复发性化脓性胆管炎的影像学诊断

Recurrent Pyogenic Cholangitis James Wilkins Harvard Medical School, year III Gillian Lieberman, MD July 19, 2010 Outline • Overview of recurrent pyogenic cholangitis (RPC). • Anatomy of the biliary tree. • Review of imaging modalities useful in the  diagnosis of RPC. • Review and evaluation of index patient. Recurrent Pyogenic Cholangitis (RPC) • Characterized by intrahepatic pigment stones,  intrahepatic biliary duct dilatation and strictures,  and recurrent bouts of cholangitis. – Typically does not involve the gallbladder. – Presents clinically with Charcot’s triad (fevers, jaundice,  and right upper quadrant (RUQ) abdominal pain). • Common problem in East and Southeast Asia. – Increasing prevalence in the US due to migration from  endemic regions. • Diagnosis is based on clinical presentation in  conjunction with imaging findings.  Pathogenesis of RPC • Pathogenesis is incompletely understood. • Possible etiologic role for biliary helminths, enteric  superinfection,  and malnutrition. – Biliary helminths, such as Ascaris lumbricoides and Clonorchis  sinensis, induce ductal injury and inflammatory response. – Secondary infection with enteric bacteria leads to pigment stone formation and recurrent cholangitis. – Enteric bacteria with beta‐glucuronidase activity enter the biliary  system via the portal vein and cause deconjugation of bilirubin  glucuronide, leading to precipitation of bilirubin‐pigment stones. – Low protein diet leads to decreased levels of an endogenously  produced inhibitor of beta‐glucuronidase activity (glucaro‐1:4‐ lactone), leading to increased susceptibility to pigment stone  formation.   Anatomy of the Biliary Tree http://usmlemd.files.wordpress.com/2008/10/biliary20tract.jpg Menu of Imaging Modalities for RPC • Ultrasonography (U/S) • Computed Tomography (CT) • Endoscopic Retrograde  Cholangiopancreatography (ERCP) • Magnetic Resonance Cholangiopancreatography  (MRCP) Ultrasonography in RPC • First line investigation. • Characteristic findings: dilatation of the biliary  tree and intrahepatic calculi (seen in up to 90% of  patients). – Calculi show variable calcification leading to variable  echogenicity and acoustic shadowing. • Advantages: Useful for diagnosis and follow‐up,  useful for needle‐guided aspiration and core  biopsies, no ionizing radiation, non‐invasive. • Disadvantages: Findings can be obscured by  pneumobilia, operator dependent. Patients 1 and 2: RPC with U/S Heffernan, E. J. et al. Am. J. Roentgenol. 2009;192:W28-W35. Patient 1: U/S. Legend: Acoustic shadowing, intrahepatic calculi in left hepatic duct. Patient 2: U/S. Legend: Dilatation of common hepatic duct. CT in RPC • Most important non‐invasive study for  diagnosis. • Characteristic findings: intrahepatic biliary duct  dilatation, intrahepatic calculi, pneumobilia,  hepatic parenchymal processes (e.g. atrophy,  abscesses, bilomas), and cholangitis. • Advantages: provides a complete picture of the  disease process. • Disadvantages: ionizing radiation, contrast  allergy. Patients 3 and 4: RPC with CT Heffernan, E. J. et al. Am. J. Roentgenol. 2009; 192: W28-W35. Hurtado, R. M. et al. N. Engl. J. Med. 2006; 354:1295-1303. Patient 3: C- axial CT of the abdomen. Legend: Calcified calculi within intrahepatic biliary ducts. Patient 4: C+ axial CT of the abdomen. Legend: Dilatation of left hepatic duct with multiple filling defects. ERCP in RPC • Direct cholangiography. • Characteristic findings: duct dilatation, calculi,  intrahepatic strictures, and decreased  arborization of the biliary tree. • Advantages: Better spatial resolution than  MRCP, allows for therapeutic intervention. • Disadvantages: May not depict all of biliary  tree, may precipitate septic shock. Patients 5 and 6: RPC with ERCP Chung, R. T. et al. N. Engl. J. Med. 2001; 345: 817-823. Heffernan, E. J. et al. Am. J. Roentgenol. 2009; 192: W28-W35. Patient 5: ERCP obtained with use of an occlusion balloon. Legend: Multiple calculi within the left hepatic duct, ductal stricture. * ** * Patient 6: ERCP. Legend: Decreased arborization of right biliary ducts, indicating obstruction or stricture. MRCP in RPC • Performed using heavily T2‐weighted images. – Bile‐filled structures appear hyperintense against a  hypointense background. • Characteristic findings: calculi, intrahepatic biliary  duct dilatation and strictures, and hepatic  parenchymal processes (hepatic abscesses are  hypointense on T1 and hyperintense on T2). • Advantages: Allows visualization of ducts proximal  to an obstruction, non‐invasive, non‐ionizing  radiation, contrast not required, allows for 3D  reconstruction of biliary tree. • Disadvantages: Does not allow for immediate  therapeutic intervention. Patients 7 and 8: RPC with MRCP Heffernan, E. J. et al. Am. J. Roentgenol. 2009; 192: W28-W35. Hurtado, R. M. et al. N. Engl. J. Med. 2006; 354:1295-1303. Patient 7: Thick-slab reconstruction of a T2-weighted MRCP. Legend: Obstructing calculus in left hepatic duct. Patient 8: T2-weighted MRCP. Legend: Multiple dark filling defects, indicating intrahepatic calculi. Our Patient: Clinical Presentation • 83 year old Chinese female with repeated  episodes of RUQ abdominal pain, fevers, and  nausea/vomiting. • Laboratory values are unremarkable except  for moderately elevated alkaline phosphatase. • Past surgical history is remarkable for an open  cholecystectomy approximately 50 years ago  and a choledochoduodenostomy. ACR Appropriateness Criteria for  RUQ Pain http://www.acr.org/secondarymainmenucategories/quality_safety/app_criteria.aspx Our Patient: U/S Findings • Summary of pertinent findings from U/S. – A large quantity of air was observed within the  biliary system. – Limited visualization of the liver due to  hyperechogenic air. – Further assessment by CT recommended. Our Patient: Initial CT Evaluation Our Patient: C+ axial CT of the abdomen in portal venous phase; PACS, BIDMC. Legend: Stone in intrahepatic biliary duct, Cystic biliary dilatation, pneumobilia, Spleen, Aorta, Stomach, Nodular, heterogeneous liver, Periductal enhancement, consistent with cholangitis. * * * * * Differential Diagnosis Based on CT  Findings • Simple obstructive cholangitis – Choledocholithiasis – Benign biliary stricture – Cholangiocarcinoma – External compression (hepatocellular carcinoma,  pancreatitis) • Primary sclerosing cholangitis – Ongoing inflammation leading to irregularly narrowed  bile ducts; beaded appearance. • Caroli disease – Congenital cystic dilatation of intrahepatic bile ducts. • Recurrent pyogenic cholangitis Differential Diagnosis Based on CT  Findings • Simple obstructive cholangitis – Choledocholithiasis – Benign biliary stricture – Cholangiocarcinoma – External compression (hepatocellular carcinoma,  pancreatitis) • Primary sclerosing cholangitis – Ongoing inflammation leading to irregularly narrowed  bile ducts; beaded appearance. • Caroli disease – Congenital cystic dilatation of intrahepatic bile ducts. • Recurrent pyogenic cholangitis Our Patient: Calculi, Pneumobilia,  and Biliary Duct Dilatation on CT Our Patient: C+ axial CT of the abdomen in arterial phase; PACS, BIDMC. * Legend: Stone in intrahepatic biliary duct, Cystic biliary dilatation, with air fluid levels, Nodular, heterogeneous liver. * Our Patient: Intrahepatic calculi on  CT Our patient: C+ sagittal reconstruction CT of the abdomen in portal venous phase; PACS, BIDMC.Legend: Intrahepatic calculi. ** * Our patient: C+ coronal reconstruction CT of the abdomen in arterial phase; PACS, BIDMC. Legend: Intrahepatic calculus. * Our patient also had a number of  ERCPs to visualize her biliary  anatomy and to remove obstructing  calculi. Our Patient: Multiple Filling  Defects on ERCP Our Patient: ERCP; PACS, BIDMC. Legend: Dilated common bile duct, Filling defects consistent with calculi in the left hepatic duct. Our patient: Filling defect in the  Right Hepatic Duct on ERCP Our Patient: ERCP; PACS, BIDMC. Legend: Filling defect consistent with a calculus in the right hepatic duct. Our Patient: Stricturing of Biliary  Radicles on ERCP Our Patient: ERCP; PACS, BIDMC. Legend: Strictures within intrahepatic biliary radicles. Our Patient: Strictures within the  Left Hepatic Duct on ERCP Legend: Dilated common bile duct, Stricturing within the left hepatic duct. Our Patient: ERCP; PACS, BIDMC. Our Patient: Heterogeneous Mass  on CT Legend: On follow-up CT scan, a heterogeneous, irregular mass was noted within the caudate lobe of the liver. The mass was found to be isodense on non-contrast CT and in the arterial phase (C+) CT but heterogeneously enhancing in the portal venous phase (C+) as shown in this image. This mass was concerning for cholangiocarcinoma. Our Patient: C+ axial CT of the abdomen in portal venous phase; PACS, BIDMC. Cholangiocarcinoma in Patients with  RPC • Incidence of cholangiocarcinoma in patients with RPC is  1.5‐11%. • Chronic bacterial infection leads to chronic proliferative  cholangitis, which in turn leads to atypical epithelial  hyperplasia and cholangiocarcinoma. • Elevation of CA19‐9 in most cases. • Characteristic findings on CT – Predominately hypoattenuating with irregular margins. – Variable enhancement after contrast administration. • No enhancement, minimal peripheral enhancement, or central  enhancement. – Delayed enhancement with increasing attenuation in ¾ of  patients. Our Patient: Biopsy of Biliary  Mucosa via ERCP • Biopsy of the irregular  mass revealed biliary  mucosa with chronic  inflammation and  granulation tissue. • There was no evidence  for cholangiocarcinoma. • CA19‐9 was within  normal limits. Our patient: Intraductal biopsy of mucosa at the takeoff of the right hepatic duct was performed with cold forceps via ERCP; PACS, BIDMC. Summary • Recurrent pyogenic cholangitis is a disease of the  intrahepatic biliary system, characterized by  intrahepatic pigment stones, strictures and dilation of  the intrahepatic biliary tree, and recurrent cholangitis. • Useful imaging modalities include U/S, CT, ERCP, and  MRCP. • We saw a patient with RPC who presented with RUQ  pain and fevers, and we reviewed the imaging findings,  which included multiple intrahepatic calculi on CT and  ERCP, dilatation and stricturing of the intrahepatic  biliary tree on CT and ERCP, and periductal  enhancement on CT, consistent with cholangitis. 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Soehnlein SL, Jacobson DR. Cholangitis, Recurrent Pyogenic. http://emedicine.medscape.com/article/365322‐ overview. Updated January 15, 2010. Acknowledgements • Mai‐Lan Ho, MD. • Gillian Lieberman, MD. • Maria Levantakis. Recurrent Pyogenic Cholangitis Outline Recurrent Pyogenic Cholangitis (RPC) Pathogenesis of RPC Anatomy of the Biliary Tree Menu of Imaging Modalities for RPC Ultrasonography in RPC Patients 1 and 2: RPC with U/S CT in RPC Patients 3 and 4: RPC with CT ERCP in RPC Patients 5 and 6: RPC with ERCP MRCP in RPC Patients 7 and 8: RPC with MRCP Our Patient: Clinical Presentation ACR Appropriateness Criteria for RUQ Pain Our Patient: U/S Findings Our Patient: Initial CT Evaluation Differential Diagnosis Based on CT Findings Differential Diagnosis Based on CT Findings Our Patient: Calculi, Pneumobilia, and Biliary Duct Dilatation on CT Our Patient: Intrahepatic calculi on CT Our patient also had a number of ERCPs to visualize her biliary anatomy and to remove obstructing calculi. Our Patient: Multiple Filling Defects on ERCP Our patient: Filling defect in the Right Hepatic Duct on ERCP Our Patient: Stricturing of Biliary Radicles on ERCP Our Patient: Strictures within the Left Hepatic Duct on ERCP Our Patient: Heterogeneous Mass on CT Cholangiocarcinoma in Patients with RPC Our Patient: Biopsy of Biliary Mucosa via ERCP Summary References Acknowledgements