Case Discussion <Case presentation:> A 76 year- old female was sent to emergency room because of general weakness, fever, and confusion for 1 day. She was well in the past except that she had diabetes mellitus for several years that was controlled with insulin injection. She began to have epigastralgia 1 week before this admission. She stated to her family that the pain was more severe after meals, and did not improve after taking an over- the- counter antacid. Fever, nausea, poor appetite and conscious change ensued 3 days later and she was sent to emergency room. <Course and treatment:> In the emergency room, the patient was disoriented and weak. Her pulse rate was 118, blood pressure 90/50, and respiratory rate 32/minute. She had a fever of 38.9°C. Her sclera and skin were markedly icteric. Right upper quadrant tenderness was elicited on deep palpation in the epigastrium. Blood tests revealed leukoctyosis with left shift, abnormal liver function tests, and an elevated C-reactive protein (CRP) level. Empirical antibiotics and other supportive treatments were initiated. Abdominal sonography showed dilated biliary trees and multiple GB stones (figure 1). An emergent abdominal CT was performed which showed a radiopaque stone in the common bile duct (figure 2). Endoscopic retrograde cholangio-pancreatography (ERCP) was performed which showed swelling of the Papilla of Vater with pus draining (figure 3) and a common bile duct (CBD) stone with CBD dilatation (figure 4). An endoscopic nasobiliary drainage (ENBD) tube was inserted into the bile duct for relieving the bile duct obstruction. The patient became stabilized after the ENBD and the CBD stone was extracted in the second ERCP session. An elective laparoscopic cholecystectomy was subsequently performed and she was discharged in stable condition. <Laboratory and image study> 1. CBC/DC   WBC  Hb  PLT Segment PT Jan. 7 K/ul g/dl K/ul % sec   24430 10.8 71 96 13.5 2. Biochemistry   Bil-T/D AST/ALT ALP/r-GT Amylase/Lipase CRP Jan.7 mg/dL U/L U/L U/L  mg/dL   8.58/5.6 69/ 103 1498/ 317 179/ 12 23.7 <Analysis> Common bile duct stones (choledocholithiasis) develop either from gallbladder stones migrating to the bile duct (secondary choledocholithiasis, 90-95%), or within the bile duct per se (primary choledocholithiasis, 5-10%). The clinical presentations range from asymptomatic imaging findings to recurrent pain (biliary colic), recurrent cholangitis, biliary pancreatitis, obstructive jaundice, and the most feared suppurative cholangitis. Acute suppurative cholangitis is a common complication of choledocholithiasis. The classical presentation of the Charcot's triad (pain, jaundice and fever/chills) is seen in 70% of the cases. The laboratory features consist of leukocytosis with shift to left, direct type hyperbilirubinemia, elevated biliary enzymes (predominant) and transaminases. Rapid deterioration with sepsis characterized by altered mentation and hypotension together with Charcot's triad constitutes the Raynold's pentad. The pathogenesis involves obstruction of the biliary tree by the stones with superimposed infection; the most commonly found organisms are enteric Gram negative bacilli (Escherichia coli, Klebsiella, Pseudomonas, Proteus species) and enterococci, and anaerobes can also be seen in 15% of the cases. The presence of pus in a completely obstructed ductal stystem resulted in elevated pressure in the biliary tree with spread of the bacteria and sepsis. Imaging studies are important in the evaluation of choledocholithiasis. Though ultrasonography has more than 95 percent sensitivity and specificity for the diagnosis of gallstones, it detects CBD stones in only approximately 50% of the cases. CBD stones frequently cause obstruction of the bile duct and ultrasound can detect dilatation of intrahepatic or extrahepatic bile ducts in about 75 % of the cases. Computed tomography and, more accureately magnetic resonance imaging (MRI) with magnetic resonance cholangiography and pancreatography (MRCP) or endoscopic ultrasonography (EUS) are less invasive than ERCP with good diagnostic accuracy. ERCP is the standard for the diagnosis of CBD stones with sensitivity and specificity rates of about 95%. Once the diagnosis is made, urgent decompression and drainage of the biliary tree is important; response to antibiotics alone is poor. The mortality rate approaches 100% if prompt relief of obstruction and drainage of infected bile are not performed. ERCP with drainage of bile duct provide dramatically lower morbidity and mortality rate compared with emergent surgery, and is the treatment of choice for both definitive diagnosis and treatment. If ERCP drainage cannot be achieved, percutaneous transhepatic cholangiography (PTC) and drainage can also be used for diagnosis and biliary drainage. This patient presented with typical manifestations of acute suppurative cholangitis. In this setting, urgent ERCP with insertion of an ENBD tube provides effective drainage of the bile duct. ERCP with endoscopic sphincterotomy (EST) for lithotripsy and laparoscopic cholecystectomy to prevent future attacks can be performed after the patient is stabilized. <Reference> Harrison's principles of internal medicine, 16th Edition, chapter 292: Diseases of the Gallbladder and Bile Ducts. Sleisenger and Fordtran’s gastrointestinal and liver disease, 7th edition, 1083-1086. Aijaz Ahmed, Ramsey C. Cheung, Emmet B. Keeffe. Management of gallstones and their complications. American family physician 2000; 61:1673-80,1687-8. Coakley FV, Schwartz LH. Magnetic resonance cholangiopancreatography. J Magn Reson Imaging 1999;9:157-62. Canto MI, Chak A, Stellato T, Sivak MV Jr. Endoscopic ultrasonography versus cholangiography for the diagnosis of choledocholithiasis. Gastrointestinal endoscopy 1998;47(6):439-48. Cotton PB. Endoscopic retrograde cholangiopancreatography and laparoscopic cholecystectomy. American journal of surgery 1993; 165(4):474-8. 繼續教育考題 1. (E) 下列何項非總膽管結石之可能症狀及併發症： A 胰臟炎 B 黃膽 C 膽管炎 D 疼痛 E 以上皆是 2. (E) 下列何項非膽管炎之可能症狀： A 黃膽 B 發燒 C 疼痛 D 低血壓 E 以上皆是 3. (A) 下列關於非急性化膿性膽管炎(acute suppurative cholangitis)之事項何者為非： A Charcot's triad為診斷之必要條件 B 黃膽為直接型黃膽(direct hyperbilirubinemia) C Raynold's pentad 表示病況危急 D 由於總膽管結石阻塞總膽管所引起 E 以上皆正確 4. (E) 關於總膽管結石之診斷,下列何者為非： A ERCP是最正確的診斷方法 B 若是超音波檢查未發現總膽管結石也無總膽管擴張, 則可排除總膽管結石之可能 C MRI也可有效地診斷總膽管結石 D 若無膽結石, 則可排除總膽管結石之可能 E B and D 5. (E) 治療急性化膿性膽管炎, 選用之抗生素應對下列何種病菌有效？ A 綠膿桿菌 (Pseudomonas) B 腸球菌 (Enterococcus) C 克雷白氏菌 (Klebsiella) D 厭氧菌 (Anaerobe) E 以上皆正確 6. (C) 關於急性化膿性膽管炎之治療方針,下列何者為非： A 有效的抗生素治療 B 引流膽管中的積膿 C 及早手術治療 D 以上皆正確 E B and C 7. (A) 關於急性化膿性膽管炎之膽管引流, 下列何者為最佳首選： A 以ERCP引流 B 以PTC引流 C 手術引流 D 三者皆可 8. (E) 關於ERCP下列何者為非： A 是診斷總膽管結石最正確的方法 B 是診斷膽結石最正確的方法 C 可以診斷總膽管結石及引流總膽管, 但是無法取石 D 以上均非 E B and C 9. (D) 關於超音波檢查下列何者為非： A 是診斷膽結石之首選方法 B 超音波檢查若是發現總膽管擴張, 表示總膽管可能有阻塞 C 診斷總膽管結石之敏感度大約為50% D 診斷總膽管結石之正確性較MRCP為高 E 以上皆正確 10. (D) 膽結石及總膽管結石併發急性化膿性膽管炎之病患, 您會為病患安排以下何種治療？ A ERCP引流總膽管及總膽管結石取石, 然後以藥物治療膽結石 B 緊急外科手術引流總膽管及總膽管結石取石及膽囊切除 C  PTC引流總膽管, 然後外科手術總膽管結石取石及膽囊切除 D ERCP引流總膽管及總膽管結石取石, 然後外科手術切除膽囊 E 使用廣效性抗生素控制感染, 然後外科手術總膽管結石取石及膽囊切除 答案解析  （E）胰臟炎、阻塞性黃膽、膽管炎、疼痛皆是總膽管結石之症狀及併發症 （E） 黃膽、發燒、疼痛稱之為Charcot's triad, 是急性化膿性膽管炎之典型表現, 約有70%之病患同時表現此三項症狀. 若是加上低血壓及意識不清則稱之為Raynold's pentad, 表示病況危急.  (A）急性化膿性膽管炎是由於總膽管結石引起總膽管阻塞及細菌感染所引起. Charcot’s triad可見於70%之病患.黃膽以直接型膽紅素為主. （E）約有10%之總膽管結石是於總膽管內形成而非來自膽囊. 超音波檢查在總膽管結石之病患約有50%可看到總膽管結石, 約有70%可看到總膽管擴張. MRI加上MRCP或是ERCP均可有效地診斷總膽管結石, 尤其以ERCP為診斷之標準. （E）綠膿桿菌 (Pseudomonas) 、腸球菌 (Enterococcus、克雷白氏菌 (Klebsiella) 、厭氧菌 (Anaerobe)皆是急性化膿性膽管炎常見之感染菌種. （C）急性化膿性膽管炎之治療方針為有效的抗生素治療及引流膽管中的積膿. 手術治療應待膽管引流、感染獲得控制, 病情穩定後施行為宜 （A）膽管引流之方法中, 以ERCP引流之侵襲性最低, PTC引流次之, 手術引流之侵襲性最高. 以ERCP引流為最佳首選. （E）ERCP是診斷總膽管結石最正確的方法, 也可以引流總膽管及進行總膽管結石取石治療, 其治療成績與手術相當. 診斷膽結石最佳之方法為超音波檢查, 其敏感度與特異度均達95%. （D）超音波檢查為診斷膽結石最佳之方法, 其敏感度與特異度均達95%. 然而在總膽管結石之病患僅有50%可看到總膽管結石, 而有70%可看到總膽管擴張. 一般而言, 總膽管擴張表示總膽管可能有阻塞. MRCP診斷總膽管結石之敏感度與特異度約為90%, 且不具侵襲性, 為一良好之診斷方法. （D）膽結石及總膽管結石併發急性化膿性膽管炎之治療首重膽管引流, 若無引流而僅以抗生素治療, 死亡率接近100%. ERCP引流之侵襲性最低為最佳首選之引流方法, 待感染獲得控制病情穩定後, 進行ERCP總膽管結石取石治療及腹腔鏡膽囊切除以避免再發, 是目前治療的準則.