Biliary Tract Diseases

Cholestasis  is  a  pathologic  state  of  reduced  bile  formation  or  flow  which  can  be  hepatocellular  (Intrahepatic),  where  an  impairment  of  bile  formation  occurs  or  ductular  (extra  hepatic),  where  impedance  to  bile  flow  occurs  after  it  is  formed.  Intrahepatic  causes  of  cholestasis  include  viral  hepatitis,  alcohol,  primary  biliary  cirrhosis,  drug  toxicity,  Hodgkin’s  lymphoma  and  pregnancy.  Extrahepatic causes include choledocholithiasis, carcinoma, and ascariasis of the biliary tree.  

Clinical presentation 

• Jaundice,
• Dark urine,
• Pale stools,
• Generalized body itching/pruritis.

Investigations 

• Laboratory  evidence  of  elevated  serum  levels  of  total  bilirubin,  direct  bilirubin,  alkaline phosphatase, gamma-glutamyl transferase, and transaminases. WITH
• Supporting radiological evidence of dilated intra or extra hepatic biliary radicles.

Pharmacological Treatment Definitive treatment:  

Identify and treat specific cause 

Supportive treatment: 

S: cholestyramine (PO) 4–16g/day 

OR 

S: ursodeoxycholic acid (PO) 20–30 mg/kg/day 

Note: Surgical intervention is indicated for extra hepatic cholestasis.

Cholelithiasis involves the presence of gallstones which are concretions that form in the biliary tract,  usually in the gallbladder. Choledocholithiasis refers to the presence of one or more gallstones in  the common bile duct (CBD). Treatment of gallstones depends on the stage of disease. 

Clinical presentation 

• Asymptomatic in majority of patients.
• Biliary pain (episodic RUQ or epigastric pain), pain radiating to scapula;
• Pain precipitated by fatty foods; nausea
• Physical exam: afebrile, and/or RUQ tenderness or epigastric pain
• Nonspecific symptoms (eg, indigestion, dyspepsia, belching, or bloating)

Investigations 

• Full Blood count
• Liver function panel
• Pancreatic enzymes (Amylase, Lipase)
• Abdominal radiography (upright and supine) – to exclude other causes of abdominal pain .
• Ultrasonography
• CT scan –superior for demonstrating stones in the distal CBD
• MRI with MRCP
• Scintigraphy (HIDA) scans
• Endoscopic retrograde cholangiopancreatography (ERCP)
• Percutaneous transhepatic cholangiography (PTC)

Complications 

• Cholecystitis
• Choledocholithiasis leading to cholangitis or gallstone pancreatitis
• Mirizzi syndrome
• Cholecystenteric fistula stone erodes through gallbladder into bowel
• Gallstone ileus: SBO (usually at term ileum) due to stone in intestine that passed through fistula
• Gallbladder carcinoma

Non-pharmacological Treatment 

Asymptomatic gallstones – Expectant management

Symptomatic gallstones – definitive surgical intervention (cholecystectomy)

Pharmacological Treatment 

A: paracetamol (PO) 1g 8hourly for 5days 

OR   

A: ibuprofen (PO) 400mg 4-6hourly for 3-5days 

OR 

B:  tramadol (PO) 50mg 12hourly for 3-5days 

AND 

S: ursodeoxycholic acid 8-10mg/kg/day divided once to three times up to 6months

Note: Cholecystectomy (open or laparoscopic) for asymptomatic gallstones may be indicated in the  following: 

• Those with large (>2 cm) gallstones
• Nonfunctional  or  calcified  (porcelain)  gallbladder  on  imaging  studies  and  at  high  risk  of gallbladder carcinoma
• Those with spinal cord injuries or sensory neuropathies affecting the abdomen
• Sickle cell anemia patients- difficult to distinguish between painful crisis and cholecystitis
• Cholecystectomy, Cholecystectomy, Endoscopic sphincterotomy,  Extracorporeal shockwave lithotripsy

Gallstone lodged in common bile duct (CBD). Occurs in some of patients with gallbladder stones; can form de novo in CBD. 

Clinical presentation 

• Asymptomatic
• RUQ pain,
• Epigastric pain due to obstruction of bile flow which leads to increase in CBD pressure

Investigations 

• LFT,
• Amylase
• Lipase.
• Abdominal (RUQ) Ultrasound
• ERCP, if ERCP unavailable or unsuccessful EUS/MRCP

Non-pharmacological Treatment 

• ERCP & papillotomy w/ stone extraction (± lithotripsy) 
• Cholecystectomy typically within 6 weeks unless contraindication 

Pharmacological Treatment 

S: cholestyramine (PO) 4-8g 12hourly when required for itching. 

Complications 

Cholangitis, cholecystitis, pancreatitis, stricture

Cholecystitis  is  inflammation  of  the  gallbladder  that  occurs  most  commonly  because  of  an  obstruction  of  the  cystic  duct  by  gallstones  (stone  impaction  in  cystic  duct  leads  to  inflammation  behind obstruction causing GB swelling and secondary infection of biliary fluid).  

Acalculous  cholecystitis:  Occurs  in  critically  ill.  GB  stasis  and  ischemia  (without  cholelithiasis)  leading to necroinflammation. 

Clinical presentation 

• RUQ/epigastric pain, radiating to right shoulder/back, nausea, vomiting, fever
• Signs of peritoneal irritation may be present
• Patients with acalculous cholecystitis may present with fever and sepsis alone
• RUQ tenderness, Murphy’s sign, palpable gallbladder, jaundice
• The absence of physical findings does not rule out the diagnosis of cholecystitis.

Investigations 

• FBP, LFT, amylase, lipase
• Ultrasonography RUQ ultrasound: high Sensitivity and specificity for stones but need specific signs of cholecystitis: GB wall thickening >4 mm, pericholecystic fluid and a sonographic Murphy’s sign
• Radiography
• Computed tomography (CT)
• Magnetic resonance imaging (MRI)
• Hepatobiliary scintigraphy (HIDA)
• Endoscopic retrograde cholangiopancreatography (ERCP)

Pharmacological Treatment In acute cholecystitis, the initial treatment includes bowel rest, IV hydration, and correction of  electrolyte abnormalities 

A: paracetamol (PO) 1g 8hourly daily ORD: paracetamol (IV) 1g 8 hourly daily (for 

AND 

C: metoclopramide (IV/PO) 10mg 12hourly daily (for intractable vomiting) 

AND  

S: piperacillin + tazobactam FDC (IV) 4.5g 6-8 hourly 7-10 days (for severe/complicated cases) 

OR  

B: ceftriaxone (IV) 1-2gm 24hourly for 7-10days  
                                                      OR  

C: ciprofloxacin (IV) 200-400mg 12hourly for 7-10days  

AND 

B: metronidazole (IV) 500mg 8hourly for 7-10days  

In cases of uncomplicated cholecystitis, outpatient treatment may be appropriate.  

A: ciprofloxacin (PO) 500mg 12hourly for 7days  

AND  

A: metronidazole (PO) 400mg 12hourly for 7days  

Note: 

• Laparoscopic cholecystectomy (standard of care for surgical treatment of cholecystitis) others include ERCP. 
• Endoscopic ultrasound-guided transmural cholecystostomy, Endoscopic gallbladder drainage.

Bile duct (BD) obstruction leads to infection proximal to the obstruction, etiologies include BD stone,  malignant (biliary, pancreatic) or benign stricture, infection with fluke (Clonorchis sinensis,  Opisthorchis viverrini) 

Clinical presentations  

• Charcot’s triad: RUQ pains, jaundice, fever/chills; present in three quarter of patients 
• Reynolds’ pentad: Charcot’s triad + shock and altered mental status; present in 15% of patients 

Investigations  

Ultrasound, RUQ USS often demonstrates dilation 

FBP, LFT, amylase, lipase  

Blood cultures 

ERCP; percutaneous transhepatic cholangiogram if ERCP unsuccessful 

Pharmacologic Treatment 

B: ceftriaxone (IV) 1-2g 24hourly for 7-10days  

OR 

S: piperacillin+tazobactam FDC (IV) 4.5g 6-8hourly for 7-10days (for severe/complicated 

cases) 

OR  

S: meropenem (IV) 1g 8 hourly for 7-10days  

AND  

C: metronidazole (IV) 500mg 8hourly for 7-10days  

Alternatively 

C: ciprofloxacin (IV) 200-400mg 12hourly for 7-10days  

AND 

B: metronidazole (IV) 500mg 8hourly for 7-10days  

Note:

• About  80%  respond  to  conservative  treatment  and  antibiotics  then,  20%  require  urgent biliary decompression via ERCP (papillotomy, stone extraction and/or stent insertion)
• If sphincterotomy cannot be performed (larger stones), decompression by biliary stent or nasobiliary catheter can be done; otherwise, percutaneous trans hepatic biliary drainage or surgery