Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings (original) (raw)

Choledochal cysts: presentation, clinical differentiation, and management

Journal of the American College of Surgeons, 2014

Choledochal cysts (CC) are a rare congenital cystic dilation of the biliary tract, first described by Vater and Ezler in 1723. 1 They present primarily in female infants and young children and are more prevalent in East Asian populations. Although benign, CC can be associated with serious complications including malignant transformation, cholangitis, pancreatitis, and cholelithiasis. 2 We herein provide a state-of-the-art, evidence-based review of CC with particular emphasis on clinical differentiation and approach to management. A search of the available electronic databases, including MEDLINE/ Pubmed, using the term choledochal cyst as well as under the MeSH database subheading choledochal cyst, was performed. Criteria for inclusion included English articles (Fig. 1). Incidence and epidemiology Approximately 80% of CC are diagnosed in infants and young children within the first decade of life. 3,4 The incidence of CC ranges from 1 in 100,000 to 1 in 150,000 individuals in Western countries 5 to 1 in 13,000 individuals in Japan. 6 Choledochal cysts are 4 times more common in females. 2,7,8 Although the exact etiology is unknown, anomalous pancreaticobiliary duct union (APBDU) is seen in 30% to 70% of all CC where the common bile duct (CBD) and pancreatic duct junction occurs outside the duodenum, allowing reflux of pancreatic fluid into the biliary tree. 9-13 The exposure of biliary epithelium to digestive and caustic pancreatic enzymes may contribute to CC formation. In 1969, Babbitt 14 initially described APBDU, and it is believed to be secondary to arrest in migration of the choledochopancreatic junction into the duodenal wall, leading to a long common channel

Choledochal cysts: lessons from a 20 year experience

Archives of Disease in Childhood, 1995

Cystic dilatation of the biliary tree is a rare congenital anomaly. To determine mode of presentation, diagnostic pitfalls, and long term outcome after surgery, 78 children (57 girls, 21 boys) with choledochal cyst treated between 1974 and 1994 were reviewed. Anatomical types were: Ic (n=44), If (n=28), IVa (n=4), and V (n=2); a common pancreaticobiliary channel was identified in 76% patients. Age at presentation ranged from 0-16 (median 2.2) years, six patients being diagnosed by prenatal ultrasonography. Of the 72 patients diagnosed postnatally, 50 (69%) presented with jaundice, associated with abdominal pain in 25 or a palpable mass in three, 13 (18%) presented with pain alone, and two (30/o) with a palpable mass. The classic triad of jaundice, pain, and a right hypochondrial mass was present in only four (6%). Four children presented acutely after spontaneous perforation of a choledochal cyst, two presented with ascites and one cyst was discovered incidentally. Plasma and/or biliary amylase values were raised in 30 of 31 patients investigated for abdominal pain; seven had evidence of pancreatitis at operation. In 35 of 67 (52%) patients referred without previous surgery, symptoms had been present for more than one month, and in 14 of them for more than one year, before diagnosis. Delayed referral was due to misdiagnosis as hepatitis (n= 12), incomplete investigation of abdominal pain (n=6), and failure to note the significance of ultrasonographic findings (n= 10). Two patients referred late died from liver failure. Of the 76 patients with type I or IV cysts, 59 underwent radical cyst excision and hepaticojejunostomy as a primary procedure and 10 as a secondary operation after previously unsuccessful surgery. Sixteen patients have been lost to follow up but most of the remainder are well after a mean period of 4-1 (0.1-13) years. Choledochal cysts are often misdiagnosed, but prognosis is excellent if radical excision is performed. (Arch Dis Child 1995; 73: 528-531)

Choledochal cysts: diagnosis and treatment

2012

The aim of this study is to show the different diagnostic procedures and treatment in patients diagnosed with congenital choledochal cysts. Choledochal cysts are congenital anomalies of the bile ducts and include cystic dilatation of the extrahepatic and intrahepatic biliary ducts or both. The study shows ten patients diagnosed as having choledochal cysts. Diagnosis was established by clinical and radiographic findings including: ultrasound (US), magnetic resonance cholangiopancreatograpy (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTC) and cytological examination of the bile juice. In the study choledochal cysts were classified according to the Todani classification. Most common cysts were type I (six cases); type III (one case), type IVa (one case) and two patients were type V cysts (Caroli disease). The most frequent symptoms were abdominal pain, jaundice and cholangitis. US findings were sensitive for the preliminary ...

Choledochal Cyst in Adults: Etiopathogenesis, Presentation, Management, and Outcome—Case Series and Review

Gastroenterology Research and Practice, 2015

Background. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in adults. Their presentations differ from children and surgical management has evolved. Methods. A retrospective review of the records of all the patients above 15 years, who underwent therapeutic intervention in our hospital, was carried out. Results. Ten cases of choledochal cyst were found; 8 female, with mean age 31 years. These included 8 cases of Todani type I and one case each of type II and type III. The predominant symptoms were abdominal pain and jaundice. Abdominal mass and past history of cholangitis and pancreatitis were seen in 2 patients. Investigations included ultrasound in 8 patients, CT in 7, ERCP in 3, and MRCP in 5. Surgical intervention included complete excision of the cyst with hepaticojejunostomy and cholecystectomy (type I), excision of the diverticulum (type II), and ERCP sphincterotomy (type III). Malignancy was not seen in any patients. The long-term postoperative complications included cholangitis in two patients. Conclusion. Choledochal cyst is rare in adults. The typical triad of abdominal pain, jaundice, and mass is uncommon in adults. The surgical strategy aims for single stage complete excision of the cyst with hepaticojejunostomy.

Choledochal cyst in pediatric patients: a 10 years single institution experience

Acta gastroenterologica Latinoamericana

Choledochal cysts (CCs) are rare congenital dilatations of the biliar tree. The incidence is 1:150,000. A correct diagnosis and surgical treatment are important because of the long term risks of infection and neoplasia. We report our experience with CCs in children. Patients with CC disease, who were seen at the Hospital Italiano de Buenos Aires between 1999 and 2009, were identified retrospectively from our prospectively acquired hepato-pancreatico-biliary database. Prenatal, clinical, surgical and pathological records were analysed. We included 12 children (10 girls and 2 boys), all of them affected by a cystic dilatation type I of Todani classification. Between the 4 patients with antenatal diagnosis only one presented symptoms before surgery, expressed in acolic feces. Between the 8 patients with postnatal diagnosis, the most common clinical presentation was abdominal pain, occurring in all patients. Ultrasound was used for the diagnosis of all patients. Mean age at surgery was ...

Choledochal cyst in pediatric patients: a 10-year single institution experience

Acta gastroenterologica Latinoamericana, 2011

Unusual Presentations of Choledochal Cyst: Case Series and Review of Literature

Indian Journal of Surgery, 2014

Congenital cystic dilation of the biliary duct is defined as choledochal cyst (CDC). CDC presenting with the classical triad of abdominal pain, lump, and jaundice is s e e n i n o n l y 6 % c a s e s. C y s t e x c i s i o n w i t h hepaticojejunostomy is the standard treatment worldwide. We hereby report five cases of CDC with unusual presentations (gastric outlet obstruction, cyst perforation, giant cystolithiasis, giant cyst, and mixed type) and discuss the challenges faced during the diagnosis and perioperative management of these cases. diagnosis and management. Herein, we report five cases of CDC who had unusual features on presentation. Case Series Of the 29 cases of CDC managed between January 2009 and December 2013, 5 (17.2 %) cases had unusual presentation. Case 1 A 12-year-old girl presented with history of pain in the abdomen for 2 years and recurrent bilious vomiting for 7-months duration. Upper gastrointestinal endoscopy showed dilated stomach with external compression at the antropyloric region. Subsequently, contrast-enhanced computed tomography (CECT) was done, which showed type I CDC with gastric outlet obstruction (Fig. 1a). On exploration, dilated stomach with a large CDC (10×7 cm) compressing the antropyloric region was present (Fig. 1b). CDC excision with Roux-en-Y hepaticojejunostomy (RYHJ) was performed. Postoperative course was uneventful and patient is doing well after a follow up of two and half years. Case 2 A 16-year-old female presented with complaints of abdominal pain for 2 months. Abdominal ultrasonography (USG) revealed a dilated common bile duct (CBD) suggesting CDC. Though surgery was advised on index admission, patient refused the same due to personal reasons. One month later, she presented with jaundice, fever, epigastric lump, and features of local peritonitis. Liver function test showed raised serum bilirubin, 17.25 mg/dl (normal range 0-1.4 mg/dl) and liver enzymes (serum aspartate transaminase, AST), 174 IU/L

Choledochal cysts in western adults: complexities compared to children

Journal of Gastrointestinal Surgery, 2004

Choledochal cysts occur most frequently in East Asian children and rarely in Western adults. Over the past two decades, pediatric treatment has been standardized, but relatively little information is available on the management of Western adults with choledochal cysts. Therefore the aims of this analysis were to compare the presentation, management, and late results of Western adults and children with choledochal cysts. Records were reviewed of patients with choledochal cysts at three academic institutions in Wisconsin. Fifty-seven patients were identified, and 51 of these patients (89%) were managed surgically. Thirtyone patients (54%) were adults, and the adults were more likely to be male (29% vs. 4%, P Ͻ 0.02). Pain (81% vs. 42%, P Ͻ 0.01) and cholangitis (35% vs. 15%) were more common in adults. Forty-one patients (71%) had type I cysts, but type IVa or V cysts with dilated intrahepatic ducts were more common in adults (39% vs. 15%, P ϭ 0.05). Seventeen adults had undergone biliary surgery prior to referral compared to only four children (59% vs. 15%, P Ͻ 0.01). Preoperative endoscopic or percutaneous stents were employed more commonly in adults (42% vs. 15%, P Ͻ 0.01). Hospital mortality was 0%, and morbidity was low in both adults and children (25% vs. 8%). An associated biliary malignancy correlated with age (P Ͻ 0.05): 0 to 30 years (0%), 31 to 50 years (19%), and 51 to 70 years (50%). In addition, adults were more likely to have late problems with cholangitis (19% vs. 4%, P Ͻ 0.07) and secondary biliary cirrhosis (13% vs. 4%). This analysis suggests that compared to children, Western adults with choledochal cysts are more likely to have (1) type IVA or V cysts, (2) undergone prior surgery, (3) preoperative biliary stents, (4) an associated biliary malignancy, and (5) late hepatobiliary problems. We conclude that surgery in Western adults with choledochal cysts is frequently complicated and should be performed by specialists in complex biliary surgery. ( J GASTROINTEST SURG 2004;8:245-252) Ć 2004 The Society for Surgery of the Alimentary Tract

Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings (original) (raw)

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