Summary
A biliary stricture is an abnormal narrowing of the bile ducts that can obstruct bile flow. Etiologies are broadly categorized as malignant (e.g., pancreatic cancer, cholangiocarcinoma) or benign (e.g., postsurgical changes, chronic pancreatitis, primary sclerosing cholangitis). Patients may be initially asymptomatic or present with symptoms such as jaundice, pruritus, fatigue, or symptoms of acute cholangitis. Diagnosis includes findings of cholestasis, biliary strictures and/or obstruction, and histopathologic confirmation. Management focuses on biliary drainage to alleviate symptoms and facilitate further treatment.
Secondary sclerosing cholangitis is a chronic cholestatic biliary disease characterized by biliary strictures resulting from identifiable causes, which can lead to biliary cirrhosis.
Primary sclerosing cholangitis is discussed separately.
Definitions
- Biliary stricture: an abnormal narrowing of the bile ducts that can obstruct the flow of bile [1]
- Secondary sclerosing cholangitis: a chronic cholestatic biliary disease characterized by biliary strictures resulting from identifiable causes, which can lead to biliary cirrhosis [2][3]
Epidemiology
- Malignant extrahepatic biliary strictures: approx. 34,000 new cases annually [1]
- Malignant perihilar strictures: approx. 3,000 new cases annually [1]
- Secondary sclerosing cholangitis is relatively rare. [4]
Epidemiological data refers to the US, unless otherwise specified.
Etiology
Biliary strictures can have malignant (more likely) or benign causes.
Malignant [1][5]
-
Primary
- Biliary malignancy (e.g., cholangiocarcinoma, gallbladder cancer, ampullary cancer, ovarian cystadenocarcinoma)
- Pancreatic cancer
- Hepatocellular carcinoma
- Lymphoma
- Combined hepatocellular-cholangiocarcinoma
- Metastatic
Benign [1][3][5][6]
-
Autoimmune or inflammatory
- Primary sclerosing cholangitis
- IgG4-mediated disease (e.g., IgG4-related sclerosing cholangitis or pancreatitis)
- Sarcoidosis
- Eosinophilic cholangitis
- Follicular cholangitis
- Chronic pancreatitis
-
Iatrogenic
- Surgical (e.g., cholecystectomy, liver transplantation)
- Locoregional cancer therapy (e.g., chemoembolization, radiation therapy)
- Drug-induced (e.g., immune checkpoint inhibitors, ketamine, amoxicillin/clavulanate)
-
Mechanical or anatomical
- Extrinsic compression by a pancreatic fluid collection
- Mirizzi syndrome
- Congenital (e.g., choledochal cysts, Caroli syndrome, biliary atresia)
- Choledochoduodenal fistula
-
Vascular
- Portal hypertensive biliopathy
- Ischemic biliary injury
- Systemic conditions
-
Infection
- Tuberculosis
- Viral (e.g., HIV/AIDS cholangiopathy)
- Parasitic (e.g., liver fluke, ascaris)
- Recurrent pyogenic cholangitis
- Other: secondary sclerosing cholangitis in critically ill patients
Subtypes and variants
Recurrent pyogenic cholangitis [7]
- Definition
- A chronic biliary disease characterized by the primary formation of infected intrahepatic stones and recurrent episodes of acute bacterial cholangitis that lead to biliary strictures, stasis, and hepatolithiasis
- Ongoing infection and inflammation result in recurrent stone formation and progressive biliary damage.
- Most common in individuals of Asian descent
- Associated with:
- Parasitic infections (e.g., Clonorchis sinensis, Ascaris lumbricoides)
- Living in rural environments
- Low socioeconomic status
- Up to 6% of patients develop cholangiocarcinoma. [7]
Clinical features
Patients with biliary strictures may be initially asymptomatic or present with nonspecific symptoms. [1][3]
- Symptoms of biliary obstruction [1][3][4]
- Jaundice
- Pruritus
- Fatigue
- Right upper quadrant abdominal pain
- Clinical features of acute cholangitis
- Symptoms of underlying condition (e.g., dyspepsia, weight loss, anorexia in pancreatic mass)
Diagnosis
General principles
- Suspect biliary stricture in patients with a cholestatic injury pattern.
- Obtain abdominal imaging in all patients to characterize strictures or obstruction.
- Evaluate all patients with sclerosing cholangitis for an underlying cause.
- Histopathology is typically required for a definitive diagnosis.
Laboratory studies [5]
Initial studies
-
LFTs: cholestatic injury pattern with conjugated hyperbilirubinemia [5]
- ↑ ALP, ↑ GGT, ↑ direct bilirubin
- ↑ AST and ↑ ALT
- CBC: assessment for leukocytosis [3]
Additional studies [3][5]
Additional testing should be obtained based on the suspected diagnosis, and include the following:
- Tumor markers (e.g., CA 19-9, CEA)
- HIV testing
- Serum immunoglobulins (e.g., IgG4); see "Diagnosis of IgG4-related disease."
Imaging [3][4][5]
Abdominal imaging should be obtained in all patients with biliary strictures and/or obstruction. Findings of secondary sclerosing cholangitis are similar to primary sclerosing cholangitis.
-
Ultrasound
- Initial imaging modality
- Used to assess for biliary dilatation, presence and location of biliary obstruction, gallstones, pyogenic liver abscess
-
MRI/MRCP (preferred) or CT with contrast [1][3][5]
- Obtained to identify the cause (e.g., mass), characterize strictures, and assess for resectability
- MRI/MRCP is considered for suspected hilar obstruction, to help identify the extent and location of biliary strictures, and to guide endoscopic therapy. [5]
- CT is considered for extrahepatic biliary obstruction and to help identify metastatic disease.
Advanced imaging [1][3][5]
- Obtained for anatomical characterization (e.g., morphology, stricture location and extent), tissue diagnosis, and identifying the underlying etiology
- Modalities
- Endoscopic ultrasound with fine-needle aspiration biopsy (FNAB)
- Endoscopic retrograde cholangiopancreatography with tissue sampling (e.g., FNAB, bile duct brushings for cytology)
Management
The primary goal of managing biliary strictures is to restore the flow of bile. [1]
- Promptly initiate treatment of acute cholangitis if present.
- Treatment of secondary sclerosing cholangitis depends on the underlying etiology.
- Consult gastroenterology to consider ERCP with stenting for biliary drainage.
- Consult interventional radiology for percutaneous transhepatic biliary drain placement if ERCP is not successful or feasible.
- Consult additional services (e.g., oncology, surgery) depending on the etiology.
Complications
- Disease-related [1][3]
-
Procedure-related [1]
- Acute cholecystitis (after stent placement)
- Post-ERCP pancreatitis
- Infection (e.g., bacteremia, pyogenic liver abscess, acute cholangitis)
- Hemorrhage (e.g., hematoma, hemobilia, post-sphincterotomy bleeding)
- Leak and/or perforation of bile duct
We list the most important complications. The selection is not exhaustive.
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