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PubMed1995

Unusual presentation of an unusual type of choledochal cyst.

Type II choledochal cysts are rare congenital malformations of the biliary ductal system. We report a case in which a type II choledochal cyst presented as a mass in the porta hepatis without any demonstrable connection with the biliary tree. In the English literature, this is the first described case of this type in the absence of biliary tract malignancy. We hypothesize that the connection to the biliary tree was lost by either torsion of the cyst or fibrosis due to chronic inflammation.

Adult↗

PubMed1994

[Colangiocarcinoma on a choledochal cyst].

Patients with choledochal cysts have an increased incidence of biliary tract carcinoma. We report a case of adenocarcinoma arising in a type I choledochal cyst which was surgically treated by resection and biliodigestive anastomosis (hepaticojejunostomy).

Adenocarcinoma↗

PubMed1998

[Deteriorating choledochal cyst. A case].

Choledochal cysts have an increased risk of biliary tract malignancy, induced by reflux of pancreatic juice and anomalous pancreatobiliary ductal junction. We report one case of congenital choledochal cyst in a 68-year-old female whose malignancy was revealed by history of pain and jaundice. Pancreatico-duodenectomy was performed and the patient was alive 6 months later.

Aged↗

PubMedDate not supplied

Malignancy in choledochal cysts.

BACKGROUND/AIMS: Malignancy in choledochal cysts is a rare condition. This study presents our experience with this condition, with emphasis on the clinical presentation, management and outcome. METHODOLOGY: Subjects included 80 adults with choledochal cysts treated from January 1979 to December 1995. Of these patients, eight were found to have malignancy in the cyst and formed the basis of this study. RESULTS: Four patients had synchronous and four had metachronous carcinoma lesions arising in the choledochal cyst. The clinical presentations were: biliary tract infection in 5 patients, gastric outlet obstruction in two and right upper quadrant pain and body weight loss in one. Operations for bile duct malignancy included total excision in two patients, choledochotomy with T-tube drainage in two patients, gastrojejunostomy in two patients, percutaneous transhepatic biliary drainage and gastrojejunostomy in one patient and metastatic lymph node biopsy only in one. One patient died due to septic shock within 30 days of operation (operative mortality). Postoperative survival time ranged from 4 to 9 months with a mean of 6.2 months. One of these patients was still alive at the time of writing (7 months). CONCLUSIONS: The frequency of malignancy in the choledochal cysts was 10% in our series. Malignancy in the choledochal cyst should be highly suspected in patients with cholangitis symptoms, body weight loss and anemia. Prognosis in this disease entity is poor.

Adenocarcinoma↗

PubMedDate not supplied

Malignancy in choledochal cysts.

BACKGROUND/AIMS: Malignancy in choledochal cysts is a rare condition. This study presents our experience with this condition, with emphasis on the clinical presentation, management and outcome. METHODOLOGY: Subjects included 80 adults with choledochal cysts treated from January 1979 to December 1995. Of these patients, 8 were found to have malignancy in the cyst and formed the basis of this study. RESULTS: Four patients had synchronous and 4 had metachronous carcinoma lesions arising in the choledochal cyst. The clinical presentation was: biliary tract infection in 5 patients, gastric outlet obstruction in 2 and right upper quadrant pain and body weight loss in 1. Operations for bile duct malignancy included total excision in 2 patients, choledochotomy with T-tube drainage in 2 patients, gastrojejunostomy in 2 patients, percutaneous transhepatic biliary drainage and gastrojejunostomy in 1 patient and metastatic lymph node biopsy only in 1. One patient died due to septic shock within 30 days of the operation (operative mortality). Postoperative survival time ranged from 4-13 months with a mean of 6.2 months. CONCLUSIONS: The frequency of malignancy in the choledochal cysts was 10% in the present series. Malignancy in the choledochal cyst should be highly suspected in patients with cholangitis symptoms, body weight loss and anemia. Prognosis in this disease entity is poor.

Adolescent↗

PubMed2003

[Indications of transduodenal papillotomy for choledochal cyst].

UNLABELLED: The Choledochal Cyst is a dilatation of the biliary tract whose etiology is still under debate. OBJECTIVE: This paper analyzes the cases of our group an contributes on the indications of transduodenal papilotomy in the surgical treatment of this process. MATERIAL AND METHODS: Seven cases diagnosed as choledochal cysts are presented with mean age between 3 and 10 years (5 females and 2 males). Six cases were presented with abdominal pain, four with jaundice, three presented with biliary stones and one cases was a casual discovery during an US examination. In all cases US and magnetic resonance cholangiopancreatography (MRCP) were performed as preoperative work-up. In one case an endoscopic retrograde cholangiopancreatography (ERCP) was made. In all cases intraoperative cholangiography was carried out. The surgical treatment was quistectomy and hepatico yeyunostomy Roux-Y in four cases; in one of them a transduodenal papilotomy was added. Cholecystectomy and transduodenal papilotomy was made in three cases, two of them presented choledocholithiasys. RESULTS: All patients are symptoms free after a mean follow up period of 2 years and 3 months (range: 1 year and 6 months to 4 years and 10 months). In one case persists dilated choledochal distal stump on MRCP.

Child↗

PubMedDate not supplied

[Anomalous choledocho-pancretic ductal junction in a choledochal cyst--a case report].

Choledochal cysts are rare congenital anomalies, mostly detected in adults. Pathogenesis of these cysts seems to be in anomalous junction between pancreatic and common bile duct, above the papillary sphincter and outside of the duodenal wall. The absence of the sphincter above the junction is followed by reflux of the pancreatic juice into the bile duct leading to dilatation and fibrous changes of bile duct wall. A 38-year-old female is presented in whom a choledochal cyst was found 11 years earlier, during the operation performed for obstructive jaundice, when cystojejunostomy with Roux-en Y Jejunal limb was carried out. In February 1990, she was admitted to our Institution forjaundice and biliary colic. The patient was reoperated. Operative cholangiography showed an anomalous pancreatobiliary junction, choledochal cyst, dilated cystic duct and moderate dilatation of intrahepatic bile ducts. Cholecystectomy, desanastomosis with partial excision of choledochal cyst, and retrocolic choledochojejunostomy with the same Roux-en-Y jejunal limb were performed. Total excision of choledochal cyst was too risky due to chronic inflammatory changes in the hepatoduodenal ligament. Postoperative recovery was uneventful and the patient remained symptom-free so far.

Adult↗

PubMed2003

Choledochal cysts and multilocular cysts of the pancreas.

Choledochal cyst is a rare congenital anomaly of the biliary system that may be associated with other abnormalities of the hepatobiliary tract. We report a case of an 11-year-old boy in whom the preoperative evaluation revealed a choledochal cyst and intraoperative cholangiopancreatography showed a cystic mass in the pancreas. Examination of the choledocho-pancreatico-duodenectomy specimen showed a multilocular cyst in the pancreas in addition to a segmental dilation of the common bile duct. The findings in our case adds pancreatic cyst to the spectrum of abnormalities associated with choledochal cyst and may also support the theory that choledochal cyst is the predominant abnormality in a widespread spectrum of the pancreatobiliary duct dysplasia.

Child↗

PubMed2005

Use of magnetic resonance cholangiopancreatography to diagnose neonatal congenital choledochal cyst.

Antenatal identification of choledochal cysts has become increasingly common with advances in prenatal ultrasonography. However, the antenatal diagnosis needs to be confirmed postnatally and a preoperative radiologic evaluation of the cyst prior to complete resection is required. There have been few reports of the application of magnetic resonance cholangiopancreatography in neonates, or in neonates with large choledochal cysts. We describe a neonate with a progressively growing giant choledochal cyst, which was initially detected at 28 weeks' gestation. After delivery, the baby did not feed well and suffered from jaundice and frequent postprandial bilious vomiting. At the age of 11 days, magnetic resonance cholangiopancreatography was used to confirm the prenatal diagnosis and provide a thorough preoperative evaluation. He underwent early resection of the cyst at 15 days of age and recovered uneventfully. Magnetic resonance cholangiopancreatography is an alternative diagnostic method for confirming the antenatal diagnosis of large choledochal cysts in neonates. It may also provide useful diagnostic information for preoperative evaluation.

Cholangiopancreatography, Magnetic Resonance↗

PubMed2001

Sphincter of Oddi dysfunction associated with choledochal cyst.

The pathophysiology of choledochal cysts remains unclear, although an association with anomalous pancreato-biliary junction and the reflux of pancreatic enzymes into the biliary tree is known. Sphincter of Oddi (SO) manometry was performed in three patients with choledochal cysts. All patients exhibited an elevated basal pressure diagnostic of sphincter of Oddi dysfunction. Two patients exhibited anomalous pancreato-biliary junction. This report suggests an association between the choledochal cyst and sphincter of Oddi dysfunction, and may suggest that SO dysfunction plays a role in choledochal cyst formation.

Adult↗

PubMed2005

Bile infection contributes to intrahepatic calculi formation after excision of choledochal cysts.

Intrahepatic calculi complicate choledochal cysts in 7-8% of patients. Although congenital stenoses and dilatation of the intrahepatic bile ducts are considered responsible for calculi formation, intrahepatic calculi are usually formed after cyst excision. In this study, bile specimens from patients with choledochal cysts were cultured for bacteria. Results were retrospectively analyzed among the following groups: the primary excision group, consisting of 97 patients undergoing cyst excision as a primary treatment (mean age 5.0 years), the internal drainage group, consisting of 13 patients who had previous cyst-enterostomy at cyst excision (mean age 20.2 years); and the hepatolithiasis group, consisting of 12 patients with postoperative hepatolithiasis (mean age 24.2 years). Bacteria were present in the bile of 10 patients (76.9%) in the internal drainage group and in all patients (100%) in the hepatolithiasis group, but present in only 17 patients (17.5%) in the primary excision group (p<0.01). Polymicrobial infection with Gram-negative enterobacteria such as Escherichia coli and Klebsiella species was predominant in the internal drainage and hepatolithiasis groups, while nonenteric bacteria were found in the primary excision group. Bile infection through bilioenterostomy may play an important role in intrahepatic calculus formation after excision of a choledochal cyst.

Adolescent↗

PubMed2005

Emergent biliary drainage for choledochal cyst.

Two patients with choledochal cyst who needed an emergent biliary drainage are presented. Case 1: Spontaneous rupture of the cyst was demonstrated by hepatobiliary scintigram in a 21-month-old girl and an emergent T-tube drainage relieved her symptom. Acute obstruction by protein plugs was considered to have caused rapid expansion of the cyst, which resulted in rupture eventually. Case 2: Marked hypoproteinemia was demonstrated in a 29-month-old boy with choledochal cyst. Bile drainage through Foley catheter promptly made the serum protein level elevated above normal range. Portal hypertension due to severely dilated cyst may have increased ascites amount, which caused extracellular fluid shift and protein to be lost into ascites. In both cases the excision of the extrahepatic bile duct and hepaticojejunostomy was carried out successfully later. Treatment of 20 pediatric cases in our institute and the literature were reviewed. Bile drainage would be safer in emergency condition even though it has been suggested that reconstructive surgery may be tolerable. Definitive surgery should be regarded as a procedure with some risk of postoperative complications because asymptomatic cases operated electively had serious ones. Treatment strategy of patients with choledochal cyst is not straightforward and should be arranged based upon their conditions.

Adult↗

PubMed1991

Occult carcinoma in an adult choledochal cyst.

The complications of choledochal cyst are avoidable if diagnosed early, and adequate resection undertaken. This case report describes the long history of right subcostal pain in a young man of 26 who had a squamous carcinoma in a choledochal cyst diagnosed after serial section of the excised cyst. Subsequent resection of the head of the pancreas showed histological residual tumour from which he died 4 months later. This case illustrates the need for complete early excision of a choledochal cyst to prevent this complication.

Adenocarcinoma↗

PubMed1990

Complications of choledochal cysts in adulthood.

Choledochal cyst is a well-recognised entity, presenting primarily in infants and young children. Where symptoms are delayed until adulthood, associated hepatobiliary pathology may complicate the presentation. These problems may be aggravated by previous treatment with bypass surgery rather than resection. We report seven cases from our recent experience presenting with complications in adulthood. These included cholangitis, hepatic abscess, pancreatitis and malignancy within the cyst. Two patients presented during pregnancy. These complications and their implications for management are discussed.

Adenoma, Bile Duct↗

PubMedDate not supplied

[Choledochal cysts in the adult patient. ].

INTRODUCTION: Choledochal cysts are a rare anomaly of the biliary system; only 20-30% are diagnosed in adults. The etiology remains uncertain; however, many patients with this pathology have had an anomalous pancreatobiliary junction. AIM: To evaluate recent trends regarding diagnosis and treatment of choledochal cysts. MATERIALS AND METHODS: Review of the world literature was performed on the matter of choledochal cysts. Incidence, signs and symptoms at presentation, and diagnostic tools and therapeutics are discussed. Results of these authors are also reported. RESULTS: Estimated incidence of choledochal cyst disease varies according to population studied. It is reported mainly in children; however, an increasing number of adult patients have been diagnosed with the disease. Symptoms are often unspecific and laboratory evaluation may demonstrate no variation. Ultrasonography (US) and cholangiography are both effective in defining biliary dilatation, but endoscopic retrograde colangiopancreatography (ERCP) and recently magnetic resonance cholangiopancreatography (MRCP) have been evaluated and best visualize the pancreatobiliary junction. Treatment of choledochal cyst disease is surgical and does not depend on age of patient; nonetheless, reach patient should be properly evaluated. CONCLUSIONS: Although choledochal cysts are typically diagnosed in infancy or childhood, the surgeon should not exclude the diagnosis because the patient is an adult. Cyst excision with hepaticojejunostomy is the definitive treatment of choice; nevertheless, surgical strategy should be selected based on type of cyst and the patient.

Adult↗

PubMed1980

CT of choledochal cyst.

Ten preoperative cases of choledochal cyst and five patients who had had complications after surgical treatment for their choledochal cysts were studied to evaluate the accuracy of computed tomography (CT) in the diagnosis of this malformation and its complications. Choledochal cysts were accurately diagnosed by CT in seven and suggested in three of the 10 preoperative patients. CT provided the correct diagnosis in all six patients with intrahepatic involvement. The CT pattern of congenital dilatation of the intrahepatic ducts was strikingly different from that of an acquired obstructive one. CT was also effective in the postoperative follow-up patients. In addition, within this group of 15 patients, three choledochal cyst cancers were detected.

Adolescent↗

PubMedDate not supplied

Ectopic pancreas associated with choledochal cyst and multiseptate gallbladder.

Congenital choledochal cyst is a rarely seen malformation of childhood, particularly when associated with multiseptate gallbladder or ectopic pancreas. The current case represents a 15-day-old boy with jaundice. Ultrasonography suggested a cystic lesion, probably of the common bile duct. The patient underwent a total excision of type I choledochal cyst and gallbladder with Roux-en-Y anastomosis, and a wedge biopsy from the liver. Gross examination revealed multiple septa dividing the gallbladder into multiple compartments. The outer and inner surfaces of the choledochal cyst were unremarkable. Microscopically, the cyst wall was composed of dense fibrous tissue with a single layer of cubic to columnar cells constituting the overlying epithelium. Serial sections incidentally revealed ectopic pancreatic tissue lying along the cyst wall characterized by acini, islets, and ductal structures with endocrine cells reactive for chromogranin A. Septa dividing the gallbladder were composed of fibrotic stalks containing smooth muscle fibers. Areas of papillary hyperplasia and intestinal metaplasia of gallbladder epithelium were also noted. The liver biopsy specimen demonstrated the presence of intrahepatic bile ducts, subsequently confirmed by cytokeratin 7. To our knowledge, this case represents the 1st one associated with these 3 entities and only the 2nd choledochal cyst with ectopic pancreatic tissue in its wall.

Choledochal Cyst↗

PubMed1977

Operative procedures for choledochal cyst.

Fifty one operations for choledochal cyst were carried out upon 46 patients: 41 as a primary operation and 10 as reoperation. An excision of choledochal cyst with hepaticojejunostomy in Roux-en-Y fashion at the second laparotomy gave a favorable results in all patients who suffered recurrent symptoms after the previous anastomotic procedure. Comparing operation time, blood loss and operative results of the reoperations with those of the primary, it was concluded that all choledochal cyst should be excised at the first operation unless other reason avoiding excision exists. Hepatic portojejunostomy is recommended for choledochal cyst with an associated distal atresia and the hypoplastic hepatic duct.

Adolescent↗