Complete cyst removal with biliary reconstruction, usually with rouxeny hepaticojejunostomy gross description. Accordingly, apbduassociated choledochal cyst patients are significantly more likely to have evidence. Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree. Most are harmless, but they should be removed when possible because they occasionally may change into malignant growths, become infected, or obstruct a gland. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal union apbdu is thought to be a major. Their diagnosis is difficult, particulary in adults.
Choledochal cyst childrens liver disease foundation. In neonates, choledochal cysts present with jaundice similar to. Get a printable copy pdf file of the complete article 2. Etiology of type i choledochal cyst is uncertain but may be related to distal bile duct obstruction, abnormal insertion of the common bile duct, or reflux of pancreatic enzymes into the biliary tree. Although they may be discovered at any age, 60% are diagnosed. Management dilemmas with choledochal cysts hepatobiliary. 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 substa. Jan 31, 2011 details of the image choledochal cyst modality. Introduction cyst dilatation thoughout the billiary tree approximately 1 in 100,000 1 in 150,000 more common in some asian countries1.
The typical patient with choledochal cyst disease has been the female infant with the triad of jaundice, an abdominal mass, and pain. Choledochal cysts are uncommon and of unknown cause, with an incidence of 0. Choledochal cyst is an unusual but serious condition which most commonly affects oriental people. There arefour basic types of choledochal cysts based on where they appear. Oct 28, 2015 choledochal cyst is a congenital dilatation of part or whole of the bile duct. A large cyst,11 cm in diameter, was considered to represent a common hepatic cyst because it was in direct contact with the liver. Although it was first described by vater and ezler in 1723, douglas published the first complete clinical description of the anomaly in a patient in 1853. The etiology of cccs remains unclear, although many theories have been put forth. 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. Fiftynine patients underwent repair of choledochal cyst. 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 definition of choledochal cyst by.
Diagnosis relies on the exclusion of other conditions e. Agent orange exposure cholangiocarcinoma bile duct. May 21, 2018 green berets ultralight bug out bag with gear recommendations duration. Get a printable copy pdf file of the complete article 3. Congenital choledochal cyst ccc is a rare disease of the biliary tree, characterized by either isolated or combined dilatations of the extra and intrahepatic bile ducts.
In order to complete this program you must have a computer with a recent browser version. Choledochal cyst and associated malignant tumors in adults. The condition usually affects the part of the bile ducts outside. Jun 11, 2019 the prognosis after excision of a choledochal cyst is usually excellent, but it is influenced by several factors, including patient age, cyst type, histologic features, and site. Choledochal cysts are classified into 5 types, based on site of the cyst or dilatation. Vater gave anatomical description of this anomaly in 1723. The choledochal cyst was mobilized and fixed with holding sutures.
The type of choledochal cyst seen in children and adults was similar. Choledochal cysts are a congenital anomaly, and they show dilatation of the intra or extrahepatic biliary tree. The prognosis after excision of a choledochal cyst is usually excellent, but it is influenced by several factors, including patient age, cyst type, histologic features, and site. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal. Choledochal cysts are being recognized with an increasing frequency among the adult population. Patient with choledochal cyst corrected are likely to represent larger cysts and inversely, smaller, static, anechoic cysts are more likely to represent cystic biliary atresia. Choledochal cysts represent congenital cystic dilatations of the biliary tree. Choledochal cysts are congenital cystic dilatations of the biliary tree which can involve the extrahepatic biliary ducts. This case report demonstrates the diagnostic and therapeutic pitfalls. These problems may be aggravated by previous treatment with bypass surgery rather than resection. The term choledochal cyst cdc describes cystic dilation of various parts of the biliary tree that have in common the potential for obstructive complications of the common bile duct, pancreatic duct, and ultimately malignant degeneration. The term choledochal cyst can be broken down into the following. The clinical triad of obstructive jaundice, right upper abdominal pain, and a palpable flactuant mass was noted in only two cases but at least one of these symptoms was present in.
Choledochal cysts are relatively rare congenital disorders of the biliary tree, usually. 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. In infancy, painless jaundice is the most common presentation, whereas older children may present with intermittent abdominal pain related to. It occurs in approximately 2 out of 100,000 people. Biliary continuity was restored by hd in 39 66% and by hj in 20 34%. For more information about applying to the research study, please visit their website. Choledochal cyst is a relatively rare disease in the west, and thus most cases have originated from asia. This means that choledochal cyst, or a subtype of choledochal cyst, affects less than 200,000 people in the us population. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in adults. Choledochal cysts are rare, with an incidence of 1. Choledochal cysts are congenital anomalies of the bile ducts that include. Choledochal cysts are congenital bile duct anomalies. Bile duct surgery with total cyst removal is the definitive treatment for choledochal cysts. Although it was first described by vater and ezler in 1723, douglas published the first complete clinical description of.
The dilated distal common bile duct was also misinterpreted as a second hepatic cyst. Choledochal cyst is an unusual but serious condition which most commonly. The centers for mendelian genomics program is working to discover the causes of rare genetic disorders. Choledochal cysts can form in the part of the bile duct inside of the liver intrahepatic or outside the liver extrahepatic. Case presentation 56 year old male referred from an outside institution for evaluation of type i choledochal cyst. Pdf choledochal cysts are cystic dilation of extrahepatic duct, intrahepatic duct, or both that may result in significant morbidity and mortality. Choledochal cyst in a female adult asymptomatic patient peertechz. Furthermore, for unknown reasons, more than half of the reported cases have occurred in japanese patients. Choledochal cysts are rare cystic transformations of the biliary tree that are increasingly diagnosed in adult patients 1,2,3,4. Scribd is the worlds largest social reading and publishing site. The cystic dilatations of the biliary tree can involve the extrahepatic andor the intrahepatic biliary tract. Cystic dilatation of the common bile duct cbd, also known as choledochal cyst, is a fairly uncommon anomaly of the biliary tract. Choledochal cysts are congenital anomalies of the biliary tract with dilatations of the extrahepatic and sometimes intrahepatic biliary system miyano and yamataka, 1997.
Choledochal cyst is defined as localized or diffuse dilatation of the extra or intra hepatic biliary tree. Mar 22, 2018 cystic dilatation of the common bile duct cbd, also known as choledochal cyst, is a fairly uncommon anomaly of the biliary tract. Choledochal cysts in adults and their complications. In 1723, vater first described dilata tion of the common bile duct 1. Choledochal cyst is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. Choledochal cysts are classified into five groups based on location or shape of the cysts.
The distalmost portion of the choledochal cyst is encircled and transected as it enters the pancreas. A copy of the license is included in the section entitled gnu free documentation license. Presentation and outcomes of choledochal cysts in children. Type iv a choledochal cyst with cystic duct cyst hpb oxford 2010. Green berets ultralight bug out bag with gear recommendations duration. A choledochal cyst is a cyst hollow outpouching of the bile ducts. Any information contained in this pdf file is automatically generated from. 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.
Comparison of therapeutic effects of laparoscopic and open. Choledochal cyst compiled free download as powerpoint presentation. The purpose of this study was to determine whether the laparoscopic procedure was feasible and safe in adult patients. In this series a correct preoperative diagnosis was established in eight of the ten patients.
Cystic disease of the bile duct is a congenital abnormality of the biliary tree that most commonly affects the neonatal population, and is. Type 1 a cyst of the extrahepatic bile duct, accounting for up to 90% of all choledochal cysts. Ultrasound study shows a cystic mass between the pancreatic head and the gallbladder. You must also have the capability to display and print pdf files in order to view and print out your certificate. Open hd patients required less total operative time than hj patients 3. Spontaneous rupture of a choledochal cyst is a rare complication biliary atresia view in chinese the main utility of the ultrasound is to exclude other anatomic causes of cholestasis ie, choledochal cyst. Bile duct cysts genetic and rare diseases information. Choledochal cyst is a wellrecognised entity, presenting primarily in infants and young children. 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.
Your cme certificate is stored in your account and is available at any time. Where symptoms are delayed until adulthood, associated hepatobiliary pathology may complicate the presentation. Source national institutes of health nih choledochal cyst as a disease. To our knowledge, available data on these rare lesions are limited to small singlecenter series. Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Choledochal cysts are diagnosed in patients of all ages and harbor a risk of becoming malignant as well as producing serious complications.
Their exact etiology remains incompletely understood. Couper introduction choledochal cyst in adults is a rare clinical entity and there has been only a few reports in the litera ture. For both children and adults, treatment consisted of excision of the cyst and biliary reconstruction with a hepaticojejunostomy. Purpose choledochal cyst is a cystic dilatation of common bile duct. Choledochal cysts an overview sciencedirect topics. In recent years in pediatric patients, laparoscopic resection of congenital choledochal cyst and choledochojejunostomy is reported to be safe. Left untreated, this frequently lead to recurrent cholangitis inflammation and infection in the bile ducts or pancreatitis inflammation of the pancreas. Although by definition the cyst can arise from anywhere within the extrahepatic biliary tree, it is \.
Choledochal cysts are treated by surgical excision of the cyst with the formation of a rouxeny anastomosis hepaticojejunostomy choledochojejunostomy to the biliary duct. 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. Jan 14, 2008 choledochal cysts are rare congenital anomalies. Malignancy after cyst excision excision of the biliary cyst does not eliminate risk of malignancy in the biliary tract enteric anastomosis intrahepatic duct lee et al 2011 south korea 0. Bile duct choledochal cysts chronic biliary and liver inflammation history of infection with the parasitic worm, liver flukes primary sclerosing cholangitis ulcerative colitis cholangiocarcinoma is rare. Choledochal cysts may cause symptoms at any age, but typically present with obstructive. Douglas wrote the first clinical report of choledochal cysts in 1852 2. Permission is granted to copy, distribute andor modify this document under the terms of the gnu free documentation license, version 1. Thus choledochal cyst as a term is a misnomer and should be called as congenital biliary dilatation. We present a case of a large type 1choledochal cyst in a 31yearoldwoman and emphasise the means of preoperative diagnosis. Fusiform or saccular dilation of the bile duct results in choledochal cyst.
These cystic dilatations of the biliary tree can involve the extrahepatic biliary radicles, the intrahepatic. Choledochal cysts are congenital anomalies of the biliary tree, and they show dilatation of the intra and extra hepatic biliary tree. Cccs occur most frequently in asian and female populations 1, 2. Classification of choledochal cyst and principles of their management. Their presentations differ from children and surgical management has evolved. Mar 31, 2017 choledochal cysts are rare congenital disorders first described by vater and ezler in 1723. Meier introduction congenital bile duct dilatation is a better term for the spectrum of anomalies known traditionally as choledochal cysts. The goal is then to excise the intrapancreatic portion of the cyst without injuring the pancreatic duct or the long common channel. Recent advances in choledochal cysts article pdf available in open journal of gastroenterology 0204. Management of choledochal cysts a 7 year experience. These cysts are uncommon in western countries, but are not rare in asian countries. 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. A choledochal cyst is a swelling widening or dilatation of the bile ducts.
Contain 1 2 liters of bile, up to 15 cm in diameter. Its safety and efficacy in adult patients remain unknown. Laparoscopic cyst excision and rouxeny hepaticojejunostomy for treating congenital choledochal cysts cccs have proved to be efficacious in children. After ba, choledochal cyst is the most common surgical cause of obstructive jaundice in the newborn period.
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