010 to April 2019. A prospective cohort (subgroup 2) of 187 patients (107 with biliary atresia and 80 without biliary atresia) underwent acoustic radiation force impulse elastography. Stepwise regression was used to identify significant predictors of biliary atresia. A sequential algorithm with risk stratification was constructed. RESULTS Among 187 patients, shear wave speed > 1.35 m/s and presence of the triangular cord sign were considered high risk for biliary atresia (red), in which 73 of 78 patients (accuracy of 93.6%) with biliary atresia were identified. Afterwards, γ-GT, abnormal gallbladder, and clay stool were introduced into the algorithm and 55 intermediate-risk infants were identified (yellow) with a diagnostic accuracy of 60% for biliary atresia. Of the remaining 54 infants who were classified as low-risk patients (green), the accuracy for excluding biliary atresia was 98.1%. By applying a three-color risk stratification tool, 70.6% patients were identified as either high risk or low risk for biliary atresia (area under the curve, 0.983; sensitivity, 98.7%; specificity, 91.4%). We also estimated the risk of biliary atresia in different color groups, which was 94.7% (95%CI, 94.3-95.5%) in the red group and 7.2% (95%CI, 6.6-8.3%) in the green group. CONCLUSIONS Our simple noninvasive approach was able to identify biliary atresia with high accuracy. KEY POINTS • Five predictors, namely shear wave speed, triangle cord sign, γ-glutamyl transferase, abnormal gallbladder, and clay stool, were selected to identify biliary atresia in cholestasis. • Shear wave speed > 1.35 m/s and presence of the triangle cord sign were considered high-risk patients with a diagnostic accuracy of 93.6% for biliary atresia. • Risk for biliary atresia was high (red), intermediate (yellow), or low (green). In the red and green group, we achieved an extremely high diagnostic performance (area under the curve, 0.983; sensitivity, 98.7%; specificity, 91.4%).
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Affiliation(s)
- Yan Chen
- Department of Neonatology, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, 1665 Kong Jiang Road, Shanghai, 200092, China
| | - Dongying Zhao
- Department of Neonatology, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, 1665 Kong Jiang Road, Shanghai, 200092, China
| | - Shengli Gu
- Department of Ultrasound, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, China
| | - Yahui Li
- Department of Neonatology, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, 1665 Kong Jiang Road, Shanghai, 200092, China
| | - Weihua Pan
- Department of Pediatric Surgery, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, 1665 Kong Jiang Road, Shanghai, 200092, China.
| | - Yongjun Zhang
- Department of Neonatology, Xinhua Hospital, Shanghai JiaoTong University School of Medicine, 1665 Kong Jiang Road, Shanghai, 200092, China.
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12
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Zhou W, Chen D, Jiang H, Shan Q, Zhang X, Xie X, Zhou L. Ultrasound Evaluation of Biliary Atresia Based on Gallbladder Classification: Is 4 Hours of Fasting Necessary? JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2019; 38:2447-2455. [PMID: 30680769 DOI: 10.1002/jum.14943] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/29/2018] [Revised: 12/12/2018] [Accepted: 12/27/2018] [Indexed: 06/09/2023]
Abstract
OBJECTIVES The aim of this study was to evaluate the influence of fasting time on the ultrasound identification and exclusion of biliary atresia in jaundiced infants through the use of the gallbladder classification scheme and to test the value of the gallbladder classification scheme in the diagnosis of biliary atresia in inexperienced individuals. METHODS A total of 188 jaundiced infants were enrolled in this study. All patients received detailed abdominal sonograms. Diagnoses were confirmed via liver biopsy, surgical findings, or follow-up. Infants were grouped into either the fasting group (fasting time ≥ 4 h) or the nonfasting group (fasting time < 4 h). According to the gallbladder classification scheme, the gallbladders were classified into 4 types. The accuracy of diagnosing biliary atresia based on the gallbladder classification scheme was compared between the 2 groups. The gallbladders of 177 infants that were detectable by ultrasonography were used to compare the diagnostic performances between a junior radiologist and an experienced radiologist. RESULTS The accuracies in the diagnosis of biliary atresia with the gallbladder classification scheme were 86.3% (82 of 95 patients) for the fasting group and 93.5% (87 of 93 patients) for the nonfasting group (P = .100). The diagnostic accuracies of biliary atresia were 88.7% (157 of 177 patients) for the junior radiologist and 90.4% (160 of 177 patients) for the experienced radiologist (P = .250). The κ value for the agreement between the 2 radiologists was 0.859. CONCLUSIONS The performance of gallbladder diagnoses was not influenced by the fasting time with the use of the gallbladder classification scheme. Additionally, the gallbladder classification scheme may help junior radiologists to more effectively identify biliary atresia and nonbiliary atresia.
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Affiliation(s)
- Wenying Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
| | - Dan Chen
- Department of Ultrasound, Guangdong Women and Children' Hospital, Guangzhou, 511400, China
| | - Hong Jiang
- Department of Pediatric Surgery, the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
| | - Quanyuan Shan
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
| | - Xiaoer Zhang
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
| | - Xiaoyan Xie
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
| | - Luyao Zhou
- Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound, the First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
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13
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Choochuen P, Kritsaneepaiboon S, Charoonratana V, Sangkhathat S. Is "gallbladder length-to-width ratio" useful in diagnosing biliary atresia? J Pediatr Surg 2019; 54:1946-1952. [PMID: 30765154 DOI: 10.1016/j.jpedsurg.2019.01.008] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/07/2018] [Revised: 11/13/2018] [Accepted: 01/09/2019] [Indexed: 10/27/2022]
Abstract
BACKGROUND The accurate assessment of gallbladder shape and wall abnormalities by ultrasound (US) in diagnosing biliary atresia (BA) remains a subjective determination. The objective of this study was to examine the reliability of gallbladder length-to-width ratio (LTWR) by US measurement for diagnosis of BA. METHODS One hundred infants with conjugated hyperbilirubinemia and unknown cause of jaundice who underwent transabdominal US from February 2009 to February 2017 were enrolled. The gallbladder classification and other detailed US findings were reviewed. RESULTS There were statistical differences in gallbladder lumen, classification, length, width and LTWR of gallbladder (all P < 0.05) between BA and non-BA groups. The gallbladder LTWR with a cutoff at 4.1 had the highest sensitivity of 71.7%, while the fibrotic cord thickness had the highest specificity of 95.9%. The combination of portal vein (PV) diameter > 4.4 mm, hepatic artery (HA) diameter > 1.2 mm, and gallbladder LTWR >4.1, provided much higher specificity (98%), odds ratio (11), and positive likelihood ratio (LR+) (10.6). CONCLUSION The gallbladder LTWR by US could be a suggestive US parameter for BA screening. The triad of PV diameter, HA diameter, and gallbladder LTWR yielded the highest specificity, odds ratio, and LR+ for diagnosing BA. LEVEL OF EVIDENCE Level III study of diagnostic test.
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Affiliation(s)
- Panjai Choochuen
- Department of Radiology, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand
| | - Supika Kritsaneepaiboon
- Department of Radiology, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand.
| | - Vorawan Charoonratana
- Department of Radiology, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand
| | - Surasak Sangkhathat
- Pediatric Surgery Unit, Department of Surgery, Faculty of Medicine, Prince of Songkla University, Hat Yai, 90110, Thailand
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Wu JF, Jeng YM, Chen HL, Ni YH, Hsu HY, Chang MH. Quantification of Serum Matrix Metallopeptide 7 Levels May Assist in the Diagnosis and Predict the Outcome for Patients with Biliary Atresia. J Pediatr 2019; 208:30-37.e1. [PMID: 30853207 DOI: 10.1016/j.jpeds.2018.12.006] [Citation(s) in RCA: 57] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2018] [Revised: 11/09/2018] [Accepted: 12/04/2018] [Indexed: 12/12/2022]
Abstract
OBJECTIVE To assess the diagnostic and prognostic usefulness of the serum matrix metallopeptidase-7 (MMP-7) level for biliary atresia in infants with cholestasis after hepatoportoenterostomy. STUDY DESIGN We enrolled 100 infants with cholestasis (age, 43.56 ± 1.97 days; 62 males) with a direct bilirubin level of >1 mg/dL, of whom 36 (36%) were diagnosed with biliary atresisa. The MMP-7 levels in serum samples collected during the cholestasis workup and 6 months after hepatoportoenterostomy were assessed by enzyme-linked immunosorbent assay. We quantified liver fibrosis by Picro Sirius red staining of collagen in specimens from the 81 infants with cholestasis. RESULTS Infants with biliary atresisa had a significantly higher serum MMP-7 level than that of non-biliary atresisa infants with cholestasis of equivalent age (P < .0001). Receiver operating characteristic analysis showed that a serum MMP-7 level of >1.43 ng/mL was predictive of biliary atresisa in infants with cholestasis (diagnostic accuracy, 88%). There was a positive correlation between the serum MMP-7 level and the severity of liver fibrosis (P = .0002). Survival analysis showed that the frequency of liver transplantation was significantly higher in infants with biliary atresisa with a serum MMP-7 level of >10.30 ng/mL compared with a serum MMP-7 level of ≤10.30 ng/mL after hepatoportoenterostomy (hazard ratio, 4.22; P = .02). CONCLUSIONS The serum MMP-7 level, which reflects the severity of liver fibrosis and can be determined noninvasively, may facilitate the diagnosis of biliary atresisa among infants with cholestasis. Moreover, the serum MMP-7 level after hepatoportoenterostomy is associated with a need for liver transplantation in infants with biliary atresisa.
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Affiliation(s)
- Jia-Feng Wu
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
| | - Yung-Ming Jeng
- Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan
| | - Huey-Ling Chen
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; Department of Hepatitis Research Center, National Taiwan University Hospital, Taipei, Taiwan
| | - Yen-Hsuan Ni
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; Department of Hepatitis Research Center, National Taiwan University Hospital, Taipei, Taiwan
| | - Hong-Yuan Hsu
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
| | - Mei-Hwei Chang
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan; Department of Hepatitis Research Center, National Taiwan University Hospital, Taipei, Taiwan.
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15
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Wu JF, Lee CS, Lin WH, Jeng YM, Chen HL, Ni YH, Hsu HY, Chang MH. Transient elastography is useful in diagnosing biliary atresia and predicting prognosis after hepatoportoenterostomy. Hepatology 2018; 68:616-624. [PMID: 29486516 DOI: 10.1002/hep.29856] [Citation(s) in RCA: 67] [Impact Index Per Article: 9.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2017] [Revised: 02/09/2018] [Accepted: 02/22/2018] [Indexed: 12/15/2022]
Abstract
UNLABELLED We investigated the utility of transient elastography (TE) for diagnosing biliary atresia (BA) in cholestatic infants and predicting the outcome of BA. Forty-eight cholestatic infants (9-87 days of age) with direct bilirubin level >1 mg/dL were enrolled. Liver stiffness measurement (LSM) by TE was performed during the cholestasis workup, and 15 subjects were diagnosed as BA. We assessed liver histology using liver biopsies from 36 subjects and graded fibrosis status using the METAVIR score. BA infants had significantly higher LSM values and METAVIR scores than non-BA cholestatic infants. A receiver operating characteristic (ROC) curve analysis showed that an LSM >7.7 kPa was predictive of BA among cholestatic infants (sensitivity = 80%; specificity = 97%; area under the curve [AUC] = 85.3%; P = 0.0001). Cholestatic infants with an LSM >7.7 kPa were more likely to be diagnosed with BA (odds ratio [OR] = 128; P < 0.001). Very early measurement of LSM after hepatoportoenterostomy (HPE) is associated with occurrence of thrombocytopenia, splenomegaly, and esophageal varices 6 months post-HPE. Five of the BA subjects were awaiting or had received liver transplantation (LT), and they had a significantly higher LSM measured 1 week post-HPE than that in the other BA subjects (26.0 vs. 10.8 kPa; P = 0.006). A Cox proportional analysis demonstrated that the need for LT was significantly higher in BA subjects with LSM >16 kPa measured 1 week post-HPE than other BA subjects (hazard ratio [HR] = 10.16; P = 0.04). CONCLUSION LSM assessment during the workup of cholestatic infants may facilitate the diagnosis of BA. LSM post-HPE may predict complications and the need for early LT in infants with BA. (Hepatology 2018).
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Affiliation(s)
- Jia-Feng Wu
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
| | - Chee-Seng Lee
- Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan
| | - Wen-Hsi Lin
- Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan
| | - Yung-Ming Jeng
- Department of Hepatitis Research Center, National Taiwan University Hospital, Taipei, Taiwan
| | - Huey-Ling Chen
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.,Department of Pediatrics, Taipei Tzu Chi Hospital, New Taipei City, Taiwan
| | - Yen-Hsuan Ni
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.,Department of Pediatrics, Taipei Tzu Chi Hospital, New Taipei City, Taiwan
| | - Hong-Yuan Hsu
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
| | - Mei-Hwei Chang
- Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.,Department of Pediatrics, Taipei Tzu Chi Hospital, New Taipei City, Taiwan
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Wang L, Yang Y, Chen Y, Zhan J. Early differential diagnosis methods of biliary atresia: a meta-analysis. Pediatr Surg Int 2018; 34:363-380. [PMID: 29397405 DOI: 10.1007/s00383-018-4229-1] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 01/11/2018] [Indexed: 11/25/2022]
Abstract
PURPOSE To evaluate the accuracy of early differential diagnosis methods of biliary atresia in patients with infantile cholestasis. METHODS We searched PubMed, EMBASE and the Web of Science databases for articles evaluated the early differential diagnosis methods of biliary atresia. The methodological quality of each study was assessed with version 2 of the Quality Assessment of Diagnostic Accuracy Studies tool. Two reviewers extracted data independently. Pooled sensitivity, specificity, positive likelihood ratio (LR +), negative likelihood ratio (LR -), diagnostic odds ratio (DOR) with 95% CIs were calculated to assess each diagnosis method. RESULTS A total of 38 articles were included. Summary sensitivity and specificity were 77% (95% CI 74-80%) and 93% (95% CI 91-94%), respectively, for B-US in 23 studies; 96% (95% CI 92-98%) and 58% (95% CI 51-65%), respectively, for MRCP in five studies; 87% (95% CI 82-91%) and 78% (95% CI 74-82%), respectively, for acholic stool in seven studies; 84% (95% CI 78-89%) and 97% (95% CI 97-98%), respectively, for serum liver function test in seven studies; 96% (95% CI 94-97%) and 73% (95% CI 70-76%), respectively, for hepatobiliary scintigraphy in 18 studies; 98% (95% CI 96-99%) and 93% (95% CI 89-95%), respectively, for percutaneous liver biopsy in 11 studies. CONCLUSION The accuracy rate of percutaneous liver biopsy is better than all of the noninvasive methods. Take into consideration the advantages and disadvantages of the six methods, combination of multidisciplinary noninvasive diagnosis methods is the first choice for differential diagnosis of BA from other causes of neonatal cholestasis.
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Affiliation(s)
- Li Wang
- Department of Pediatric Surgery, Tianjin Children's Hospital, Tianjin, 300134, China
| | - Yuan Yang
- Department of Pediatric Surgery, Tianjin Children's Hospital, Tianjin, 300134, China
| | - Yang Chen
- Department of Pediatric Surgery, Tianjin Children's Hospital, Tianjin, 300134, China
| | - Jianghua Zhan
- Department of Pediatric Surgery, Tianjin Children's Hospital, Tianjin, 300134, China.
- Tianjin Pediatrics Research Institute, Tianjin, 300134, China.
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Ghazy RM, Adawy NM, Khedr MA, Tahoun MM. Biliary atresia recent insight. EGYPTIAN PEDIATRIC ASSOCIATION GAZETTE 2018. [DOI: 10.1016/j.epag.2017.12.001] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
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Zhou LY, Chen SL, Chen HD, Huang Y, Qiu YX, Zhong W, Xie XY. Percutaneous US-guided Cholecystocholangiography with Microbubbles for Assessment of Infants with US Findings Equivocal for Biliary Atresia and Gallbladder Longer than 1.5 cm: A Pilot Study. Radiology 2018; 286:1033-1039. [DOI: 10.1148/radiol.2017170173] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Affiliation(s)
- Lu-yao Zhou
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., S.L.C., Y.H., X.Y.X.), Department of Pediatric Surgery (H.D.C.), and Department of Anesthesiology (Y.X.Q.), the First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Rd 2, Guangzhou 510080, P.R. China; and Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou, P.R. China (W.Z.)
| | - Shu-ling Chen
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., S.L.C., Y.H., X.Y.X.), Department of Pediatric Surgery (H.D.C.), and Department of Anesthesiology (Y.X.Q.), the First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Rd 2, Guangzhou 510080, P.R. China; and Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou, P.R. China (W.Z.)
| | - Hua-dong Chen
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., S.L.C., Y.H., X.Y.X.), Department of Pediatric Surgery (H.D.C.), and Department of Anesthesiology (Y.X.Q.), the First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Rd 2, Guangzhou 510080, P.R. China; and Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou, P.R. China (W.Z.)
| | - Yang Huang
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., S.L.C., Y.H., X.Y.X.), Department of Pediatric Surgery (H.D.C.), and Department of Anesthesiology (Y.X.Q.), the First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Rd 2, Guangzhou 510080, P.R. China; and Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou, P.R. China (W.Z.)
| | - Yu-xin Qiu
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., S.L.C., Y.H., X.Y.X.), Department of Pediatric Surgery (H.D.C.), and Department of Anesthesiology (Y.X.Q.), the First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Rd 2, Guangzhou 510080, P.R. China; and Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou, P.R. China (W.Z.)
| | - Wei Zhong
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., S.L.C., Y.H., X.Y.X.), Department of Pediatric Surgery (H.D.C.), and Department of Anesthesiology (Y.X.Q.), the First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Rd 2, Guangzhou 510080, P.R. China; and Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou, P.R. China (W.Z.)
| | - Xiao-yan Xie
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., S.L.C., Y.H., X.Y.X.), Department of Pediatric Surgery (H.D.C.), and Department of Anesthesiology (Y.X.Q.), the First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Rd 2, Guangzhou 510080, P.R. China; and Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou, P.R. China (W.Z.)
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Zhou Y, Jiang M, Tang ST, Yang L, Zhang X, Yang DH, Xiong M, Li S, Cao GQ, Wang Y. Laparoscopic finding of a hepatic subcapsular spider-like telangiectasis sign in biliary atresia. World J Gastroenterol 2017; 23:7119-7128. [PMID: 29093620 PMCID: PMC5656459 DOI: 10.3748/wjg.v23.i39.7119] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2017] [Revised: 08/23/2017] [Accepted: 09/13/2017] [Indexed: 02/06/2023] Open
Abstract
AIM To assess the diagnostic value of a laparoscopic finding of a hepatic subcapsular spider-like telangiectasis (HSST) sign in biliary atresia.
METHODS A retrospective study was conducted first and then a validation set was used to investigate the value of an HSST sign in predicting biliary atresia (BA). In the retrospective study, laparoscopic images of the liver surface were reviewed in 126 patients with infantile cholestasis (72 BA patients and 54 non-BA cholestasis patients) and a control group of 38 patients with non-hepatic conditions. Analysis was first made by two observers separately and finally, a consensus conclusion was achieved. Then, the diagnostic value of the HSST sign was validated in an independent cohort including 45 BA and 45 non-BA patients.
RESULTS In the retrospective investigation, an amplified HSST sign was found in all BA patients, while we were unable to detect the HSST sign in 98.1% of the 54 non-BA patients. There was no HSST sign in any of the control subjects. In the first review, the sensitivity and specificity from one reviewer were 100% and 98.1%, respectively, and the results from the other reviewer were both 100%. The consensus sensitivity and specificity were 100% and 98.1%, respectively. The HSST sign was defined as being composed of several enlarged tortuous spider-like vascular plexuses with two to eight branches distributed on all over the liver surface, which presented as either a concentrated type or a dispersed type. In the independent validation group, the sensitivity, specificity, positive predictive value and negative predictive value of the HSST sign were 100%, 97.8%, 97.8% and 100%, respectively.
CONCLUSION The HSST sign is characteristic in BA, and laparoscopic exploration for the HSST sign is valuable in the diagnosis of BA.
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Affiliation(s)
- Ying Zhou
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Meng Jiang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Shao-Tao Tang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Li Yang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Xi Zhang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - De-Hua Yang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Meng Xiong
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Shuai Li
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Guo-Qing Cao
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Yong Wang
- Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
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20
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Yoon HM, Suh CH, Kim JR, Lee JS, Jung AY, Cho YA. Diagnostic Performance of Sonographic Features in Patients With Biliary Atresia: A Systematic Review and Meta-analysis. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2017; 36:2027-2038. [PMID: 28556285 DOI: 10.1002/jum.14234] [Citation(s) in RCA: 26] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/14/2016] [Accepted: 12/30/2016] [Indexed: 06/07/2023]
Abstract
OBJECTIVES To evaluate the diagnostic performance of sonographic features of biliary atresia in patients with infantile cholestasis. METHODS The Ovid-MEDLINE and EMBASE databases were searched for studies of the diagnostic performance of sonographic features of biliary atresia in patients with infantile cholestasis. A meta-analysis was performed to evaluate the diagnostic performance of the triangular cord sign and other sonographic features in patients with biliary atresia. RESULTS Seventeen eligible studies with 1444 patients were included. The triangular cord sign had high accuracy for diagnosing biliary atresia: the meta-analytic summary sensitivity and specificity were 85% (95% confidence interval, 77%-90%) and 97% (95% confidence interval, 94%-99%), respectively. The area under the hierarchical summary receiver operating characteristic curve was 0.97. Meta-regression analysis revealed that the cutoff thickness of the triangular cord sign (3 or 4 mm) was a significant factor affecting study heterogeneity (P < .01). The proportions of nonidentification of the gallbladder (GB) ranged from 0% to 53%. Abnormal GB morphologic characteristics, nonvisualization of the common bile duct, and the presence of hepatic subcapsular flow showed relatively high sensitivities and specificities. CONCLUSIONS The triangular cord sign and other sonographic features, including abnormal GB morphologic characteristics, nonvisualization of the common bile duct, and the presence of hepatic subcapsular flow, had high diagnostic performance for the diagnosis of biliary atresia.
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Affiliation(s)
- Hee Mang Yoon
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
| | - Chong Hyun Suh
- Department of Radiology, Namwon Medical Center, Namwon, Korea
| | - Jeong Rye Kim
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
| | - Jin Seong Lee
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
| | - Ah Young Jung
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
| | - Young Ah Cho
- Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
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Pediatric cholangiopathies: diseases of the gallbladder and biliary tract. Abdom Radiol (NY) 2017; 42:69-85. [PMID: 27518785 DOI: 10.1007/s00261-016-0865-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
Abstract
Pediatric gallbladder and bile duct disease encompasses a broad spectrum of processes, from congenital to developmental to neoplastic. We describe normal pediatric biliary anatomy and summarize the most common pathologic entities, with a focus on non-invasive imaging techniques and findings. Ultrasound is the first-line imaging modality in children with suspected biliary pathology based on its widespread availability, cost effectiveness, and lack of ionizing radiation. MRI and MRCP are often used for further evaluation in cases of diagnostic uncertainty and for surgical planning.
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Govindarajan KK. Biliary atresia: Where do we stand now? World J Hepatol 2016; 8:1593-1601. [PMID: 28083081 PMCID: PMC5192550 DOI: 10.4254/wjh.v8.i36.1593] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/03/2016] [Revised: 09/09/2016] [Accepted: 11/01/2016] [Indexed: 02/06/2023] Open
Abstract
The pathway from clinical suspicion to establishing the diagnosis of biliary atresia in a child with jaundice is a daunting task. However, investigations available help to point towards the correct diagnosis in reasonable time frame. Imaging by Sonography has identified several parameters which can be of utility in the diagnostic work up. Comparison of Sonography with imaging by Nuclear medicine can bring out the significant differences and also help in appropriate imaging. The battery of Biochemical tests, available currently, enable better understanding of the line-up of investigations in a given child with neonatal cholestasis. Management protocols enable standardized care with optimal outcome. The place of surgical management in biliary atresia is undisputed, although Kasai procedure and primary liver transplantation have been pitted against each other. This article functions as a platform to bring forth the various dimensions of biliary atresia.
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Affiliation(s)
- Krishna Kumar Govindarajan
- Krishna Kumar Govindarajan, Department of Pediatric Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry 605006, India
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Koob M, Pariente D, Habes D, Ducot B, Adamsbaum C, Franchi-Abella S. The porta hepatis microcyst: an additional sonographic sign for the diagnosis of biliary atresia. Eur Radiol 2016; 27:1812-1821. [PMID: 27553925 DOI: 10.1007/s00330-016-4546-5] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2015] [Revised: 07/06/2016] [Accepted: 08/08/2016] [Indexed: 12/27/2022]
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Abstract
Cholestasis in preterm infants has a multifactorial etiology. Risk factors include degree of prematurity, lack of enteral feeding, intestinal injury, prolonged use of parenteral nutrition (PN), and sepsis. Soy-based parenteral lipid emulsions have been implicated in the pathophysiology of PN-associated liver injury. Inflammation plays an important role. Medical therapies are used; however, their effects have not consistently proven effective. Evaluation of cholestasis involves laboratory work; direct bilirubin levels are used for diagnosis and trending. Adverse outcomes include risk for hepatobiliary dysfunction, irreversible liver failure, and death. Early enteral feedings as tolerated is the best way to prevent and manage cholestasis.
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Affiliation(s)
- Katie Satrom
- Division of Neonatology, Department of Pediatrics, University of Minnesota, 2450 Riverside Avenue, 6th Floor, East Building, Delivery Code: 8952A, Minneapolis, MN 55454, USA.
| | - Glenn Gourley
- Pediatric Gastroenterology, Department of Pediatrics, University of Minnesota, 2450 Riverside Avenue, 6th Floor, East Building, 8952A, Minneapolis, MN 55454, USA
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25
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26
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Cho HH, Kim WS, Choi YH, Cheon JE, Lee SM, Kim IO, Shin SM, Ko JS, Moon JS. Ultrasonography evaluation of infants with Alagille syndrome: In comparison with biliary atresia and neonatal hepatitis. Eur J Radiol 2016; 85:1045-52. [PMID: 27161050 DOI: 10.1016/j.ejrad.2016.03.023] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2015] [Revised: 03/01/2016] [Accepted: 03/21/2016] [Indexed: 10/22/2022]
Abstract
OBJECTIVE To evaluate the ultrasonography (US) features of Alagille syndrome (ALGS), as compared with biliary atresia (BA) or neonatal hepatitis (NH). METHODS Our study included 23 ALGS, 75 BA and 70 NH patients. The initial US images were retrospectively reviewed for gallbladder (GB) morphology with systemic classification, GB length and luminal area, presence of triangular-cord (TC) sign and hypertrophied hepatic-artery. The presence of anomalies associated with ALGS was evaluated. The diagnostic values of each finding and their combinations were evaluated. RESULTS Both ALGS (57%) and BA (79%) were more frequently associated with abnormal GB shapes than NH (19%, all P<0.001). The short and small GBs were more frequently observed in ALGS and BA than in NH (all P<0.001). None in the ALGS and NH showed TC sign, while 41% in the BA did (all P<0.001). Hypertrophied hepatic-artery was noted less frequently in both ALGS (13%) and NH (14%) than in BA (83%, all P<0.001). The combination of US criteria with associated anomalies increased the positive-predictive-value for ALGS. CONCLUSION Abnormal shaped GB with absence of the TC sign and hypertrophied hepatic-artery and presence of associated anomalies can be a differential point of ALGS.
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Affiliation(s)
- Hyun-Hae Cho
- Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea
| | - Woo Sun Kim
- Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Institute of Radiation Medicine, Seoul National University Medical Research Center, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea.
| | - Young Hun Choi
- Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea
| | - Jung-Eun Cheon
- Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Institute of Radiation Medicine, Seoul National University Medical Research Center, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea
| | - So Mi Lee
- Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea
| | - In-One Kim
- Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Institute of Radiation Medicine, Seoul National University Medical Research Center, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea
| | - Su-Mi Shin
- Department of Radiology, SMG-SNU Boramae Medical Center, Seoul 07061, Republic of Korea
| | - Jae Sung Ko
- Department of Pediatrics, Seoul National University Hospital, Seoul National University College of Medicine, Republic of Korea
| | - Jin Soo Moon
- Department of Pediatrics, Seoul National University Hospital, Seoul National University College of Medicine, Republic of Korea
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He JP, Hao Y, Wang XL, Yang XJ, Shao JF, Feng JX. Comparison of different noninvasive diagnostic methods for biliary atresia: a meta-analysis. World J Pediatr 2016; 12:35-43. [PMID: 26684313 DOI: 10.1007/s12519-015-0071-x] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/04/2014] [Accepted: 09/02/2014] [Indexed: 01/20/2023]
Abstract
BACKGROUND This study was undertaken to retrospectively analyze the accuracy of different methods in differentiating biliary atresia from neonatal jaundice. METHODS A search was made in MEDLINE, and the Web of Science for relevant original articles published in English; methodological quality of the included studies was also assessed. Two reviewers extracted data independently. Studies were pooled, summary receiver operating characteristics curve and diagnostic odds ratio (DOR) with corresponding confidence intervals were calculated. RESULTS For diagnosis of biliary atresia, ultrasonography (US), hepatic scintigraphy (HBS), and magnetic resonance cholangiography (MRCP) had a pooled sensitivity of 74.9% (range: 70.4%-79.1%), 93.4% (range: 90.3%-95.7%) and 89.7% (range: 84.8%-93.4%), a specificity of 93.4% (range: 91.4%-95.1%), 69.2% (range: 65.1%-73.1%) and 64.7% (range: 58.0%-71.0%), a positive likelihood ratio of 12.16 (range: 6.41-23.08), 3.01 (range: 2.15-4.20) and 3.10 (range: 1.59-6.06), a negative likelihood ratio of 0.23 (range: 0.13-0.38), 0.13 (range: 0.06-0.25) and 0.16 (range: 0.06-0.44), DOR of 72.56 (range: 27.34-192.58), 29.88 (range: 12.82-69.64) and 32.48 (range: 8.22-128.29), with an area under the curve of 0.96, 0.91, and 0.92, and Q value of 0.90, 0.85, and 0.85, respectively. CONCLUSIONS US, HBS and MRCP can be very useful for the diagnostic work-up of neonatal cholestasis. To improve the sensitivity and specificity, several additional measures can be used.
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Affiliation(s)
- Jin-Peng He
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Yun Hao
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Xiao-Lin Wang
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Xiao-Jin Yang
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Jing-Fan Shao
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Jie-Xiong Feng
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
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Lee SM, Cheon JE, Choi YH, Kim WS, Cho HH, Cho HH, Kim IO, You SK. Ultrasonographic Diagnosis of Biliary Atresia Based on a Decision-Making Tree Model. Korean J Radiol 2015; 16:1364-72. [PMID: 26576128 PMCID: PMC4644760 DOI: 10.3348/kjr.2015.16.6.1364] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2015] [Accepted: 09/19/2015] [Indexed: 01/11/2023] Open
Abstract
Objective To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). Materials and Methods From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Results Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Conclusion Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology.
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Affiliation(s)
- So Mi Lee
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - Jung-Eun Cheon
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - Young Hun Choi
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - Woo Sun Kim
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | | | - Hyun-Hye Cho
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - In-One Kim
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
| | - Sun Kyoung You
- Department of Radiology, Seoul National University College of Medicine, Seoul 03080, Korea
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Zhou LY, Wang W, Shan QY, Liu BX, Zheng YL, Xu ZF, Xu M, Pan FS, Lu MD, Xie XY. Optimizing the US Diagnosis of Biliary Atresia with a Modified Triangular Cord Thickness and Gallbladder Classification. Radiology 2015; 277:181-191. [PMID: 25955579 DOI: 10.1148/radiol.2015142309] [Citation(s) in RCA: 47] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/24/2025]
Abstract
Purpose To evaluate the diagnostic performance of ultrasonography (US) in the identification and exclusion of biliary atresia with a modified triangular cord thickness metric together with a gallbladder classification scheme, as well as hepatic artery (HA) diameter and liver and spleen size, in a large sample of jaundiced infants. Materials and Methods The ethics committee approved this study, and written informed parental consent was obtained. In 273 infants with conjugated hyperbilirubinemia (total bilirubin level ≥ 31.2 μmol/L, with direct bilirubin level > indirect bilirubin level), detailed abdominal US was performed to exclude biliary atresia. Biliary atresia was found in 129 infants and ruled out in 144. A modified triangular cord thickness was measured at the anterior branch of the right portal vein, and a gallbladder classification scheme was identified that incorporated the appearance of the gallbladder and a gallbladder length-to-width ratio of up to 5.2 when the lumen was visualized, as well as HA diameter and liver and spleen size. Reference standard diagnosis was based on results of one or more of the following: surgery, liver biopsy, cholangiography, and clinical follow-up. Area under the receiver operating characteristic curve (AUC) analysis, binary logistic regression analysis, Fisher exact test, and unpaired t test were performed. Results Triangular cord thickness, HA diameter, ratio of gallbladder length to gallbladder width, liver size, and spleen size exhibited statistically significant differences (all P < .05) between the group with biliary atresia and the group without. AUCs of triangular cord thickness, ratio of gallbladder length to width, and HA diameter were 0.952, 0.844, and 0.838, respectively. Logistic regression analysis demonstrated that these three US parameters were significantly associated (all P < .05) with biliary atresia. The combination of triangular cord thickness and gallbladder classification could yield comparable AUCs (0.915 vs 0.933, P = .400) and a higher sensitivity (96.9% vs 92.2%), compared with triangular cord thickness alone. Conclusion By using the combination of modified triangular cord thickness and gallbladder classification scheme, most infants with biliary atresia could be identified. (©) RSNA, 2015.
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Affiliation(s)
- Lu-Yao Zhou
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
| | - Wei Wang
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
| | - Quan-yuan Shan
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
| | - Bao-xian Liu
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
| | - Yan-ling Zheng
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
| | - Zuo-feng Xu
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
| | - Ming Xu
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
| | - Fu-shun Pan
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
| | - Ming-de Lu
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
| | - Xiao-yan Xie
- From the Department of Medical Ultrasonics, Institute for Diagnostic and Interventional Ultrasound (L.Y.Z., W.W., Q.Y.S., B.X.L., Y.L.Z., Z.F.X., M.X., F.S.P., X.Y.X.), and Department of Hepatobiliary Surgery (M.D.L.), First Affiliated Hospital, Sun Yat-Sen University, 58 Zhongshan Road 2, Guangzhou 510080, People's Republic of China
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Goel P, Bhatnagar V, Das N, Kalaivani M. Evaluation of blood levels of nitric oxide as a means of differentiation between neonatal hepatitis and extrahepatic biliary atresia: A pilot study. J Indian Assoc Pediatr Surg 2015; 20:139-42. [PMID: 26166985 PMCID: PMC4481626 DOI: 10.4103/0971-9261.159029] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
Aim: The differentiation between neonatal hepatitis (NH) and extrahepatic biliary atresia (EHBA) is not always possible despite all the currently available diagnostic modalities. In this study, an attempt has been made to evaluate the role of nitric oxide (NO) levels in the peripheral blood to differentiate between the two conditions, one requiring early surgical intervention (EHBA) and the other amenable to conservative medical management (NH). Patients and Methods: Twenty patients who presented to the pediatric surgical service, over a 2 years period, with features of neonatal cholestasis were enrolled in the study. The diagnostic workup included documentation of history and clinical examination, biochemical liver function tests, ultrasonography, hepatobiliary scintigraphy (HS), and magnetic resonance cholangio-pancreaticography (MRCP). These patients did not show excretion on HS and intrahepatic ducts on MRCP. Hence, they were subjected to mini-laparotomy and operative cholangiography (OC). The EHBA patients were treated with the Kasai's portoenterostomy procedure, and the extrahepatic ducts were flushed with normal saline in NH patients. All patients were evaluated preoperatively for levels of NO in the peripheral blood by the Greiss reaction spectrophotometrically at 540 nm. Normal values were determined from a cohort of controls. The median (range) levels of NO in patients with EHBA and NH were compared, and the statistical significance of the difference was calculated by applying the Wilcox Rank Sum test. A P = 0.05 was considered as significant. Results: Of the 20 patients enrolled in the study, 17 patients were treated for EHBA (Group I) and the remaining 3 patients had patent ducts on OC and were thus diagnosed as NH (Group II). The mean age of the patients in Groups I and II was comparable: 2.79 ± 0.75 and 2.67 ± 0.58 months, respectively (P = 0.866). The median NO levels were significantly elevated in each of the two groups as compared to the controls (5.6 μmol/l, range 1.26-11.34 μmol/l); when compared among themselves, the NO levels were significantly higher in Group I, 64.05 μmol/l (range 24.11-89.43 μmol/l), when compared with Group II, 41.72 μmol/l (range 23.53-45.63 μmol/l) (P = 0.022). Conclusion: The serum levels of NO were found to be significantly higher in patients with EHBA as compared to those with NH. Hence, this may be a useful biochemical marker for the preoperative differentiation of EHBA from NH. However, a larger study is required for establishing the validity of the statistical significance.
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Affiliation(s)
- Prabudh Goel
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
| | - Veereshwar Bhatnagar
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
| | - Nibhriti Das
- Department of Biochemistry, All India Institute of Medical Sciences, New Delhi, India
| | - Mani Kalaivani
- Department of Biostatistics, All India Institute of Medical Sciences, New Delhi, India
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Queiroz TCN, Ferreira AR, Fagundes EDT, Roquete MLV, Penna FJ. Biliary atresia: evaluation on two distinct periods at a reference pediatric service. ARQUIVOS DE GASTROENTEROLOGIA 2014; 51:53-8. [PMID: 24760065 DOI: 10.1590/s0004-28032014000100011] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/11/2013] [Accepted: 11/18/2013] [Indexed: 11/21/2022]
Abstract
CONTEXT Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period. OBJECTIVES To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. METHODS Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. RESULTS Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001). On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15), but difference was found when comparing the age at surgery (P = 0.002). Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. CONCLUSIONS Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.
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Affiliation(s)
- Thais Costa Nascentes Queiroz
- Departamento de Gastroenterologia Pediátrica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil
| | - Alexandre Rodrigues Ferreira
- Departamento de Gastroenterologia Pediátrica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil
| | - Eleonora Druve Tavares Fagundes
- Departamento de Gastroenterologia Pediátrica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil
| | - Mariza Leitão Valadares Roquete
- Departamento de Gastroenterologia Pediátrica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil
| | - Francisco José Penna
- Departamento de Gastroenterologia Pediátrica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brasil
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Kianifar HR, Tehranian S, Shojaei P, Adinehpoor Z, Sadeghi R, Kakhki VRD, Keshtgar AS. Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature. Pediatr Radiol 2013; 43:905-19. [PMID: 23519699 DOI: 10.1007/s00247-013-2623-3] [Citation(s) in RCA: 64] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/08/2012] [Revised: 12/21/2012] [Accepted: 12/27/2012] [Indexed: 12/30/2022]
Abstract
Hepatobiliary scintigraphy is an important diagnostic modality for work-up of neonatal cholestasis. Therefore, our objective was to evaluate the literature regarding the accuracy of hepatobiliary scintigraphy in differentiating biliary atresia from non-biliary atresia causes of cholestasis (collectively called neonatal hepatitis). Our search included Medline, SCOPUS and Google Scholar. Only studies using Tc-99 m-labeled immunodiacetic acid (IDA) derivatives were included. Overall, 81 studies were included in the meta-analysis. Pooled sensitivity and specificity were 98.7% (range 98.1-99.2%) and 70.4% (range 68.5-72.2%), respectively. Factors that increased specificity included the use of radiotracers with high hepatic extraction, administration of hepatic-inducing drugs (such as phenobarbital), use of a calculated dose/kg and administration of a booster dose in cases of non-excretion of the tracer in the bowel. SPECT imaging and duodenal fluid sampling also had high specificity; however, they need further validation because of the low number of studies. Semiquantitative imaging methods do not seem to have any incremental value. We conclude that hepatobiliary scintigraphy using IDA derivatives can be very useful for diagnostic work-up of neonatal cholestasis. To improve the specificity, several measures can be followed regarding type and dose of the radiotracer and imaging protocols. Non-imaging methods seem to be promising and warrant further validation.
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Affiliation(s)
- Hamid Reza Kianifar
- Paediatric Gastroenterology Ward, Mashhad University of Medical Sciences, Ghaem Hospital, Mashhad, Iran
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Jiang LP, Chen YC, Ding L, Liu XL, Li KY, Huang DZ, Zhou AY, Zhang QP. The diagnostic value of high-frequency ultrasonography in biliary atresia. Hepatobiliary Pancreat Dis Int 2013; 12:415-22. [PMID: 23924500 DOI: 10.1016/s1499-3872(13)60065-x] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
BACKGROUND It is a globally challenging problem to differentially diagnose biliary atresia (BA) from other disease processes causing infantile cholestatic jaundice. The high-frequency ultrasonography (HUS) yields much improved spatial resolution and therefore, might show better image in BA diagnostic examination. The present study was to evaluate the HUS on the diagnosis of BA in infants with jaundice. METHODS Fifty-one infants with neonatal jaundice were scanned with ultrasonography. Images included gallbladder, bile duct, right hepatic artery (RHA), portal vein (PV) and triangular cord (TC) sign, magnetic resonance imaging and additionally, laboratory tests and histopathology reports were assessed. RESULTS Twenty-three BA and 28 non-BA cases were confirmed. The sensitivity, specificity, and accuracy of HUS were 91.3%, 92.9%, and 92.2%, respectively. All of these indices were significantly higher than those of conventional ultrasonography (P<0.01) and MR cholangiopancreatography (P<0.05). The HUS features, included a positive TC sign, an increased RHA diameter and RHA-diameter to portal-vein-diameter ratio (RHA/PV) and abnormal gallbladder, were important in the diagnosis of BA. CONCLUSION HUS provided better imaging of BA and should be considered as a primary modality in the differential diagnosis of infantile jaundice.
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Affiliation(s)
- Li-Ping Jiang
- Department of Ultrasound, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China
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Lee SY, Kim GC, Choe BH, Ryeom HK, Jang YJ, Kim HJ, Park JY, Cho SM. Efficacy of US-guided percutaneous cholecystocholangiography for the early exclusion and type determination of biliary atresia. Radiology 2011; 261:916-922. [PMID: 21998045 DOI: 10.1148/radiol.11110665] [Citation(s) in RCA: 29] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/09/2025]
Abstract
PURPOSE To evaluate the efficacy of ultrasonographically (US)-guided percutaneous cholecystocholangiography (PCC) for early diagnosis and characterization of biliary atresia in infants with cholestatic liver disease. MATERIALS AND METHODS Institutional review board approval was obtained for this study. Parental informed written consent was obtained. From October 2003 to August 2010, 22 infants (12 male, 10 female; age range, 1-138 days) were referred to the radiology department for PCC. Indications for PCC were suspected biliary atresia at 24-hour delayed technetium 99m-diisopropyl-phenylcarbamoylmethyl-iminodiacetic acid (DISIDA) scintigraphy because no excretion was observed in the small bowel (n = 17) or when the results of the scan or liver biopsy could not be obtained within 3 days because of a delay in schedule (n = 5). A diagnosis of biliary atresia was excluded when there was contrast material visualized in the gallbladder, biliary system, and passage to the duodenum. Patients with biliary atresia underwent surgery as the reference standard. RESULTS Among the 18 patients who underwent successful PCC, biliary atresia was excluded in 13, with diagnoses as follows: total parenteral nutrition-associated cholestasis (TPNAC) (n = 6), neonatal hepatitis (n = 4), congenital syphilis (n = 1), neonatal lupus (n = 1), and congenital cytomegalovirus hepatitis (n = 1). Biliary atresia was diagnosed in five patients (four with type IIIb and one with type IIIa) and was confirmed at surgery. In four infants in whom US-guided gallbladder puncture had failed, biliary atresia (n = 2) and TPNAC (n = 2) were diagnosed. CONCLUSION PCC is a safe and useful technique for early exclusion when biliary atresia cannot be ruled out after traditional screening tests; in addition, it may be useful for preoperative type determination of biliary atresia. © RSNA, 2011.
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Affiliation(s)
- Sang Yub Lee
- Department of Radiology, Kyungpook National University School of Medicine, 101 Dongin dong 2 Ga, Jung gu, Daegu 700-422, Republic of Korea
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Comparison of different diagnostic methods for differentiating biliary atresia from idiopathic neonatal hepatitis. Clin Imaging 2010; 33:439-46. [PMID: 19857804 DOI: 10.1016/j.clinimag.2009.01.003] [Citation(s) in RCA: 64] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2008] [Accepted: 01/08/2009] [Indexed: 01/11/2023]
Abstract
AIM To retrospectively analyze different methods in differentiating biliary atresia from idiopathic neonatal hepatitis. METHODS Sixty-nine infants with cholestatic jaundice and final diagnosis of idiopathic neonatal hepatitis (INH) and biliary atresia (BA) were studied retroprospectively from January 2004 to December 2006. A thorough history and physical examination were undertaken. All cases underwent abdominal magnetic resonance cholangiography (MRCP), ultrasonography (US), hepatobiliary scintigraphy (HBS), HBS single-photon emission computer tomography (HBS SPECT), and operation or percutaneous liver biopsy. The accuracy, sensitivity, specificity, and predictive values of these various methods were compared. RESULTS There were 39 girls and 30 boys, among whom 35 had INH (age, 61+/-17 days) and 34 had BA (age, 64+/-18 days). The mean age at onset of jaundice was significantly lower in cases of BA when compared to INH cases (9+/-13 vs. 20+/-21 days; P=.032). The diagnostic accuracy of different methods was as follows: liver biopsy, 97.1%; HBS SPECT, 91.30%; MRCP, 71.01%; HBS, 66.67%; US, 65.22%. CONCLUSION Our results indicate that biopsy of the liver is considered as the most reliable method to differentiate INH from BA. The accuracy of HBS SPECT is higher than that of MRCP, HBS, and US. There was no significant difference in diagnostic accuracy among MRCP, HBS, and US.
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Abstract
Extrahepatic biliary atresia (EHBA), an inflammatory sclerosing cholangiopathy, is the leading indication for liver transplantation in children. The cause is still unknown, although possible infectious, genetic, and immunologic etiologies have received much recent focus. These theories are often dependent on each other for secondary or coexisting mechanisms. Concern for EHBA is raised by a cholestatic infant, but the differential diagnosis is large and the path to diagnosis remains varied. Current treatment is surgical with an overall survival rate of approximately 90%. The goals of this article are to review the important clinical aspects of EHBA and to highlight some of the more recent scientific and clinical developments contributing to our understanding of this condition.
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Affiliation(s)
- Mikelle D. Bassett
- Department of Pediatrics, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, WA
| | - Karen F. Murray
- Division of Gastroenterology and Nutrition, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, WA
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Li SX, Zhang Y, Sun M, Shi B, Xu ZY, Huang Y, Mao ZQ. Ultrasonic diagnosis of biliary atresia: A retrospective analysis of 20 patients. World J Gastroenterol 2008; 14:3579-82. [PMID: 18567090 PMCID: PMC2716624 DOI: 10.3748/wjg.14.3579] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To investigate the clinical value of ultrasonographic diagnosis of biliary atresia (BA), a retrospective analysis of the sonogram of 20 children with BA was undertaken.
METHODS: Ultrasonography (US) was performed in 20 neonates and infants with BA, which was confirmed with cholangiography by operation or abdominoscopy. The presence of triangular cord, the size and echo of liver, the changes in empty stomach gallbladder and postprandial gallbladder were observed and recorded.
RESULTS: The triangular cord could be observed at the porta hepatis (thickness: 0.3-0.6 cm) in 10 cases. Smaller triangular cord (0.2-0.26 cm) can be observed in 3 cases. The gallbladder was not observed in 2 cases, and 1 case showed a streak gallbladder without capsular space. The gallbladders of 15 cases were flat and small. The gallbladders of 2 cases were of normal size and appearance, however, there was no postprandial contraction. The livers of all cases showed hepatomegaly and heterogeneous echogenicity. Statistical analysis was performed to compare the hepatomegaly and heterogeneous echogenicity and the stage of hepatic fibrosis.
CONCLUSION: The presence of the triangular cord at the porta hepatis is specific. However, it is not the only diagnostic criterion, since flat and small gallbladder and poor contraction are also of important diagnostic and differential diagnostic significance. The degree of hepatomegaly and heterogeneous echogenicity is proportional with liver fibrosis, and able to indicate the duration of course and prognosis.
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Kim WS, Cheon JE, Youn BJ, Yoo SY, Kim WY, Kim IO, Yeon KM, Seo JK, Park KW. Hepatic arterial diameter measured with US: adjunct for US diagnosis of biliary atresia. Radiology 2007; 245:549-55. [PMID: 17890351 DOI: 10.1148/radiol.2452061093] [Citation(s) in RCA: 75] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
PURPOSE To prospectively evaluate the accuracy of hepatic artery diameter and hepatic artery diameter-to-portal vein diameter ratio for ultrasonographic (US) diagnosis of biliary atresia, with cholangiographic or clinical information as reference standard. MATERIALS AND METHODS Institutional review board approval and informed consent were obtained. US was performed in 68 neonates and infants with cholestatic jaundice (mean age, 61 days; male-to-female ratio, 38:30). Biliary atresia (n = 38) was confirmed with cholangiography, and hepatitis (n = 30) was diagnosed with clinical (n = 24) or cholangiographic (n = 6) findings. Diameter of the right hepatic artery was measured with US. Right hepatic artery diameter-to-right portal vein diameter ratio was measured to determine relative enlargement of the hepatic artery. As a control group, 17 neonates and infants (mean age, 67 days; male-to-female ratio, 12:5) without jaundice underwent US of the porta hepatis. Statistical analysis was performed to compare US parameters among three groups with one-way analysis of variance. Optimal cutoff values of the hepatic artery diameter and hepatic artery diameter-to-portal vein diameter ratio for biliary atresia diagnosis were obtained with receiver operating characteristic analysis. RESULTS The diameter of the right hepatic artery in biliary atresia group (1.9 mm +/- 0.4 [standard deviation]) was significantly larger than that in the hepatitis (1.4 mm +/- 0.3) and control (1.2 mm +/- 0.2) groups (P < .001). Hepatic artery diameter-to-portal vein diameter ratio in the biliary atresia group (0.52 +/- 0.12) was larger than that in hepatitis (0.40 +/- 0.07) and in control (0.40 +/- 0.10) groups (P < .001). Optimum cutoff values for diagnosis of biliary atresia were 1.5 mm (sensitivity, 92%; specificity, 87%; accuracy, 89%) for hepatic artery diameter and 0.45 for hepatic artery diameter-to-portal vein diameter ratio (sensitivity, 76%; specificity, 79%; accuracy, 78%). CONCLUSION Measurement of hepatic artery diameter can be helpful in the US diagnosis of biliary atresia.
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Affiliation(s)
- Woo Sun Kim
- Department of Radiology, Institute of Radiation Medicine, Seoul National University College of Medicine, Seoul National University Hospital, Chongno-Gu, Seoul, Korea
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Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop. Hepatology 2007; 46:566-81. [PMID: 17661405 PMCID: PMC3888317 DOI: 10.1002/hep.21790] [Citation(s) in RCA: 181] [Impact Index Per Article: 10.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Biliary atresia is the most common cause of end-stage liver disease in the infant and is the leading pediatric indication for liver transplantation in the United States. Earlier diagnosis (<30-45 days of life) is associated with improved outcomes following the Kasai portoenterostomy and longer survival with the native liver. However, establishing this diagnosis is problematic because of its rarity, the much more common indirect hyperbilirubinemia that occurs in the newborn period, and the schedule for routine infant health care visits in the United States. The pathogenesis of biliary atresia appears to involve immune-mediated fibro-obliteration of the extrahepatic and intrahepatic biliary tree in most patients and defective morphogenesis of the biliary system in the remainder. The determinants of the outcome of portoenterostomy include the age at surgery, the center's experience, the presence of associated congenital anomalies, and the postoperative occurrence of cholangitis. A number of screening strategies in infants have been studied. The most promising are early measurements of serum conjugated bilirubin and a stool color card given to new parents that alerts them and their primary care provider to alcholic stools. This report summarizes a National Institutes of Health workshop held on September 12 and 13, 2006, in Bethesda, MD, that addressed the issues of outcomes, screening, and pathogenesis of biliary atresia.
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Affiliation(s)
- Ronald J Sokol
- Department of Pediatrics, University of Colorado School of Medicine, The Children's Hospital, Denver, CO, USA.
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Takaya J, Nakano S, Imai Y, Fujii Y, Kaneko K. Usefulness of magnetic resonance cholangiopancreatography in biliary structures in infants: a four-case report. Eur J Pediatr 2007; 166:211-4. [PMID: 16960695 DOI: 10.1007/s00431-006-0230-0] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/12/2006] [Accepted: 06/21/2006] [Indexed: 12/17/2022]
Abstract
In this paper, we report the usefulness of magnetic resonance cholangiopancreatography (MRCP) in excluding biliary atresia (BA) as the cause of neonatal cholestasis. MRCP with a 1.5-T magnetic resonance (MR) imaging unit was performed on four jaundiced neonates and infants aged from 38 days to 106 days. The diagnosis of BA (n=2) was confirmed with surgery, liver biopsy and surgical cholangiography. Diagnosis of neonatal hepatitis (NH, n=2) was confirmed with clinical follow-up until jaundice resolved, while one of them was diagnosed with surgical cholangiography. In all discoloured acholic stools, increased direct bilirubin (4.4-11.3 mg/dl) with positive lipoprotein X prompted technetium 99mTc disofenin scanning, which showed no excretion. Computed tomography (CT) showed a gallbladder in one with hepatitis but no intrahepatic bile duct in two with BA. The Kasai operation was performed in two patients with BA. In two patients with BA, neither the common bile duct nor the common hepatic ducts were visible at MRCP. In two patients with NH, MRCP clearly depicted both the common hepatic and the common bile ducts. MRCP was accurate in excluding BA as the cause of neonatal cholestasis, while 99mTc disofenin cholescintigraphic findings were false-positive in two patients with non-obstructive cholestasis. We conclude that MRCP can be used to depict the major biliary structures of neonates and small infants and to exclude BA as the cause of neonatal cholestasis by allowing visualisation of the biliary tract.
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Affiliation(s)
- Junji Takaya
- Department of Pediatrics, Kansai Medical University, Moriguchi-shi, Osaka 570-8506, Japan.
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Dehghani SM, Haghighat M, Imanieh MH, Geramizadeh B. Comparison of different diagnostic methods in infants with Cholestasis. World J Gastroenterol 2006; 12:5893-5896. [PMID: 17007060 PMCID: PMC4100675 DOI: 10.3748/wjg.v12.i36.5893] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/19/2006] [Revised: 08/05/2006] [Accepted: 08/15/2006] [Indexed: 02/06/2023] Open
Abstract
AIM To evaluate different methods in differentiating idiopathic neonatal hepatitis from biliary atresia. METHODS Sixty-five infants with cholestatic jaundice and final diagnosis of idiopathic neonatal hepatitis and biliary atresia were studied prospectively from September 2003 to March 2006. A thorough history and physical examination were undertaken and the liver enzymes were examined. All cases underwent abdominal ultrasonography, hepatobiliary scintigraphy, and percutaneous liver biopsy. The accuracy, sensitivity, specificity and predictive values of these various methods were compared. RESULTS There were 34 girls and 31 boys, among them 46 subjects had idiopathic neonatal hepatitis (age, 61 +/- 17 d) and 19 had biliary atresia (age, 64 +/- 18 d). The mean age at onset of jaundice was significantly lower in cases of biliary atresia when compared to idiopathic neonatal hepatitis cases (9 +/- 13 d vs 20 +/- 21 d; P = 0.032). The diagnostic accuracy of different methods was as follows: liver biopsy, 96.9%; clinical evaluation, 70.8%; ultrasonography, 69.2%; hepatobiliary scintigraphy, 58.5%; and liver enzymes, 50.8%. CONCLUSION Our results indicate that clinical evaluation by an experienced pediatric hepatologist and a biopsy of the liver are considered as the most reliable methods to differentiate idiopathic neonatal hepatitis and biliary atresia.
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Affiliation(s)
- Seyed Mohsen Dehghani
- Department of Pediatric Gastroenterology, Gastroenterohepatology Research Center of Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
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Esmaili J, Izadyar S, Karegar I, Gholamrezanezhad A. Biliary atresia in infants with prolonged cholestatic jaundice: diagnostic accuracy of hepatobiliary scintigraphy. ACTA ACUST UNITED AC 2006; 32:243-7. [PMID: 16967250 DOI: 10.1007/s00261-006-9049-4] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2006] [Accepted: 07/11/2006] [Indexed: 11/28/2022]
Abstract
BACKGROUND Cholestatic jaundice during infancy is one of the most problematic challenges for pediatricians. Biliary atresia (BA) and neonatal hepatitis syndrome (NHS) are major causes of cholestatic jaundices. Our aim was to compare the diagnostic accuracy of hepatobiliary scintigraphy with liver biopsy and ultrasonography in excluding BA. METHODS Seventy consecutive patients, all suffering from prolonged cholestatic jaundice (>1 month), were included. Laparotomy with surgical cholangiography was considered as the gold standard; however, in nine patients, based on the patient's recovery from jaundice and the normalization of laboratory values during the clinical follow-up period (=6-12 months), the diagnosis of NHS was verified and performing laparotomy was unnecessary. All patients underwent hepatobiliary scintigraphy, liver biopsy and ultrasonography and their results were compared. RESULTS Based on the gold standards mentioned above, 46 patients (46/70 = 65.7%) had BA. The sensitivity, specificity, PPV, NPV, and accuracy of the hepatobiliary scintigraphy in diagnosis of BA were 90%, 80%, 91.8%, 76.2%, and 84.5%, respectively. The respective values for liver biopsy were 92.5%, 88.9%, 94.9%, 84.2%, and 90.1% and for ultrasonographic analysis were 41.7%, 90.9%, 90.9%, 41.7%, and 66.3%. CONCLUSION Hepatobiliary scintigraphy is an important imaging technique in the diagnostic evaluation of infants with prolonged cholestatic jaundice. It is a convenient and reliable method of differentiating BA from NHS, with a diagnostic accuracy superior to that of US but slightly inferior to that of liver biopsy. Ultrasonography is the least sensitive and specific available modality and its findings should be confirmed by scintigraphy or liver biopsy.
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Affiliation(s)
- Javad Esmaili
- Department of Nuclear Medicine, Imam Khomeini Hospitals Complex, Tehran University of Medical Sciences, Keshavarz Blvd., Tehran, Iran
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Ryeom HK, Choe BH, Kim JY, Kwon S, Ko CW, Kim HM, Lee SB, Kang DS. Biliary atresia: feasibility of mangafodipir trisodium-enhanced MR cholangiography for evaluation. Radiology 2005; 235:250-258. [PMID: 15749972 DOI: 10.1148/radiol.2343040028] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
Abstract
The study was approved by the institutional review board, and informed consent was obtained from the patients' parents. Twenty-three consecutive infants suspected of having biliary atresia (BA) were prospectively examined by using mangafodipir trisodium (Mn-DPDP)-enhanced magnetic resonance (MR) cholangiography. Sequential T1-weighted spoiled gradient-echo MR cholangiograms were obtained 1, 2, and 3 hours after intravenous administration of Mn-DPDP. The possibility of BA was excluded if bowel excretion of contrast material was noted at contrast material-enhanced MR cholangiography. The diagnostic specificity and accuracy of contrast-enhanced MR cholangiography were compared with those of conventional MR cholangiography, technetium 99m Tc ((99m)Tc)-disofenin (DISIDA) scintigraphy, and the triangular cord sign at ultrasonography (US). MR cholangiography was used to accurately distinguish four cases of BA from 19 cases of other cholestatic liver diseases, without false-positive results. Conventional MR cholangiography, (99m)Tc-DISIDA scintigraphy, and the triangular cord sign at US respectively yielded false-positive results of 42% (eight of 19 infants), 35% (six of 17 infants), and 11% (two of 19 infants) in patients without BA. Mn-DPDP-enhanced MR cholangiography appears to be a promising modality for early diagnosis of BA as the cause of neonatal cholestasis.
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Affiliation(s)
- Hun Kyu Ryeom
- Department of Radiology, Kyungpook National University School of Medicine, Samduk 2Ga, Jung-Gu, Daegu 700-721, Republic of Korea
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Moyer V, Freese DK, Whitington PF, Olson AD, Brewer F, Colletti RB, Heyman MB. Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 2004; 39:115-28. [PMID: 15269615 DOI: 10.1097/00005176-200408000-00001] [Citation(s) in RCA: 222] [Impact Index Per Article: 10.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]
Abstract
For the primary care provider, cholestatic jaundice in infancy, defined as jaundice caused by an elevated conjugated bilirubin, is an uncommon but potentially serious problem that indicates hepatobiliary dysfunction. Early detection of cholestatic jaundice by the primary care physician and timely, accurate diagnosis by the pediatric gastroenterologist are important for successful treatment and a favorable prognosis. The Cholestasis Guideline Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition has formulated a clinical practice guideline for the diagnostic evaluation of cholestatic jaundice in the infant. The Cholestasis Guideline Committee, consisting of a primary care pediatrician, a clinical epidemiologist (who also practices primary care pediatrics), and five pediatric gastroenterologists, based its recommendations on a comprehensive and systematic review of the medical literature integrated with expert opinion. Consensus was achieved through the Nominal Group Technique, a structured quantitative method. The Committee examined the value of diagnostic tests commonly used for the evaluation of cholestatic jaundice and how those interventions can be applied to clinical situations in the infant. The guideline provides recommendations for management by the primary care provider, indications for consultation by a pediatric gastroenterologist, and recommendations for management by the pediatric gastroenterologist. The Cholestasis Guideline Committee recommends that any infant noted to be jaundiced at 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin. However, breast-fed infants who can be reliably monitored and who have an otherwise normal history (no dark urine or light stools) and physical examination may be asked to return at 3 weeks of age and, if jaundice persists, have measurement of total and direct serum bilirubin at that time. This document represents the official recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition on the evaluation of cholestatic jaundice in infants. The American Academy of Pediatrics has also endorsed these recommendations. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all patients with this problem.
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Kanegawa K, Akasaka Y, Kitamura E, Nishiyama S, Muraji T, Nishijima E, Satoh S, Tsugawa C. Sonographic diagnosis of biliary atresia in pediatric patients using the "triangular cord" sign versus gallbladder length and contraction. AJR Am J Roentgenol 2003; 181:1387-90. [PMID: 14573442 DOI: 10.2214/ajr.181.5.1811387] [Citation(s) in RCA: 62] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Abstract
OBJECTIVE A retrospective review was performed to evaluate the importance of the "triangular cord" sign in comparison with gallbladder length and contraction for the diagnosis of biliary atresia in pediatric patients. MATERIALS AND METHODS Fifty-five fasting infants with cholestatic jaundice were examined on sonography. The examinations focused on the visualization of the triangular cord sign and assessment of gallbladder length and contraction. The diagnosis of neonatal hepatitis or of other causes of infantile cholestasis was made if symptom resolution occurred during follow-up. RESULTS A triangular cord sign was found in 27 of 29 infants with biliary atresia and in one of 26 infants with neonatal hepatitis or other causes of infantile cholestasis. The diagnostic accuracy was 95%, sensitivity was 93%, and specificity was 96%. The gallbladder was thought to be abnormal if it was less than 1.5 cm long, was not detectable, or was detectable but had no lumen. The gallbladder was abnormal in 21 of 29 infants with biliary atresia, whereas it was abnormal in eight of 26 infants with neonatal hepatitis or other causes of infantile cholestasis. The diagnostic accuracy was 71%, sensitivity was 72%, and specificity was 69%. The gallbladder was detectable on sonography in 13 infants with biliary atresia and 26 infants with neonatal hepatitis or other causes of infantile cholestasis. Gallbladder contraction was not confirmed in 11 of 13 infants with biliary atresia and seven of 26 infants with neonatal hepatitis or other causes of infantile cholestasis. The diagnostic accuracy was 77%, sensitivity was 85%, and specificity was 73%. CONCLUSION The triangular cord sign was a more useful sonographic finding for diagnosing biliary atresia than gallbladder length and contraction.
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Affiliation(s)
- Kimio Kanegawa
- Department of Radiology, Kobe Children's Hospital, 1-1-1 Takakuradai Suma-ku, Kobe 654-0081, Japan
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47
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Lee HJ, Lee SM, Park WH, Choi SO. Objective Criteria of Triangular Cord Sign in Biliary Atresia on US Scans. Radiology 2003; 229:395-400. [PMID: 14595143 DOI: 10.1148/radiol.292020472] [Citation(s) in RCA: 78] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
PURPOSE To develop objective criteria for the ultrasonographic (US) appearance of the triangular cord (TC) sign for the diagnosis of biliary atresia. MATERIALS AND METHODS US was performed in 86 infants with jaundice. Biliary atresia (n = 20) was confirmed with hepatoportoenterostomy. Neonatal hepatitis (n = 66) was diagnosed with needle biopsy (n = 5), cholescintigraphy (n = 19), or clinical findings (n = 42). Thickness of the echogenic anterior wall of the right portal vein (EARPV) was measured. The TC sign was defined as thickness of the EARPV of more than 4 mm on a longitudinal scan. Biliary atresia was diagnosed when the TC sign was present. Statistical analyses were performed to compare the thickness of the EARPV between patients with biliary atresia and those with neonatal hepatitis and to test the significance of a 4-mm thickness as the criterion for the TC sign in the differentiation of biliary atresia from neonatal hepatitis (P <.05). RESULTS The TC sign was present in 16 (80%) of 20 patients with biliary atresia and in one of 66 patients with neonatal hepatitis. Mean thickness of the EARPV was significantly greater in patients with biliary atresia (5.39 mm) than in patients with neonatal hepatitis (2.17 mm) (P <.05). Use of 4-mm thickness as the criterion for TC sign was statistically significant (P <.05), resulting in a sensitivity of 80%, specificity of 98%, and positive and negative predictive values of 94% for the diagnosis of biliary atresia. CONCLUSION An objective criterion of the TC sign is an EARPV thicker than 4 mm on a longitudinal scan.
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Affiliation(s)
- Hee-Jung Lee
- Department of Diagnostic Radiology, School of Medicine & Institute for Medical Science, Keimyung University Dongsan Medical Center, 196 Dongsandong, Chunggu, Taegu 700-310, Korea.
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Visrutaratna P, Wongsawasdi L, Lerttumnongtum P, Singhavejsakul J, Kattipattanapong V, Ukarapol N. Triangular cord sign and ultrasound features of the gall bladder in infants with biliary atresia. AUSTRALASIAN RADIOLOGY 2003; 47:252-6. [PMID: 12890244 DOI: 10.1046/j.1440-1673.2003.01172.x] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
The aim of this study was to reassess the accuracy of the triangular cord sign, the triangular cord sign coupled with abnormal gall-bladder length, and an irregular gall-bladder wall in the diagnosis of biliary atresia. The ultrasonograms of 46 infants with cholestatic jaundice were reviewed for the triangular cord sign, gall-bladder length and gall-bladder wall without knowledge of the clinical data. Of the 23 infants with biliary atresia, 22 had the triangular cord sign whereas 17 infants with other causes of cholestatic jaundice did not have the triangular cord sign. The sensitivity, specificity, accuracy and positive predictive value of the triangular cord sign in the diagnosis of biliary atresia were 95.7, 73.9, 84.8 and 78.6%, respectively. The sensitivity, specificity, accuracy and positive predictive value of the triangular cord sign coupled with abnormal gall-bladder length in the diagnosis of biliary atresia were all 95.7%. Gall-bladder wall irregularity was seen in seven of 14 infants (50%) with biliary atresia whose gall bladders contained bile on ultrasound and in two of 22 infants (9.1%) without biliary atresia whose gall bladders contained bile on ultrasound. At the medical centre where this study was performed and where infants present with cholestatic jaundice at an advanced stage, the ultrasonographic triangular cord sign coupled with abnormal gall-bladder length is more reliable than the ultrasonographic triangular cord sign alone or gall-bladder wall irregularity in the diagnosis of biliary atresia.
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Affiliation(s)
- Pannee Visrutaratna
- Department of Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
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49
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Tan Kendrick APA, Phua KB, Ooi BC, Tan CEL. Biliary atresia: making the diagnosis by the gallbladder ghost triad. Pediatr Radiol 2003; 33:311-5. [PMID: 12695863 DOI: 10.1007/s00247-003-0867-z] [Citation(s) in RCA: 59] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/05/2002] [Accepted: 12/12/2002] [Indexed: 01/11/2023]
Abstract
OBJECTIVE To describe the gallbladder ghost triad and evaluate its usefulness in the sonographic diagnosis of extrahepatic biliary atresia (BA). METHODS From October 1997 to February 2002, 217 fasted infants with cholestatic jaundice aged 2-12 weeks were examined sonographically. We defined the gallbladder ghost triad as gallbladder length <1.9 cm, lack of smooth/complete echogenic mucosal lining with an indistinct wall and irregular/lobular contour, and used it as a criteria for BA. Gallbladder wall thickness, triangular cord, diffuse periportal echogenicity and hepatic artery calibre were also recorded. Diagnosis of BA was confirmed surgically and histologically. RESULTS Thirty of 31 babies with BA demonstrated the gallbladder ghost triad. No false-positives were recorded. The 31st BA baby showed a normal gallbladder at 6 weeks, but developed the ghost triad at 8 weeks. Gallbladder wall thickening was seen in 46/186 non-BA babies, but not in BA. Triangular cord was observed in 24/31 babies. Twenty-two of 186 non-BA babies and 5/31 BA babies showed diffuse periportal echogenicity. The hepatic artery appeared more prominent in BA. All 31 babies diagnosed sonographically as BA had surgery. Three non-BA babies had "negative" laparotomies showing hypoplastic bile ducts. CONCLUSIONS The gallbladder ghost triad is a very accurate sign of BA. Indeterminate cases require close follow-up.
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Affiliation(s)
- Anne Poh Ann Tan Kendrick
- Department of Diagnostic Imaging, Kandang Kerbau Women's and Children's Hospital, 100 Bukit Timah Road, 229899, Singapore.
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Avni FE, Segers V, De Maertelaer V, Cadranel S, Dassonville M, Delaet MH, Nicaise N, Metens T. The evaluation by magnetic resonance imaging of hepatic periportal fibrosis in infants with neonatal cholestasis: preliminary report. J Pediatr Surg 2002; 37:1128-33. [PMID: 12149687 DOI: 10.1053/jpsu.2002.34457] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
PURPOSE The aim of this study was to evaluate the potential role of magnetic resonance (MR) imaging cholangiography for the assessment of periportal fibrosis associated with neonatal cholestasis. METHODS The authors have compared the findings on MR imaging cholangiography and on pathology in 10 infants evaluated because of neonatal cholestasis. The series included 3 patients with biliary atresia (BA), 3 patients with choledocal cyst, 2 with a neonatal hepatitis, and 2 with an inspissated bile syndrome. MR examinations were performed on a 0.5 Tesla magnet unit using Turbo Field Echo T1 and Turbo spin echo T2 sequences. A periportal hyposignal paralleling the portal vein branches and disappearing after Gadolinium injection was considered consistent with periportal fibrosis. The final type of hepatobiliary anomaly was established based on surgery (n = 6) or on laparoscopic cholangiogram (n = 10). The degree of periportal fibrosis was evaluated on pathology using a grading system from grade 0 (no fibrosis) to 4 (fibrosis with cirrhosis). The relationship between periportal hyposignal and fibrosis was tested using the exact chi2 test. RESULTS MR imaging assessed correctly and more completely than ultrasound scan the morphology of the biliary tract in all 10 patients. A periportal hyposignal was present in the 3 patients with BA (2 patients displayed a grade 3 and one a grade 4 fibrosis on pathology) and in one with choledocal cyst (grade 3 fibrosis on pathology). No hyposignal was visualized in the 2 other patients with a choledocal cyst (grades 1 and 2), in the 2 patients with neonatal hepatitis (grades 1 and 2), or in the 2 patients with inspissated bile syndrome (both grade 0). A relationship between the hyposignal seen on MR and the degree of fibrosis seen on pathology was confirmed by the exact chi2 test (P =.019). CONCLUSIONS This preliminary series confirms the potential role of MR imaging for the assessment of the morphology of the abnormal biliary tract and of the degree of periportal fibrosis. The presence of an hyposignal on an echo gradient TFE T1 sequence suggests an advanced fibrosis (grade 3 and higher).
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Affiliation(s)
- Fred E Avni
- Department of Pediatric Imaging, University Children Hospital, Brussels, Belgium
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