Chen cp, cheng sj, chang ty, yeh lf, lin yh, wang w. Choledochal cyst is a relatively rare disease in the west, and thus most cases have originated from asia. Choledochal cysts in adults hepatobiliary disease jama. Choledochal cyst is the cystic dilatation of the extrahepatic bile ducts, with or without. Choledochal cysts are classified into five groups based on location or shape of the cysts. Choledochal cysts represent congenital cystic dilatations of the biliary tree. Presentation this is a young patient who recently underwent surgery for scoliosis repair and had an abdominal ct. Type v is intrahepatic cystic biliary dilatation or carolis disease.
Types i and iva cysts are the most common types, which are associated with anomalous pancreaticobiliary. Therefore, after continuous followup, elective removal of the choledochal cyst will be performed. Complete cyst removal with biliary reconstruction, usually with rouxeny hepaticojejunostomy gross description. 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. 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.
Ba is a progressive obstructive cholangiopathy diagnosed in the newborn period and characterized by partial or complete lack of the extrahepatic bile ducts. To our knowledge, it is the first report of choledochal cyst as the cause of acute acalculous cholecystitis. 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. Iiimd, resident of radiology and imaging diagnosis, universidade federal do. Choledochal cysts are rare congenital anomalies which are principally. The choledochal cyst is a rare disease of unknown etiology, most prevalent in childhood, however, occurs in 20% of adult patients. This is either a type iii or ivb choledochal cyst, depending on whether the ed is considered to be involved.
The todani classification of bile duct cysts divides choledochal cysts into five groups. Most are harmless, but they should be removed when possible because they occasionally may change into malignant growths, become infected, or obstruct a gland. Only 7 cases were definitely diagnosed preoperatively. The choledochal cysts, which refer to the cystic dilatation of the biliary duct, are rare lesions generally seen in children. Pdf recent advances in choledochal cysts semantic scholar. Neonatal type 1 choledochal cyst journal of perinatology. Diagnosis requires excluding other causes of biliary dilatation, including tumor, stone, or stricture commonly associated with cholelithiasis, cystolithiasis, choledocholithiasis, and hepatolithiasis classified into 5 types based on todani classification type i. Diagnosis relies on the exclusion of other conditions e. Oldham section of pediatric surgery, department of surgery, the university of michigan medical center, ann arbor, mi 48109, usa abstract. Choledochal cysts cc are a rare congenital cystic dilation of the biliary tract, first described by vater and ezler in 1723. Choledochal cysts appear as cystic or fusiform di latation of the common bile duct at radiography. 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. 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. Historically, choledochal cysts have been classified according to the descriptions published in 1959 by alonsolej, with subsequent modifications.
Five had antenatal us revealing cystic abdominal masses. The various types of choledochal cysts have been classified on the basis of. Cystic dilatation of the common bile duct cbd, also known as choledochal cyst, is a fairly uncommon anomaly of the biliary tract. A new concept of the etiology of choledochal cyst is presented.
Choledochal cyst and associated malignant tumors in adults. Bile duct surgery with total cyst removal is the definitive treatment for choledochal cysts. The choledochal cyst was mobilized and fixed with holding sutures. Management of choledochal cyst with portal hypertension. 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. Case report antenatal sonographic diagnosis of choledochal cyst. Mr cholangiopancreatography a and endoscopic retrograde cholangiopancreatography b images show saccular dilatation of intrahepatic duct and common bile duct, which joined with pancreatic duct at right angle. Type iv is a choledochal cyst associated with other intrahepatic and extrahepatic cysts or carolis disease.
Their presentations differ from children and surgical management has evolved. A case of an 18yearold patient, with a recurrent abdominal pain, was diagnosed with the performance of an abdominal ultrasound and the magnetic resonance image. To date, specific pathogenic and diagnostic criteria for. Todani classification of bile duct cysts radiology. Magnetic resonance cholangiopancreatography diagnosis of choledochal cyst involving the cystic duct. Also, it is associated with a hepatobiliary disease. Choledochal cyst childrens liver disease foundation. Based on the classification of congenital choledochal cysts, images of different. The dilated distal common bile duct was also misinterpreted as a second hepatic cyst. Choledochal cyst of the cystic duct indian journal of radiology. Mallinckrodt institute of radiology, st louis childrens hospital department of radiology. Introduction cyst dilatation thoughout the billiary tree approximately 1 in 100,000 1 in 150,000 more common in some asian countries1. Antenatal sonographic diagnosis of choledochal cyst. Choledochocele, or type iii choledochal cyst, is a.
An ultrasound was performed to compliment the ct findings. Right coronal mrcp in the same patient nicely shows the choledochocele protruding into the duodenal lumen and the mildly dilated extrahepatic duct, along with numerous stones in the gallbladder. Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree. 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.
Type iv a choledochal cyst with cystic duct cyst hpb oxford 2010. Our study details the diagnosis, treatment and outcome of six such children, four girls and two boys. The highest percentage of definitive positive examinations was found in the 1 i rose bengal scan 3 of 4, but the. The objective of the study was to study the presentation, diagnosis, treatment and outcome of choledochal cyst in college of medical sciences, bharatpur. The diagnosis of choledochal cyst is usually made in the first few years of life, and more than 60% of all cases are diagnosed in the first decade. Early reports suggested that drainage operations including cystoduodenostomy or cystojejunostomy without excision of the cyst resulted in satisfactory. The disease is mostly that of infancy and childhood with 80% being diagnosed in the first ten years of life. 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. Article pdf available in indian journal of radiology and imaging. Biliary atresia ba and choledochal cyst cc are two wellknown causes of obstructive jaundice in neonates and young infants and have similar clinical presentation.
These cysts are uncommon in western countries, but are not rare in asian countries. The incidence of cancer in choledochal cyst increases with age and occurs around 32 years old. 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. Pancreatic juice leads to cholangitis and dilatation of the common duct. Choledochal cyst singh h, khanna v, puri b, maurya v. Their diagnosis is difficult, particulary in adults. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in adults. Choledochal cysts are treated by surgical excision of the cyst with the formation of a rouxeny anastomosis hepaticojejunostomy choledochojejunostomy to the biliary duct. Fusiformcystic dilation of extrahepatic duct type ii.
The management of choledochal cysts in the newborn. Choledochal cysts are a congenital anomaly, and they show dilatation of the intra or extrahepatic biliary tree. Left untreated, this frequently lead to recurrent cholangitis inflammation and infection in the bile ducts or pancreatitis inflammation of the pancreas. We report the imaging findings in three cases of choledochal cyst involving the cystic duct.
The choledochal cyst is a rare congenital malformation of the bile ducts. This anomaly allows reflux of pancreatic juice into the common bile duct. Choledochal cyst with anomalous union of pancreaticobile duct komi ib, todani iva combined stone. Prenatal diagnosis of choledochal cyst using ultrasound and magnetic resonance imaging. This report describes the first case of acute acalculous cholecystitis associated with choledochal cyst. Pdf the choledochal cysts, which refer to the cystic dilatation of the biliary. 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. Choledochal cysts in adults are uncommon, and cystic dilatation of the cystic duct type vi choledochal cyst is a unique occurrence. Choledochal cyst definition of choledochal cyst by.
This case report demonstrates the diagnostic and therapeutic pitfalls. We are reporting here the case of a cholangiocarcinoma in choledochal cyst in young adult. Imaging evaluation of congenital cystic lesions of the biliary tract. Choledochal cysts classification, physiopathology, and. The etiology of this entity is based on an anomalous relationship between the common bile duct and the pancreatic ducts. Choledochal cysts are now being diagnosed before birth on routine maternal sonography us.
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