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Choledochal malformation
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Mark Davenport, Nguyen Thanh Liem
Although the traditional term “choledochal cyst” is still frequently overused, irrespective of the actual choledochal morphology and shape, this practice should be avoided. Congenital choledochal malformation (CCM) is a better term for the spectrum of biliary abnormalities characterized by dilatation. The incidence of CCM is not known though it is clearly much more common in East Asian countries and may even be increasing. All the common types have a female predominance.
Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Which choledochal cyst distribution is consistent with this diagnosis?Dilatation of distal extrahepatic duct within the duodenal wallDilatation of the intrahepatic ductsDiverticulum of the extrahepatic ductExtrahepatic duct dilatationIntra- and extrahepatic duct dilatation
Normal and Abnormal Development of the Biliary Tree
Published in Gianfranco Alpini, Domenico Alvaro, Marco Marzioni, Gene LeSage, Nicholas LaRusso, The Pathophysiology of Biliary Epithelia, 2020
The pathogenesis of choledochal cysts is unknown. With the lack of a pattern of inheritance and no relationship to ductal plate development, there is little upon which to speculate.
Type 1 Choledochal Cyst with Ectopic Pancreas and Septate Gallbladder
Published in Fetal and Pediatric Pathology, 2022
Amir-Hossein Akbari, Juan Putra
Choledochal cysts (CCs) are cystic dilatation of the biliary tract which result from congenital developmental abnormalities of the bile duct system (1). The frequency on CCs is 1 in 100,000–150,000 live births in western population (1). They are 4 times more common in females than males (1). CCs are classified based on the anatomical location of biliary duct dilation into 5 types (I–V) (2). The most common type is type I (50–80%), followed by types IV (15–35%), V (20%), III (4.5%) and II (2%) (2). Clinically, CCs present as right upper quadrant mass, abdominal pain and occasionally jaundice (3). Complications include pancreatitis, cholangitis, spontaneous rupture of cyst and cholangiocarcinoma (reported in 5–10% of the cases) (4, 5). The mainstay of treatment is complete surgical resection (5). CCs are associated with a myriad of different developmental anomalies such as colonic atresia, duodenal atresia, multiseptated gallbladder, and pancreatic divisum (6–11). To our knowledge there is only one published case of co-occurrence of CCs with ectopic pancreas and septate gallbladder (8). Here we present the clinicopathologic findings of another case demonstrating this co-occurrence.
Cholangiocarcinoma: novel therapeutic targets
Published in Expert Opinion on Therapeutic Targets, 2020
Keisaku Sato, Shannon Glaser, Domenico Alvaro, Fanyin Meng, Heather Francis, Gianfranco Alpini
Various factors are associated with CCA development. Liver fluke infections caused by parasites such as Opisthorchis viverrini or Clonorchis sinensis are a common risk factor for CCA especially in Southeast Asia [4]. Parasite infection often induces hepatolithiasis, which is the existence of gallstones in the bile ducts and increases the risk of CCA [7]. Since CCA is a biliary tract cancer derived from the biliary tree, biliary disorders such as primary sclerosing cholangitis (PSC) are risk factors for CCA development [8]. Inflammatory bowel disease (IBD) is closely associated with PSC and often co-exists which is referred to as PSC-IBD. PSC-IBD patients are at risk for CCA development, and the long duration of IBD is associated with the increased risk of CCA [9]. Patients with choledochal cysts also have a high risk of CCA [4,10]. Chronic hepatitis and cirrhosis caused by a viral infection such as hepatitis B or C virus have been recognized as the major risk factor, especially for intrahepatic CCA [3,10]. Genetic traits that induce mutations, polymorphisms, or genetic aberrations may increase the risk of CCA development [11]. Other reported factors include obesity, smoking, and alcohol drinking [10]. Previous studies performed in the US and China have found that metabolic syndrome increases the risk of CCA [12,13]. The incidence of CCA has been increasing worldwide in recent years as evidenced by increased patients with obesity or metabolic syndrome, suggesting that CCA is a growing health concern [14].
Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy
Published in Fetal and Pediatric Pathology, 2019
Santosh Kumar Mahalik, Suvradeep Mitra, Susama Patra, Kanishka Das
Biliary atresia (BA) is the commonest cause of surgically correctable infantile obstructive cholangiopathy. It typically involves the extrahepatic biliary tree but the intrahepatic biliary tree also shows progressive fibro-obliterative changes, the latter limiting the success of Kasai portoenterostomy. Cystic biliary atresia (cBA) is a variant that accounts for 5–10% of all BA, has fewer associated congenital anomalies and an overall better prognosis than typical BA [1]. Choledochal cyst (CC) is a cystic dilatation of the extrahepatic and/or intrahepatic biliary tree without atretic changes in the biliary channels. The pathogenesis of BA, cBA, and CC is elusive and proposed theories include immune dysregulation, viral infections, developmental anomaly and ciliopathy [1]. CC and BA are sometimes considered to be different stages in the spectrum of the same disease. We detail a case of congenital biliary dilatation and infantile obstructive cholangiopathy with clinical, radiological, and histomorphological features of both CC and BA.