Terry M Humphrey, Mark D Stringer
Biliary atresia: US diagnosis.
Radiology. 2007 Sep;244(3):845-51. doi: 10.1148/radiol.2443061051.
Abstract/Text
PURPOSE: To evaluate prospectively the sensitivity of ultrasonography (US) in the diagnosis of biliary atresia (BA), with surgery as the reference standard.
MATERIALS AND METHODS: After institutional ethical approval and with informed parental consent, 90 consecutive fasting infants with conjugated hyperbilirubinemia underwent detailed US studies performed by a single operator with a 7.5-MHz curvilinear transducer and a 13.5-MHz linear-array transducer. The following features were prospectively recorded: gallbladder morphology, triangular cord sign, presence of a common bile duct, liver size and echotexture, splenic appearance, and vascular anatomy. The operator was blinded to results of other investigations. Sensitivity, specificity, and positive and negative predictive values were calculated for each US variable. BA and non-BA groups were compared by means of the Fisher exact test for categorical variables and an unpaired t test for continuous variables.
RESULTS: Thirty infants (13 male, 17 female) had surgically confirmed BA, and 60 (35 male, 25 female) had other documented causes of neonatal jaundice; the mean ages at US assessment were 48.5 and 52.4 days, respectively (P>.5). Eight US features showed a significant difference between BA and non-BA groups (P<.001, Fisher exact test). The features with the greatest individual sensitivity and specificity, respectively, in the diagnosis of BA were triangular cord sign (73% and 100%), abnormal gallbladder wall (91% and 95%) and shape (70% and 100%), and an absent common bile duct (93% and 92%). The hepatic artery diameter was significantly larger in infants with BA than in those without BA (mean+/-standard deviation, 2.2 mm+/-0.59 vs 1.6 mm+/-0.40, P<.001), but portal vein diameters were not significantly different. By means of all these US features, 88 of 90 infants were correctly classified as having or not having BA, for an overall accuracy of 98%.
CONCLUSION: BA can be distinguished with US from other causes of conjugated hyperbilirubinemia in 98% of infants if multiple US features are carefully evaluated.
Copyright (c) RSNA, 2007.
Woo Sun Kim, Jung-Eun Cheon, Byung Jae Youn, So-Young Yoo, Wha Young Kim, In-One Kim, Kyung Mo Yeon, Jeong Kee Seo, Kwi-Won Park
Hepatic arterial diameter measured with US: adjunct for US diagnosis of biliary atresia.
Radiology. 2007 Nov;245(2):549-55. doi: 10.1148/radiol.2452061093. Epub 2007 Sep 21.
Abstract/Text
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.
Richard A Schreiber, Collin C Barker, Eve A Roberts, Steven R Martin, Fernando Alvarez, Lesley Smith, J Decker Butzner, Iwona Wrobel, David Mack, Stanley Moroz, Mohsin Rashid, Rabin Persad, Dominique Levesque, Herbert Brill, Garth Bruce, Jeff Critch, Canadian Pediatric Hepatology Research Group
Biliary atresia: the Canadian experience.
J Pediatr. 2007 Dec;151(6):659-65, 665.e1. doi: 10.1016/j.jpeds.2007.05.051. Epub 2007 Oct 22.
Abstract/Text
OBJECTIVE: To determine the outcomes of Canadian children with biliary atresia.
STUDY DESIGN: Health records of infants born in Canada between January 1, 1985 and December 31, 1995 (ERA I) and between January 1, 1996 and December 31, 2002 (ERA II) who were diagnosed with biliary atresia at a university center were reviewed.
RESULTS: 349 patients were identified. Median patient age at time of the Kasai operation was 55 days. Median age at last follow-up was 70 months. The 4-year patient survival rate was 81% (ERA I = 74%; ERA II = 82%; P = not significant [NS]). Kaplan-Meier survival curves for patients undergoing the Kasai operation at age < or = 30, 31 to 90, and > 90 days showed 49%, 36%, and 23%, respectively, were alive with their native liver at 4 years (P < .0001). This difference continued through 10 years. The 2- and 4-year post-Kasai operation native liver survival rates were 47% and 35% for ERA I and 46% and 39% for ERA II (P = NS). A total of 210 patients (60%) underwent liver transplantation; the 4-year transplantation survival rate was 82% (ERA I = 83%, ERA II = 82%; P = NS).
CONCLUSIONS: This is the largest outcome series of North American children with biliary atresia at a time when liver transplantation was available. Results in each era were similar. Late referral remains problematic; policies to ensure timely diagnosis are required. Nevertheless, outcomes in Canada are comparable to those reported elsewhere.
Mark Davenport, Enrica Caponcelli, Emily Livesey, Nedim Hadzic, Edward Howard
Surgical outcome in biliary atresia: etiology affects the influence of age at surgery.
Ann Surg. 2008 Apr;247(4):694-8. doi: 10.1097/SLA.0b013e3181638627.
Abstract/Text
OBJECTIVE: The exact nature of the relationship between age at Kasai portoenterostomy (KP) for biliary atresia (BA) and outcome is controversial.
METHODS: Single-center retrospective analysis of consecutive cases (January 1994-December 2005) undergoing KP, using 2 dichotomous measures of outcome (clearance of jaundice to <20 micromol/L and native liver survival at 2 years post-KP). Outcome was analyzed by age cohort (<30 days, 30-40 days, etc) and cumulatively. Data are quoted as median (interquartile range). Statistical analysis was by extended Fisher r x c analysis. P < 0.05 was regarded as significant.
RESULTS: Two hundred twenty-five infants with BA were divided into 3 groups based on possible etiology: isolated BA (n = 177), biliary atresia splenic malformation (BASM) syndrome (n = 28), and cystic BA (n = 23). Three infants had both BASM and a cyst. Age at KP was significantly greater in those with isolated BA [58 (48-61)] compared with both BASM [47 (39-59); P = 0.02] and cystic BA [47 (39-54); P = 0.02]. Overall, 127 (56%) cleared their jaundice and 145 (65%) survived to 2 years with their native liver. Children with isolated BA showed no statistical difference by age cohort for clearance of jaundice (P = 0.75) or for native liver survival (P = 0.14). In contrast, there was a marked detrimental effect of age at KP for both BASM and cystic BA groups (P = 0.02).
CONCLUSION: There is a marked detrimental effect of age at KP on groups with a presumed "developmental" origin, not seen in the majority with isolated BA.
日本胆道閉鎖症研究会: 胆道閉鎖症全国登録2017年集計結果. 日小外会誌 2019; 55: 291-297.
F M Karrer, J R Lilly, B A Stewart, R J Hall
Biliary atresia registry, 1976 to 1989.
J Pediatr Surg. 1990 Oct;25(10):1076-80; discussion 1081.
Abstract/Text
The Registry provides information about 904 children with biliary atresia from more than 100 institutions. There was a 1.4 to 1 female predominance; racial distribution was 62% caucasian, 20% black, 11% Hispanic, 4.2% asian, and 1.5% American Indian. Eight hundred sixteen (90%) underwent corrective surgery (median age at operation, 69 days). Intraoperatively, 70% had totally obliterated extrahepatic bile ducts, 22% had patency of the gallbladder and distal common duct, whereas only 8% had "correctable" biliary atresia (proximal duct patency). A variety of reconstructions were used, but the majority of patients had a Roux-en-Y portoenterostomy with or without exteriorization. Follow-up was available for 670 children (74%) with average length of follow-up of 5 years (range, 1 to 16 years). Five-year actuarial survival was 48% following Kasai's operation, but was less than 10% (at 3 years) if no operative correction was done. Survival was unaffected by sex, type of reconstruction, or cholangitis. Predictors of a bad outcome were (1) caucasian race; (2) operative age greater than 60 days; (3) presence of cirrhosis at initial biopsy; (4) totally nonpatent extrahepatic ducts; (5) absent ducts at the level of transection in the liver hilus; and (6) subsequent development of varices or ascites. Identification of factors predictive of the ultimate outcome provide a basis for either continued efforts with management of Kasai's operation or for early referral for liver transplantation.
Masato Shinkai, Youkatsu Ohhama, Hiroshi Take, Norihiko Kitagawa, Hironori Kudo, Kyoko Mochizuki, Tomoko Hatata
Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children's hospital.
J Pediatr Gastroenterol Nutr. 2009 Apr;48(4):443-50.
Abstract/Text
OBJECTIVE: Sequential strategies combining the Kasai operation as a first-line treatment and liver transplantation as a second-line option, if necessary, have been accepted for patients with biliary atresia (BA). To understand the role of the Kasai operation in the treatment of BA, it is necessary to analyze the long-term outcome of the operation alone and to evaluate the present status of survivors retaining their native livers.
MATERIALS AND METHODS: A retrospective chart review was carried out for a group of 80 patients who had undergone the Kasai operation between 1970 and 1986 at the Kanagawa Children's Medical Center.
RESULTS: The 5-, 10-, and 20-year survival rates of patients with their native livers were 63%, 54%, and 44%, respectively. The survival rates varied significantly depending on the type of BA, age at initial Kasai operation, era of surgery, and surgical method. By age 20, nearly half of the adult survivors had already developed liver cirrhosis and its sequelae. Episodes of cholangitis and gastrointestinal bleeding occurred after 20 years of age in 37% and 17% of the adult patients, respectively, and 20% of the adult patients died of liver failure or underwent living-related partial liver transplantation in their 20s. Five female patients gave birth to a total of 9 children, and 1 male patient fathered a child.
CONCLUSIONS: Although increasing numbers of patients with BA survive 20 years or more after the Kasai operation, meticulous lifelong postoperative care should be continued for the survivors because of the possibility of hepatic deterioration.
Cheng-Hui Hsiao, Mei-Hwei Chang, Huey-Ling Chen, Hung-Chang Lee, Tzee-Chung Wu, Chieh-Chung Lin, Yao-Jong Yang, An-Chyi Chen, Mao-Meng Tiao, Beng-Huat Lau, Chia-Hsiang Chu, Ming-Wei Lai, Taiwan Infant Stool Color Card Study Group
Universal screening for biliary atresia using an infant stool color card in Taiwan.
Hepatology. 2008 Apr;47(4):1233-40. doi: 10.1002/hep.22182.
Abstract/Text
UNLABELLED: Biliary atresia is the most common cause of death from liver disease in children. Although the Kasai operation before 60 days of age can significantly improve prognosis, delay in referral and surgery remains a formidable problem worldwide because of difficulties in differentiating it from benign prolonged neonatal jaundice. We established a universal screening system using an infant stool color card to promote the early diagnosis and treatment of biliary atresia. After a pilot regional study in 2002-2003, a national stool color screening system was established by integrating the infant stool color card into the child health booklet given to every neonate in Taiwan since 2004. Within 24 hours of the discovery of an abnormal stool color, this event is reported to the registry center. The annual incidence of biliary atresia per 10,000 live births in 2004 and 2005 was 1.85 (40/216,419) and 1.70 (35/205,854), respectively. The sensitivity of detecting biliary atresia using stool cards before 60 days of age was 72.5% in 2004, which improved to 97.1% in 2005. The national rate of the Kasai operation before 60 days of age increased from 60% in 2004 to 74.3% in 2005. The jaundice-free rate (<2 mg/dL) at 3 months after the Kasai operation among infants with biliary atresia in 2004-2005 was 59.5% (44 of 74), significantly higher than the historical data of 37.0% in 1976-2000 before the stool card screening program (P = 0.002).
CONCLUSION: Universal screening using the stool color cards can enhance earlier referral, which may ultimately lead to timely performance of the Kasai operation and better postoperative outcome in infants with biliary atresia.
Sleisenger and Fordtran’s Gastrointestinal and Liver Disease, 9th ed. Saunders , 2010.
