1.Choledochal cyst associated the with anomalous union of pancreaticobiliary duct (AUPBD) has a more grave clinical course than choledochal cyst alone.
Hye Kyoung SONG ; Myung Hwan KIM ; Seung Jae MYUNG ; Sung Koo LEE ; Hong Ja KIM ; Kyo Sang YOO ; Dong Wan SEO ; Hyun Joo LEE ; Byeong Cheol LIM ; Young Il MIN
The Korean Journal of Internal Medicine 1999;14(2):1-8
OBJECTIVE: Since choledochal cyst is frequently associated with the anomalous union of pancreaticobiliary duct (AUPBD), AUPBD has been regarded to be the etiologic factor of choledochal cyst. However, the clinical significance of AUPBD an patients with choledochal cyst has not been clearly defined. Therefore, to clarify the significance of AUPBD in choledochal cyst patients, we compared the clinical features of patients with choledochal cyst according to the presence or absence of AUPBD. METHODS: Among 52 cases which were diagnosed as choledochal cyst out of 5,037 ERCP referrals between August 1990 and December 1996, we selected 44 cases, in which the pancreaticobiliary junction was clearly visualized on cholangio-pancreaticography. These cases were divided into AUPBD-present group (n = 28) and AUPBD-absent group (n = 16). Clinical features were compared between the two groups. Furthermore, in AUPBD-present group, clinical data were also analyzed according to Kimura's classification of AUPBD. RESULTS: In our study, AUPBD was associated with choledochal cyst in 28 (64%) cases. AUPBD was found only in type I and IV according to Todani's classification of choledochal cyst. There were no significant differences between the AUPBD-present group and the AUPBD-absent group in the incidence of gallstone disease, while the incidence of acute inflammation was 93% (26/28) in the AUPBD-absent group (p < 0.01). Carcinoma developed only in the AUOBD-present group (9/28, 32%) (p < 0.05). Pancreatic disorders (i.e. pancreatic stone, pancreatitis or pancreatic cancer) occurred in 12 of 28 cases in the AUPBD-present group (43%), while only in 1 of 16 cases in the AUPBD-absent group (6%) (p < 0.05). CONCLUSION: AUPBD associated with choledochal cyst may have implications not only as a possible etiologic factor but also as an important factor that may affect the clinical course, surgical planning and prognosis. In cases with choledochal cyst, we should make an effort to evaluate the presence of AUPBD.
Adolescence
;
Adult
;
Aged
;
Bile Ducts/abnormalities*
;
Calculi/complications
;
Cholangiography
;
Choledochal Cyst/radiography
;
Choledochal Cyst/pathology
;
Choledochal Cyst/complications*
;
Female
;
Human
;
Inflammation/complications
;
Male
;
Middle Age
;
Neoplasms/complications
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Pancreatic Ducts/radiography
;
Pancreatic Ducts/abnormalities*
;
Prognosis
2.Rhabdomyosarcoma of the common bile duct mimicking choledochal cyst: a rare cause of obstructive jaundice.
Hassan NAZMUL ; Islam RAFIQUL ; Fathema KANIZ ; Sayeed MAIMUNA ; Wahiduzzaman Mazumder MD ; Bazlul Karim ASM ; Pada Dey BISHNU
Chinese Journal of Contemporary Pediatrics 2020;22(12):1338-1343
Extrahepatic biliary tract tumors are rare and among them rhabdomyosarcoma is most common. Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor and is a very rare malignancy of the common bile duct in children. It usually presents as obstructive jaundice and/or pruritus. If there is no local invasion to the adjacent tissues, the radiological appearance of the tumor lesion is like a choledochal cyst. So the diagnosis is usually made at surgery or by preoperative biopsy. It is important to diagnose early and differentiate it from choledochal cyst and start treatment as early as possible for long time survival of the patient. This case report presented a case of a 10-year-old boy with recurrent onset of obstructive jaundice and fever preoperatively who was diagnosed as choledochal cyst and postoperatively as embryonal rhabdomyosarcoma of the common bile duct. After surgical resection and postoperative chemotherapy, the child had a good prognosis. So it is crucial to know that this rare tumor can mimic congenital choledochal cyst and it should be considered in the differential diagnosis of obstructive jaundice in children.
Child
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Choledochal Cyst
;
Common Bile Duct/pathology*
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Diagnosis, Differential
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Humans
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Jaundice, Obstructive/etiology*
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Male
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Rhabdomyosarcoma, Embryonal/diagnosis*
3.Relationship between Anomalous Pancreaticobiliary Ductal Union and Pathologic Inflammation of Bile Duct in Choledochal Cyst.
So Won PARK ; Hong KOH ; Jung Tak OH ; Seok Joo HAN ; Seung KIM
Pediatric Gastroenterology, Hepatology & Nutrition 2014;17(3):170-177
PURPOSE: Choledochal cyst is a cystic dilatation of common bile duct. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal union (APBDU) is thought to be a major etiology of choledochal cyst. In this study, we analyzed the clinical and anatomical characteristics and pathologies of patients diagnosed with choledochal cyst in a single institute for 25 years. METHODS: A total of 113 patients, diagnosed with choledochal cyst and who received an operation in Severance Children's Hospital from January 1988 to May 2013, were included. Medical records were reviewed, including clinical and demographic data, surgical procedures. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, and intraoperative cholangiography were used as diagnostic tools for evaluation and classification of choledochal cyst and the presence of anomalous pancreaticobiliary ductal union. Todani's classification, and relationship between APBDU and surgical pathology. RESULTS: Among 113 patients, 77 patients (68.1%) presented symptoms such as hepatitis, pancreatitis and/or cholecystitis. Eighty three patients (73.5%) had APBDU, and 94 patients (83.2%) showed inflammatory pathologic changes. APBDU, pathologic inflammation, and serological abnormalities such as hepatitis or pancreatitis showed a statistically significant correlation to one another. CONCLUSION: APBDU is thought to be one of the etiologic factors of choledochal cyst. It is related to the inflammatory changes in bile duct that can lead to the cystic dilatation.
Bile Ducts*
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Child
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Cholangiography
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Cholangiopancreatography, Magnetic Resonance
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Cholecystitis
;
Choledochal Cyst*
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Classification
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Common Bile Duct
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Dilatation
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Hepatitis
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Humans
;
Inflammation*
;
Medical Records
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Pancreatitis
;
Pathology
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Pathology, Surgical
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Ultrasonography
4.A Case of Early Bile Duct Cancer Arising from Villous Adenoma in Choledochal Cyst.
Tae Seung LEE ; Hae Kyung KIM ; Hong Min AHN ; Uh Joo LEE ; Young Chul CHOI ; Byung Min JOHN ; Tae Il PARK ; Jin Hoi KOO
The Korean Journal of Gastroenterology 2009;54(1):55-59
Choledochal cyst is an uncommon premalignant anomaly. The morphology and pathogenesis of the premalignant lesion of cholangiocarcinoma arising from the choledochal cyst has not been well described. Herein, we report a rare case of bile duct adenoma arising from choledochal cyst with anomalous union of pancreaticobiliary duct (AUPBD). 50-year-old woman was admitted to our hospital with the complaint of epigastric pain. She had received common bile duct (CBD) exploration and choledocholithotomy and cholecystectomy 3 months earlier under the diagnosis of multiple CBD stones. Intraoperalive cholangiogram was not remarkable except CBD dilatation at that time. Endoscopic retrograde cholangiopancreatography revealed choledochal cyst with AUPBD and round filling defect which disappeared easily on the balloon cholaniogram. On magnetic resonance cholangiopancreatography, the filling defect was confirmed as 2 cm polypoid mass attached to the distal bile duct wall. At laparotomy, a soft whitish mass was palpable on the lower CBD. On histological examination, adenoma with focal carcinoma change arising from choledochal cyst was diagnosed.
Adenoma, Villous/*diagnosis/pathology/radiography
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Bile Duct Neoplasms/*diagnosis/pathology/radiography
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Cholangiopancreatography, Magnetic Resonance
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Choledochal Cyst/*radiography/secretion/surgery
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Female
;
Humans
;
Middle Aged
;
Tomography, X-Ray Computed
5.MRI Findings of Intrinsic and Extrinsic Duodenal Abnormalities and Variations.
Ebru DUSUNCELI ATMAN ; Ayse ERDEN ; Evren USTUNER ; Caglar UZUN ; Mehmet BEKTAS
Korean Journal of Radiology 2015;16(6):1240-1252
This pictorial review aims to illustrate the magnetic resonance imaging (MRI) findings and presentation patterns of anatomical variations and various benign and malignant pathologies of the duodenum, including sphincter contraction, major papilla variation, prominent papilla, diverticulum, annular pancreas, duplication cysts, choledochocele, duodenal wall thickening secondary to acute pancreatitis, postbulbar stenosis, celiac disease, fistula, choledochoduodenostomy, external compression, polyps, Peutz-Jeghers syndrome, ampullary carcinoma and adenocarcinoma. MRI is a useful imaging tool for demonstrating duodenal pathology and its anatomic relationships with adjacent organs, which is critical for establishing correct diagnosis and planning appropriate treatment, especially for surgery.
Ampulla of Vater/anatomy & histology/radiography
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Choledochal Cyst/pathology/radiography
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Diverticulum/radiography
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Duodenal Diseases/pathology/*radiography
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Duodenum/*anatomy & histology/radiography
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Humans
;
*Magnetic Resonance Imaging
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Pancreas/abnormalities/anatomy & histology/radiography
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Pancreatic Diseases/radiography
6.A Case of Intrahepatic Cholangiocarcinoma Associated with Type IV Choledochal Cyst.
Suk Hun KIM ; Hyung Wook KIM ; Dae Hwan KANG ; Min Dae KIM ; Jin Ho LEE ; Jae Hyung LEE ; Bong Gap KIM ; Jong Hwan PARK
The Korean Journal of Gastroenterology 2012;60(2):123-127
Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.
Adult
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Bile Duct Neoplasms/*diagnosis/etiology/pathology
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*Bile Ducts, Intrahepatic
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Cholangiocarcinoma/*diagnosis/etiology/pathology
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Cholangiopancreatography, Endoscopic Retrograde
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Cholangiopancreatography, Magnetic Resonance
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Choledochal Cyst/complications/*diagnosis
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Female
;
Humans
;
Positron-Emission Tomography and Computed Tomography
;
Tomography, X-Ray Computed
7.Value of Manganese-Enhanced T1- and T2-Weighted MR Cholangiography for Differentiating Cystic Parenchymal Lesions from Cystic Abnormalities which Communicate with Bile Ducts.
Mi Suk PARK ; Jeong Sik YU ; Jae Hee LEE ; Ki Whang KIM
Yonsei Medical Journal 2007;48(6):1072-1074
We present a case report to show how manganese-enhanced T1- and T2-weighted MR cholangiography could differentiate cystic parenchymal lesions from cystic abnormalities which communicate with the bile ducts.
Bile Ducts/*pathology
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Cholangiopancreatography, Magnetic Resonance/*methods
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Choledochal Cyst/diagnosis
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Contrast Media/chemistry
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Cysts/diagnosis
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Diagnosis, Differential
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Humans
;
Liver/*pathology
;
Male
;
Manganese/*chemistry
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Middle Aged
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Reproducibility of Results
;
Sensitivity and Specificity
8.Unilocular Extrahepatic Biliary Cystadenoma Mimicking Choledochal Cyst: A Case Report.
Ju Hyun PARK ; Dong Ho LEE ; Hyoung Jung KIM ; Young Tae KO ; Joo Won LIM ; Moon Ho YANG
Korean Journal of Radiology 2004;5(4):287-290
We report here on a case of extrahepatic biliary cystadenoma arising from the common hepatic duct. A 42-year-old woman was evaluated by us to find the cause of her jaundice. Ultrasonography and CT showed a cystic dilatation of the common hepatic duct and also marked dilatation of the intrahepatic duct. Direct cholangiography demonstrated a large filling defect between the left hepatic duct and the common hepatic duct; dilatation of the intrahepatic duct was also demonstrated. Following excision of the cystic mass, it was pathologically confirmed as a unilocular biliary mucinous cystadenoma arising from the common hepatic duct.
Adult
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Bile Duct Neoplasms/*diagnosis
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Cholangiopancreatography, Magnetic Resonance
;
Choledochal Cyst/*diagnosis
;
Cystadenoma/*diagnosis
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Diagnosis, Differential
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Female
;
Hepatic Duct, Common/*pathology/radiography/ultrasonography
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Humans
;
Tomography, X-Ray Computed
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Ultrasonography, Interventional
9.A Case of Choledochal Cyst Complicated by Liver Cirrhosis on Pathology in a 20-Month-Old-Girl.
Sun Hwan BAE ; Sung Yun CHOI ; Tae Seok LEE ; Ho Jeong LEE
Korean Journal of Pediatrics 2005;48(1):104-107
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.
Alkaline Phosphatase
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Biliary Tract
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Biopsy
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Child
;
Cholecystectomy
;
Choledochal Cyst*
;
Choledochostomy
;
Cholestasis
;
Classification
;
Diagnostic Imaging
;
Dilatation
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Female
;
Fibrosis
;
Hepatomegaly
;
Hospitals, General
;
Humans
;
Infant
;
Liver Cirrhosis*
;
Liver*
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Pathology*
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Physical Examination
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Spleen
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Splenomegaly
10.Management Strategy for Congenital Choledochal Cyst with Co-existing Intrahepatic Dilation and Aberrant Bile Duct As Well As Other Complicated Biliary Anomalies.
Qian DONG ; Buxian JIANG ; Hong ZHANG ; Zhong JIANG ; Hongting LU ; Chuanmin YANG ; Yu CHENG ; Xiwei HAO
Yonsei Medical Journal 2006;47(6):826-832
The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- xisting intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.
Tomography, X-Ray Computed
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Postoperative Complications/ultrasonography
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Male
;
Liver Diseases/complications/*radiography/surgery
;
Infant, Newborn
;
Infant
;
Humans
;
Female
;
Choledochal Cyst/complications/*radiography/surgery
;
Cholangiography
;
Child, Preschool
;
Child
;
Bile Ducts/*abnormalities/pathology/surgery