Overview
Definition:
Intrahepatic duct stones (cholangiolithiasis) are gallstones located within the bile ducts inside the liver parenchyma
Surgical clearance refers to the operative removal of these stones to restore bile flow and prevent complications
This differs from common bile duct stones which are located in the extrahepatic bile ducts.
Epidemiology:
Intrahepatic stones are less common than common bile duct stones, occurring in approximately 1-2% of patients undergoing bile duct surgery
They are more prevalent in certain Asian populations and are often associated with recurrent pyogenic cholangitis, liver fluke infestations (e.g., Clonorchis sinensis), and biliary strictures.
Clinical Significance:
Untreated intrahepatic stones can lead to significant morbidity and mortality due to recurrent cholangitis, liver abscess formation, biliary cirrhosis, and portal hypertension
Early and effective surgical clearance is crucial for preventing these life-threatening complications and improving long-term liver function.
Clinical Presentation
Symptoms:
Right upper quadrant abdominal pain, often colicky
Intermittent jaundice and pruritus
Fever, chills, and malaise, indicative of cholangitis
Nausea and vomiting
Weight loss and fatigue in chronic cases
Anorexia.
Signs:
Jaundice, particularly scleral icterus
Tenderness in the right upper quadrant
Palpable liver (hepatomegaly) or an enlarged gallbladder (hydrops)
Fever
Signs of sepsis in severe cholangitis
Cachexia in advanced disease.
Diagnostic Criteria:
No specific universally accepted diagnostic criteria exist solely for intrahepatic stones
Diagnosis is typically made based on a combination of clinical suspicion, laboratory findings, and imaging suggestive of intrahepatic biliary dilatation with intraluminal filling defects or stones.
Diagnostic Approach
History Taking:
Detailed history of abdominal pain pattern, duration, and radiation
History of gallstones, previous biliary or liver surgery
Travel history to endemic areas for liver flukes
Symptoms of cholangitis (fever, jaundice, pain) – Charcot's triad
Reynolds' pentad (Charcot's triad plus altered mental status and shock) indicates severe disease.
Physical Examination:
Systematic abdominal examination focusing on tenderness, guarding, organomegaly
Assessment for jaundice and signs of sepsis
Cardiopulmonary examination to rule out associated complications.
Investigations:
Laboratory tests: Complete blood count (leukocytosis)
Liver function tests (elevated bilirubin, alkaline phosphatase, GGT, AST, ALT)
Coagulation profile
Blood cultures if sepsis is suspected
Imaging modalities: Ultrasound (initial assessment for intrahepatic duct dilatation and stones)
CT scan (better delineation of stone burden, ductal anatomy, and abscesses)
MRCP (gold standard for defining ductal anatomy, stone location, and extent of dilatation)
ERCP (diagnostic and therapeutic, but less preferred for solely intrahepatic stones due to higher risk of cholangitis and pancreas injury)
Percutaneous transhepatic cholangiography (PTC) for access and clearance in selected cases.
Differential Diagnosis:
Intrahepatic abscess
Pyogenic liver abscess
Hydatid cyst
Other intrahepatic cystic lesions
Liver tumors with biliary involvement
Biliary strictures without stones
Parasitic infections causing biliary obstruction.
Management
Initial Management:
Fluid resuscitation and correction of electrolyte imbalances
Empiric broad-spectrum antibiotics for suspected cholangitis
Analgesia for pain relief
Nutritional support
Urgent consultation with a hepatobiliary surgeon.
Medical Management:
Antibiotics targeting common biliary pathogens (e.g., E
coli, Klebsiella, Enterococcus)
Aim for coverage of gram-negative and anaerobic organisms
Typical regimen includes a beta-lactam/beta-lactamase inhibitor or a carbapenem, adjusted based on culture and sensitivity
Antipyretics and analgesics.
Surgical Management:
Surgical indications include symptomatic stones, recurrent cholangitis, liver abscess, biliary strictures, and suspected malignancy
Surgical approaches are tailored to the stone burden, location, and patient factors:
1
Laparoscopic or Open Bile Duct Exploration (BDE): For stones that can be accessed and removed from dilated intrahepatic ducts, often with a trans-choledochal approach
May involve choledochotomy and primary closure or T-tube drainage
2
Hepaticojejunostomy (Roux-en-Y): Preferred for extensive intrahepatic stones, dominant strictures, or when repeated clearance is anticipated
Bypasses the diseased intrahepatic ducts
3
Percutaneous Transhepatic Approach: For stones in peripheral intrahepatic ducts or when a trans-duodenal/trans-choledochal approach is not feasible
Involves percutaneous drainage and stone extraction
4
Hepatectomy: Reserved for localized, non-resectable intrahepatic stones, particularly in association with dominant strictures or liver abscesses not amenable to less invasive methods.
Supportive Care:
Close monitoring of vital signs, urine output, and fluid balance
Nutritional support, including adequate protein and calorie intake
Pain management
Physiotherapy to prevent respiratory complications.
Complications
Early Complications:
Bleeding from the operative site
Bile leak (choleperitoneum, biloma)
Cholangitis recurrence
Pancreatitis (especially after ERCP)
Injury to adjacent organs (e.g., bowel, vascular structures)
Anesthesia-related risks.
Late Complications:
Biliary strictures
Recurrent cholangitis
Liver abscess formation
Biliary cirrhosis
Portal hypertension
Stone recurrence
Cholangiocarcinoma (long-term risk associated with chronic biliary stasis and inflammation).
Prevention Strategies:
Aggressive management of suspected cholangitis pre-operatively
Thorough operative exploration and clearance of all accessible stones
Appropriate biliary drainage (T-tube, hepaticojejunostomy) to facilitate early detection of leaks and potential retained stones
Prophylactic antibiotics post-operatively in high-risk patients.
Prognosis
Factors Affecting Prognosis:
Extent and location of stones
Presence of dominant biliary strictures
Development of cholangitis or liver abscess
Overall liver function and degree of biliary cirrhosis
Patient's age and comorbidities
Skill and experience of the surgical team.
Outcomes:
With timely and appropriate surgical management, many patients can achieve stone-free status and resolution of symptoms
However, recurrence is a significant concern, and long-term follow-up is essential
Patients with advanced biliary cirrhosis or recurrent severe infections have a poorer prognosis.
Follow Up:
Regular outpatient follow-up is recommended, typically including clinical assessment and serial imaging (ultrasound, MRCP) to monitor for stone recurrence, strictures, or other complications
Frequency depends on the initial presentation and surgical procedure
Lifelong surveillance may be necessary for high-risk individuals.
Key Points
Exam Focus:
Differentiate intrahepatic from extrahepatic stones
Understand risk factors (parasites, strictures)
Key imaging modalities: US, CT, MRCP
Surgical strategies: BDE, hepaticojejunostomy, percutaneous, hepatectomy
Management of cholangitis is paramount
Recurrence is common.
Clinical Pearls:
Suspect intrahepatic stones in patients with recurrent cholangitis despite cholecystectomy and common bile duct clearance
MRCP is invaluable for anatomical delineation
Consider parasitic etiology in endemic regions
Hepaticojejunostomy offers a durable solution for extensive disease.
Common Mistakes:
Inadequate preoperative assessment of stone burden and ductal anatomy
Incomplete stone clearance
Failure to address dominant biliary strictures
Over-reliance on ERCP for solely intrahepatic stones
Neglecting long-term follow-up leading to undetected recurrence.