Historically, surgical treatment of choledochal cyst consisted of cyst enterostomy. A new classification system is proposed according to location and extension of the bile duct dilatation and clinical features. Various pathological conditions develop in the biliary tract, pancreas, and liver. The condition usually affects the part of the bile ducts outside. Complete cyst removal is necessary for the first surgical treatment. Surgical treatment is recommended for choledochal cysts localized on extrahepatic biliary ducts, which reduces cystrelated complicat ions and the risk of malignant transformations. Epidemiology the estimated incidence rate of choledochal cysts is 1 in 2 million live births 2. The choledochal cyst was mobilized and fixed with holding sutures. 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. Introduction cyst dilatation thoughout the billiary tree approximately 1 in 100,000 1 in 150,000 more common in some asian countries1. More than 90 % of patients with choledochal cysts have an apbj with the pancreatic duct joining the common bile duct 1 cm proximal to the ampulla. 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 etiology remains unknown, but choledochal cysts are likely to be congenital in nature.
Medicinenet does not provide medical advice, diagnosis or treatment. Diagnosis of choledochal cyst is made based on a disproportionate dilation of extrahepatic biliary duct, without ruling out the possibility of a tumor, stone, or inflammation as a cause of this dilation. Today, the surgical treatment choledochal cysts cyst is completed excision followed by reconstruction of the biliary tree with hepaticojejunostomy in a rouxeny fashion has been widely accepted as the procedure of choice. Choledochal cysts are treated by surgical excision of the cyst with the formation of a rouxeny anastomosis hepaticojejunostomy choledochojejunostomy to the biliary duct. Choledochal cysts are also 4 times more common in females than males. 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. 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%.
This case report demonstrates the diagnostic and therapeutic pitfalls. Bile duct surgery with total cyst removal is the definitive treatment for choledochal cysts. 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. Type i choledochal cyst radiology reference article. Proper bile duct flow, rather than radical excision, is the most critical factor determining treatment outcomes of bile duct cysts. Proper bile duct flow, rather than radical excision, is the most critical factor determining treatment outcomes of. The type iv cyst is the second most common type in adults, and represents 10% 15% of adult cases 24. Choledochal cyst excision is a topic covered in the pediatric surgery nat to view the entire topic, please sign in or purchase a subscription apsa pediatric surgery library combines pediatric surgery not a textbook nat with apsa expert, a powerful platform for earning moc cme credits all powered by unbound medicine. Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree. Treatment most patients underwent a resection, as shown in table 4.
Most appear to be of congenital origin probably related to distal bile duct stenosis, and almost 15% can now be detected antenatally. This consisted of resection of the extrahepatic cyst type i, ii and the extrahepatic part of type. Choledochal cyst todani ia case report sciencedirect. When a child has a choledochal cyst, a swelling of that duct, bile may back up in the liver. Choledochal cyst presentation and treatment in an adult. A case of an 18yearold patient, with a recurrent abdominal pain, was d iagnosed with the performance of an abdominal ultrasound and the magnetic resonance image. Surgical options in treatment of choledochal cyst are excisionremoval of the. More than 60% of them present during the first year of life. Accordingly, apbduassociated choledochal cyst patients are significantly more likely to have evidence. Diagnosis of choledochal cyst is concluded upon disproportionate expansion of extrahepatic bile duct. Medcram medical lectures explained clearly 488,942 views. The incidence of bile duct cysts ranges from 1 in,000 to 1 in 2 million births 1. We present a case of a large type 1choledochal cyst in a 31yearoldwoman and emphasise the means of preoperative diagnosis.
Choledochal cysts in adults are uncommon, and cystic dilatation of the cystic duct type vi choledochal cyst is a unique occurrence. Cyst excision is the definitive treatment of choice for choledochal cyst. 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. Management of choledochal cyst with portal hypertension. The dilated distal common bile duct was also misinterpreted as a second hepatic cyst. A recent article published in journal of surgery suggested that choledochal cysts could. Choledochal cysts are segments of the biliary tree that have undergone local distention.
Childrens liver disease foundation this leaflet has been written for. Left untreated, this frequently lead to recurrent cholangitis inflammation and infection in the bile ducts or pancreatitis inflammation of the pancreas. 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. Understanding choledochal malformation archives of. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in adults. Choledochocele, or type iii choledochal cyst, is a. The disease is mostly that of infancy and childhood with 80% being diagnosed in the first ten years of life. In japan, it accounts for one in admissions to hospital, compared with one in 00 in the united states. Their exact etiology remains incompletely understood.
Diagnosis and management of choledochal cyst are important because patients may develop cholangiocarcinoma. Liver function tests lfts explained clearly by duration. Choledochal cysts classification, physiopathology, and. Clinical significance of intrapancreatic choledochal cyst excision in surgical management of type i choledochal cyst. Choledochal cysts an overview sciencedirect topics. Choledochal cyst and associated malignant tumors in adults. Key words choledochal cyst cancer congenital anomalies bile ducts introduction the incidence of choledochal cysts in the west is between one in 00 and one in 190000 live births. Choledochal malformations also known as choledochal cysts may be characterised as an abnormal dilatation of the biliary tract, in the absence of any acute obstruction. Considering the diagnosis and good patients general condition, we opted for surgical treatment. The frequency of choledochal cysts is about 1 in 15,000 live births in western countries, and as high as 1 in live births in japan suchy et al, 2001. This should be done in a centre which has experience of looking after children who have choledochal cysts and surgeons experienced with this kind of operation.
Choledochal cysts in adults hepatobiliary disease jama. The performance of cystenterostomy has recently been. First reported by vater and ezler in 1723, choledochal cysts ccs are rare cystic dilations of the biliary tract. Their diagnosis is difficult, particulary in adults. Any type of cyst is susceptible to malignancy, but the greatest prevalence is observed with types i, iv, and v. Malignancies in choledochal cyst can arise from the distal cbd, the wall of the cyst even after successful drainage at cystoenterostomy, or the intrahepatic bile ducts. N2 choledochal cyst is a relatively uncommon entity in western countries. Choledochal cyst is a congenital cystic dilation of a part of bile duct that occurs most commonly in the main part of common bile duct. Choledochal cysts are treated by surgical excision of the cyst with the formation of a rouxeny anastomosis hepaticojejunostomy choledochojejunostomy to the biliary duct future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree.
Oct 28, 2015 choledochal cyst is a congenital dilatation of part or whole of the bile duct. Fetal choledochal cyst radiology reference article. The risk of carcinogenesis in congenital choledochal cyst patients. The goal is then to excise the intrapancreatic portion of the cyst without injuring the pancreatic duct or the long common channel. In earlier decades, an operation known as cystenterostomy was performed that only drained the cyst and the bilary reconstruction left the cyst behind. Choledochal cysts have been classified into several different types depending on where. 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. 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. Excision and biliary reconstruction using a roux loop as an open operation is still the. Presentation and clinical outcomes of choledochal cysts in. Choledochal cysts are diagnosed in children and adults, although most 80% develop in children younger than 10 years. The choledochal cyst is a rare disease of unknown etiology, most prevalent in childhood, however, occurs in 20% of adult patients.
The cystic dilatations of the biliary tree can involve the extrahepatic andor the intrahepatic biliary tract. Their presentations differ from children and surgical management has evolved. Studies now conclude that for all types of choledochal cyst, a total choledochal cyst excision with rouxeny hepaticojejunostomy is the treatment of choice. 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. Management choledochal cysts treatment is one of the challenging. This retrospective study complied with the ethical guidelines of the declaration of. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis.
Choledochal cyst is a rare congenital anomaly characterized by. Choledochal cyst in two pediatric heart transplant patients. Treatment entails removal of the dilated portion of the bile duct and reconnection of the biliary system, usually requiring a hepaticojejunostomy. Miyano g, yamataka a, shimotakahara a 2005 cholecystectomy alone is inadequate for treatment of form fruste choledochal cyst. Treatment varies depending on type of choledochal cyst, but type i and iv cysts typically undergo surgical excision and reconstruction by rouxeny hepaticojejunostomy left this graphic shows the todani classification of choledochal cysts. An illustrated guide to pediatric surgery pp 283289 cite as. After ba, choledochal cyst is the most common surgical cause of obstructive jaundice in the newborn period.
Factors thought to contribute to the development of malignancy. Furthermore, for unknown reasons, more than half of the reported cases have occurred in japanese patients. In 1995, videoguided laparoscopic treatment was initially used in clinical practice for the management of choledochal cyst. Fetal choledochal cysts are choledochal cysts diagnosed in utero.
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 choledochal cysts were classified as todani and coworkers type i in 20 patients, type ii in 2 patients, and type iv in 8 patients. Oldham section of pediatric surgery, department of surgery, the university of michigan medical center, ann arbor, mi 48109, usa abstract. A large cyst,11 cm in diameter, was considered to represent a common hepatic cyst because it was in direct contact with the liver. 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. Paediatric surgery unit, prince hamza hospital, amman, jordan. Parentscarers of a child diagnosed with a choledochal cyst young people diagnosed with a choledochal cyst others who may find this leaflet helpful. A choledochal cyst is a cyst hollow outpouching of the bile ducts. Choledochal cysts are rare congenital disorders first described by vater and ezler in 1723. Mar 31, 2017 choledochal cysts are rare congenital disorders first described by vater and ezler in 1723. Aupbd into 3 types6, type 1 is joining of the ducts at a right angle to.
Often huge cysts microscopic histologic description. Surgical treatment of choledochal cysts in children. Retrospective study of choledochal cyst international journal of. Management of choledochal cysts a 7 year experience. Choledochal cysts are a congenital anomaly, and they. There is a recognsed female predilection and a greater occurrence in asian popu. Management issues of pht patients are inadequately addressed, as its incidence is low and underlying causes variable. Also, it is associated with a hepatobiliary disease.
There is a reported risk of malignancy in type i choledochal cysts ranging from 2. Choledochal cyst is often associated with pancreatobiliary malunion, and pancreatic juice usually refluxes into the bile duct via the malunion. Conclusion there are several surgical techniques that can be used in the treatment of choledochal cysts, wherein each is intended as a complete resection of the cyst with histological confirmation. A diagnosis of a congenital type 1c choledochal cyst was made in an. T1 choledochal cyst in two pediatric heart transplant patients. Management of choledochal cysts a 7 year experience p j m h s vol. The term choledochal cyst can be broken down into the following. Early reports suggested that drainage operations including cystoduodenostomy or cystojejunostomy without excision of the cyst resulted in satisfactory. The joining of the right and left hepatic ducts forms the common hepatic duct.
Franko j, nussbaum ml, morris jb 2006 choledochal cyst cholangiocarcinoma arising from adenoma. Cystic dilatation of the common bile duct cbd, also known as choledochal cyst, is a fairly uncommon anomaly of the biliary tract. Thyroglossal duct cyst causes, symptoms, diagnosis, treatment. Accordingly, apbduassociated choledochal cyst patients are significantly more likely to have. Choledochal cyst is a relatively rare disease in the west, and thus most cases have originated from asia. In recent years in pediatric patients, laparoscopic resection of congenital choledochal cyst and choledochojejunostomy is reported to be safe. Choledochal cyst is a rare benign biliary disease mostly presenting during childhood. Choledochal cysts are congenital anomalies of the bile ducts and are defined as abnormal, disproportionate, cystic dilatation of the biliary duct. Couper introduction choledochal cyst in adults is a rare clinical entity and there has been only a few reports in the litera ture.
Choledochal cyst childrens liver disease foundation. However, incomplete cyst excision can result in recurrent symptoms and malignant. The distalmost portion of the choledochal cyst is encircled and transected as it enters the pancreas. Rarely, reconstruction above the confluence of the left and right hepatic ducts is. Radical resection and rouxeny anastomosis is the standard method for the management of congenital choledochal cyst. Adult presentation is rare and associated diseases and complications are common. A choledochal cyst is a congenital anomaly of the duct tube that transports bile from the liver to the gall bladder and small intestine. In conclusion, we aimed to resect extrahepatic biliary ducts as much as possible preserving the last segment of the bile duct joining the main pancreatic duct. Choledochal cyst is a congenital cystic dilation of a part of bile duct that occurs most commonly in the main portion of common bile duct.