# Clinical Cases: Biliary Diseases ## Case 1: Acute Cholecystitis ### Patient Presentation **Demographics:** 45-year-old female **Chief Complaint:** Right upper quadrant pain, fever, and nausea for 18 hours **History of Present Illness:** The patient developed severe, constant right upper quadrant pain that started after eating a fatty meal last night. Unlike previous episodes of "gallbladder attacks" that resolved within hours, this pain has persisted and worsened. She has had fever, chills, and multiple episodes of nausea with vomiting. The pain radiates to her right shoulder. **Past Medical History:** Known gallstones (diagnosed 2 years ago), obesity, two prior pregnancies **Social History:** Non-smoker, occasional alcohol ### Physical Examination - **Vital Signs:** Temperature 38.6C, BP 128/82 mmHg, HR 102 bpm - **General:** Uncomfortable female lying still, appears unwell - **Abdomen:** Right upper quadrant tenderness with guarding; Murphy's sign positive (inspiratory arrest during RUQ palpation); no peritoneal signs; hypoactive bowel sounds ### Workup and Results - **CBC:** WBC 14,800 with 85% neutrophils - **Liver Function Tests:** AST 52 U/L, ALT 68 U/L, ALP 145 U/L, Total bilirubin 1.4 mg/dL - **Lipase:** Normal - **Right Upper Quadrant Ultrasound:** Gallstones with posterior acoustic shadowing; gallbladder wall thickening (5 mm); pericholecystic fluid; positive sonographic Murphy sign - **HIDA Scan (confirmatory):** Non-visualization of gallbladder at 4 hours (indicates cystic duct obstruction) ![Acute Cholecystitis Ultrasound](case_01_image.jpg) *Ultrasound demonstrating acute cholecystitis with gallstones (echogenic focus with acoustic shadow), gallbladder wall thickening (>3mm), and pericholecystic fluid. The sonographic Murphy sign was positive.* **Image Source:** Case courtesy of Radiopaedia.org ### Diagnosis **Acute Calculous Cholecystitis (Tokyo Grade II - Moderate)** ### Clinical Correlation Acute cholecystitis develops when a gallstone impacts in the cystic duct and fails to dislodge, causing persistent gallbladder distension and inflammation. The pathophysiology progresses from obstruction to distension, wall ischemia, chemical inflammation, and secondary bacterial infection (50% of cases). This differs from biliary colic, which resolves within 6 hours when the stone dislodges. Murphy's sign is the hallmark physical finding - inspiratory arrest occurs as the inflamed gallbladder descends during inspiration and contacts the examining hand. Complications include gangrenous cholecystitis, perforation, and emphysematous cholecystitis. Tokyo Guidelines classify severity to guide management. ### Treatment - NPO, IV fluid resuscitation - IV antibiotics: Piperacillin-tazobactam OR ceftriaxone + metronidazole - Analgesia - **Early laparoscopic cholecystectomy** (within 72 hours of symptom onset) - Grade II without organ dysfunction is amenable to early surgery - If high surgical risk or organ dysfunction: percutaneous cholecystostomy followed by interval cholecystectomy - Intraoperative cholangiogram to rule out CBD stones if indicated --- ## Case 2: Ascending Cholangitis ### Patient Presentation **Demographics:** 68-year-old male **Chief Complaint:** Fever, jaundice, and right upper quadrant pain for 2 days **History of Present Illness:** The patient developed fever with rigors, followed by right upper quadrant pain and progressive jaundice over 2 days. He has become increasingly confused. He had an ERCP 2 weeks ago for known common bile duct stones, but not all stones could be removed due to technical difficulty. **Past Medical History:** Choledocholithiasis with prior incomplete stone removal, atrial fibrillation, hypertension ### Physical Examination - **Vital Signs:** Temperature 39.4C, BP 88/52 mmHg, HR 124 bpm, RR 24/min - **General:** Toxic-appearing, jaundiced, confused male - **HEENT:** Icteric sclerae - **Abdomen:** Right upper quadrant tenderness, no peritoneal signs - **Neurologic:** Disoriented to time and place ### Workup and Results - **CBC:** WBC 22,400 with bandemia - **Liver Function Tests:** Total bilirubin 8.5 mg/dL, Direct bilirubin 6.8 mg/dL, ALP 485 U/L, GGT 380 U/L, AST 245 U/L, ALT 312 U/L - **BMP:** Cr 2.1 mg/dL (baseline 0.9) - **Lactate:** 4.8 mmol/L - **Blood Cultures:** Drawn (later positive for E. coli) - **CT Abdomen:** Dilated common bile duct (15 mm) with filling defect consistent with stone; pneumobilia from prior sphincterotomy ![Cholangitis MRCP](case_02_image.jpg) *MRCP demonstrating a dilated common bile duct with filling defect (choledocholithiasis) causing biliary obstruction. In the clinical context of fever, RUQ pain, and jaundice (Charcot's triad), this indicates acute cholangitis.* **Image Source:** Case courtesy of Radiopaedia.org ### Diagnosis **Acute Cholangitis - Severe (Reynold's Pentad present: Charcot's triad + hypotension + confusion)** ### Clinical Correlation Acute cholangitis requires two elements: biliary obstruction AND bacterial infection. Obstruction (choledocholithiasis, stricture, malignancy) elevates intrabiliary pressure, disrupting the blood-bile barrier and allowing bacterial translocation into the bloodstream. Charcot's triad (fever, RUQ pain, jaundice) is present in 50-70% of patients. Reynold's pentad adds hypotension and altered mental status, indicating severe septic cholangitis. Common organisms include E. coli, Klebsiella, Enterococcus, and anaerobes. Tokyo Guidelines classify severity: Grade I responds to antibiotics; Grade II requires early drainage within 24 hours; Grade III (severe with organ dysfunction) requires emergency drainage within hours. ### Treatment - **Emergent ICU admission** - Aggressive IV fluid resuscitation with vasopressors (norepinephrine) - Broad-spectrum antibiotics: Piperacillin-tazobactam OR meropenem (cover biliary pathogens and possible resistant organisms) - **Emergency biliary drainage** - ERCP with stone extraction/stent placement within hours - If ERCP fails or unavailable: percutaneous transhepatic cholangiography with drainage - Monitor for response: defervescence, improving hemodynamics - Definitive stone clearance once stabilized (may require repeat ERCP or surgery) --- ## Case 3: Cholangiocarcinoma (Klatskin Tumor) ### Patient Presentation **Demographics:** 65-year-old male **Chief Complaint:** Painless jaundice, pruritus, and weight loss for 3 weeks **History of Present Illness:** The patient noticed progressive yellowing of his skin and eyes over 3 weeks. He has intense generalized pruritus, worse at night, interfering with sleep. He has lost 12 pounds unintentionally. He denies abdominal pain but reports clay-colored stools and dark urine. **Past Medical History:** Primary sclerosing cholangitis diagnosed 15 years ago, ulcerative colitis (well-controlled on mesalamine) **Social History:** Never smoker, no alcohol ### Physical Examination - **Vital Signs:** Normal - **General:** Jaundiced, cachectic male with excoriations from scratching - **HEENT:** Icteric sclerae - **Abdomen:** Hepatomegaly, no palpable gallbladder (unlike distal CBD obstruction), no ascites - **Skin:** Jaundice, multiple scratch marks ### Workup and Results - **Liver Function Tests:** Total bilirubin 18.2 mg/dL, Direct 15.6 mg/dL, ALP 680 U/L, GGT 520 U/L, AST 85 U/L, ALT 92 U/L - **CA 19-9:** 485 U/mL (elevated) - **MRCP:** Biliary stricture at the confluence of left and right hepatic ducts with intrahepatic ductal dilation bilaterally; no single dominant mass - **CT Chest/Abdomen/Pelvis:** Hilar mass with vascular encasement; no distant metastases - **ERCP with Brushings:** Stricture at hepatic confluence; cytology shows adenocarcinoma ![Klatskin Tumor MRCP](case_03_image.jpg) *MRCP demonstrating a Klatskin tumor (perihilar cholangiocarcinoma) at the confluence of the right and left hepatic ducts. Note the dilated intrahepatic ducts bilaterally with abrupt cutoff at the hilum.* **Image Source:** Case courtesy of Radiopaedia.org ### Diagnosis **Perihilar Cholangiocarcinoma (Klatskin Tumor) - Bismuth Type IIIA/B** ### Clinical Correlation Cholangiocarcinoma arises from bile duct epithelium and is classified by location: intrahepatic, perihilar (Klatskin tumor - most common), or distal. Primary sclerosing cholangitis is the strongest risk factor, conferring 10-15% lifetime risk. Other risk factors include liver flukes, choledochal cysts, and chronic hepatitis. The Bismuth-Corlette classification stages perihilar tumors by extent of ductal involvement. Klatskin tumors present with painless obstructive jaundice; the absence of a palpable gallbladder (unlike distal tumors) reflects intrahepatic obstruction above the cystic duct insertion. CA 19-9 is elevated in most cases but lacks specificity. Prognosis is poor, with 5-year survival <20% even with resection. ### Treatment - Biliary drainage: ERCP with bilateral stent placement for jaundice palliation - Multidisciplinary tumor board review - Staging evaluation: EUS, consider staging laparoscopy - **If resectable:** Extended hepatectomy with bile duct resection and regional lymphadenectomy (requires adequate future liver remnant) - **Selected patients:** Liver transplant following neoadjuvant chemoradiation protocol (at specialized centers) - **If unresectable/metastatic:** Palliative chemotherapy (gemcitabine + cisplatin) - Pruritus management: Cholestyramine, ursodiol, consider rifampin or naltrexone - Enhanced PSC surveillance in remaining patients with PSC (annual imaging + CA 19-9)