Protein stone formation in an intrapancreatic remnant cyst after resection of a choledochal cyst.
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AIM: To explore the potential carcinogenicity of bile from congenital choledochal cyst (CCC) patients and the mechanism of the carcinogenesis in congenital choledochal cyst patients. METHODS: 20 bile samples from congenital choledochal cyst patients and 10 normal control bile samples were used for this study. The proliferative effect of bile was measured by using Methabenzthiazuron (MTT) assay; Cell cycle and apoptosis were analyzed by using flow cytometry (FCM), and the PGE(2) levels in the supernatant of cultured cholangiocarcinoma cells were quantitated by enzyme-linked immunoabsorbent assay (ELISA). RESULTS: CCC bile could significantly promote the proliferation of human cholangiocarcinoma QBC939 cells compared with normal bile (P=0.001) and negative control group (P=0.002), and the proliferative effect of CCC bile could be abolished by addition of cyclooxygenase-2 specific inhibitor celecoxib (20 microM). The QBC939 cells proliferative index was increased significantly after treated with 1 % bile from CCC patient (P=0.008) for 24 h, the percentage of S phase (29.48+/-3.27)% was increased remarkably (P<0.001) compared with normal bile (11.72+/-2.70) %, and the percentage of G0/G1 phase (54.19+/-9.46) % was decreased remarkably (P=0.042) compared with normal bile (69.16+/-10.88) %, however, bile from CCC patient had no significant influence on apoptosis of QBC939 cells (P=0.719). CONCLUSION: Bile from congenital choledochal cyst patients can promote the proliferation of human cholangiocarcinoma QBC939 cells via COX-2 and PGE(2) pathway.
The scintigraphic appearances of 12 surgically proven cases of choledochal cyst were retrospectively reviewed. In seven of 12 cases, radionuclide accumulated in the choledochal cyst (i.e., the dilated common bile duct) in less than 1 hr. In three additional cases, delayed accumulation (1-24 hr) within the cyst was seen. In two of the 12 cases, no ductal activity appeared and the diagnosis of choledochal cyst could not be made, although in one of these two cases delayed images were not obtained. Other frequent findings included delayed or nonvisualization of the gallbladder (11 of 12) and the appearance of prominent intrahepatic ducts (five of 12). We conclude that hepatobiliary scintigraphy is a noninvasive test useful in the diagnosis of choledochal cyst.
BACKGROUND: There are few studies that report on stenosis of the intrahepatic bile ducts associated with choledochal cysts. We investigated the presence and clinical significance of stenosis of the intrahepatic bile ducts associated with choledochal cysts. STUDY DESIGN: We examined intrahepatic bile ducts in patients with choledochal cysts using cholangiography (93 patients), endoscopy and direct observation during surgery (19 patients), and histologic examination (12 patients). RESULTS: Stenosis of the intrahepatic bile duct was present in 75 (80.6 percent) of 93 patients with choledochal cysts. Endoscopic and direct observation detected membranous stenosis, which consisted of a diaphragm, in 11 of 19 patients. Septal stenosis, which consisted of a bridge-like septum, was present in eight of 19 patients. Stenoses consisted of mucosal and fibromuscular layers. Intrahepatic calculi developed in eight patients with stenosis following resection of the choledochal cysts. CONCLUSIONS: Membranous or septal stenosis of the intrahepatic bile duct is a characteristic feature of choledochal cysts. Our findings suggest that these stenoses should be treated surgically because of the risk of intrahepatic calculi.
A classification scheme for choledochal cysts is outlined and their appearance on cholangiograms is illustrated. Choledochal cysts are uncommon anomalies of the biliary system and are probably congenital in origin. They are manifested by cystic dilatation of the extra- or intrahepatic biliary tree or both. The classification system described here divides choledochal cysts into one of five main types. The most common, which is manifested by cystic or focal segmental dilatation of the common bile duct or fusiform choledochal dilatation, accounts for 80-90% of cases.
Hepaticojejunostomy Roux-en-Y after excision of choledochal cyst is the treatment of choice for children with choledochal cyst, to prevent the risk of postoperative cholangitis, stone formation and malignancy. The author reports two children with previous biliary surgery for choledochal cyst, one with cystojejunostomy without cholecystectomy and the other with cholecystectomy alone. Two children were admitted to the Pediatric Surgical Unit, Ratchaburi Hospital, with the complaint of chronic abdominal pain. After investigation the two children had cyst excision and hepaticojejunostomy Roux-en-Y. After six and one year follow-up the patients remain asymptomatic. The aim of this report was to show the complication of two previous biliary surgeries for choledochal cyst and support total cyst excision combined with hepaticojejunostomy Roux-en-Y being the treatment of choice for choledochal cyst.
We report a case of 35-yr-old woman with early cancer in congenital choledochal cyst. She had a five-year history of intermittent right upper abdominal pain and intermittent jaundice. In this period she had a few abdominal ultrasonographies, but the cholelithiasis had not been found. Now, she was admitted to our hospital because she felt right upper abdominal pain with slight jaundice and subfebrile temperature four weeks ago. Abdominal ultrasonography showed enormous dilatation of the common bile duct, which was suspected as choledochal cyst. Computed tomography and endoscopic retrograde cholangiopancreatography revealed cystic dilatation of extrahepatic bile duct. An anomalous pancreaticobiliary junction was not found. The patient with congenital choledochal cyst was operated on. The excision of choledochal cyst was done with hepaticojejunostomy Roux-en-Y. There were no lymph nodes metastases. On the central part of choledochal cysts mucosa, it was shown a white plain area of thickness 0.3 cm and 0.8 cm in diameter. Histologically it was well-differentiated tubular adenocarcinoma, which was limited to the mucosa and which did not penetrate to other parts of the bile duct wall. From our knowledge, only small number cases of early cholangiocarcinoma in choledochal cyst were until now reported. Nearly eight years after the operation the patient feels very well, and has optimal working ability (Karnofsky 100%).
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OBJECTIVE: We report our experience using MR cholangiography and CT cholangiography in pediatric patients with choledochal cysts. SUBJECTS AND METHODS: Fourteen patients (two boys, 12 girls; mean age, 7.8 years) with either a preoperative diagnosis of choledochal cyst or a surgical finding of choledochal cyst underwent non-breath-hold MR cholangiography using T2-weighted fat-suppressed fast spin-echo sequences with a 1.5-T magnet, and CT cholangiography with IV infusion of meglumine iodoxamic acid. Radiologic findings were correlated with findings from surgery, operative cholangiography, or percutaneous transhepatic cholangiography. RESULTS: The biliary tree was visualized in all 14 patients with MR cholangiography and in 13 patients (92.9%) with CT cholangiography. In the 11 preoperative cases of choledochal cyst, MR cholangiography correctly showed all cysts and CT cholangiography showed 10 cysts (90.9%). The quality of images on CT cholangiography and MR cholangiography was comparable. The sensitivity of CT cholangiography and MR cholangiography in revealing intrahepatic stones was 83.3% and 66.7%, respectively; the specificity for both techniques was 100%. The rate of detecting the pancreatic duct and the common channel by CT cholangiography and MR cholangiography was 63.6% and 45.5% respectively. After surgery, CT cholangiography was superior to MR cholangiography in revealing the location of biliary-enteric anastomosis and the extent of anastomotic narrowing. CONCLUSION: Because non-breath-hold MR cholangiography is not invasive and does not use ionizing radiation and potentially toxic contrast agents, it is recommended as the imaging technique of choice in children with choledochal cysts. CT cholangiography can be considered as an adjunct after surgery and in patients in whom MR cholangiography is unsatisfactory.
A choledochal cyst is an uncommon anomaly of the biliary system requiring surgical intervention. A case of a choledochal cyst imaged by computed tomography following oral administration of cholangiography contrast material (Telepaque) is reported. Telepaque-enhanced computed tomography is an easy, noninvasive method to demonstrate pertinent preoperative anatomy in cases of choledochal cysts, and is especially useful in the pediatric patient.
A 20-year-old woman presented with dull ache in the abdomen. Ultrasonography and CT scan showed a solitary liver cyst. Roux-en-Y cystojejunostomy was done since there was bile in the cyst. Histology of the cyst wall revealed it to be a choledochal cyst. Solitary intrahepatic choledochal cyst and presentation in adult life are rare.
Based on a case report, we describe choledochal cysts in adults. Choledochal cysts are rare, and are often overlooked. The aetiology is unknown. The symptoms are pain or discomfort, episodes of jaundice, cholangitis and pancreatitis. Increased liver function tests and gallstone disease are common. There is an increased risk of malignant tumours in the cysts and adjacent organs. Ultrasonography, computer tomography, endoscopic retrograde cholangiopancreaticography, percutaneous cholangiography and magnetic resonance imaging with magnetic resonance cholangiopancreaticography are relevant diagnostic tools. Extrahepatic cysts should be operated with radical excision, with Roux-Y hepaticojejunostomy or other reconstructions allowing later diagnostic and therapeutic access to the bile ducts. Long term results are good.
Although once considered rare, choledochal cysts now are extensively reported on and have an official anatomic classification. The authors report a case of a 4-year-old girl whom on routine abdominal ultrasound as follow-up for vesicoureteral reflux was found to have a choledochal cyst. Follow-up hepatobiliary scan together with the ultrasound findings was consistent with a rare type II choledochal cyst. On laparoscopy, a type II choledochal cyst was confirmed on dissection and amenable to laparoscopic excision. Intraoperative cholangiogram after excision showed normal remaining biliary anatomy. The patient was discharged the following day without delayed complications on follow-up visits. This report suggests a role for laparoscopic surgery in the definitive management of selected cases of choledochal cystic disease. (Included is relevant radiological documentation, illustration of technique, and review of the literature.)
PURPOSE: This retrospective study was designed to classify choledochal cysts on the basis of the findings of hepatobiliary scintigraphy. METHODS: Twenty-one patients with choledochal cysts (15 female, 6 male; mean age, 20 years) proved on the findings of endoscopic retrograde cholangiopancreatography (ERCP) or surgery and histopathologic analysis were included in the study. Two nuclear medicine physicians, blinded with regard to cholangiographic and operative details, were asked to review and to classify the type of choledochal cyst seen on the hepatobiliary scan. Later, scintigraphic results were compared with ERCP and surgical findings for a reference standard. RESULTS: The findings of hepatobiliary scintigraphy correlated with ERCP and surgical findings in 18 of 21 cases (86%). Scintiscans correctly identified all type 1 cysts (12/12). The sensitivity of scintigraphy in diagnosing type 4 cysts was 66% (6 of 9 cases). It underestimated the intrahepatic extent of disease in type 4a biliary cysts (37%). CONCLUSION: This study illustrates the utility of hepatobiliary scintigraphy in diagnosing type 1 and 4 choledochal cysts.
We report herein a rare case of choledochal cyst diagnosed at 14 weeks gestation and treated with percutaneous transhepatic drainage until postpartum. A 26-year-old primigravid woman at 14 weeks gestation presented with epigastric pain, slight fever, and nausea of 3 days duration. Abdominal ultrasonography revealed a 6-cm-diameter cystic mass between the porta hepatis and the pancreas head, which was diagnosed as a type I choledochal cyst. At 18 weeks of gestation, her upper abdominal pain became severe, and the size of the choledochal cyst increased to 12 cm in diameter. Laboratory data revealed a biliary obstruction. Percutaneous transhepatic drainage was performed immediately. She delivered a healthy male baby by elective cesarean section at 37 weeks of gestation. At 6 weeks postpartum, the patient underwent surgical excision of the choledochal cyst, cholecystectomy, and formation of a Roux-en-Y hepaticojejunostomy. Although choledochal cysts are rare during pregnancy, obstetricians should be familiar with the condition to ensure prompt diagnosis and adequate definitive management, given that the implications of missed or delayed diagnosis may be detrimental to both mother and fetus.
A case of cholangiocarcinoma arising in an unresected choledochal cyst in adulthood is presented. Although typically diagnosed in pediatric population, as many as 20% to 30% of choledochal cysts can be discovered in adulthood. Unresected choledochal cyst is clearly associated with increased risk of cholangiocarcinoma. Proper surgical treatment includes cyst resection and bilioenteric anastomosis. Asymptomatic patients with choledochal cyst previously treated by biliary diversion without cyst resection present a challenging issue and should be considered for cyst resection. Association of choledochal cyst with pancreaticobiliary anomalies is reviewed.
OBJECTIVE: The purpose of this study was to determine whether MR cholangiopancreatography can accurately depict anomalous pancreaticobiliary ductal union in children with choledochal cysts. SUBJECTS AND METHODS: Twenty children (age range, 1 month-13 years; mean age, 4.6 years; all girls) who were diagnosed with choledochal cyst by sonography underwent MR cholangiopancreatography with a single-shot fast spin-echo sequence. The type of choledochal cyst and anomalous pancreaticobiliary ductal union were characterized on the basis of MR cholangiopancreatographic findings and were compared with the finding of intraoperative cholangiography. RESULTS: The type of choledochal cyst (type Ia, n = 4; type Ic, n = 7; type IVa, n = 7; type IVb, n = 2) determined on MR cholangiopancreatography correlated with that identified on intraoperative cholangiography in each patient. Anomalous pancreaticobiliary ductal union was detected by MR cholangiopancreatography and intraoperative cholangiography in 12 (60%) and 16 (80%) of 20 patients, respectively. The types of anomalous pancreaticobiliary ductal union as determined on MR cholangiopancreatography (type A, n = 2; type B, n = 7; type C, n = 3) were concordant with those of intraoperative cholangiography in 11 of 12 patients. In five of eight patients with choledochal cyst (type Ia, n = 1; type IVa, n = 5; type IVb, n = 2) in whom MR cholangiopancreatography could not depict anomalous pancreaticobiliary ductal union, anomalous pancreaticobiliary ductal union was documented on intraoperative cholangiography that was performed after choledochal cyst resection. CONCLUSION: MR cholangiopancreatography provides diagnostic information about anomalous pancreaticobiliary ductal union in children with choledochal cyst.
We report a very rare case of type I choledochal cyst associated with a polycystic kidney disease. A 48-year-old female had been dependent on hemodialysis for chronic renal failure due to polycystic kidney disease and was incidentally diagnosed to have a dilated common bile duct by an ultrasonography. An endoscopic retrograde cholangiopancreatography showed a spindle-shaped, dilated common bile duct (type I choledochal cyst) without visualization of the pancreatic duct. She underwent a resection of the choledochal cyst. Intraoperative cholangiography showed no reflux of contrast medium into the pancreatic duct. Amylase level of the aspirated bile from the bile duct was not elevated. In the case of choledochal cyst combined with renal fibropolycystic disease, pancreaticobiliary maljunction may not contribute to the etiology of choledochal cyst. In such cases, management of choledochal cyst is still controversial and requires further discussion.