Choledochal cysts classification, physiopathology, and. Clinical significance of intrapancreatic choledochal cyst excision in surgical management of type i choledochal cyst. Surgical treatment of choledochal cysts in children. Choledochal cyst definition of choledochal cyst by the. Children with choledochal cysts are typically treated surgically to remove them, either by an open procedure or laparoscopically. Filler and gustavo stringel toronto, ontario, canada since 1969 excision of choledochal cyst and establishment of bile drainage by rouxeny hepati cojejunostomy has been performed in 7 children whose ages ranged from 10 mo to 11 yr. The two most frequent complications of choledochal cysts are stone formation and malignancy.
The tubes get bigger than normal dilate or pouches form on the tubes. Franko j, nussbaum ml, morris jb 2006 choledochal cyst cholangiocarcinoma arising from adenoma. The aim of this report was to present our experience with a modified surgical technique designed for the treatment of choledochal cysts cc in children. Retrospective study of operated cases of choledochal cyst. Choledochal cyst types, causes, symptoms, diagnosis. Choledochal cyst is an unusual but serious condition which most commonly. Choledochal cyst definition of choledochal cyst by. This keeps bile from flowing well, which can cause liver problems. Miyano g, yamataka a, shimotakahara a 2005 cholecystectomy alone is inadequate for treatment of form fruste choledochal cyst. The management of choledochal cysts in the newborn.
Studies now conclude that for all types of choledochal cyst, a total choledochal cyst excision with rouxeny hepaticojejunostomy is the treatment of choice. The distal classification and management of choledochal cyst. There is a reported risk of malignancy in type i choledochal cysts ranging from 2. Choledochal cysts are a congenital anomaly, and they. Surgical treatment of choledochal cysts wiley online library. The term choledochal cyst can be broken down into the following. Between june 2004 and february 2005, we operated on 6 patients with a diagnosis of type i cc by means of a leakfree technique that consists of a transient complete sealing of the hepatic duct for the. An abnormal membranous sac in the body containing a gaseous, liquid, or semisolid substance.
The condition usually affects the part of the bile ducts outside. 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 in adults article pdf available in annals of the royal college of surgeons of england 796. Paediatric surgery unit, prince hamza hospital, amman, jordan. 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. In recent years in pediatric patients, laparoscopic resection of congenital choledochal cyst and choledochojejunostomy is reported to be safe. In this study, the clinical difference of laparoscopic surgery and laparotomy in the treatment of pediatric choledochal cyst was analyzed to explore the advantages, disadvantages of laparoscope in the treatment of congenital choledochal cyst, which provides a reference and theoretical basis for the application of laparoscope in the treatment of. Also, it is associated with a hepatobiliary disease.
When a child has a choledochal cyst, a swelling of that duct, bile may back up in the liver. Diagnosis and management of choledochal cysts ncbi. 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. Choledochal cyst presentation and treatment in an adult. Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree. Treatment entails removal of the dilated portion of the bile duct and reconnection of the biliary system, usually requiring a hepaticojejunostomy. Cyst excision is the definitive treatment of choice for choledochal cyst. In some countries, such as japan, the disease is still most commonly seen in young childhood, while in the united states and europe an increasing number of patients present for diagnosis and treatment in adulthood. The type iv cyst is the second most common type in adults, and represents 10% 15% of adult cases 24. Definitive surgery in the form excision of the cc and hepaticoenterostomy has been described as the ideal treatment option. Surgical treatment of choledochal cysts lipsett 2003. Management of choledochal cysts a 7 year experience. Proper bile duct flow, rather than radical excision, is the most critical factor determining treatment outcomes of. Laparoscopic leakfree technique for the treatment of.
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. Choledochal cyst american pediatric surgical association. Choledochal cyst treatment due to reported risk of malignancy in type 1 choledochal cysts ranging from 2. Girls are four times more likely to be affected than boys. 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.
We present a case of a large type 1choledochal cyst in a 31yearoldwoman and emphasise the means of preoperative diagnosis. Choledochal cysts an overview sciencedirect topics. Choledochal cysts in adults hepatobiliary disease jama. A choledochal cyst is a congenital anomaly of the duct tube that transports bile from the liver to the gall bladder and small intestine. Children with choledochal cysts have a higher rate of cancer of the bile duct in adulthood.
The choledochal cyst is a rare disease of unknown etiology, most prevalent in childhood, however, occurs in 20% of adult patients. Choledochal cyst is a rare benign biliary disease mostly presenting during childhood. Typically a rouxeny hepaticojejunostomy is performed 1. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Management of choledochal cysts a 7 year experience p j m h s vol. Choledochal cysts have been classified into several different types depending on where. The only feasible treatment is surgical excision, with reconstruction. Surgical treatment of choledochal cysts lipsett 2003 journal of.
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. Their presentations differ from children and surgical management has evolved. Treatment of choledochal cyst by excision sciencedirect. Choledochal cysts in adults are uncommon, and cystic dilatation of the cystic duct type vi choledochal cyst is a unique occurrence.
The risk of carcinogenesis in congenital choledochal cyst patients. Thus, jaundice, cholangitis, stone formation, biliary cirrhosis, or malignancy may develop in the future. Rarely, reconstruction above the confluence of the left and right hepatic ducts is. To date, specific pathogenic and diagnostic criteria for. Complications of choledochal cysts can happen cancer can grow in the cyst over time. A diagnosis of a congenital type 1c choledochal cyst was made in an.
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. The etiology remains unknown, but choledochal cysts are likely to be congenital in nature. Presentation and clinical outcomes of choledochal cysts in. Choledochal cysts in adults hepatobiliary disease jama surgery. We report a successful management of a unique case of perforated choledochal cyst in an infant who presented with biliary peritonitis. Choledochal cysts are congenital anomalies of the bile ducts and are defined. To identify cases of choledochal cyst in adult 12years. The treatment of choice of type i bile duct cysts in adults is total. Choledochal cyst childrens liver disease foundation. Either choledochal cysts remain clinically silent until adulthood or may.
Choledochal cyst is a problem with the tubes ducts that carry bile from your childs liver to their gallbladder and intestine. Complete excision of choledochal cyst with rouxeny hepaticojejunostomy is the treatment of choice for patients without malignancy and can be. Patients with biliary cysts may present as infants, children, or adults. Adult presentation is rare and associated diseases and complications are common. Infection of the bile ducts cholangitis inflammation of the pancreas pancreatitis stones and sludge can form in the cyst and cause abdominal pain or inflammation of the gallbladder cholecystitis. Treatment of choledochal cyst by excision by robert m. Mrcp is emerging as a highly sensitive, safe, and noninvasive diagnostic preoperative technique for the detection of choledochal cysts. A number of possible approaches exist, depending on cyst location and other factors. Most are harmless, but they should be removed when possible because they occasionally may change into malignant growths, become infected, or obstruct a gland. Choledochal cyst article about choledochal cyst by the. Surgical treatment depends on the anatomic findings and extent of biliary involvement according to the widely used todani classification.