Caroli syndrome

Harjai, MM; Bal, R K

doi:10.1007/s003839900323

Cited by 18 publications

(13 citation statements)

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“…If the large IBDs are affected, the result is Caroli disease, whereas abnormal development of the small interlobular bile ducts results in congenital hepatic fibrosis. If all levels of the intrahepatic biliary tree are involved, features of both congenital hepatic fibrosis and Caroli disease are present; this condition has been termed Caroli syndrome (43,44).…”

Section: Biliary Systemmentioning

confidence: 99%

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Multimodality Imaging of Pancreatic and Biliary Congenital Anomalies

Mortelé¹,

Rocha²,

Streeter³

et al. 2006

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A wide spectrum of anomalies of the pancreas, the pancreatic ductal system, and the biliary tree are commonly encountered at radiologic evaluation. These anomalies may simulate various neoplastic, inflammatory, and posttraumatic conditions and should be part of the differential diagnosis for a variety of abnormalities found at diagnostic imaging. Anatomic variants, developmental anomalies (eg, pancreas divisum, annular pancreas, ectopic pancreas, pancreatic agenesis and hypoplasia), and congenital diseases (congenital pancreatic cysts, von Hippel-Lindau disease, choledochal cysts), in addition to potential imaging pitfalls (uneven distribution of fat, "pseudomasses"), can all pose a diagnostic challenge for the radiologist. Familiarity with these anomalies, the imaging techniques available for their study, and their variable imaging manifestations is necessary for differentiating them from other biliary and pancreatic conditions. A basic understanding of the embryologic development and normal anatomy of the pancreas and biliary tree is also essential for identifying these anomalies.

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Section: Biliary Systemmentioning

confidence: 99%

“…If all levels of the intrahepatic biliary tree are involved, features of both congenital hepatic fibrosis and Caroli disease are present; this condition has been termed Caroli syndrome (43,44).…”

Section: Page 721mentioning

confidence: 99%

Multimodality Imaging of Pancreatic and Biliary Congenital Anomalies

Mortelé¹,

Rocha²,

Streeter³

et al. 2006

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278

174

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“…21 In the majority of published cases, children with CS have an early onset of symptoms and a rapidly progressive disease, which is caused by the combination of cholangitis and portal hypertension due to CHF. [22][23][24] In contrast, CD is thought to have its clinical manifestation in patients older than 20-year old.…”

Section: General Aspects Of Caroli Disease or Syndromementioning

confidence: 99%

Long-Term Outcome of Liver Resection and Transplantation for Caroli Disease and Syndrome

Ulrich

Pratschke²,

Pascher³

et al. 2008

Annals of Surgery

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“…If concomitant renal failure ensues from the dysplastic kidneys, liver transplantation combined with renal transplantation might be warranted. Acute cholangitis is treated by prompt application of antibiotics and percutaneous transhepatic biliary drainage in adults, with a complication rate of 7-40%,9,10 but its efficacy in infants of our patient's age is not known. Ursodeoxycholic acid is a useful treatment of primary hepatolithiasis in patients with Caroli's syndrome 11…”

Section: Discussionmentioning

confidence: 99%