Gore RM, Ghahremani GG et al. Anomalies and - Department of ...

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f ~I ., , :< " I c , '(,II ~I ! . ..... Figure 78-8. Type I choledochal cysts. A. Coronal oblique multiplanar reformatted image shows fusiform dilatation of the common bile duct (arrow). Note also the dilatation of the intrahepatic biliary tract (arrowhead). B. Percutaneous transhepatic cholangiogram shows a large choledochal cyst (C) at the level of the extrahepatic bile duct. Note the aberrant entry of the common bile duct at the side of the pancreatic duct (arrowhead). C. Photograph shows an excised type 1 choledochal cyst of the common bile duct (arrow) in continuity with the cystic duct and the gallbladder (arrowhead).Scale is in centimeters. D. MRCP dilated main common bile duct (arrow). (C from Brancatelli G, Federle Mp, Vilagrain V, et al: Fibropolycystic liver disease: CT and MR imaging findings. RadioGraphies 25:659-670, 2005. o from Mortele KR, Rochar TC, Stretter JL, et al: Multimodality imaging of pancreatic and biliary congenital anomalies. RadioGraphies 26:715-731,2006.)
.i 1 'I ii !I " I~ r~ I 'I il il il :1 " i~ hepatic ducts, stones, strictures, and communicating hepatic abscesses. CT can also demonstrate tiny dots with strong contrast enhancement within dilated intrahepatic bile ducts (the "central dot" sign). These intraluminal dots correspond to intraluminal portal veins.64.68 CT and ultrasound demonstrate multiple cystic areas within the liver69-71(Fig. 78-11). Technetium Tc 99m sulfur colloid scans show multiple cold defects, and hepatobiliary scans show an unusual pattern of retained activity throughout the liver.54,55 MRCP with three-dimensional display is an accurate method for demonstrating Caroli's disease because the luminal contents of the bile ducts appear hyperintense in contrast to the portal vein, which usually appears as signal void. Cystic expansions of the intrahepatic biliary tract are depicted as oval-shaped structures in continuity with the biliary tract (Fig. 78-12). They are nearly signal void on black bile techniques and have a high signal intensity on bright bile or MR cholangiopancreatography sequences.72 Treatment depends on the clinical features and location of the biliary abnormality. When the disease is localized to one hepatic lobe, hepatectomy relieves symptoms and appears to remove the risk of malignancy. In diffuse Caroli's disease, treatment options include conservative or endoscopic therapy, internal biliary bypass procedures, and liver transplantation in carefully selected cases. Figure 78-11. Caroli's disease: CT findings. The dilated segments of the intrahepatic biliary tract may be visualized as "cysts" (straight arrows), which are occasionally attached to more proximal ectatic segments of the biliary radicles (curved arrow). The defining CT feature of Caroli's disease is the central dot sign (open arrow). There is ectasia of the distal nephrons in the kidneys. (From Taylor AJ, Bohorfoush AG: Interpretation of ERCP with Associated Digital Imaging. Philadelphia, Lippincott- Raven, 1997, p 52, with permission.) Figure 78-12. Caroli's disease: MR findings. A. Coronal oblique MR cholangiopancreatography demonstrates multiple segmental saccular dilatations of the intrahepatic bile ducts (arrows). Curved arrow, common bile duct. B. Coronal half-Fourier rapid acquisition with relaxation enhancement (RARE) image of the kidneys shows multiple fluid-containing foci (arrows) in the papillae indicating renal tubular ectasia (medullary sponge kidney). References 1. Fitoz S, Erden A, Bomban S: Magnetic resonance cholangiopancreatography of biliary system abnormalities in children, Clin Imaging 31:93-101,2007. 2. Savader SI, Venbrux AC, Faerber AC, et al: Biliary tract anomalies, congenital and neonatal disorders, and hepatobiliary cystic malformations. In Friedman AC, Dachman AH (eds): Radiology of the Liver, Biliary Tract, and Pancreas. SI. Louis, CV Mosby, 1994, pp 397 -444. 3, Kamath BM, Piccoli DA: Heritable disorders of the bile ducts, Gastroenterol Clin North Am 32:857-875,2003. 4. Taylor AI, Bohorfoush AG: Interpretation of ERCP with Associated Digital Imaging Correlation, Philadelphia, Lippincott-Raven, 1997, pp 41-58. 5. Bader TR, Semelka RC, Reinhold C: Gallbladder and biliary system. In Semelka RC (ed): Abdominal.Pelvic MRI. New York, Wiley-Liss, 2002, pp 319.372. 6, Lack EE: Pathology of the Pancreas, Gallbladder, Extrahepatic Biliary Tract, and Ampullary Region. Oxford, Oxford University Press, 2003, pp 395-413. 7. Haller JO, Slovis TL: Pediatric gallbladder and biliary tract: Sonographic evaluation. Ultrasound Q 9:271-311,1992. I '.
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