• Advances in pediatric surgery
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• v.11 no.1
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• pp.19-26
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• 2005

Choledochal cysts have been associated with complications such as cholangitis, pancreatitis, and malignancy of the biliary tract. Recently, the incidence of choledochal cyst in neonate and young infant is increasing due to advances in diagnostic imaging. The aim of this study is to investigate the rationale of excision of choledochal cyst during the neonatal period. The clinical outcome and correlation between age at surgery and the degree of liver fibrosis were reviewed retrospectively. A total of 198 patients with choledochal cyst who were managed surgically between January 1985 and December 2000 at the Department of Surgery, Seoul National University Children's Hospital were included in this study. The overall outcome and the outcome of patients who were managed surgically during the neonatal period were compared. Correlation between age and the degree of liver fibrosis was evaluated by chi-square test and Pearson exact test. The mean age of the patients was 2 years 7 months (ranged from 5 days to 15 years). Mean postoperative follow-up period was 7 years 1 month (7 months to 20 years). The results are as follows. Twelve patients (6%) had postoperative complications, cholangitis (7), bleeding (4) and ileus (1). Eleven patients operated during the neonatal period had no postoperative complications. The positive correlation between age group and degree of liver fibrosis was statistically significant (chi-square: p=0.0165, Pearson exact test: p=0.019). The results support the rationale that excision of choledochal cyst can be performed safely without increasing morbidity in neonates.

• Journal of Yeungnam Medical Science
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• v.35 no.1
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• pp.109-113
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• 2018

A 75-year-old man with chronic cholangitis and a common bile duct stone that was not previously identified was admitted for right upper quadrant pain. Acute cholecystitis with cholangitis was suspected on abdominal computed tomography (CT); therefore, endoscopic retrograde cholangiopancreatography with endonasal biliary drainage was performed. On admission day 5, hemobilia with rupture of two intrahepatic artery pseudoaneurysms was observed on follow-up abdominal CT. Coil embolization of the pseudoaneurysms was conducted using percutaneous transhepatic biliary drainage. After several days, intrahepatic artery pseudoaneurysm rupture recurred and coil embolization through a percutaneous transhepatic biliary drainage tract was conducted after failure of embolization via the hepatic artery due to previous coiling. After the second coil embolization, a common bile duct stone was removed, and the patient presented no complications during 4 months of follow-up. We report a case of intrahepatic artery pseudoaneurysm rupture without prior history of intervention involving the hepatobiliary system that was successfully managed using coil embolization through percutaneous transhepatic biliary drainage.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.2
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• pp.164-172
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• 2021

Purpose: The use of Endoscopic ultrasonography (EUS) in pediatric patients is not as common as in adults. The aim of this study is to evaluate the role of EUS in the diagnosis of pancreatobiliary disease in childhood. Methods: Between December 2016 and January 2018, the findings of patients who underwent EUS were evaluated retrospectively. Results: Of the 41 patients included in the study 25 were girls (61.0%), mean age was 12.2±4.2 years. EUS was performed for biliary colic in 21 (51.2%), for recurrent pancreatitis in 12 (29.2%), for cholecystitis/cholangitis in 5 (12.2%), and for acute pancreatitis in 3 (7.4%) patients. EUS had a significant clinical effect in the decision of treatment and follow-up of 6/21 biliary colic cases, in diagnosis and follow-up of 6/12 recurrent pancreatitis cases, in decision-making and monitoring of invasive procedures (ERCP/surgery) of 3/5 acute cholecystitis/cholangitis and 2/3 of acute pancreatitis cases as well as in follow-up of the other cases. The effectiveness of EUS in determining direct treatment and invasive intervention was 43.9%. None of the patients had complications related to the EUS procedure. Conclusion: Although current guidelines show that EUS can be used in pediatric patients, this is limited to a few published studies. In this study, it is shown that EUS is a safe method for the diagnosis, follow-up and treatment of common pancreatobiliary pathologies in childhood.

• Advances in pediatric surgery
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• v.3 no.2
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• pp.93-97
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• 1997

Extended porta hepatis dissection and hepatic portojejunostomy was performed on 14 biliary atresia patients during last 13 years by a single surgeon. The average age at operation was 68 days(range from 37 days to 98 days). The patients were admitted for 8 weeks postoperatively for administration of parenteral antibiotics. There was one operative mortality due to acute hepatic necrosis. Among 13 patients remaining, 12(92.5 %) became chemically jaundice-free within 36 weeks postoperatively(average 16.8 weeks). the earliest 8 weeks, and in one patients jaundice persisted. Five(38.5 %) patients developed cholangitis after operation. Among jaundice-free patients, one patient died of unrelated disease 2 years after hepatic portojejunostomy, who underwent left lateral segmentectomy because of a biloma. Eleven survivors(78.6 %) are jaundice-free. The oldest one is 13 years old, enjoying a normal life. The mean period of follow-up is 7 years and 3 months.

• Advances in pediatric surgery
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• v.17 no.1
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• pp.1-14
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• 2011

Biliary atresia (BA) is an infantile cholestatic disease of progressive obliterative cholangiopathy with varying degrees of damage to both extra and intrahepatic bile ducts due to unknown causes. The diagnostic studies should be done to diagnose or exclude BA without unnecessary delay. Kasai portoenterostomy is the first choice of treatment for bile drainage from microscopic bile ductules present in the portal fibrous mass. The medical management after Kasai portoenterostomy should be done carefully to maintain bile excretion and prevent and treat complications Including cholangitis, hepatic fibrosis, portal hypertension and nutritional problem. The reported five years-survival rates after Kasai portoenterostomy range from 30 to 60 %. About 20 % of all patients undergoing Kasai portoenterostomy during infancy survive into adulthood with their native liver. Even if Kasai portoenterostomy remains as the first line of treatment In BA, liver transplantation serves as a good salvage treatment when portoenterostomy fails or liver function gradually deteriorates after initially successful establishment of bile flow, Overall 5-year survival rate in BA is about 90 % in recent series.

• Advances in pediatric surgery
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• v.14 no.2
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• pp.178-182
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• 2008

Forme fruste choledochal cyst (FFCC) is one of the diverse types of choledochal cyst with little or no dilatation of the extrahepatic bile duct (EHBD). It is considered that FFCC has to do with the pancreatobiliary malunion (PBM). In children, 3 to 6 millimeters of EHBD is assumed to be normal. Even though there is no clear-cut definition, FFCC is likely to be associated with bile duct dilatation less than 10 millimeters. Almost all cases have PBMs and symptoms of the pancreatitis or cholangitis. We experienced a case of FFCC in a 4-year-old boy. His EHBD measures 10 mm diameter. He had symptoms of pancreatitis and elevated hepatic transaminases. The pancreatobiliary common channel was 28 millimeters. He underwent EHBD resection and Roux-en-Y hepaticojejunostomy and was discharged with no specific complications.

• Journal of Yeungnam Medical Science
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• v.38 no.1
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• pp.1-9
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• 2021

Gallbladder (GB) polyp is a mucosal projection into the GB lumen. With increasing health awareness, GB polyps are frequently found using ultrasonography during health screening. The prevalence of GB polyps ranges between 1.3% and 9.5%. Most patients are asymptomatic and have benign characteristics. Of the nonneoplastic polyps, cholesterol polyps are most common, accounting for 60%-70% of lesions. However, a few polyps have malignant potential. Currently, the guidelines recommend laparoscopic cholecystectomy for polyps larger than 1 cm in diameter due to their malignant potential. The treatment algorithm can be influenced by the size, shape, and numbers of polyps, old age (>50 years), the presence of primary sclerosing cholangitis, and gallstones. This review summarizes the commonly recognized concepts on GB polyps from diagnosis to an algorithm of treatment.

• Journal of Digestive Cancer Research
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• v.5 no.1
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• pp.55-57
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• 2017

A 64-year-old man was admitted due to jaundice for 2 weeks. Radiologic findings revealed biliary stricture at the hepatic hilum with intrahepatic duct dilation suggesting Bismuth type IV Klatskin tumor. Jaundice improved spontaneously several days after hospitalization. Surgical treatment was considered but he only wanted to observe without specific treatment. Ten months later, he was re-admitted due to the recurrence of jaundice. Computed tomography (CT) showed no significant difference compared to previous results. Serum cancer antigen 19-9 and Immunoglobulin G4 were normal. Endoscopic forcep biopsy during endoscopic retrograde cholagiopancreatography (ERCP) revealed chronic inflammation. After steroid use under possible diagnosis of IgG4 related cholangiopathy, biliary stricture improved slightly. Four years later, he was hospitalized with the occurrence of acute cholangitis. Endoscopic retrograde biliary drainage was performed following endobiliary forcep biopsy. Pathology revealed well-differentiated adenocarcinoma at this time. Combined chemotherapy based on gemcitabine and cisplatin was performed. Six months later, CT revealed partial response.

• Gut and Liver
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• v.12 no.6
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• pp.714-721
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• 2018

Background/Aims: Recently reported prognostic models for primary biliary cholangitis (PBC) have been shown to be effective in Western populations but have not been well-validated in Asian patients. This study aimed to compare the performance of prognostic models in Korean patients and to investigate whether inflammation-based scores can further help in prognosis prediction. Methods: This study included 271 consecutive patients diagnosed with PBC in Korea. The following prognostic models were evaluated: the Barcelona model, the Paris-I/II model, the Rotterdam criteria, the GLOBE score and the UK-PBC score. The neutrophil-to-lymphocyte ratio (NLR) was analyzed with reference to its association with prognosis. Results: For predicting liver transplant or death at the 5-year and 10-year follow-up examinations, the UK-PBC score (areas under the receiver operating characteristic curve [AUCs], 0.88 and 0.82) and GLOBE score (AUCs, 0.85 and 0.83) were significantly more accurate in predicting prognosis than the other scoring systems (all p<0.05). There was no significant difference between the performance of the UK-PBC and GLOBE scores. In addition to the prognostic models, a high NLR (>2.46) at baseline was an independent predictor of reduced transplant-free survival in the multivariate analysis (adjusted hazard ratio, 3.74; p<0.01). When the NLR was applied to the prognostic models, it significantly differentiated the prognosis of patients. Conclusions: The UK-PBC and GLOBE scores showed good prognostic performance in Korean patients with PBC. In addition, a high NLR was associated with a poorer prognosis. Including the NLR in prognostic models may further help to stratify patients with PBC.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.60-64
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• 2008

Kabuki syndrome is characterized by peculiar facial features, developmental delay, and mental retardation. Congenital hepatic abnormalities in Kabuki syndrome patients have been sporadically reported in the literature and consist of extrahepatic biliary atresia, neonatal sclerosing cholangitis, and transient neonatal cholestasis. We report here a case of congenital hepatic fibrosis in a patient with Kabuki syndrome. To our knowledge, only one case of congenital hepatic fibrosis has been reported in the setting of Kabuki syndrome.