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Benign biliary strictures

Section: Hepatobiliary Sub-section: Biliary Curriculum: Curriculum, page 89

Aetiology

Strictures of the extrahepatic biliary tree

  • Iatrogenic biliary injury
    • Post cholecystectomy
    • Trauma
    • Other
  • Gallstone-related
    • Mirizzi syndrome
  • Inflammatory
    • Recurrent pyogenic cholangitis
    • Parasitic infestation
    • Clonorchis sinensis
    • Opisthorchis viverrini
    • Echinococcus
    • Ascaris
  • HIV/AIDS cholangiopathy
  • Primary sclerosing cholangitis
  • Benign strictures imitating malignancy
  • Pancreatitis
  • IgG4-related disease
  • Autoimmune pancreatitis
  • IgG4-related cholangiopathy
    • Inflammatory pseudotumour

Liver flukes (Trematode)

  • From raw fish.
  • Immature fluke passes into biliary tree, grow to maturity.
  • Ova passed into GI tract and subsequently water supplies.
  • Clinical presentation:
    • Asymptomatic or acute febrile illness or chronic symptoms.
    • Chronic infestation can cause hepatolithiasis.
  • Diagnosis
    • Detection of ova within stool or in duodenal aspirates and eosinophilia may be present on blood film.
    • ERCP may show slender filling defects within CBD +/- fibrosis & calculus formation.
  • Treatment

Echinococcus

  • Biliary obstruction can occur due to local compression of the common hepatic duct by the expanding cyst, or when daughter cysts pass down the common hepatic duct following rupture of the cyst into intrahepatic radicles.
  • Secondary sclerosing cholangitis has been described following inappropriate injection of scolicidal agents into the hepatic cyst when there is communication with the biliary tree.

Ascaris lumbricoides

  • Roundworm – commonest worm to infect humans.
  • Rarely presents with obstructive jaundice.
  • Can be due to migration of worm into biliary tree or worm traversing the ampulla.
  • Associated with recurrent pyogenic cholangitis.
  • USS sometimes sees long linear filling defect within biliary tree.
  • ERCP – may identify, with endoscopic extraction possible.
  • Medical treatment with anthelmintics
  • Late complication of papillary stenosis
    • Can be treated with sphincterotomy.

HIV/AIDS-associated cholangiopathy

  • Thought secondary to opportunistic infection of biliary tree by CMV, cryptosporidium or other organisms.
  • Presentation: RUQ pain, abnormal LFTs. Jaundice is unusual.
  • MRCP – characteristic ductal abnormalities of multiple intrahepatic strictures, papillary stenosis and long segmental extrahepatic strictures.
  • ERCP is gold standard for diagnosis.
  • Provides symptomatic relief following sphincterotomy.
  • Antiretroviral therapy associated with regression of cholangiographic abnormalities, but endoscopic interventions have not been associated with improvements in prognosis.

Biliary strictures imitating malignancy

  • Most common pathology is chronic pancreatitis.
  • Others include IgG4-related disease, PSC and choledocholithiasis.

Primary sclerosing cholangitis

Defintion

Idiopathic chronic and progressive disorder manifesting as stricturing, inflammation, and fibrosis of intra- and extrahepatic bile ducts.

Aetiology

  • Rare, precise cause not determined but increasing evidence of immunological basis.
  • Majority have UC or rarely Crohn’s.
  • Association with number of human leucocyte antigens.

Ulcerative colitis

  • 5% of patients with UC develop it
  • 75% of patients with PSC are found to have concurrent UC
  • Patients with coexisting PSC and UC are at a markedly increased risk for colonic neoplasia (five times)
  • Independent on activity of colitis
  • An increased risk of development has been demonstrated in patients with human leucocyte antigen (HLA) B8, DR2, DR3 or DR6 haplotype positivity.
  • Treatment of PSC with steroids, colectomy or antibiotics is ineffectual.
  • Patients undergoing restorative proctocolectomy have a higher subsequent incidence of pouchitis and dysplasia in the ileal pouch mucosa

Presentation

  • Early
    • Asymptomatic.
  • Late
    • Jaundice, pruritis, pain, fever and weight loss.
    • Abnormal LFTs during investigation of IBD.
  • Advanced
    • Liver failure.
    • Sudden deterioration – may be cholangiocarcinoma (which there is strong association).

Investigation

  • LFTs – cholestatic.
  • ANCA present in majority, however autoantibodies tested to exclude Primary biliary cirrhosis
  • Cholangiography
    • Demonstrates diffuse stricturing and attenuated intrahepatic bile ducts.
    • ERCP enables brushing and endoscopic therapy.
    • MRCP preferred for diagnosis – highly sensitive.

Prognosis

  • Poor prognosis (median survival 10 years from diagnosis)
  • Development of cholangiocarcinoma or colorectal cancer has become leading cause of death.

Management

  • Endoscopic or transhepatic dilatation of short dominant strictures with or without endoscopic stenting has been described as effective
  • Liver transplant necessary for end-stage liver disease.
    • Recurrence has been reported at 15-25% in transplanted liver
    • Indications: persistent jaundice, intractable pruritis, recurrent cholangitis, malnutrition or fatigue. Many undergo before liver failure occurs or when cholangiocarcinoma suspected.

Exclusion of malignant stricture

  • Important to exclude cholangiocarcinoma or GB cancer (can occur in 10-36% of PSC)
  • Rapid deterioration or dominant stricture should be investigated extensively.
  • Serum Ca 19-9 used.
  • CT/MRI may show mass lesion in biliary tree.
  • PET is superior to differentiate between PSC and cholangiocarcinoma.
  • ERCP + brush cytology may give diagnosis;
  • ERCP cholangioscopy + targeted biopsies most accurate.
  • Laparoscopy – may be used in assessing for transplant.

IgG4-related disease

Defintion

IgG4-related disease is an immune-mediated condition that mimics many malignant, infectious and inflammatory disorders, but the aetiology remains unknown.

Presentation

  • Can cause biliary stricutres
    • Autoimmune pancreatitis
      • Two types of autoimmune pancreatitis are reported, with type I associated with IgG4 disease. Although only accounting for about 2.4% of pancreatic resections, the condition is important since a proportion of patients will develop either biliary anastomotic strictures or intrahepatic strictures following resection.
    • IgG4-related sclerosing cholangitis
      • IgG4-related sclerosing cholangitis is commonly associated with type I autoimmune pancreatitis and can be difficult to differentiate from cholangiocarcinoma.
    • Inflammatory pseudotumours
  • Retroperitoneal fibrosis
  • Periaortitis
  • Sclerosing mesenteritis
  • Multifocal fibrosclerosis
  • Extra-abdominal diseases such as Riedel’s thyroiditis.

Diagnosis

  • Radiological – enlarged pancreatic head mass with delayed enhancement without pancreatic duct dilatation.
  • Tissue is gold standard for diagnosis (demonstration of high number of IgG4-positive plasma cells).
    • EUS biopsy – however can be difficult to obtain adequate tissue

Treatment

  • Glucocorticoids are mainstay
    • Resolution within 1–2 weeks so avoidance of endoscopic stenting may be beneficial to aid in the diagnostic process
    • In the absence of biochemical or histological proof of IgG4 disease but typical radiological appearance, treatment with high-dose glucocorticoids usually leads to prompt resolution of jaundice and imaging findings.
  • PET may be useful to assess treatment response.

Sphincter of Oddi dysfunction

  • Often had cholecystectomy, middle aged, female Recurrent abdominal pain after cholecystectomy
  • Three types:
    • Type 1 – abdominal pain, obstructive LFTs, biliary dilatation and delayed emptying of contrast at ERCP
    • Type 2 – pain with only one or two of above
    • Type 3 – recurrent biliary pain
  • Diagnosis usually by exclusion. Morphine-neostigmine and secretin provocation MRCP may be of diagnostic value.
  • Biliary manometry should be reserved for patients whose diagnosis is unclear (type 2)
  • Medical therapy with calcium channel blockers, nitrates and botox is available, long term results unknown.
  • Avoidance of opioid may prevent onset of pain. Development of pain following codeine is diagnostic.
  • ERCP potential treatment. 5-16% post ERCP pancreatitis. Good responses in 69%.
  • Surgical sphincterotomy, only if ERCP unsuccessful.

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