• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.104-107
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• 2005

Choledochal cyst is considered to be congenital anomalies of the biliary tract, characterized by varying degrees of cystic dilatation at various segments of the biliary tract. A 20-month-old girl was admitted to Eul-Ji general hospital because of abdominal distension. Physical examination revealed marked splenomegaly and hepatomegaly with nodular surface and hard consistency. Laboratory examination showed elevated transaminase level, alkaline phosphatase level and gamma glutamyltranspeptidase level without evidence of cholestasis. Diagnostic imaging study revealed choledochal cyst with Todani classification type 1. Cholecystectomy and Roux-en-Y choledochojejunostomy was performed, and wedge liver biopsy showed diffuse periportal fibrosis with cirrhotic change and ductular proliferation in the portal area. After operation, hepatosplenomegaly and abnormal laboratory examinations improved rapidly, and in 9 months, the liver and spleen became not palpable. We experienced a case of choledochal cyst complicated by liver cirrhosis on pathology in a 20 month-old girl, and removal of choledochal cyst improved clinical manifestations rapidly.

• 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.

• 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.

• The Korean Journal of Nuclear Medicine
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• v.20 no.2
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• pp.105-107
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• 1986

It is well known that $^{99m}Tc-DISIDA$ hepatobilliary scan is a noninvasive and accurate method for detecting choledochal cyst. In the case we are reporting, $^{99m}Tc-DISIDA$ scan of a 17 year old female patient showed the characteristic pattern of a choledochal cyst, confirmed by surgery.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.186-191
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• 2002

Choledochal cyst is a congenital anomaly with classic triad of abdominal pain, jaundice and right upper abdominal mass. Bile peritonitis caused by cyst rupture is relatively not rare in infancy. The mechanism of rupture must be epithelial irritation of the biliary tract by refluxed pancreatic juice caused by pancreatico-biliary malunion associated with mural immaturity in infancy, rather than an abnormal rise in ductal pressure or congenital mural weakness at a certain point. We experienced a case of bile peritonitis caused by spontanenous rupture of choledochal cyst in a 10-month-old girl presented with abdominal distension, persistent fever, diarrhea, irritability and intractable ascites. She was presumed as having bile peritonitis by bile colored ascitic fluid with elevated bilirubin level and diagnosis was made by $^{99m}Tc$ DISIDA hepatobiliary scan showing extrahepatic biliary leak. The perforated cyst was surgically removed and the biliary tree was reconstructed with a Roux-en-Y hepaticojejunostomy.

• Korean Journal of Radiology
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• v.23 no.2
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• pp.271-279
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• 2022

Objective: To compare the clinical and radiologic findings between perforated and non-perforated choledochal cysts in children. Materials and Methods: Fourteen patients (mean age ± standard deviation, 1.7 ± 1.2 years) with perforated choledochal cysts (perforated group) and 204 patients (3.6 ± 3.8 years) with non-perforated choledochal cysts (non-perforated group) were included between 2000 and 2019. All patients underwent choledochal cyst excision after ultrasound, CT, or MR cholangiopancreatography. Relevant data including demographics, clinical symptoms, laboratory findings, imaging findings, and outcomes were analyzed. Statistical differences were compared using the Mann-Whitney U test and Fisher's exact test. Results: Choledochal cyst perforation occurred only in children under the age of 4 years. Acute symptoms, including fever (p < 0.001), were more common in the perforated group than in the non-perforated group. High levels of white blood cells (p = 0.004), C-reactive protein (p < 0.001), and serum amylase (p = 0.002), and low levels of albumin (p < 0.001) were significantly associated with the perforated group. All 14 patients with perforated choledochal cysts had ascites, whereas only 16% (33/204) of patients in the non-perforated group had ascites (p < 0.001). In the subgroup of patients who had ascites, a large amount of ascites (p = 0.001), increase in the amount of ascites in a short time (p < 0.001), complex ascites (p < 0.001), and perihepatic pseudocysts (p < 0.001) were more common in the perforated group than in the non-perforated group. Conclusion: Children with perforated choledochal cysts have characteristic clinical and radiologic findings compared to those with non-perforated choledochal cysts. In young children with choledochal cysts, perforation should be differentiated in cases with acute symptoms, laboratory abnormalities, and characteristic ascites findings.

• Advances in pediatric surgery
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• v.15 no.1
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• pp.11-17
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• 2009

Choledochal cyst is a congenital dilatation of the bile duct. Intrahepatic bile duct dilatation of type IVa by Todani's classification at the time of diagnosis resolved spontaneously after cyst excision and hepaticojejunostomy in many cases. It should be distinguished from the true cystic dilatation of the intrahepatic ducts, which tends to persist, albeit after some regression. We therefore studied postoperative intrahepatic duct dilatation changes in choledochal cyst. A total of seventy-six choledochal cysts were managed at the Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center from May 1995 to December 2005. The ratio of males to females was 1:2.8. Preoperative radiologic diagnosis by Todani's classification was Type I (n=52, 68.4 %), II (n=1, 1.3 %), IVa (n=23, 30.3 %). Among fifty-five patients with intrahepatic bile duct dilatation we were able to follow up forty-eight by ultrasonography. Twenty-two patients were type IVa, and twenty-six patients were type I and showed intrahepatic duct dilatation. Mean follow-up duration was 35.3 months (9~105 months). Complete regression of dilated intrahepatic duct was observed in fifteen patients of type IVa and twenty-four patients of type I. Incomplete regression of dilated intrahepatic duct was observed in six patients in type IVa and two patients in type I. Only one patient in type IVa showed no change in ductal dilatation during a follow-up period of 15 months. We conclude that true type IVa is much less frequent than what was diagnosed preoperatively by imaging study. Therefore in type IVa patients who are diagnosed preoperatively the decision to perform liver resection should be carefully considered. Postoperative long term follow up of choledochal cyst with intrahepatic bile duct dilation is needed.

• 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.

• The Korean Journal of Nuclear Medicine
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• v.27 no.1
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• pp.71-80
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• 1993

In order to evaluate the scintigraphic features of choledochal cyst and these diagnostic value, authors investigated the findings of fourteen patients with choledochal cyst undergone hepatobiliary scan with $^{99m}Tc$-DISIDA before surgery. Five cases demonstrated the decreased hepatic uptake at 5-minute image of which four cases revealed severe jaundice. Seven cases demonstrated visualization of the cystic dilated common bile duct within 1 hour after injection. Two cases showed the cyst activity between 1 and 12 hours, but the cyst activity was not visible in five cases. Nonvisualization of the gall bladder was noted in ten cases, while four cases demonstrated visualization of the gall bladder within 1 hour. The time of visualization of gut activity was variably delayed. The intestinal activity was found in three cases within 1 hour and appeared in three cases between 1 and 2 hours and eight cases showed no visible gut activity. In four cases, intrahepatic ductal prominence was visible on the scintigram. Seven cases showed early and persistent accumulation of tracer in the common bile duct. Three cases showed persistent photon-deficient area in the gall bladder region. Two cases showed early photon-deficient area around gall bladder region with progressive accumulation of tracer in the same region. Two cases showed no evidence of activity in the biliary tract but noted late excretion into the small intestine. We concluded that hepatobiliary scan using $^{99m}Tc$-DISIDA is a noninvasive test useful in the evaluation and the diagnosis of choledochal cyst.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.3
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• pp.259-265
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• 2020

Purpose: Perforation of choledochal cyst (CC) is a relatively rare clinical presentation in pediatric populations and difficult to predict preoperatively. We assess the clinical implications by comparing clinical parameters based on a single-center experience between perforated and nonperforated CC to facilitate the appropriate management for future interventions. Methods: A total of 92 cases of CC in pediatric patients (aged <18 years) who received surgical management between January 2003 and December 2018 at a Pusan National University Children's Hospital were reviewed. After screening the clinical features of perforated cases, we compared the demographic findings, clinical characteristics, and some laboratory results between the perforated and nonperforated groups. Results: Perforated CC was identified in 8 patients (8.7%), and nonperforated CC in 84 patients (91.3%). Perforation can be classified into three categories: free perforation of cyst (3 cases), pinpoint perforation of cyst (2 cases), and necrotic change of cyst (3 cases). CC perforation occurred significantly more commonly in patients aged <24 months. Clinically, the perforated group showed significantly higher frequency of fever and higher C-reactive protein (CRP) level during the initial visit. Conclusion: Perforation is more likely to be suspected in patients aged <24 months presenting together with fever and high CRP level in the initial visit. It is also necessary to keep in mind that it indicates not only a possibility of complicated disease status regardless of its association with stones but also a difficulty of applying a minimal invasive procedure and relatively increased length of hospital stay.