• 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.

• 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.13 no.1
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• pp.1-12
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• 2007

Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.

• Journal of Yeungnam Medical Science
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• v.30 no.1
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• pp.39-42
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• 2013

Cholelithiasis, duodenal ulcer, duodenal perforation and tumor invasion may lead to choledochoduodenal fistula (CDF). CDF often has no specific symptoms and may be incidentally detected in an upper gastrointestinal radiographic study or endoscopy; but in some cases, it may be accompanied by recurrent cholangitis and liver abscess. In this paper, a case of recurrent liver abscess caused by CDF is reported. A 62-year-old female was admitted to the authors' hospital because of right upper quadrant pain and fever. The abdominal computed tomography showed a liver abscess in the right lobe. A duodenal fistulous orifice was detected with endoscopy, and a contrast was injected through the duodenal orifice using a catheter under fluoroscopy. The injection of the contrast revealed a fistulous track between the duodenal bulb and the common hepatic duct. In fistulas complicated by recurrent liver abscess, surgery or medical management may be needed. The CDF in this case study was treated via endoscopic clipping.

• The Korean Journal of Nuclear Medicine
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• v.21 no.1
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• pp.25-32
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• 1987

Authors analysed biochemical studies and scintigraphic findings of obstructive jaundice and nonobstructive jaundice in 44 cases of $^{99m}Tc-DISIDA$ scintigraphy with nonvisualization of biliary excretion till 120 min or 240 min after injection of $^{99m}Tc-DISIDA$. Causative diseases of $^{99m}Tc-DISIDA$ scintigraphy with nonvisualization of biliary excretion were in order to choledocholithiasis (25%), hepatitis (25%), cholangiocarcinoma (14%), cholangitis (14%) and pancreas head tumor (11%). In obstructive jaundice, statistically significant findings were elevated alkaline phosphatase above 300 IU/L on biochemical study and single lobe enlargement of the liver, irregular radioisotope uptake of the liver and concave indentation of the gall bladder fossa of the liver on scintigraphy. In nonobstructive jaundice, statistically significant findings were persistent renal excretion of $^{99m}Tc-DISIDA$ and more increased uptake density of the heart than the liver on scintigraphy.

• Advances in pediatric surgery
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• v.14 no.2
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• pp.125-133
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• 2008

To evaluate the long-term prognosis of biliary atresia after Kasai operation, a total of 14 patients (of the 41 patients operated upon from 1982 to 1997), who had been followed up for more than 10 years, were included in this retrospective study. Eleven out of 14 patients survived with their native livers, and their data analyzed for age at operation, clearing time of jaundice, histological outcome, postoperative complications, effectiveness after the application of an intussusception anti-reflex valve, and quality of life. Average age at surgery was 62.8 days. Serum bilirubin was normalized within three months in all patients. Six among the eleven long-term survivors had ascending cholangitis as one of the postoperative complications. The application of an intussusception anti-reflux valve did not show any statistical significance in long-term survival. Most of long-term survivors appeared to enjoy good quality of life. Kasai operation might not be the definitive treatment for biliary atresia; however, Kasai operation made it possible to achieve long-term survival for patients with biliary atresia when the patients were detected and treated as early as possible.

• Journal of Yeungnam Medical Science
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• v.2 no.1
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• pp.299-306
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• 1985

Congenital dilatation of the common bile duct is relatively rare anomaly. Its pathogenesis has not been completely understood. Complications of the choledochal cyst are mainly suppurative cholangitis, liver cirrhosis, stone formation, malignant change, bile peritonitis due to spontaneous and traumatic rupture. We experienced one case of choledochal cyst associated with hemorrhagic tendency and a cerebral hematoma, which is extremely rare complication. The 3 monthes old male patient reported here was treated with complete excision of cyst and Roux-en-Y choledochojejunostomy after correction of bleeding tendency and removal of crebral hematoma. Postoperative course was relatively uneventful, 11 days after operation, the patient was discharged with full improvement.

• Parasites, Hosts and Diseases
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• v.45 no.4
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• pp.301-306
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• 2007

We attempted to identify parasite DNA in the biliary stones of humans via PCR and DNA sequencing. Genomic DNA was isolated from each of 15 common bile duct (CBD) stones and 5 gallbladder (GB) stones. The patients who had the CBD stones suffered from cholangitis, and the patients with GB stones showed acute cholecystitis, respectively. The 28S and 18S rDNA genes were amplified successfully from 3 and/or 1 common bile duct stone samples, and then cloned and sequenced. The 28S and 18S rDNA sequences were highly conserved among isolates. Identity of the obtained 28S D1 rDNA with that of Clonorchis sinensis was higher than 97.6%, and identity of the 18S rDNA with that of other Ascarididae was 97.9%. Almost no intra-specific variations were detected in the 28S and 18S rDNA with the exception of a few nucleotide variations, i.e., substitution and deletion. These findings suggest that C. sinensis and Ascaris lumbricoides may be related with the biliary stoneformation and development.

• Clinical Endoscopy
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• v.51 no.6
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• pp.596-599
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• 2018

An 89-year-old man was referred to our hospital for treatment of hepatolithiasis causing recurrent cholangitis. He had undergone a prior Whipple procedure. Computed tomography demonstrated left-sided hepatolithiasis. First, we conducted peroral direct cholangioscopy (PDCS) using an ultraslim endoscope. Although PDCS was successfully conducted, it was unsuccessful in removing all the stones. The stones located in the B2 segment were difficult to remove because the endoscope could not be inserted deeply into this segment due to the small size of the intrahepatic bile duct. Next, we substituted the endoscope with an upper gastrointestinal endoscope. After positioning the endoscope, the SpyGlass digital system (SPY-DS) was successfully inserted deep into the B2 segment. Upon visualizing the residual stones, we conducted SPY-DS-guided electrohydraulic lithotripsy. The stones were disintegrated and completely removed. In cases of PDCS failure, a treatment strategy using the SPY-DS can be considered for patients with hepatolithiasis after a Whipple procedure.

• Korean Journal of Transplantation
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• v.28 no.3
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• pp.160-164
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• 2014

Background: In solid organ transplantation patients, complications of cholelithiasis may run a fulminant course, resulting in high morbidity and mortality under immunosuppression and may even result in rejection. Here, we reviewed medical records of 66 patients in order to determine the outcome of management approach for asymptomatic gallstones in renal transplantation patients. Methods: We retrospectively reviewed clinical courses of 66 cases of renal transplantation performed between 2000 and 2012 at Kosin University Gospel Hospital. Results: Among 66 cases, eight had gallstones before transplantation. Three of these cases had undergone previous cholecystectomy for symptomatic gallstones, one had a simultaneous laparoscopic cholecystectomy and renal transplantation, and four were observed by regular abdominal ultrasonography. One patient was found to have cholangitis, and endoscopic retrograde biliary drainage was performed, resulting in alleviation of symptoms. Among 58 cases without preoperative gallstones, three developed gallstones after transplantation. One patient had cholecystitis, and the symptoms subsided after conservative treatment. Conclusions: For patients with asymptomatic gallstones who are awaiting renal transplantation, expectant management should be considered.