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Gastroenterology - Biliary Disease - Fast Facts | NEJM Resident 360

The most commonly encountered biliary diseases are cholelithiasis (gallstones in the gallbladder), cholecystitis (inflammation of the gallbladder), choledocholithiasis (gallstones in the common bile duct), and gallstone pancreatitis (inflammation of the pancreas from gallstone blockage of the pancreas duct). Although 10%–15% of adults develop cholelithiasis in their lifetime, only about 1% of cases per year become symptomatic. These patients are commonly managed as inpatients. In this section, we review the following biliary diseases:

• Cholelithiasis

• Biliary Strictures

• Primary Sclerosing Cholangitis

The Biliary Tract

(Source: Biliary Tract. USMLEMD 2008.)

Cholelithiasis

Gallstone-Related Complications

• Biliary colic occurs when the gallbladder contracts against a gallstone that has passed into the cystic duct. This leads to steady cramping pain in the right upper quadrant that may last for 30 to 60 minutes, then dissipate and return. Biliary pain often begins a few hours after ingesting fatty food and resolves within several hours.

• Cholecystitis occurs when an impacted gallstone in the cystic duct leads to gallbladder distention and subsequent ischemia and inflammation of the gallbladder wall. Prolonged ischemia and inflammation of the gallbladder can result in bacterial translocation and infection; thus management of cholecystitis includes:

• • intravenous antibiotics: with coverage of gram-positive, gram-negative, and anaerobic bacteria

  • surgical intervention: usually early laparoscopic cholecystectomy (as early as possible during hospitalization) unless the patient is at high risk for surgical complications, when initial nonoperative management is preferred

• Choledocholithiasis occurs when a gallstone is impacted in the common bile duct. Sometimes the stone will pass spontaneously. However, in some cases an endoscopic retrograde cholangiopancreatography (ERCP) is required to extract the sludge and stones from the duct. Choledocholithiasis can lead to acute ascending cholangitis.

• Acute ascending cholangitis is a medical emergency associated with high morbidity and mortality if it is not diagnosed and treated promptly. It is characterized by inflammation and infection that involves the whole biliary tree. Cholangitis can also develop as a result of other causes of biliary obstruction. The classic presentation of septic cholangitis is Charcot triad (right-upper-quadrant pain, jaundice, and fever) or Reynolds pentad (with added altered mental status and hypotension).

• • Management includes broad-spectrum antibiotics to cover biliary organisms.

  • Emergency ERCP is required to remove obstructing stones and allow pus to drain into the duodenum. In certain situations, a stent is placed in the distal common bile duct, with or without sphincterotomy, to allow ongoing drainage of pus and debris.

Diagnosis

The following table outlines the diagnostic criteria for acute cholecystitis. Diagnosis requires one local sign or symptom (e.g., right-upper-quadrant pain), one systemic sign (e.g., fever), and confirmation on an imaging test.

(Source: Acute Calculous Cholecystitis. N Engl J Med 2008.)

Management

Management of gallstone disease depends on whether the patient is symptomatic. In many patients who undergo abdominal imaging for other reasons, asymptomatic gallstones are detected in the gallbladder. In the absence of symptoms, cholecystectomy is usually not recommended. However, in specific circumstances (e.g., patients with hemolytic anemia), detection of asymptomatic gallstones may require surgical intervention.

In symptomatic patients with complications related to cholelithiasis, and particularly in patients with acute cholecystitis, surgical intervention is indicated.

The following table summarizes the advantages and disadvantages of various surgical interventions for complications of gallstones.

(Source: Interventional Approaches to Gallbladder Disease. N Engl J Med 2015.)

Biliary Strictures

Biliary strictures (which can predispose to cholangitis) can result from chronic choledocholithiasis, chronic pancreatitis, primary sclerosing cholangitis, or biliary tract malignancies (e.g., cholangiocarcinoma or ampullary carcinoma).

Management of benign biliary strictures is generally via ERCP with dilatation, stent placement, or both. Malignant strictures, however, more often require biliary stenting and specific therapies directed at treating malignancies.

Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is an idiopathic, chronic, cholestatic liver disease characterized by persistent, progressive biliary inflammation and fibrosis of intrahepatic and extrahepatic bile ducts that lead to multifocal bile-duct strictures, consequent inflammation, and even cirrhosis.

Clinically, PSC manifests as insidious, worsening cholestasis with progressive jaundice. Approximately 70% of patients with PSC also have ulcerative colitis. There are several subtypes of PSC, with the classic subtype occurring in 90% of patients.

(Source: Primary Sclerosing Cholangitis. N Engl J Med 2016.)

Diagnosis

Diagnosis of PSC is made from laboratory and imaging findings and exclusion of secondary causes:

• lab tests: increased serum alkaline phosphatase level that persists for >6 months

• imaging: cholangiographic findings of multiple bile-duct strictures (on magnetic resonance cholangiopancreatography [MRCP] or ERCP)

• exclusion of sclerosing cholangitis (e.g., amyloidosis, iatrogenic biliary strictures, and IgG4-associated cholangitis)

Treatment

Treatment of PSC is not well established and no effective medical therapy exists.

Some guidelines suggest treatment with low-dose ursodeoxycholic acid because in early studies it led to lower serum alkaline phosphatase levels (but no improvement in overall survival or need for liver transplantation). However, in a 2009 randomized, double-blind, placebo-controlled trial, ursodeoxycholic acid was associated with a 2.3 times higher risk of liver transplantation, cirrhosis, and death in patients. Thus, the American Association for the Study of Liver Diseases (AASLD) does not support use of ursodeoxycholic acid in patients with PSC.

Due to the lack of treatment options and the progressive nature of PSC, approximately 40% of patients require liver transplantation. However, the disorder may recur after transplantation in about 25% of patients.

In addition to ulcerative colitis, PSC is also associated with conditions summarized in the table below:

(Source: Primary Sclerosing Cholangitis. N Engl J Med 2016.)

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