• Journal of Veterinary Clinics
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• v.22 no.4
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• pp.401-403
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• 2005

A seven-year old female captive Japanese macaque (Macaca fuscata) was mobbed by its cage mates and subsequently died due to trauma. An incidental finding of multiple biliary cystadenoma in the liver is herein reported. Grossly, multiple small cysts were observed throughout the surface of the liver. One particular cyst that measured $1.3\times1.2\times1.0cm$ and contained mucinous fluid was observed in the center of the liver. Microscopically the cysts were lined by biliary epithelium that varied from simple cuboidal to columnar cells. Signs of malignancy and metastasis to other organs were not observed. This is the first report of biliary cystadenoma in Japanese macaque.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.4
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• pp.433-436
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• 2023

Forty-five adults with type IV-A choledochal cysts (CDC) who underwent extrahepatic cyst excision from January 2013 to December 2021 were followed up for a median interval of 25 months (range, 2 to 10 years) to observe the long-term complications in the remaining intrahepatic cyst. Late complications in varying combinations were seen in 10 patients, which included cholangitis and/or intrahepatic stones in 9 patients, intrahepatic bile duct stenosis with stones in 2 patients, anastomotic stricture in 6 patients, and left lobar atrophy with intrahepatic stones in 3 patients. Out of 6 patients who required re-do hepaticojejunostomy (HJ), three patients had left lobe atrophy with patent HJ anastomosis and a recurrent attack of cholangitis on follow-up at 3, 8, and 10 years. Complications occur frequently after extrahepatic cyst excision for type IV-A CDC and require a long-term follow-up.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.27 no.1
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• pp.62-70
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• 2024

Purpose: Choledochal cysts are congenital anomalies that occur as localized cystic or fusiform dilatations of the biliary tree. Reflux and stasis of pancreatic enzymes in the biliary duct may relate to the development of intestinal metaplasia which might be an important factor related to the carcinogenesis of choledochal cyst, thus the expression of beta-catenin in the metaplastic epithelium might be associated with malignant transformation of choledochal cyst epithelium. Methods: This study was conducted at a tertiary care pediatric center between October 2014 and March 2017. Forty patients were evaluated for epithelial lining, mural ulceration, fibrosis, inflammation, and metaplasia. Results: Out of 40, 12 cases (30.0%) were the infantile age group and 28 cases (70.0%) were in the classic pediatric group. Ulceration was classified as grade 0 (14 cases, 35.0%), grade 1 (17 cases, 42.5%), or grade 2 (nine cases, 22.5%). Inflammation was classified as grade 0 (2 cases, 5.0%), grade 1 (26 cases, 65.0%), or grade 2 (12 cases, 30.0%). Fibrosis was classified as grade 0 (five cases, 12.5%), grade 1 (11 cases, 27.5%), grade 2 (17 cases, 42.5%), or grade 3 (seven cases, 17.5%). Metaplasia was noted in five (12.5%) out of 40 cases. All choledochal cysts with metaplasia showed beta-catenin nuclear positivity on immunohistochemistry and were followed up. Conclusion: This study emphasizes the importance of detailed histopathological examination and documentation of metaplastic changes. Metaplasia was associated with beta-catenin nuclear positivity. These findings suggest a potential role for beta-catenin as a marker of metaplastic changes in choledochal cysts.

• Advances in pediatric surgery
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• v.10 no.2
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• pp.136-141
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• 2004

Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) is a rare disease. It has been generally recognized as type I (correctable with cystic dilatation), which means a good prognosis. From a total of 73 patients with BA who underwent operation from September 1988 to September 2003 at our institute, 7 (9.6 %) cases of type III BA with EHBC (uncorrectable with cystic dilatation) are reviewed. Clinical findings, laboratory data, radiologic findings, treatment methods and outcomes were reviewed. Female was more prevalent (male to female ratio; 2:5). All cases were type III with EHBC according to the intraoperative cholangiography, and underwent Kasai' s portoenterostomy. The mean age was 57 days at operation. Three patients(42.9 %) are long term survivors. Further evaluation is needed to determine the correlation between prognostic factors and outcome for.

• Journal of Yeungnam Medical Science
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• v.29 no.2
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• pp.136-140
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• 2012

A 61-year-old male who complained of right upper quadrant pain was referred to the authors for evaluation after his computed tomography suggested biliary adenocarcinoma. The lesion consisted of multiple cysts with papillary mass and peri-ampullay mass. The patient underwent an operation due to a clinical suspicion of biliary cystadenocarcinoma, but the pathology confirmed biliary papillomatosis (BP) after diagnosing intrahepatic papillary neoplasm with high-grade dysplasia and invasive adenocarcinoma with papillary neoplasm from the distal common bile duct to the duodenum. BP is a disease characterized by multiple papillary masses. Its cause has yet to be discovered. It commonly manifests as bile duct dilation but rarely as a ductal cystic change. Under computed tomography or magnetic resonance imaging, both the BP and the cystic neoplasm can show bile duct dilation and a papillary mass, which makes their differential diagnosis difficult. A confirmative diagnosis can be made through a pathologic examination. BP is classified as a benign disease that can become malignant and may recur, though rarely. Its treatment of choice is surgical resection. Laser ablation or photodynamic therapy can be used for unresectable lesions. In the case featured in this paper, biliary papillomatosis was difficult to differentiate from cystic adenocarcinoma due to diffusely scattered multiple large cystic lesions in the liver, and it was histologically confirmed to have become malignant with cystic duct dilation after the operation. This case is reported herein with a literature review.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.3
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• pp.180-186
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• 2015

Purpose: Bile duct dilatation is a relatively common sonographic finding; nevertheless, its clinical significance in children is controversial because little research has been done in the area. Therefore, we investigated the natural course and clinical significance of biliary duct dilatation in children. Methods: We performed a retrospective study of 181 children (range, 1-day-old to 17-year-old) in whom dilatation of the intrahepatic duct and/or common hepatic duct and/or common bile duct was detected by abdominal ultrasonography at the Severance Children's Hospital between November 2005 and March 2014. We reviewed and analyzed laboratory test results, clinical manifestations, and clinical course in these patients. Results: Pediatric patients (n=181) were enrolled in the study and divided into two groups. The first group included 59 subjects, without definitive cause of bile duct dilatation, who did not require treatment; the second group included 122 subjects, with definitive cause of bile duct dilatation or underlying biliary disease, who did require treatment. In the first group, 24 patients (40.7%) showed spontaneous resolution of bile duct dilatation, 20 patients (33.9%) showed no change, and 15 patients (25.4%) were lost to follow-up. In the second group, 31 patients were diagnosed with choledochal cysts, and 91 patients presented with biliary tract dilatations due to secondary causes, such as gallbladder or liver disease, post-operative complications, or malignancy. Conclusion: Biliary dilatation in pediatric patients without symptoms, and without laboratory and other sonographic abnormalities, showed a benign clinical course. No pathologic conditions were noted on follow-up ultrasonography.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.2
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• pp.195-200
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• 2023

Backgrounds/Aims: We aimed to build a machine learning tool to help predict low-grade intraductal papillary mucinous neoplasms (IPMNs) in order to avoid unnecessary surgical resection. IPMNs are precursors to pancreatic cancer. Surgical resection remains the only recognized treatment for IPMNs yet carries some risks of morbidity and potential mortality. Existing clinical guidelines are imperfect in distinguishing low-risk cysts from high-risk cysts that warrant resection. Methods: We built a linear support vector machine (SVM) learning model using a prospectively maintained surgical database of patients with resected IPMNs. Input variables included 18 demographic, clinical, and imaging characteristics. The outcome variable was the presence of low-grade or high-grade IPMN based on post-operative pathology results. Data were divided into a training/validation set and a testing set at a ratio of 4:1. Receiver operating characteristics analysis was used to assess classification performance. Results: A total of 575 patients with resected IPMNs were identified. Of them, 53.4% had low-grade disease on final pathology. After classifier training and testing, a linear SVM-based model (IPMN-LEARN) was applied on the validation set. It achieved an accuracy of 77.4%, with a positive predictive value of 83%, a specificity of 72%, and a sensitivity of 83% in predicting low-grade disease in patients with IPMN. The model predicted low-grade lesions with an area under the curve of 0.82. Conclusions: A linear SVM learning model can identify low-grade IPMNs with good sensitivity and specificity. It may be used as a complement to existing guidelines to identify patients who could avoid unnecessary surgical resection.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.121-125
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• 1999

To study the role of anomalous pancreatico-biliary ductal union (APBDU) in the development of choledochal cyst, we reviewed 23 cases. APBDU is defined as a long common channel(>0.4 mm). The patients ages ranged from 1 week to 112 months and the mean age was 22.5 months. Right upper quadrant pain was the most prevalent symptom. The diagnosis was made by ultrasonography and operative cholangiography in most patient. The preoperative diagnosis was made in 100 % of the cases. Gallstones were found in 5 cases. Todani type I and type IV were prevalent. A long common channel was found in all cases. The operative treatment consisted of cyst excision and Reux-en-Y hepaticojejunostomy or choledochojejunosotmy. One patient had postoperative pancreatitis. There was no mortality. We conclude that detection of choledochal cyst is occurring at a younger age and APBDU seems to play an important role in the pathogenesis of type I and IV cysts. Cyst excision is the treatment of choice to eliminate repeated cholangitis and malignant transformation.

• Advances in pediatric surgery
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• v.4 no.2
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• pp.156-162
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• 1998

Choledochal cyst is rare in the western countries, but common in oriental countries. Complicatioins include ascending cholangitis, recurrent pancreatities, progressive biliary cirrhosis, portal hypertension, stone formation and later malignant transformation. Bile peritonitis secondary to rupture is one of the rarest complications, with an incidence of 1.8 % to 18 %. The anomalous arrangement of the pancreatobiliary ductal system with a long common channel may cause inflammation leading to perforation of the cyst. The authors found 4 cases (14.2 %) of bile peritonitis among 28 cases of choledochal cyst treated from Jan. 1983 to Jan. 1998. The patients ages ranged from 6 months to 3 years and three were female. The perforation sites were located on the common bile duct at its junction with the cystic duct in 2 cases, the distal cyst wall in 1 case and the left hepatic duct at its junction with cyst in 1 case. The types of choledochal cysts by Todani's classification were Type IVa in 3 cases and type I in 1 case. By the new Komi's classification utilizing operative cholangiogram there were 2 cases of Type Ia, 1 case of type IIb and 1 case of type III. One stage cyst excision and hepaticojejunostomy(Roux-en Y type) was done in 3 cases, and two staged operation in 1 case. All patients had an uneventful course postoperatively. The average day of discharge was 9.8th postoperatively. In conclusion, primary excision of the choledochal cyst and biliary reconstruction is a safe and effective treatment of ruptured choledochal cyst in infants.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.1
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• pp.1-11
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• 2016

Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is imperative that the clinician differentiates between intrahepatic and extrahepatic origin of cholestasis. Treatment may be supportive or curative and depends on the etiology. Recent literature shows that optimal nutritional and medical support also plays an integral role in the management of pediatric patients with chronic cholestasis. This review will provide a broad overview of the pathophysiology, diagnostic approach, and management of cholestasis beyond the neonatal and infancy periods.