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Lee, Ming-Yen Gillian Lieberman, MD September 2010 Oriental Cholangiohepatitis (OCH): Radiologic Features Lee, Ming-Yen, Kaohsiung Medical University, Year IV Gillian Lieberman, MD Lee, Ming-Yen Gillian Lieberman, MD Agenda • Patient presentation − Initial presentation − Radiologic findings • Oriental Cholangiohepatitis − − − − Pathogenesis Differential diagnosis Relative Roles of Imaging Tests Management 2 Lee, Ming-Yen Gillian Lieberman, MD Our Patient: Initial Presentation • 83-year-old female who has a history of having undergone cholecystectomy and choledochoduodenostomy • Apparently, over the past five years she has been having repeated episodes of cholangitis manifest as fevers and right upper quadrant abdominal pain. 3 Lee, Ming-Yen Gillian Lieberman, MD Our Patient: Biliary duct air on CT • Scattered pockets of air are seen throughout the biliary tree − consistent with the patient's prior choledochoduodenost omy. • Diffuse dilation of the intrahepatic biliary ducts C4 BIDMC PACS Lee, Ming-Yen Gillian Lieberman, MD Our Patient: biliary duct dilatation on CT • Scattered pockets of air are seen throughout the biliary tree − consistent with the patient's prior choledochoduodenost omy. • Diffuse dilation of the intrahepatic biliary ducts C+ 3mins delay 5 BIDMC PACS Lee, Ming-Yen Gillian Lieberman, MD Our patient: biliary ducts dilatation with air on coronal CT Arterial phase Venous phase 6 BIDMC PACS Lee, Ming-Yen Gillian Lieberman, MD Our patient:filling defect on ERCP • Air fills the intrahepatic biliary ducts. • There is markedly dilated left intrahepatic duct which demonstrates irregular filling. • There is an irregular filling defect at the right central intrahepatic biliary duct. BIDMC PACS 7 Lee, Ming-Yen Gillian Lieberman, MD Oriental Cholangiohepatitis Lee, Ming-Yen Gillian Lieberman, MD Oriental Cholangiohepatitis • Oriental cholangiohepatitis (OCH), also known as − Oriental cholangitis − recurrent pyogenic cholangitis − Intrahepatic pigmented stone disease • characterized by recurrent attacks of fever, chills, abdominal pain, and jaundice (Charcot's triad of acute cholangitis ) 9 Lee, Ming-Yen Gillian Lieberman, MD Oriental Cholangiohepatitis: pathogenesis • The bile ducts are markedly abnormal, characterized by extrahepatic and intrahepatic ductal dilatation with focal areas of stricturing in the intrahepatic biliary tree. • The biliary wall is fibrotic with inflammatory cell infiltration. 10 Website: The radiology assistant Lee, Ming-Yen Gillian Lieberman, MD Comparison our p’t #1: OCH on ultrasound • Extrahepatic bile ducts dilation :85-100% • Intrahepatic bile ducts dilation: 66-79% • Stones (85-90%) : are more echogenic than the liver parenchyma and adjacent tissue 11 AJR 157:1-8, July 1991 Lee, Ming-Yen Gillian Lieberman, MD Comparison our p’t #2: OCH on CT w/o contrast • Full extent of ductal dilatation − Dilatation of the extrahepatic ducts is detected clearly. − Central, larger intrahepatic biliary dilatation − bile ducts tapering abruptly toward periphery 12 AJR 157:1-8, July 1991 Lee, Ming-Yen Gillian Lieberman, MD Comparison our p’t #3: OCH on CT w/ contrast • Localized dilatation from obstruction by stricture or stone. − Often the stones are hypodense detection rate :6381% • wall of the bile ducts may enhance − acute cholangitic episode 13 AJR 157:1-8, July 1991 Lee, Ming-Yen Gillian Lieberman, MD Our patient: comparison OCH on CT w/o contrast BIDMC PACS • stones are detected more easily on unenhanced scans, and enhanced CT scans are better in the detection of subtle intrahepatic biliary dilatation. Chan F-L, Man S-W, Leong LLY, Fan S-T. Evaluation ofrecurrent pyogenic cholangitis with CT: analysis of 50 patients. Radiology 1989;170: 165-1 69 14 Lee, Ming-Yen Gillian Lieberman, MD Comparison our p’t #4: OCH on ERCP • Disproportionately severe dilatation of the extrahepatic ducts with mild or no dilatation of the intrahepatic ducts − Acute tapering − Straightening − Rigidity − Multiple focal strictures − Decrease in arborization Filling defects of stones stricture Facet stones in CBD 15 AJR 157:1-8, July 1991 Lee, Ming-Yen Gillian Lieberman, MD Our patient :ERCP • There is markedly dilated left intrahepatic duct which demonstrates irregular filling. • Irregular filling defect at the right central intrahepatic biliary duct. • Stones! Strictures! Acute tapering! BIDMC PACS 16 Lee, Ming-Yen Gillian Lieberman, MD Differential diagnosis of bile duct dilatation 17 Website: The radiology assistant Lee, Ming-Yen Gillian Lieberman, MD DDx.1: gallstones caused dile duct dilatation on CT • Gallstones passed into the extrahepatic duct − dilatation mainly proximal to the stone − In OCH: dilated diffusely regardless of the level of the stone RadioGraphics 2001; 21:3–22 18 Lee, Ming-Yen Gillian Lieberman, MD DDx2: Clonorchiasis on CT • Clonorchiasis − diffuse dilatation of the intrahepatic bile ducts with no or minimal dilatation of the large bile ducts − Periductal changes are more severe in clonorchiasis − Stones and flukes of C. sinensis can be differentiated easily. RadioGraphics, 28, 1307-1323, 19 Lee, Ming-Yen Gillian Lieberman, MD DDx3: cholangio CA. on CT • Biliary obstruction by malignant tumors − cholangiocarcinoma and cancer of the pancreas or ampulla of Vater − The entire biliary tree proximal to the mass is dilated − An obstructing mass can be detected!! Holland-Frei Cancer Medicine, bile duct cancer 20 Lee, Ming-Yen Gillian Lieberman, MD DDx4: Sclerosing cholangitis on CT • Sclerosing cholangitis − dilatation is focal and discontinuous (beaded appearance and serpiginous course) 21 The radiology assistant, Biliary Ducts : Benign and Malignant Diseases Lee, Ming-Yen Gillian Lieberman, MD DDx5: Caroli disease on CT • Caroli disease − A developmental anomaly • segmental saccular dilatation of the intrahepatic ducts • Result in stasis, cholangitis, liver abscess, and stone formation − Occurs in a younger age group − Differentiation is possible by noting the dilated saccules in the intrahepatic bile ducts The radiology assistant, Biliary Ducts : Benign and Malignant Diseases 22 Lee, Ming-Yen Gillian Lieberman, MD Imaging Tests: us + CT • Sonography − the main technique used for screening and diagnosis in suspected OCH • CT is not a screening procedure, but it is recommended when − sectional imaging is mandatory but sonography is not confirmative or is equivocal − when space-occupying lesions complicate OCH − when hepatic resection is planned − When imaging guidance is needed for complex drainage procedures 23 Lee, Ming-Yen Gillian Lieberman, MD Imaging Tests: Direct cholangiography • Direct cholangiography − “Road map” in patients undergoing surgical intervention − Necessary for the detection of residual stones after surgery − Assessment of biliary stricture and choledochoenteric fistulas − Preprocedural biliary intervention 24 Lee, Ming-Yen Gillian Lieberman, MD Mangement of our patient • Treatment of acute complications ,such as cholangitis − fluid resuscitation, antibiotics, and biliary drainage. • Prevention of the long-term complications − Clearance of stones − Hepatic resection 25 Lee, Ming-Yen Gillian Lieberman, MD Reference • Jae Hoon Lim, Oriental Cholangiohepatitis: Pathologic, Clinical, and Radiologic Features,AJR 157:1-8, July 1991 • Uptodate, Oriental cholangiohepatitis • Chan F-L, Man S-W, Leong LLY, Fan S-T. Evaluation ofrecurrent pyogenic cholangitis with CT: analysis of 50 patients. Radiology 1989;170: 165-1 69 • Gillian Lieberman, MD, G. Lieberman’s Primary Care Radiology. http://eradiology.bidmc.harvard.edu • Cheng YF; Lee TY; Sheen-Chen SM; Huang TL; Chen TY, Treatment of complicated hepatolithiasis with intrahepatic biliary stricture by ductal dilatation and stenting: long-term results, World J Surg 2000 Jun;24(6):712-6. • Mary Ann Turner, MD, Ann S. Fulcher, MD, The Cystic Duct: Normal Anatomy and Disease Processes, RadioGraphics 2001; 21:3–22 • Donald W Kufe, MD,Raphael E Pollock, et al. Holland-Frei Cancer Medicine,6th edition, Section 28: Gastrointestinal Tract, Bile Duct Cancer, http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed6 • The radiology assistant, http://www.radiologyassistant.nl, Biliary Ducts : Benign and Malignant Diseases, Angela D. Levy MDChief Gastrointestinal Radiology, University Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington DC 26 Lee, Ming-Yen Gillian Lieberman, MD Acknowledgements • Gillian Lieberman, MD • Robert Sheiman, MD • Girish Tyagi, MD • Leo Tsai, MD, PhD, MSc • David Glazier, MD • Elizabeth Asch, MD • Wabmaster, Larry Barbaras • Emily Hanson Acting Medical Student Education Coordinator 27 Lee, Ming-Yen Gillian Lieberman, MD Thanks for your attention….