Episode 1
October 2023
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Presenter:
Hawwa Alao, MD
Advanced Endoscopy Fellow
Harbor UCLA Medical Center, Torrance CA, US
31 y/o male with ulcerative colitis and presumed primary sclerosing cholangitis (PSC) presented with RUQ pain, chills, vomiting, and abnormal hepatic panel. CT abdomen and pelvis with contrast showed cholelithiasis, distended gallbladder with pericholecystic fluid, and decreased enhancement of the gallbladder wall, concerning for acute cholecystitis with gallbladder wall ischemia consistent with gangrenous/hemorrhagic cholecystitis. Moderate to severe intrahepatic biliary ductal dilatation, hepatomegaly, and mildly nodular liver, suggestive of fibrofatty cirrhosis were seen. There was splenomegaly with splenic and paraesophageal varices, consistent with portal hypertension.
Initial labs revealed WBC 19.7 ,Alk phos-515 U/L, ALT- 91 U/L, AST- 69 U/L, T. Bili- 2.2 mg/dL, D. Bili- 1.3 mg/dL, Alb- 3 g/dL, PT-15.7. Chronic liver disease panel including autoimmune markers were negative and IgG4 levels were normal.
The patient had an emergent laparoscopic cholecystectomy for acute gangrenous cholecystitis with intra-operative cholangiogram showing bile duct stones. Subsequent intraoperative ERCP was performed with removal of 3 pigmented stones and placement of a biliary stent. Cholestatic hepatitis worsened post-operatively (Labs showed Alk Phos- 945 U/L, ALT- 156 U/L, AST-141 U/L, T. Bili-6 mg/dL, D. Bili-3.4 mg/dL). Repeat endoscopic exam showed 4 columns of < 5mm distal esophageal varices. EUS showed multiple collateral vessels around the portal confluence and turbulent anterograde flow in the portal vein consistent with portal HTN. ERCP cholangiogram revealed filling defects in the common bile duct and the right posterior hepatic duct. Multiple intrahepatic pigmented stones were removed with a single-operator peroral cholangioscope/EHL (SpyGlass, Boston Scientific) over several sessions with ductal clearance achieved.
Discussion Questions:
Are the findings consistent with PSC?
How would you manage this patient moving forward?
Given portal HTN findings, would you evaluate this further? If yes, how?
Expert Comments:
The expert panel concluded that the cholangiograms were atypical for PSC and presentation more consistent with recurrent pyogenic cholangitis as opposed to PSC. Chronic intrahepatic stone disease might have led to secondary cirrhosis and subsequent portal HTN. The patient had adequate imaging and endoscopic evidence of cirrhosis despite a normal platelet count and a liver biopsy or endoscopic portal pressure measurements were not warranted. The panel agreed that repeat ERCP-cholangioscopy was necessary to remove the large stone burden to improve cholestatic hepatitis and achieve ductal clearance.
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Presenter:
Worapoth Yingyongthawat, MD
Advanced Endoscopy Fellow
Siriraj Hospital, Bangkok, Thailand
A 62-year-old woman presented with 4 weeks of progressive jaundice, dull epigastric abdominal pain, and 4 kg weight loss. Laboratory analysis showed ALT 46 U/L, AST 58 U/L, ALP 248 U/L, total bilirubin 14 umol/L, direct bilirubin 13 umol/L, CA19-9 was 161 U/mL.
CT abdomen and pelvis demonstrated 4.5 cm circumferential wall thickening with luminal narrowing along the common bile duct, common hepatic duct, and hepatic hilar region causing intrahepatic dilatation, concerning for periductal infiltrating type cholangiocarcinoma.
Based on the clinical presentation and the imaging, the preoperative diagnosis was type IIIA hilar cholangiocarcinoma Bismuth-Corlette classification. Two options for surgical management depending on the extent of the disease were extended right hepatectomy with pancreaticoduodenectomy and right trisectionectomy with pancreaticoduodenectomy. Preoperative biliary drainage was recommended. ERCP with direct single-operator peroral cholangioscopy (SpyGlass, Boston Scientific) revealed diffuse erythematous, edematous bile duct wall and polypoid protrusion with bile duct luminal narrowing extending from hepatic hilum to distal common bile duct. The cholangioscope could not be advanced above the stricture. Biopsies of the stricture were obtained. A transpapillary biliary stent was placed across the common bile duct stricture for biliary drainage with subsequent normalization of liver tests.
The pathology revealed moderately differentiated adenocarcinoma. Right portal vein embolization was performed to augment left lobe liver volume. The 3D CT liver volumetry after right portal vein embolization showed the future liver remnant of 45%, a minimal acceptable level for surgery. In the interim newly developed distant nodal metastasis with peritoneal carcinomatosis were found and bilateral side-by-side metal stents were placed for palliation. The patient passed away several months later from necrotizing pneumonia.
Discussion Questions:
Timing of direct cholangioscopy: at the index procedure vs. only when non-diagnostic brushings/biopsies from the initial procedure.
Role of cholangioscopy in the pre-operative planning to evaluate the extent of the disease
Role of portal vein embolization for augmentation of liver volume prior to surgical resection.
Expert Comments:
Experts uniformly agreed that it is preferable to have cholangioscopy performed at the index procedure if resources are available. They felt that cholangioscopy in addition to tissue sampling provides important information on the extent of disease and could be helpful for preoperative planning. EUS may be beneficial during the index procedure to define the extent of biliary involvement and to evaluate for metastasis not seen on imaging; such as peritoneal carcinomatosis. Portal vein embolization could be considered for liver volume optimization in challenging liver resection cases.
