This paper includes our 9-year experience of 34 infants with biliary atresia with introduction of a new non-invasive diagnostic method, that is, ultrasonographic "triangular cord" (TC) sign. TC sign was defined as visualization of a triangular or a band-like echogenicity just cranial to the portal vein. Ultrasonographic TC sign seemed to be a simple, non-invasive, time-saving and useful tool in the diagnosis of biliary atresia, representing 84% sensitivity. Active bile excretion was restored in 90% of the patients who were treated between 31-60days, 78% of those between 61-90 days, and 33% of those being 91days or older. The incidence of postoperative cholangitis was 36%, and construction of antireflux valve in the Roux-en -Y loop did not affect the incidence of postoperative cholangitis (P=0.18). As for the surgical outcome, of 34 infants with biliary atresia, 23 (68%) are alive for 2-102 months period, and 12 of them are alive for more than 5 years . Five-year estimate survival by Kaplan-Meier method was 66 %.
Bile ; Biliary Atresia* ; Cholangitis ; Diagnosis* ; Humans ; Incidence ; Infant ; Portal Vein ; Ultrasonography

Congenital duodenal atresia is the most common cause of perinatal intestinal obstruction. Abdominal distension and projectile vomiting are the specific symptoms of neonatal gastro-intestinal obstruction. The incidence of duodenal atresia is between 1 in 2,710 and 1 in 10,000 live births.1 Most cases are thought to be failure of recanalization of the duodenal lumen during embryonic period.2 Duodenal atresia is associated with a high incidence of other associational anomalies, including esophageal atresia, biliary atresia, congenital heart disease and vertebral anomalies. Prenatal diagnosis, the ealry operation and the facotrs such as prematurity, combined abnormalities, nutirtion have a significant influence on complication and mortality.3,4 We report a case of congenital duodenal atresia diagnosed by ultrasonography prenatally in Andong general hostpital at 26 gestational weeks and operated the 7th day of cesarean section in Yeungnam university hospital with a brief review of literature.
Biliary Atresia ; Cesarean Section ; Esophageal Atresia ; Female ; Gyeongsangbuk-do ; Heart Defects, Congenital ; Incidence ; Intestinal Obstruction ; Pregnancy ; Prenatal Diagnosis ; Ultrasonography ; Ultrasonography, Prenatal* ; Vomiting

Biliary atresia (BA) is very difficult to distinguish from neonatal hepatitis (NH) and its prognosis depends on the age at the time of Kasai operation. Therefore early differentiation between these two conditions is very important. Although various clinical and laboratory tests have been reported to differentiate between them, they are still of limited value. From 1980 to 1999, forty-five infants were referred to our pediatric surgical unit for operation for suspected BA. Eight patients underwent Kasai operation immediately because late diagnosis. These were excluded from the study. The clinical history, physical findings, radiologic and laboratory examinations of 37 cases were analyzed retrospectively. The average age of BA (n=20) was 55.1+/-6.7 days, and that of NH (n=17) was 55.8+/-5.6 days. The sex ratio of BA was 13:7, and that of NH was 14:3. All the patients had obstructive jaundice and acholic stool except 4 BA and 6 NH patients. Acholic stool with yellow component was more frequent in NH. Onset of jaundice was within 2 weeks after birth in 85% of BA, and in 65% of NH. The onset of acholic stool was within 2 weeks after birth in 60% of BA, and in 23.5% of NH. The duration of jaundice and acholic stool of BA were 50.9+/-6.6 days and 41.3+/-8.4 days and those of NH were 40.1+/-3.1 days and 26.6+/-5.4 days respectively. The ultrasonogram and hepatobiliary scan were useful, but not a definitively reliable method for the differentiation of these two diseases. There was no difference in laboratory data. Seventeen cases had NH among 45 referred cases for Kasai operation with the clinical impression of BA, and 4 cases of 17 NH cases needed to be explored to rule out BA. In conclusion, false positive rate of clinical impression of BA was 37.8%, and negative exploration rate was 8.9%. Therefore, careful clinical observation for 1-2 weeks by an experienced pediatric surgeon was very important to avoid unnecessary operation to rule out NH up to the age of 8 - 10 weeks, so long as the stool had yellow component.
Biliary Atresia* ; Delayed Diagnosis ; Diagnosis, Differential ; Hepatitis ; Humans ; Infant ; Jaundice ; Jaundice, Obstructive ; Parturition ; Prognosis ; Retrospective Studies ; Sex Ratio ; Ultrasonography

PURPOSE: Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. METHODS: From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and MRCP. The final diagnosis was based on the operative and pathologic findings with biopsy specimen including clinical and laboratory findings. RESULTS: A total of 70 patients, consisting of 31 males and 39 females, with a mean age of 2.6+/-3.3 years were studied. The final diagnosis was biliary atresia in 25, neonatal cholestasis in 18, choledochal cyst without anomalous pancreatobiliary duct union(APBDU) in nine, choledochal cyst with APBDU in seven, cholestatic hepatitis in five, chronic recurrent pancreatitis in three, sclerosing cholangitis in two, and secondary biliary cirrhosis in one case. The overall diagnostic accuracy of abdominal ultrasonography was 75.7% and that of MRCP was 97.1%. The sensitivity and specificity of MRCP were 100% and 98% for biliary atresia, 87.5% and 100% for choledochal cyst with APBDU, 100% and 100% for choledochal cyst without APBDU, sclerosing cholangitis and chronic recurrent pancreatitis, respectively. CONCLUSION: MRCP is a fast, non-invasive and reliable method for diagnosing pancreaticobiliary diseases in children and will be the standard diagnostic procedure in the future.
Biliary Atresia ; Biopsy ; Child* ; Cholangitis, Sclerosing ; Choledochal Cyst ; Cholestasis ; Diagnosis ; Female ; Hepatitis ; Humans ; Jaundice, Obstructive ; Liver Cirrhosis, Biliary ; Male ; Pancreatitis ; Sensitivity and Specificity ; Ultrasonography

PURPOSE: The aim of this study is to determine the feasibility and effectiveness of performing an ultrasound-guided percutaneous cholecysto-cholangiogram (PCC) for excluding biliary atresia as the cause of neonatal jaundice. MATERIALS AND METHODS: Between Oct. 2003 and Feb. 2005, six ultrasound-guided PCC procedures were performed to five jaundiced infants (4 females and 1 male; mean age: 60 days old) for whom possibility of biliary atresia could not be ruled out by the DISIDA scan as the cause of their neonatal jaundice. Gallbladder puncture was performed under ultrasound guidance with a 23-gauge needle. Contrast material injection during fluoroscopic examination was performed after dilatation of the gallbladder lumen with normal saline under ultrasound guidance. The criteria used for excluding biliary atresia were complete visualization of the extrahepatic biliary trees and/or contrast excretion into the duodenum. The complications and final diagnosis was assessed according to the clinical and laboratory findings. RESULTS: The procedures were successful in all the patients without any complication. Biliary atresia could be ruled out in all the patients. The final diagnosis was neonatal cytomegalovirus hepatitis in two patients, total parenteral nutrition-associated cholestasis in two patients, and combined cytomegalovirus hepatitis and total parenteral nutrition-associated cholestasis in one patient. CONCLUSION: Ultrasound-guided PCC is a feasible and effective method for the early definitive exclusion of biliary atresia as the cause of neonatal jaundice. By the technique of injecting normal saline before contrast injection, PCC can be done even in a totally collapsed or very small gallbladder.
Biliary Atresia* ; Cholangiography ; Cholecystography ; Cholestasis ; Cytomegalovirus ; Diagnosis ; Dilatation ; Duodenum ; Female ; Gallbladder ; Gastrointestinal Tract ; Hepatitis ; Humans ; Infant* ; Infant, Newborn ; Jaundice, Neonatal ; Male ; Needles ; Punctures ; Ultrasonography

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.
Adolescent ; Anoxia ; Arteriovenous Fistula/etiology ; Biliary Atresia/*diagnosis/etiology ; Cyanosis/complications ; Dyspnea/complications ; Echocardiography, Transesophageal ; End Stage Liver Disease/complications/*surgery ; Female ; Hepatic Artery/abnormalities ; Hepatopulmonary Syndrome/*diagnosis/ultrasonography ; Humans ; *Liver Transplantation ; Osteoarthropathy, Secondary Hypertrophic/complications

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.
Area Under Curve ; Biliary Atresia/*diagnosis/ultrasonography ; Common Bile Duct/ultrasonography ; Decision Making ; Diagnosis, Differential ; Female ; Gallbladder/ultrasonography ; Hepatic Artery/ultrasonography ; Humans ; Infant ; Infant, Newborn ; Jaundice, Obstructive/complications/diagnosis ; Logistic Models ; Male ; Portal Vein/ultrasonography ; ROC Curve ; Retrospective Studies ; Sensitivity and Specificity

Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a liver biopsy was suggestive of biliary atresia. This finding forced us to perform intraoperative cholangiography, which revealed a patent common bile duct with impacted thick bile. We performed normal saline irrigation and the symptom was improved, the final diagnosis was IBS. Thus, we herein report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.
Bile Ducts, Extrahepatic ; Bile* ; Biliary Atresia ; Biopsy ; Cholangiography ; Cholangiopancreatography, Magnetic Resonance ; Cholestasis ; Common Bile Duct ; Diagnosis ; Diagnostic Tests, Routine ; Fatty Acids, Omega-3 ; Fatty Acids, Unsaturated* ; Female ; Humans ; Infant ; Jaundice ; Liver ; Sewage ; Ultrasonography