William C. Beck
Benjamin K. Poulose
Presentation
A 68-year-old man with a history of adult-onset diabetes, obesity, and tobacco use presents to the emergency department. He is febrile on arrival with a temperature of 102.7, a blood pressure of 95/50, and a heart rate of 106. His primary complaint is of right upper-quadrant pain of 24-hour duration. He notes that he has had similar pain on occasion before but always had complete resolution of pain within a couple of hours. He reports his urine has been very dark for the last 12 hours. On exam, he has tenderness of his right upper quadrant with voluntary guarding. The sclerae are mildly icteric. No jaundice is present.
Differential Diagnosis
The typical presentation of cholangitis involves the combination of right upper-quadrant pain, fever, and jaundice, commonly known as Charcot’s triad. Only 50% to 70% of patients, however, present with all three elements. The addition of mental status changes and hypotension comprise Reynold’s pentad, which is indicative of systemic sepsis. Although biliary colic may be elicited, patients with cholangitis usually also manifest with fever and jaundice. In a patient presenting with a history of gallstones or right upper-quadrant pain, the presence of jaundice, hypotension, or altered mental status should alert the provider to the diagnosis of cholangitis. Peritonitis is uncommon and should prompt the examiner to look for other causes of abdominal pain such as diverticulitis, perforated ulcer, or pancreatitis. Careful consideration should be given to acute cholecystitis in the differential diagnosis, as the immediate treatment would differ considerably. A previous history of biliary interventions should prompt providers to consider the diagnosis of acute cholangitis. Those patients with prior biliary operations, endoscopically or radiologically placed biliary stents, and history of chronic biliary conditions (i.e., primary sclerosing cholangitis) are at increased risk of developing cholangitis.
Presentation Continued
This patient undergoes further evaluation of his abdominal pain, fevers, and jaundice with a complete blood count (CBC), comprehensive metabolic panel (CMP), amylase, lipase, coagulation profile, and two sets of blood cultures. He has a leukocytosis of 13,300/mL with a left shift, a bilirubin of 4.4 mg/dL, and alkaline phosphatase of 500 IU/L. Both the AST and the ALT are elevated at 210 and 334 IU/L, respectively.
Workup
An abdominal ultrasound (US) is the preferred initial imaging study. In the above patient, it reveals cholelithiasis with mild intra- and extrahepatic biliary duct dilation. Computed tomography (CT) scan and magnetic resonance cholangiopancreatography (MRCP) are not needed for the diagnosis of cholangitis but may prove helpful to identify the underlying etiology of the biliary obstruction that predisposed the patient to cholangitis, such as benign or malignant biliary strictures, or periampullary mass. In the case of malignant causes of biliary obstruction, pain is usually less of a component of presentation and the onset is more insidious. In general, a single, good-quality noninvasive study (US, CT, or MRCP) can suffice to establish biliary ductal dilation and provide clues to the etiology of biliary obstruction. A high suspicion for common bile duct stones is present with clinical ascending cholangitis without other obvious etiology, or total bilirubin >4 mg/dL. Other strong predictors of common bile duct stones include a dilated common bile duct (>6 mm) on US with gallbladder in situ and bilirubin between 1.8 and 4 mg/dL.
Many patients do not present with Charcot’s triad, and in 2006 an international consensus meeting was held in Tokyo, Japan, with the goal of developing guidelines useful in establishing the diagnosis of acute cholangitis. The consensus was reached that Charcot’s triad was sufficient to diagnose acute cholangitis. Additionally, the Tokyo guidelines suggest that if two of the three elements of Charcot’s triad are present, along with (1) laboratory evidence of inflammatory response, (2) abnormal liver function tests, and (3) abnormal imaging studies demonstrating biliary dilatation, inflammatory findings, or the presence of an etiology such as a biliary stricture, calculus, stent, or mass that the diagnosis of cholangitis can be made (Figure 1).
FIGURE 1 • Diagnosis of cholangitis may be made using either the traditional Charcot’s triad, or Tokyo criteria.
Blood test results can vary in patients with acute cholangitis, and must be taken in the context of the history and physical exam of the individual patient. The white blood cell count is elevated above 10,000/mL in 60% to 80% of patients that present with acute cholangitis and is the most often noted abnormal result. The absence of an elevated total bilirubin should question the diagnosis of cholangitis. Liver tests (AST, ALT, GGT) are usually elevated, but no specific pattern has been demonstrated. Alkaline phosphatase is also usually elevated and is typically more elevated in biliary obstruction due to malignant etiologies as compared to choledocholithiasis.
Diagnosis and Treatment
In this patient presenting with two elements of Charcot’s triad (fever, abdominal pain, and jaundice), biliary disease should be at the top of the differential diagnosis. In the setting of altered mental status and relative hypotension in a normally hypertensive man, the provider should strongly suspect cholangitis as the diagnosis. Many patients have a history of prior calculous biliary disease or biliary operation. In Western countries, choledocholithiasis is the most common etiology, followed by benign and malignant biliary strictures. Other etiologies include autoimmune cholangitis, parasitic infections, prior biliary operations, indwelling stents, and chronic pancreatitis.
When cholangitis is suspected, the patient should be admitted to the hospital for intravenous fluid resuscitation, initiation of appropriate antibiotics, hemodynamic monitoring, and prompt biliary decompression if indicated. Blood cultures should be sent prior to the initiation of antibiotics to allow for the tailoring of antibiotic therapy after the causative organism is identified. Timely initiation of empiric intravenous antibiotic therapy is crucial to successful treatment. Therapy should target gram-negative bacteria and anaerobes. A fluoroquinolone with added metronidazole or extended-spectrum beta lactam (piperacillintazobactam) can provide adequate empiric coverage. Occasionally, coagulopathy is present, which needs to be corrected prior to undergoing any intervention. If the patient responds well to antibiotic therapy, and there is no hemodynamic instability, further imaging (CT or MRCP) may be performed to better elucidate the underlying cause of the cholangitis. However, if the patient appears septic, urgent biliary decompression is required either by endoscopic, percutaneous, or surgical means.
Those patients who respond well to initial resuscitation and antibiotic therapy without biliary drainage are deemed as having mild cholangitis. Continued clinical improvement in these patients may delay the urgency of biliary decompression as the etiology for biliary obstruction is sought. Patients who do not respond to resuscitation and antibiotics alone will need urgent biliary decompression.
Surgical Approach
Urgent endoscopic decompression of the biliary tree has been established as the treatment of choice for the management of acute cholangitis. Endoscopic retrograde cholangiography (ERC) is successful in over 90% of patients in decompressing the biliary tree. The timing of endoscopic biliary decompression should be individualized and often occurs within 24 to 48 hours of initial admission. The urgency of intervention should be dictated by the patient’s clinical response to resuscitation and antibiotic therapy. An initially good response followed by clinical deterioration prompts urgent biliary decompression. More urgent or emergent decompression is required in patients who remain hypotensive despite aggressive resuscitation and antibiotic therapy. When ERC expertise is not readily available and urgent biliary decompression is needed, percutaneous or surgical biliary decompression should be employed.
ERC can be performed under moderate or deep sedation and is associated with decreased rates of postoperative mechanical ventilation and death as opposed to traditional open bile duct exploration. ERC is typically performed with the patient in the prone or semiprone position. The duodenoscope is advanced through the oropharynx, esophagus, and stomach to the second portion of the duodenum. The ampulla is visualized and engaged with a cannula or sphincterotome. Wire access to the biliary tree is achieved and the cannula advanced into the bile duct. Before a large volume of contrast in instilled into the biliary tree, bile is aspirated to assist with decompression and to obtain biliary cultures. The act of biliary cannulation alone will often result in rapid biliary decompression in patients with acute suppurative cholangitis (Figure 2). Radiopaque contrast is then injected into the bile duct and cholangiography performed to ascertain the etiology of biliary obstruction. Should a common bile duct calculus be discovered, a judgment is made regarding appropriateness of biliary sphincterotomy prior to stent placement. If the calculus is relatively small and there is little associated ampullary edema, biliary sphincterotomy can usually be safely performed to help facilitate ductal clearance either with a biliary extraction balloon or a Dormia basket. A temporary transampullary biliary stent is then placed to ensure continued biliary drainage (Figure 3). If during cholangiography a large, challenging stone or complex stricture is encountered, the main priority should be biliary decompression with stenting to quickly improve the patient’s clinical condition. This is done at the expense of repeated diagnostic and therapeutic procedures to help define the precise etiology of biliary obstruction and definitively treat the patient. However, these additional procedures can often be performed on an elective basis. In experienced hands, ERC can be performed with minimal risk. Post-ERC pancreatitis is the most frequently encountered complication, followed by hemorrhage, cholangitis, and perforation. In patients with severe sepsis and hypotension who do not have suppurative cholangitis at the time of ERC, serious consideration should be given to an alternate diagnosis.
FIGURE 2 • Suppurative cholangitis.
FIGURE 3 • Transampullary stent placement.
If ERC is not available, other options for biliary decompression include percutaneous transhepatic drainage and surgical decompression. If the intrahepatic ducts are dilated, thus providing a transhepatic target for biliary access, a percutaneous route to biliary drainage is favored as it is less invasive than surgical decompression and provides adequate drainage. In addition, a percutaneously placed biliary drain drainage can usually be converted to an internal endoscopically placed stent once acute issues have resolved via a rendezvous technique.
Surgical decompression of the biliary tree is largely of historical interest given the high success of ERC and percutaneous techniques, avoiding the additional physiologic insult of a major operation. However, there are scenarios where surgical decompression may be the preferred method of decompression. These atypical scenarios arise in patients with altered upper gastrointestinal anatomy (e.g., post Roux-en-Y gastric bypass) and where endoscopic or percutaneous radiologic expertise is not readily available. If a patent cystic duct can be demonstrated with an in situ gallbladder, an open or laparoscopic cholecystostomy tube may be an efficient, life-saving intervention until further expertise can be obtained. If common bile duct access is necessary, an open approach is usually employed for common bile duct exploration and t-tube placement (Table 1). Surgeons with advanced laparoscopic skills and experience may consider a laparoscopic approach. If a laparotomy is performed, an upper midline or right subcostal incision is utilized to approach the biliary tree. A self-retaining retractor is used to retract the liver cephalad and colon caudally. If a gallbladder is present, it is mobilized in a dome-down fashion until the cystic duct is identified. Calot’s triangle is defined and the cystic artery is ligated and divided. The anterior surface of the cystic duct is dissected toward the common bile duct, which usually is readily identified. Care is then taken to dissect the anterior surface of the bile duct only to avoid the flanking blood supply coursing at the 3 and the 9 o’clock positions. The caliber of the bile duct and associated inflammation are assessed to help further guide surgical intervention. The cholecystectomy is completed. The intended area of choledochotomy should be 1 to 2 cm distal to the insertion of the cystic duct toward the ampulla. Two separate mural sutures, using a fine 4-0 or 5-0 suture, are placed on either side of the anterior portion of the bile duct and a longitudinal choledochotomy made with a no. 15 blade scalpel. At this point, a decision is made to proceed with common bile duct exploration or to insert a t-tube for decompression in the unstable patient. If common bile duct stones are suspected, simple irrigation of the ductal lumen with a small bore red rubber catheter is usually adequate to mobilize most calculi out through the choledochotomy. The red rubber catheter can be advanced both proximally and distally to clear the bile duct. A balloon-tipped catheter (“biliary Fogarty”) may also be used. Rigid instrumentation and extraction forceps should be avoided especially with inflamed tissues increasing the chance of ductal injury. Choledochoscopy is a very useful adjunct to ensure ductal clearance. A 3-mm or a 5-mm choledochoscope can be used with continuous saline irrigation to adequately and efficiently visualize the ductal lumen. Difficult stones can be retrieved with wire baskets placed through the scope. It is essential that the surgeon be familiar with the equipment intended for choledochoscopy prior to the procedure. In addition, biliary endoscopy can be challenging even for experienced surgical endoscopists; familiarity with endoscopic techniques in general greatly facilitates this procedure, especially when through-the-scope therapeutics are employed. Once the duct is cleared, a t-tube is usually placed in the setting of acute cholangitis. For adequate drainage, a 14 F or 16 F guttered t-tube should be placed within the bile duct. The choledochotomy is closed using absorbable sutures over the t-tube, which is brought through the abdominal wall. Some redundancy of the tubing should be left within the abdomen to avoid tension, but a long and tortuous course should be avoided to help facilitate possible future percutaneous techniques. If time and resources permit, a completion t-tube cholangiogram can be performed to confirm bile duct clearance and integrity of the ductal closure. A closed-suction drain is placed in the area of the choledochotomy and the abdomen closed in the standard fashion.
TABLE 1. Key Technical Steps and Potential Pitfalls of Open Biliary Decompression
Special Intraoperative Considerations
The surgical approach to the common bile duct should be avoided in patients with smaller ducts (<5 mm) where identification and manipulation would be technically difficult, especially in an inflamed field. In these situations, cholecystostomy tube placement may be an ideal method of biliary decompression, should endoscopy not be feasible. Should the surgeon encounter severely inflamed tissues around the main portal triad, every effort to minimize dissection and safely enter the common bile duct need to be employed. Keen judgment should be used to avoid injury to the portal vein, hepatic artery, or duodenum in the inflamed field. Identification of the common bile duct can be facilitated by using a small needle (22 to 25 g) to aspirate bile from the duct before an incision is made within it. This technique is especially helpful in patients who have had prior cholecystectomy. Laparoscopy has limited use in the acute decompression of the common bile duct except among surgeons and surgical teams who have considerable expertise with these techniques.
Postoperative Management
Following biliary decompression, patients are observed in the hospital for resolution of their symptoms. Antibiotics may be tailored following the results of cultures and continued for 5 to 7 days. A CBC and CMP may be sent to follow improving white blood cell counts and liver profile. Definitive therapy is dictated by the underlying cause of biliary obstruction. In most patients who undergo ERC for choledocholithiasis, elective cholecystectomy can be performed at a later date with ductal clearance usually achieved via ERC.
For those patients in whom a t-tube was placed, the tube is initially left to gravity drainage in the acute setting. After discharge, a contrast study is obtained via the tube to confirm clearance of the bile duct and patency of the biliary tree. With this study, usually performed 2 to 3 weeks after insertion, decision can then be made to “internalize” biliary drainage (i.e., cap the t-tube). Another study is repeated at 4 to 6 weeks and the tube is removed should this study demonstrate normal patency and drainage of the biliary system.
Case Conclusion
In the patient presented, a high suspicion of common bile duct stones exists based on documented cholelithiasis, extrahepatic biliary ductal dilation, clinical ascending cholangitis, and a total bilirubin level greater than 4mg/dL. The patient underwent successful ERC with biliary sphincterotomy and extraction of common bile duct stones. Once discharged, an elective laparoscopic cholecystectomy was performed.
TAKE HOME POINTS
· Prompt diagnosis, antibiotic administration, and fluid resuscitation are of utmost importance in the patient with acute cholangitis.
· The most common cause of acute cholangitis in Western countries is choledocholithiasis, followed by malignancy.
· Urgent biliary decompression is the essential treatment in acute cholangitis not responsive to resuscitation and antibiotics.
· Endoscopic biliary decompression is associated with lower morbidity and mortality than with conventional surgical bile duct exploration.
· Percutaneous or surgical decompression should be employed when endoscopic intervention is not feasible.
SUGGESTED READINGS
ASGE Standards of Practice Committee, Maple JT, Ben-Manachem T, et al. The role of endoscopy in the evaluation of suspected choledocholithiasis. Gastrointest Endosc. 2010;71:1–9.
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Lai EC, Mok FP, Tan ES, et al. Endoscopic biliary drainage for severe acute cholangitis. N Engl J Med. 1992;326:1582–1586.
Mayumi T, Takada T, Kawarda Y, et al. Results of the Tokyo Consensus Meeting Tokyo Guidelines. J Hepatobiliary Pancreat Surg. 2007;14:114–121.
Thompson JE, Tompkins RK, Longmire WP. Factors in management of acute cholangitis. Ann Surg. 1982;195:137–145.