Hepatolithiasis: Description, Causes and Risk Factors:HepatolithiasisHepatolithiasis is endemic in the Asia-Pacific region, where its prevalence can be as high as 30-50%. The disease is rare in the West. However, increased rates of immigration from endemic areas are causing an ongoing rise in the incidence of hepatolithiasis in the West.Hepatolithiasis is defined as the presence of gallstones in the bile ducts proximal to the confluence of the right and left hepatic ducts, irrespective of the co-existence of gallstones in the common bile duct (CBD) and/or gallbladder. The etiology of hepatolithiasis is not fully understood, but genetic, dietary and environmental factors are thought to be contributory. Malnutrition and low socioeconomic conditions are associated with a high incidence of intrahepatic stones. Hence, economic advancement and the Westernization of lifestyle are associated with an apparent decline in the incidence of the disease in some East Asian countries. Intrahepatic stones occur more commonly in the 5th and 6th decades of life and do not demonstrate a gender preference. However, concomitant intrahepatic and extrahepatic stones occur commonly in older age groups (7th and 8th decades) and are found in approximately 70% of all hepatolithiasis cases.Relative proportion of hepatolithiasis:Taiwan 53.5%
  •  Shenyang 21.1%
  • Beijing 9.2%
  • Fukuoka 4.6%
  • Hong Kong 3.1%
  • Japan 2.2%
  • Singapore 1.7%
Hepatolithiasis is complex disease which requires a transhepatic team approach individualized for each patient according to the site of involvement & presence of strictures. Multidisciplinary approach including surgical, endoscopic and interventional radiological techniques has the best outcome.Symptoms:Signs and symptoms:Recurrent abdominal pain.
  • RUQ (right upper quadrant) pain.
  • Fever.
  • Portal hypertension.
  • Bouts of cholangitis.
  • Hepatic abscesses.
  • Lobar atrophy.
  • Cholangitis & cholangiocarcinoma.
  • Abnormal LFTs with elevation of alkaline phosphatase.
Diagnosis:Radiological studies and percutaneous procedures are keys in the diagnosis and treatment of hepatolithiasis. Non-invasive imaging modalities such as ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) accurately depict the normal anatomy and presence of intrahepatic stones. It should be stressed that each modality has its pros and cons, and imaging studies should be performed on the basis of understanding the pathophysiology. As the diagnostic role of magnetic resonance cholangiopancreatography (MRCP) evolves, the roles of both endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC), and their most significant advantage, is primarily therapeutic with their ability to extract stones, biopsy intraductal lesions, and place stents easily.Treatment:The primary treatment goal in hepatolithiasis is to resolve ongoing infections, prevent recurrent cholangitis and subsequent hepatic fibrosis.Non-surgical options: Non-surgical approaches to hepatolithiasis consist of the extraction of stones under radiological (PTC with or without lithotripsy or access through a T-tube) or endoscopic (ERCP with or without lithotripsy) guidance. Extracorporeal shock wave lithotripsy (ESWL) is particularly useful for cholesterol stones, and the use of holmium (Ho): YAG laser for calcium bilirubinate hepatolithiasis has shown favorable results. Although an ERCP (endoscopic retrograde cholangiopancreatography) is less invasive and easier in most cases, access is limited by the location of the strictures, ductal angulation and the degree of stone impaction. Percutaneous transhepatic cholangioscopic lithotomy or lithotripsy (PTCSL) is a widely used procedure for stone removal and dilation of strictures, particularly in right-sided, bilateral or recurrent disease. Note, however, that almost 40% of cases of hepatolithiasis have intrahepatic bile duct strictures which make stone extraction difficult, and the inability of non-surgical techniques to resolve strictures results in disease recurrence. Hence, non-surgical therapy is most useful in bilobar hepatolithiasis without strictures, or in patients who pose high surgical risk or have short life expectancies.Surgical options: The surgical approach to hepatolithiasis typically involves the removal of the affected segment(s). Hepatectomy removes stones, eliminates strictures and the consequent bile stasis which is responsible for stone formation, and eradicates the risk of cholangiocarcinoma; in rare cases it removes a known intraductal tumor. Indications for hepatectomy in hepatolithiasis are: (i) unilobar hepatolithiasis, particularly left-sided; (ii) atrophy, fibrosis and multiple abscesses secondary to cholangitis; (iii) suspicion of concomitant intrahepatic cholangiocarcinoma, and (iv) multiple intrahepatic stones with biliary strictures that cannot be treated percutaneously or endoscopically. Complete removal of the diseased lobe or segment is crucial to prevent recurrence and progressive liver disease.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.


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