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Approach to right upper quadrant pain-lessons from a case
This document discusses a case of right upper quadrant abdominal pain. Imaging including ultrasound, CT, and MRI found irregular gallbladder wall thickening, gallstones, and increased signal surrounding the gallbladder. Pathology after cholecystectomy found xanthogranulomatous cholecystitis, a rare form of chronic cholecystitis characterized by a thickened fibrotic gallbladder wall with foamy histiocytes and bile extravasation. A final diagnosis of xanthogranulomatous cholecystitis was made based on gross and histologic findings.
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Approach to right upper quadrant pain-lessons from a case
- 1. Approach to rightupper quadrant pain. Illustrated through a case report Ultrasound MRI CT
- 2. Vascular Infarct Pyelophlebitis Mesenteric thrombosis Adrenal infarct Occlusion Embolism Renalvein thrombosis Clinical DDx: RUQ abd pain (by mnemonic) “V I N D I C A T E” Inflammation/Infection Cellulitis, Osteomyelitis Diaphragmatic abscess Trichinosis, TB, Herpes zoster Hepatitis, Hepatic abscess Cholecystitis, Cholangitis Duodenitis, Diverticulitis, Colitis Pancreatitis, Pyelonephritis Ulcer, Mesenteric adenitis Waterhouse-Friderichsen syndrome Neoplasm Carcinoma Cholangioma Pancreatic carcinoma Hodgkin disease Lymphosarcoma Neuroblastoma Adrenal carcinoma Multiple myeloma Intoxication/ Idiopathic Alcoholic hepatitis Ulcer Gout Degenerative Osteoarthritis Allergic/ Autoimmune Rheumatoid spondylitis Congenital/ Acquired Anomaly Ventral hernia Incisional hernia Diverticulum Obstruction Cyst Hydronephrosis Trauma Contusion Cough Hemorrhage Laceration Rupture Herniated disc Spine fracture Endocrine Hyperparathyroidism
- 3. Gallbladder carcinoma Cholecystitis and cholelithiasis Hepaticflexure syndrome Carcinoma of the colon with obstruction Colitis Diverticulitis Pyelonephritis Embolic nephritis Renal calculus Carcinoma of the pancreas Pancreatic calculus Pancreatitis Duodenal ulcer Common duct stone Cholangitis LacerationBudd-Chiari syndrome Carcinoma Subphrenic abscess Hepatitis Liver abscess DDx: RUQ abd pain (by anatomy) Legend: Liver Pancreas Bile duct Small bowel Gallbladder Large Bowel Renal System Others Budd-Chiari syndrome Liver abscess Laceration Hepatitis Carcinoma Common duct stone Cholangitis Pancreatitis Pancreatic calculus Carcinoma of the pancreas Duodenal ulcer Colitis Diverticulitis Carcinoma of the colon with obstruction Hepatic flexure syndrome Cholecystitis and cholelithiasis Pyelonephritis Embolic nephritis Renal calculus Gallbladder carcinoma Subphrenic abscess Pneumonia/empyema pleurisy
- 4. Imaging Modalities: • Ultrasound(US): abdomen/gallbladder to look for gallstones, aneurysm • Nuclear Medicine: cholescintigraphy (or HIDA scan) with or w/out cholecystokinin to evaluate the function of the gallbladder and the bile ducts • X-ray: Upper GI series to rule out stomach/duodenum conditions; abdomen; colon barium enema; chest x-ray to rule out pneumonia • Computed Tomography (CT): abdomen to further evaluate the gallbladder for mass/carcinoma as well as other abd organs such as the nearby pancreas • Magnetic Resonance Imaging (MRI): T1 with fat saturation, T2 to assess soft tissue changes such as fluid, inflammation, edema; MR cholangiopancreatography (MRCP) to visualize the biliary tract and pancreatic ducts • Invasive: cholangiography, percutaneous cholecystostomy, endoscopic retrograde cholangiopancreatography (ERCP)
- 5. Step by StepDiagnosis Clinical DDx: • Cholecystitis • Cholelithiasis • Choledocholithiasis • Cholangitis • Hepatitis • Pancreatitis Imaging: Ultrasound H&P: • Hx – RUQ abd pain • Exam – (+) Murphy sign • Labs – Leukocytosis
- 6. Our Patient: Findings onUltrasound Patient √ Marked irregular GB wall thickening √ Cholelithiasis with (+) US Murphy sign Abd aorta Impression: Gangrenous cholecystitis vs GB carcinoma Partners CAS Normal Liver Gallbladder Courtesy of Dr. MaryEllen Sun (BIDMC PACS) Hyperechoic fatty liver with abnormality in the region contiguous to gallbladder Film Findings: hyperechoic fatty liver, markedly thickened gallbladder wall, cholelithiasis with (+) US Murphy sign SagittalSagittal
- 7. Arrive at OurDx, Step by Step … Clinical DDx: • Cholecystitis • Choledocholithiasis • Cholangitis • Hepatitis • Pancreatitis Imaging: CT to evaluate gallbladder wall thickening vs “mass”; why? • gallbladder carcinoma has a poor prognosis of 85% mortality within 1 year of diagnosis • need to further evaluate the US findings with more imaging studies before embarking on any treatment H&P: • Hx – RUQ abd pain • Labs – Leukocytosis • Exam – (+) Murphy sign US DDx: • Gangrenous cholecystitis • Gallbladder carcinoma US Findings: • Irregular gallbladder wall thickening
- 8. Our Patient: Findingson CT scan Partners CAS Axial, oral C+ Cystic structure Cystic duct Common hepatic duct Common bile duct Gallbladder Neck Body Fundus www.wiltshiresurgery.com Heterogeneous low density in the adjacent liver Irregular wall thickening involving the gallbladder fundus Film Findings: Irregularly thickened wall at the gallbladder fundus, low attenuation in liver adjacent to the gallbladder, cyst at the fundus.
- 9. Partners CAS Impression: CTfindings suspicious for malignancy. Infection much less likely given no pericholecystic fluid or inflammation. Our Patient: Pertinent negative findings on CT scan Coronal, oral and IV C+ No wall thickening in the inferior and medial aspect of the gallbladder No pericholecystic fluid or inflammation No intra or extrahepatic biliary ductal dilatation Cystic structure Irregular wall thickening involving the gallbladder fundus
- 10. Arrive at OurDx, Step by Step … Clinical DDx: • Cholecystitis • Choledocholithiasis • Cholangitis • Hepatitis • Pancreatitis CT Findings: • Irregular wall thickening at the gallbladder fundus • Cystic structure at the gallbladder fundus • No pericholecystic fluid or inflammation • No biliary ductal dilatation H&P: • Hx – RUQ abd pain • Labs – Leukocytosis • Exam – (+) Murphy sign US DDx: • Gangrenous cholecystitis • Gallbladder carcinoma US Findings: • Irregular gallbladder wall thickening CT DDx: gallbladder malignancy Imaging: MR to further evaluate soft tissue changes in the gallbladder and the adjacent liver to assess inflammatory changes and confirm or rule out malignancy
- 11. Our Patient: Findings onMR imaging Axial T1-weighted Gradient Echo with Fat Sat; Post-Gadolinium Arterial Phase Partners CAS Axial T1-weighted Hi-Resolution with Fat Sat; Post-Gadolinium Partners CAS Wall thickening along the fundus measuring up to 15mm in maximum thickness Slight enhancement of GB wall mucosa, most prominently involving the fundal portion Film Findings: thickened gallbladder wall with hyper-intensity of the mucosa mostly involving the fundus
- 12. Our Patient: Findings onMR imaging Axial T1-weighted Gradient Echo with Fat Sat; Post-Gadolinium, Arterial Phase Partners CAS Axial T1-weighted Hi-Resolution with Fat Sat; Post-Gadolinium Partners CAS Small cystic area adjacent to the fundus measuring up to 2.0 cm, (+) rim enhancement No clear communication between the fundus and this cystic collection could be demonstrated Film Findings: small cyst at the fundus with ? communication to the gallbladder that cannot be clearly identified on MR
- 13. Axial T2-weighted withFat Saturation Partners CAS Our Patient: Findings on MR imaging Gallbladder sludge and stones Coronal T2-weighted Single-Shot Fast Spin Echo (SSFSE) Partners CAS Irregular wall thickening involving the gallbladder fundus Film Findings: Gallstones and, again, irregularly thickened gallbladder wall involving the fundus
- 14. Our Patient: Findings onMR imaging Partners CAS Coronal 2D Thick-Slab Abdomen (MR Cholangiopancreatography, or MRCP) Copyright ® The McGraw-Hill Companies, Inc. Gallbladder Duodenum Cystic duct Right hepatic duct Left hepatic duct Common hepatic duct Common bile duct Gallbladde r carcinoma Common hepatic duct Common Common bile duct Common Common R and L hepatic ducts Cystic duct CoGallbladder Com Main pancreatic duct Com Hepatopancreatic ampulla Com Major duodenal papilla ComDuodenum (1) (2) (3) (4) Pancreatic duct Hepatopancreatic ampulla Major duodenal papilla http://academic.kellogg.cc.mi.us/herbrandsonc/bio201_McKinley/Digestive%20System.htm Film Findings: No biliary/pancreatic duct obstruction/dilatation Impression: Normal biliary/pancreatic ductal system.
- 15. (1) R andL hepatic ducts merge to form a common hepatic duct Quick Review: The Biliary and Pancreatic Ducts Copyright ® The McGraw-Hill Companies, Inc. Gallbladder carcinoma Common hepatic duct Common Common bile duct Common Common R and L hepatic ducts Cystic duct CoGallbladder Com Main pancreatic duct Com Hepatopancreatic ampulla Com Major duodenal papilla ComDuodenum (2) (3) (4) (1) (4) Bile and pancreatic juices enter duodenum at the major duodenal papilla (2) Common hepatic and cystic ducts merge to form a common bile duct (3) Pancreatic duct merges with common bile duct at the hepatopancreatic ampulla http://academic.kellogg.cc.mi.us/herbrandsonc/bio201_McKinley/Digestive%20System.htm
- 16. Our Patient: Findings onMR imaging Axial T2-weighted with Fat Saturation Partners CAS ↑ T2 signal abnormality (hyper-intensity) surrounding the gallbladder and adjacent liver parenchyma No enlarged lymph nodes. Patent hepatic vasculature. No ascites. Film Findings: ↑ T2 signal surrounding the fundus, patent hepatic vasculature, no lymphadenopathy or ascities Impression: Overall MRI findings suggestive of fatty infiltration, adenomyomatosis likely complicated by chronic cholecystitis; gallbladder adenocarcinoma cannot be entirely excluded.
- 17. MRI Dx: Whatis Adenomyomatosis? • Definition: benign, abnormal though non-premalignant gallbladder mucosal hyperplasia, muscular wall thickening, and formation of intramural diverticula or sinus tracts called Rokitansky- Aschoff sinuses • Radiologic Finding: Pearl Necklace Sign uodenuuodenu mm Haradome, H. et al. Radiology 2003. 227(1): 80-8. Very sm all cystic structures Very sm all cystic structures (Pearl Necklace Sign) (Pearl Necklace Sign) MultipleMultiple gallbladder stonesgallbladder stones
- 18. Arrive at OurDx, Step by Step … Clinical DDx: • Cholecystitis • Choledocholithiasis • Cholangitis • Hepatitis • Pancreatitis H&P: • Hx – RUQ abd pain • Labs – Leukocytosis • Exam – (+) Murphy sign US DDx: • Gangrenous cholecystitis • Gallbladder carcinoma CT DDx: gallbladder malignancy Pathology/Management: Open cholecystectomy to make the definitive, final Dx by histology and determine future management of our patient MR DDx: • Adenomyomatosis • Gallbladder adenocarcinoma MR Findings: • Thickened gallbladder wall • Fundus cyst with ?communication • Gallbladder stones • No biliary obstruction/dilatation • ↑ T2 signal surrounding the fundus
- 19. Our Companion Patient: Findingson Gross Pathology Diffuse wall thickening Serosa covered with dense fibrous adhesions Ulcerated mucosal surface Yellow nodules/plaques, orYellow nodules/plaques, or xanthogranulomatous foci, extend intoxanthogranulomatous foci, extend into adjacent liver through the walladjacent liver through the wall Levy, A. et al. Radiographics. 2002. 22(2): 387-413. Cross section of the resected gallbladderCross section of the resected gallbladder Disruption of the gallbladder wall Gross Pathology Findings: (1) fibrosis and wall thickening (2)
- 20. Our Companion Patients: Findingson Histology Varadarajulu S, et al. Up-to-Date Fibroblasts,Fibroblasts, inflammatory cellsinflammatory cells Spindle-shaped cellsSpindle-shaped cells with more granularwith more granular cytoplasm andcytoplasm and elongated nucleielongated nuclei Lipid-laden mø: 2 morphological types Levy, A. et al. Radiographics. 2002. 22(2): 387-413. Xanthogranulomatous cholecystitisXanthogranulomatous cholecystitis focus (blackarrows above)focus (blackarrows above) H&E stainH&E stain Thickened, fibrotic wall Contains: (1) bile pigment (2) chronic inflammatory cells (3) foamy pigment-laden macrophages (mø) No dysplasia or malignancy! Rounded foamyRounded foamy macrophagesmacrophages (1)(1) (2)(2)
- 21. Arrive at OurDx Clinical DDx: • Cholecystitis • Choledocholithiasis • Cholangitis • Hepatitis • Pancreatitis H&P: • Hx – RUQ abd pain • Labs – Leukocytosis • Exam – (+) Murphy sign US DDx: • Gangrenous cholecystitis • Gallbladder carcinoma CT DDx: gallbladder malignancy MR DDx: • Adenomyomatosis • Gallbladder adenocarcinoma Pathology (Final) Dx: Xanthogranulomatous cholecystitis Gross/Histologic Findings: • Wall thickening with fibrotic serosa • Xanthogranulomatous foci • Bile extravasation through disrupted wall • Lipid-laden macrophages • Chronic inflammatory cells
- 22. Dx: What is XanthogranulomatousCholecystitis? • Definition: unusual form of benign, chronic cholecystitis with focal or diffuse destructive inflammatory process • Signs and symptoms: RUQ abd pain, fever, leukocytosis, vomiting, (+) Murphy sign • Hallmarks: (1) thickened, fibrotic, disrupted gallbladder wall (2) foamy histiocytes (3) bile extravasation
- 23. Dx: What is XanthogranulomatousCholecystitis? • Pathophysiology: gallbladder or cystic duct obstruction ↑ gallbladder intraluminal pressure rupture of Rokitansky-Aschoff sinuses or mucosal ulceration extravasation of bile into the gallbladder wall s63.jpgs63.4x1.jpg bile bile s63.jpg http://anatomy.iupui.edu/courses/histo_D502/D502f04/Labs.f04/digestive%20III%20lab/Lab13index.htm
- 24. Management: Significance of XanthogranulomatousCholecystitis • Significance: may simulate malignancy clinically, radiologically, and pathologically • Management of XG cholecystitis: open cholecystectomy with complete resection of the gallbladder due to dense fibrosis, extensive inflammation, ?coexistent malignancy • Management of GB carcinoma: (1) aggressive surgery – partial/segmental hepatic resection or Whipple procedure (2) no resection at all with chemo/radiation instead
- 25. • XG cholecystitis:benign yet focally/diffusely destructive inflammatory gallbladder disease with (1) fibrosis and wall thickening, (2) bile extravasation, (3) lipid-laden mø, (4) acute/chronic inflammatory cells • XG cholecystitis vs GB carcinoma: Patients with carcinoma are more likely to present with anorexia, weight loss, palpable mass, and jaundice • Preoperative Dx by radiographs: may significantly alter therapy and patient prognosis – be careful! Take Home Points:
- 26. References Chun KA, HaHK, Yu ES, Shinn KS, Kim KW, Lee DH, Kang SW, Auh YH. Xanthogranulomatous cholecystitis: CT features with emphasis on differentiation from gallbladder carcinoma. Radiology. 1997 Apr; 203(1): 93-7. Guermazi A. Are there other imaging features to differentiate xanthogranulomatous cholecystitis from gallbladder carcinoma? Eur Radiol. 2005 Jun; 15(6): 1271-2. Haradome H, Ichikawa T, Sou H, Yoshikawa T, Nakamura A, Araki T, Hachiya J. The pearl necklace sign: an imaging sign of adenomyomatosis of the gallbladder at MR cholangiopancreatography. Radiology. 2003 Apr; 227(1): 80-8. Levy AD, Murakata LA, Rohrmann CA Jr. Gallbladder carcinoma: radiologic-pathologic correlation. Radiographics. 2001 Mar-Apr; 21(2): 295-314. Levy AD, Murakata LA, Abbott RM, Rohrmann CA Jr. From the archives of the AFIP. Benign tumors and tumorlike lesions of the gallbladder and extrahepatic bile ducts: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 2002 Mar-Apr; 22(2): 387-413. Review. Shuto R, Kiyosue H, Komatsu E, Matsumoto S, Kawano K, Kondo Y, Yokoyama S, Mori H. CT and MR imaging findings of xanthogranulomatous cholecystitis: correlation with pathologic findings. Eur Radiol. 2004 Mar; 14(3): 440-6. Srivastava M, Sharma A, Kapoor VK, Nagana Gowda GA. Stones from cancerous and benign gallbladders are different: A proton nuclear magnetic resonance spectroscopy study. Hepatol Res. 2008 May 27. Varadarajulu S, Zakko SF. Xanthogranulomatous cholecystitis. Up-to-date. 2007. Slides 16 and 17 – http://academic.kellogg.cc.mi.us/herbrandsonc/bio201_McKinley/Digestive %20System.htm Slide 25 – http://anatomy.iupui.edu/courses/histo_D502/D502f04/Labs.f04/digestive%20III %20lab/Lab13index.htm