Choledochal cysts an overview sciencedirect topics. A choledochal cyst is a swelling widening or dilatation of the bile ducts. Oct 28, 2015 choledochal cyst is a congenital dilatation of part or whole of the bile duct. Diagnosis relies on the exclusion of other conditions e. To study the age and sex incidence of choledochal cyst. The choledochal cysts were classified as todani and coworkers type i in 20 patients, type ii in 2 patients, and type iv in 8 patients. Nov 10, 2016 biliary atresia ba and choledochal cyst cc are two wellknown causes of obstructive jaundice in neonates and young infants and have similar clinical presentation. A choledochal cyst is a cyst hollow outpouching of the bile ducts. Not actually a cyst but a dilation of common bile duct which may secondarily obstruct other biliary ducts or the duodenum 2 8% develop biliary tract carcinoma 20x normal risk at mean age 34 years, lower risk if surgery earlier in life age 10 years or less, carcinoma may develop within wall of cyst, within gallbladder or bile ducts.
Five had antenatal us revealing cystic abdominal masses. Request pdf liver histology in choledochal cyst pathological changes and response to surgery. The outer wall of the cyst contains almost exclusively duodenal mucosa while the inner wall may include duodenal or biliary epithelium. Primary epidermoid cyst of biliary duct presenting as. Dec 10, 2011 malignant change in association with choledochal cysts has been reported in pediatric cases, and it should therefore be suspected in any choledochal cyst appearing after infancy.
Introduction cyst dilatation thoughout the billiary tree approximately 1 in 100,000 1 in 150,000 more common in some asian countries1. Here we are reporting a case of type vi cdc with papillary gbc. What is a choledochal cyst and what is its appearance on an mrcp. The condition usually affects the part of the bile ducts outside.
A choledochal cyst is not just an aneurysmal dilatation of the common bile duct, but it is the most obvious manifestation of a more widespread pancreaticobiliary ductal dysplasia. Choledochal cysts are treated by surgical excision of the cyst with the formation of a rouxeny anastomosis hepaticojejunostomy choledochojejunostomy to the biliary duct. Spontaneous rupture of a choledochal cyst is a rare complication caroli disease view in chinese some confusion with extrahepatic choledochal cysts, one form of which can extend into the intrahepatic bile ducts type v choledochal cysts however, choledochal cysts are not a form of caroli disease. Choledochal cysts are congenital anomalies of the biliary tree, and they show dilatation of the intra and extra hepatic biliary tree.
Management of choledochal cyst with portal hypertension. Choledochal cysts in adults are uncommon, and cystic dilatation of the cystic duct type vi choledochal cyst is a unique occurrence. Characterised by a dilatation limited to the duodenal wall in the distal part. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in. To date, specific pathogenic and diagnostic criteria for. Cirrhotic changes in choledochal cyst in relevance to. As the epithelium of these cysts are prone to develop biliary intraepithelial neoplasia bin, onus must be placed on complete surgical excision and multiple cut sections of the histopathology specimen must be analyzed. Smooth muscle distribution patterns of choledochal cysts. They are considered congenital because they occur in fetuses and neonates. Protocol for the examination of specimens from patients with. Choledochal cyst occasionally causes liver fibrosis caused by obstructive jaundice. A case report of an unusual type of choledochal cyst with.
Regression of biliary cirrhosis following choledochal cyst. Early diagnosis and prompt treatment is necessary for a good outcome. Their origin may be related to an abnormal connection between the pancreatic duct and common bile duct and chronic reflux of pancreatic juice into the bile duct, resulting in irritation of the duct. Type vi choledochal cyst with gall bladder carcinoma bmj. Ba is a progressive obstructive cholangiopathy diagnosed in the newborn period and characterized by partial or complete lack of the extrahepatic bile ducts. Histopathologically, the type i, ii, and iv choledochal cysts are similar and consist of a wall of dense collagenous tissue with. However, because of an increased amount of routine health checkups and advancement of noninvasive hepatobiliary im aging, diagnosis of choledochal cyst in adults has increased. Analysis of choledochal cyst cases presenting to osmania general hospital over the period of january 2016november 2017. Choledochal cyst is a rare benign biliary disease mostly presenting during childhood. Choledochal cyst congenital anomaly involving cystic dilation of various ducts of the biliary tract. An isolated dilatation of the cystic duct type vi choledochal cyst cdc is extremely rare with only 21 cases reported in the world literature until now. Choledochal cysts have been classified into several different types depending on where. Choledochal cyst, liver biopsy,liver cirrhosis introduction choledochal cyst. Choledochal cysts are rare congenital or acquired cystic dilatations of the intra or extrahepatic bile ducts.
Choledochal cysts are more common in asian populations reported incidence of 1 in than in the western hemisphere, where the incidence is only 1 in 100,000 to 150,000 live births. Massive dilatation of the common bile duct resembling a choledochal cyst. Choledochal cysts classification, physiopathology, and. Choledochal cyst is a relatively rare disease in the west, and thus most cases have originated from asia. Although benign, cc can be associated with serious complications including malignant transformation, cholangitis, pancreatitis, and cholelithiasis. Type vi choledochal cysts can further be described based on morphology as fusiform more common and saccular. They are typically a surgical problem of infancy and childhood, but in nearly 20% of the patients the diagnosis is delayed until adulthood.
Adult presentation is rare and associated diseases and complications are common. Congenital dilatation of the extrahepatic biliary tract with or without dilatation of the intrahepatic biliary tract known as. Choledochal cysts cc are a rare congenital cystic dilation of the biliary tract, first described by vater and ezler in 1723. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in adults. Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree. In 1723, vater first described dilata tion of the common bile duct 1. The disease is mostly that of infancy and childhood with 80% being diagnosed in the first ten years of life. Congenital cystic lesions of the biliary tract include ductal plate malformations and choledochal cysts and can be recognized with characteristic imaging findings and basic knowledge of the embryologic development of the biliary tree. The choledochal cyst was mobilized and fixed with holding sutures. Liver fibrosis is more common and severe in infants than in children.
Choledochal cysts are classified into 5 types, based on site of the cyst or dilatation. Choledochal cyst childrens liver disease foundation. The management of choledochal cysts in the newborn springerlink. A singlecenter experience with 73 patients of adult. Clinical significance of intrapancreatic choledochal cyst excision in surgical management of type i choledochal cyst. Sometimes, a choledochal cyst is found when a child is having an abdominal ultrasounds scan for an unconnected reason like pain in the abdomen. Type iv a choledochal cyst with cystic duct cyst hpb oxford 2010. They are usually lined by bile duct epithelium, which may undergo intestinal and squamous metaplasia. Biliary tract cysts are a rare biliary malformative pathology, accounting for 1% of benign biliary diseases 1. Not actually a cyst but a dilation of common bile duct which may secondarily obstruct other biliary ducts or the duodenum. Choledochal cystsclassification, physiopathology, and.
Histopathology of synovial cysts of the spine chebib. Yotsuyanagi s 1936 contribution to aetiology and pathology of idiopathic cystic. This keeps bile from flowing well, which can cause liver problems. Cirrhosis develops more frequently with increasing age. Biliary atresia ba and choledochal cyst cc are two wellknown causes of obstructive jaundice in neonates and young infants and have similar clinical presentation. Choledochal cysts are rare, with an incidence of 1.
More severe symptoms, younger age and apbj are associated with higher degree of liver damage. Choledochal cysts are rare disease and of unknown etiology. However, various aspects of etiology and pathophysiology are not clear particularly with reference to the liver histology, bile composition, the pancreatobiliary junction, and the intracystic pressure. A large cyst,11 cm in diameter, was considered to represent a common hepatic cyst because it was in direct contact with the liver. Histology can play vital role in characterization, as often clinical findings and radiology can overlap. Pdf choledochal cysts are a congenital anomaly, and they show dilatation of the. There is a higher incidence of ccs in asian populations compared with other. Except portal fibrosis and central venous distension, all other pathological changes regress after surgery. First reported by vater and ezler 1 in 1723, choledochal cysts ccs are rare cystic dilations of the biliary tract. Madadisanjani o, wirth tc, kuebler jf, petersen c, ure bm.
Their diagnosis is difficult, particulary in adults. Choledochal cysts have been classified into several different types depending on where they are located and whether they can be seen as separate structures from the ducts diverticulumlike. Approximately 80% of cc are diagnosed in infants and young children within the first decade of life. Cystic dilation of biliary ducts, otherwise known as choledochal cyst, is an uncommon condition frequently present in infancy and childhood but increasingly diagnosed in adults.
Choledochal cyst and associated malignant tumors in adults. Contain 1 2 liters of bile, up to 15 cm in diameter. Choledochal cyst rupture with an intrahepatic pseudocyst mimicking hepatic mesenchymal hamartoma in an infant clinical imaging, vol. The term choledochal cyst can be broken down into the following. Choledochal cysts have been classified into several different types depending on where they are located and whether they can be seen as separate structures from the ducts diverticulum like. Choledochal cysts are abnormal dilatations of the biliary tree, generally believed to be congenital in origin. There is no report in the literature outlining the management of newborns with choledochal cysts, many of whom are asymptomatic. Sep 21, 20 choledochal cysts are now being diagnosed before birth on routine maternal sonography us. Correlation of intracystic pressure with cyst volume. Histology of synovial cysts of the spine shows varied types of cyst. The liver was hard and nodular and had the characteristic features of cirrhosis. Until now, no unifying etiologic theory exists for choledochal cysts, but most literature would conclude they are congenital bile duct anomalies. The tubes get bigger than normal dilate or pouches form on the tubes. Management of choledochal cyst with portal hypertension saluja s s, mishra p k, sharma b c, narang p abstract portal hypertension pht is a rare complication associated with choledochal cysts.
Our study details the diagnosis, treatment and outcome of six such children, four girls and two boys. Choledochal cysts are diagnosed in children and adults, although most 80% develop in children younger than 10 years. The condition usually affects the part of the bile ducts outside the liver the common bile duct and the hepatic ducts but sometimes it also affects those inside the liver the intrahepatic ducts. Laparotomy confirmed a large choledochal cyst and splenomegaly. Most patients have abnormalities of the pancreatic ducts and, frequently, multiple areas of dilatation involving the intrahepatic bile ducts.
Therefore, the current treatment of choice in choledochal. The surgical treatment of choledochal cysts, with resection and bilioenteric. Choledochal cysts are rare and usually manifest in infancy and childhood. Bile that is produced in the liver flows through increasingly larger channels ducts within the liver and finally into even larger ducts that leave the liver, pass through the substance of the pancreas, and then empty into the duodenum a choledochal cyst is a cyst hollow outpouching of the bile ducts. Complications after cyst excision with hepaticoenterostomy for choledochal cysts and. Complete cyst removal with biliary reconstruction, usually with rouxeny hepaticojejunostomy gross description. Diagnosis and management of choledochal cysts ncbi. Request pdf histopathologic improvement in biliary cirrhosis after definitive surgery for choledochal cyst choledochal cyst causes liver fibrosis, the extent of which varies with each case. Choledochal cyst is a rare congenital cystic dilation of the biliary tract with an incidence that ranges from one in 100,000 to 150,000 in western countries to 1 in,000 in east asian populations. Liver histology in choledochal cyst pathological changes. Choledochal cysts are uncommon developmental anomalies characterized by the dilation of the bile duct, which is more frequent in the asian population, especially in japanese, than in western populations. Choledochal cyst liver disease research and support. To study the diagnostic modalities used in detection of choledochal cyst.
Histopathology showed an inflamed cystic structure lined with biliary epithelium, consistent with a choledochal cyst. Associated with other hepatobiliary tract abnormalities. Influence of age on the presentation and outcome of choledochal cyst. The cystic dilatations of the biliary tree can involve the extrahepatic andor the intrahepatic biliary tract. Liver histology in choledochal cyst pathological changes and. Majority of these patients are diagnosed during the first decade of life, however, presentation in late adulthood is not uncommon. May rupture spontaneously, be associated with reflux of pancreatic enzymes into bile duct. Choledochal cysts in adults hepatobiliary disease jama. Choledochal cysts cdc are rare congenital disorder characterized by abnormal dilation of the bile ducts. The dilated distal common bile duct was also misinterpreted as a second hepatic cyst.
Epidemiology incidence western population 1 in 100,000 to 150000 live births more common in asia 1 in live births in japan sex prevalence female dominance 34. Proper bile duct flow, rather than radical excision, is the most critical factor determining treatment outcomes of bile duct cysts. Choledochal cyst is mostly diagnosed in the first decade of life. The presentation and therapeutic strategies for choledochal cysts in adult may differ from that of childhood. Choledochal cyst is a problem with the tubes ducts that carry bile from your childs liver to their gallbladder and intestine. Choledochal cysts are usually diagnosed in childhood, although in utero and adult diagnosis is also common. This is the first report of clinically diagnosed type ii choledochal cyst that is entirely lined by stratified squamous epithelium, unlike most other. Type i choledochal cyst radiology reference article. Choledochal cyst a different disease in newborns and. Choledochal cyst is a cystic dilation of biliary tree that can increase the risk of malignancy in bile ducts and gallbladder. Choledochal cysts ccs are rare medical conditions, which are congenital cystic dilatations of any portion of the bile ducts, most often occurring in the main portion of the common bile duct.
Furthermore, for unknown reasons, more than half of the reported cases have occurred in japanese patients. Although they may be discovered at any age, 60% are diagnosed. The diagnosis and management of choledochal cyst has standard procedures and strategies. Management issues of pht patients are inadequately addressed, as its incidence is low and underlying causes variable. In some cases the child may have symptoms such as jaundice, a mass in the abdomen, pancreatitis or cholangitis. To prevent costintensive and potentially lifethreating complications, a choledochal cyst must be considered in the differential diagnosis whenever the rather common diagnosis of a hepatic cyst is considered. This case report demonstrates the diagnostic and therapeutic pitfalls. Typical common duct cd cyst histology consists of relatively flat line. Surgical conditions like choledochal cyst should be considered within the context of hepatocellular elevation with or without cholestasis, as.
Jun 11, 2019 based on findings from a retrospective analysis of 32 children and 47 adults with choledochal cysts, shah et al concluded that because of differences with regard to the presentation, management, and histopathology of, as well as the outcomes related to, these lesions, choledochal cysts in children should be considered separate entities from. There is only one case of in situ gall bladder cancer gbc reported in association with type vi cdc in the literature. Histopathologic improvement in biliary cirrhosis after definitive surgery for choledochal cyst article in journal of pediatric surgery 455. The cyst was drained into the jejunum in the rouxeny fashion. The incidence of gallbladder carcinoma in patients with ductal malunion without choledochal cyst forme fruste choledochal cyst was 50% in one study, and only 5%. Histopathologic improvement in biliary cirrhosis after. To study the clinical presentation of choledochal cyst. Interpretation the preliminary imaging modality for detecting choledochal cyst is ultrasonography which delineates a cystic. Their exact pathophysiology remains unclear pancreaticobiliary ductal dysplasia. Douglas wrote the first clinical report of choledochal cysts in 1852 2. Choledochal cysts represent congenital cystic dilatations of the biliary tree. Choledochal cysts and multilocular cysts of the pancreas. Pdf recent advances in choledochal cysts researchgate. Their presentations differ from children and surgical management has evolved.
Between 1960 and 1975, 17 patients with congenital cystic dilatation of the common bile duct choledochal cyst were treated and three were associated with malignant tumors in the cysts and one. However, on histopathology, the bile duct was also found to. Epidemiology although uncommon in western countries 1. Choledochal cysts, are rare congenital dilations enlargements of the bile ducts, a network of long tubelike structures that carry bile from the liver to small intestine for digestion. Choledochal cyst a different disease in newborns and infants.
Choledochal cyst is considered a conglomerate of pancreatobiliary anomalies consisting of a choledochal cyst, a commonchanneltype pancreaticobiliary junction, intraheptic cystic disease, and a partially obstructed distal common bile duct. Singleincision versus conventional laparoscopic cyst excision and rouxy hepaticojejunostomy for children with choledochal cysts. Choledochal cysts cc are a rare congenital cystic dilation of the. Presentation and clinical outcomes of choledochal cysts in. Choledochal cyst is a known premalignant condition that causes gallbladder cancer, and the gallbladder is the second most common site of malignancy after cholangiocarcinoma in a choledochal cyst. A biliary tract pathology case study what is a choledochal cyst and what is its appearance on an mrcp. Choledochal cysts can be diagnosed prenatally on routine ultrasonography.