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. Although they may be discovered at any age, 60% are diagnosed. In 1723, vater first described dilata tion of the common bile duct 1. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in adults. 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.
In some cases the child may have symptoms such as jaundice, a mass in the abdomen, pancreatitis or cholangitis. Sep 21, 20 choledochal cysts are now being diagnosed before birth on routine maternal sonography us. Cirrhotic changes in choledochal cyst in relevance to. 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.
Presentation and clinical outcomes of choledochal cysts in. Primary epidermoid cyst of biliary duct presenting as. Choledochal cysts are abnormal dilatations of the biliary tree, generally believed to be congenital in origin. Choledochal cyst is a relatively rare disease in the west, and thus most cases have originated from asia. To study the age and sex incidence of choledochal cyst. Five had antenatal us revealing cystic abdominal masses. Choledochal cyst and associated malignant tumors in adults. May rupture spontaneously, be associated with reflux of pancreatic enzymes into bile duct. Yotsuyanagi s 1936 contribution to aetiology and pathology of idiopathic cystic. Their diagnosis is difficult, particulary in adults. Choledochal cyst a different disease in newborns and. Liver fibrosis is more common and severe in infants than in children. Type vi choledochal cysts can further be described based on morphology as fusiform more common and saccular.
Choledochal cyst is a problem with the tubes ducts that carry bile from your childs liver to their gallbladder and intestine. Choledochal cysts are congenital anomalies of the biliary tree, and they show dilatation of the intra and extra hepatic biliary tree. Histopathologically, the type i, ii, and iv choledochal cysts are similar and consist of a wall of dense collagenous tissue with. Choledochal cysts in adults hepatobiliary disease jama. Management issues of pht patients are inadequately addressed, as its incidence is low and underlying causes variable. Introduction cyst dilatation thoughout the billiary tree approximately 1 in 100,000 1 in 150,000 more common in some asian countries1. Our study details the diagnosis, treatment and outcome of six such children, four girls and two boys. Diagnosis and management of choledochal cysts ncbi. A choledochal cyst is a cyst hollow outpouching of the bile ducts.
Choledochal cyst a different disease in newborns and infants. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in. 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. Epidemiology although uncommon in western countries 1. 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. 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. Therefore, the current treatment of choice in choledochal. This is the first report of clinically diagnosed type ii choledochal cyst that is entirely lined by stratified squamous epithelium, unlike most other. Influence of age on the presentation and outcome of choledochal cyst. Choledochal cysts are rare disease and of unknown etiology.
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. Type vi choledochal cyst with gall bladder carcinoma bmj. Complete cyst removal with biliary reconstruction, usually with rouxeny hepaticojejunostomy gross description. There is only one case of in situ gall bladder cancer gbc reported in association with type vi cdc in the literature.
Choledochal cysts have been classified into several different types depending on where. Histopathologic improvement in biliary cirrhosis after definitive surgery for choledochal cyst article in journal of pediatric surgery 455. 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 singlecenter experience with 73 patients of adult. Although benign, cc can be associated with serious complications including malignant transformation, cholangitis, pancreatitis, and cholelithiasis. Choledochal cysts are rare and usually manifest in infancy and childhood. 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. Choledochal cysts cc are a rare congenital cystic dilation of the. More severe symptoms, younger age and apbj are associated with higher degree of liver damage.
A case report of an unusual type of choledochal cyst with. The condition usually affects the part of the bile ducts outside. Pdf choledochal cysts are a congenital anomaly, and they show dilatation of the. A large cyst,11 cm in diameter, was considered to represent a common hepatic cyst because it was in direct contact with the liver. Ba is a progressive obstructive cholangiopathy diagnosed in the newborn period and characterized by partial or complete lack of the extrahepatic bile ducts. Management of choledochal cyst with portal hypertension. Histopathologic improvement in biliary cirrhosis after. A biliary tract pathology case study what is a choledochal cyst and what is its appearance on an mrcp. 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. Choledochal cyst childrens liver disease foundation.
All patients with choledochal cyst show pathological changes in liver of varying severity. 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. Type iv a choledochal cyst with cystic duct cyst hpb oxford 2010. Type i choledochal cyst radiology reference article. Interpretation the preliminary imaging modality for detecting choledochal cyst is ultrasonography which delineates a cystic. Choledochal cysts cc are a rare congenital cystic dilation of the biliary tract, first described by vater and ezler in 1723. Contain 1 2 liters of bile, up to 15 cm in diameter. Surgical conditions like choledochal cyst should be considered within the context of hepatocellular elevation with or without cholestasis, as. Histopathology of synovial cysts of the spine chebib. Congenital dilatation of the extrahepatic biliary tract with or without dilatation of the intrahepatic biliary tract known as. Their exact pathophysiology remains unclear pancreaticobiliary ductal dysplasia. Choledochal cyst congenital anomaly involving cystic dilation of various ducts of the biliary tract. Majority of these patients are diagnosed during the first decade of life, however, presentation in late adulthood is not uncommon.
Until now, no unifying etiologic theory exists for choledochal cysts, but most literature would conclude they are congenital bile duct anomalies. Choledochal cyst occasionally causes liver fibrosis caused by obstructive jaundice. Sometimes, a choledochal cyst is found when a child is having an abdominal ultrasounds scan for an unconnected reason like pain in the abdomen. 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. Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree. A choledochal cyst is a swelling widening or dilatation of the bile ducts. Histopathology showed an inflamed cystic structure lined with biliary epithelium, consistent with a choledochal cyst. There is no report in the literature outlining the management of newborns with choledochal cysts, many of whom are asymptomatic. Smooth muscle distribution patterns of choledochal cysts. Analysis of choledochal cyst cases presenting to osmania general hospital over the period of january 2016november 2017. 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. Protocol for the examination of specimens from patients with.
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%. 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 are rare, with an incidence of 1. Their presentations differ from children and surgical management has evolved. Singleincision versus conventional laparoscopic cyst excision and rouxy hepaticojejunostomy for children with choledochal cysts.
Choledochal cyst liver disease research and support. Diagnosis relies on the exclusion of other conditions e. Choledochal cysts are usually diagnosed in childhood, although in utero and adult diagnosis is also common. 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. Clinical significance of intrapancreatic choledochal cyst excision in surgical management of type i choledochal cyst. The management of choledochal cysts in the newborn springerlink. Choledochal cysts are rare congenital or acquired cystic dilatations of the intra or extrahepatic bile ducts.
Choledochal cysts and multilocular cysts of the pancreas. Proper bile duct flow, rather than radical excision, is the most critical factor determining treatment outcomes of bile duct cysts. 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. The term choledochal cyst can be broken down into the following. To date, specific pathogenic and diagnostic criteria for. Choledochal cysts an overview sciencedirect topics. Approximately 80% of cc are diagnosed in infants and young children within the first decade of life. Choledochal cysts cdc are rare congenital disorder characterized by abnormal dilation of the bile ducts. Choledochal cysts are classified into 5 types, based on site of the cyst or dilatation. Choledochal cysts are diagnosed in children and adults, although most 80% develop in children younger than 10 years. Correlation of intracystic pressure with cyst volume.
Douglas wrote the first clinical report of choledochal cysts in 1852 2. Cirrhosis develops more frequently with increasing age. Characterised by a dilatation limited to the duodenal wall in the distal part. Typical common duct cd cyst histology consists of relatively flat line. Biliary atresia ba and choledochal cyst cc are two wellknown causes of obstructive jaundice in neonates and young infants and have similar clinical presentation. Regression of biliary cirrhosis following choledochal cyst. 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. There is a higher incidence of ccs in asian populations compared with other. Massive dilatation of the common bile duct resembling a choledochal cyst. The surgical treatment of choledochal cysts, with resection and bilioenteric. First reported by vater and ezler 1 in 1723, choledochal cysts ccs are rare cystic dilations of the biliary tract. The tubes get bigger than normal dilate or pouches form on the tubes. Oct 28, 2015 choledochal cyst is a congenital dilatation of part or whole of the bile duct. 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.
Pdf recent advances in choledochal cysts researchgate. Laparotomy confirmed a large choledochal cyst and splenomegaly. 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. The liver was hard and nodular and had the characteristic features of cirrhosis. Choledochal cysts can be diagnosed prenatally on routine ultrasonography. 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. 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. Biliary tract cysts are a rare biliary malformative pathology, accounting for 1% of benign biliary diseases 1.
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. This keeps bile from flowing well, which can cause liver problems. Choledochal cysts in adults are uncommon, and cystic dilatation of the cystic duct type vi choledochal cyst is a unique occurrence. Choledochal cyst is a rare benign biliary disease mostly presenting during childhood. Madadisanjani o, wirth tc, kuebler jf, petersen c, ure bm. Request pdf liver histology in choledochal cyst pathological changes and response to surgery. However, on histopathology, the bile duct was also found to. They are typically a surgical problem of infancy and childhood, but in nearly 20% of the patients the diagnosis is delayed until adulthood. They are usually lined by bile duct epithelium, which may undergo intestinal and squamous metaplasia. 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. Complications after cyst excision with hepaticoenterostomy for choledochal cysts and. Except portal fibrosis and central venous distension, all other pathological changes regress after surgery. What is a choledochal cyst and what is its appearance on an mrcp.
Choledochal cysts classification, physiopathology, and. Adult presentation is rare and associated diseases and complications are common. Choledochal cyst is mostly diagnosed in the first decade of life. They are considered congenital because they occur in fetuses and neonates.
Here we are reporting a case of type vi cdc with papillary gbc. Choledochal cyst rupture with an intrahepatic pseudocyst mimicking hepatic mesenchymal hamartoma in an infant clinical imaging, vol. The diagnosis and management of choledochal cyst has standard procedures and strategies. 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.
Choledochal cysts are treated by surgical excision of the cyst with the formation of a rouxeny anastomosis hepaticojejunostomy choledochojejunostomy to the biliary duct. To study the clinical presentation of choledochal cyst. Choledochal cystsclassification, physiopathology, and. The choledochal cyst was mobilized and fixed with holding sutures. Choledochal cyst, liver biopsy,liver cirrhosis introduction choledochal cyst. Associated with other hepatobiliary tract abnormalities. Choledochal cysts represent congenital cystic dilatations of the biliary tree.
Early diagnosis and prompt treatment is necessary for a good outcome. 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. 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. This case report demonstrates the diagnostic and therapeutic pitfalls. Not actually a cyst but a dilation of common bile duct which may secondarily obstruct other biliary ducts or the duodenum.
Furthermore, for unknown reasons, more than half of the reported cases have occurred in japanese patients. The cystic dilatations of the biliary tree can involve the extrahepatic andor the intrahepatic biliary tract. 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. The disease is mostly that of infancy and childhood with 80% being diagnosed in the first ten years of life. Histology of synovial cysts of the spine shows varied types of cyst. The presentation and therapeutic strategies for choledochal cysts in adult may differ from that of childhood. Liver histology in choledochal cyst pathological changes and. The outer wall of the cyst contains almost exclusively duodenal mucosa while the inner wall may include duodenal or biliary epithelium. 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. The cyst was drained into the jejunum in the rouxeny fashion. Liver histology in choledochal cyst pathological changes.
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