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Amar, Al-Ozaibi, and Badri: Hepatolithiasis: a case report and literature review


Hepatolithiasis is defined as calculi in the biliary ducts proximal to the joining of the right and left hepatic ducts regardless of the presence of concurrent gallstones in either the gall bladder or common bile duct.1 Its prevalence reaches 30–50% in East Asia.2

In most cases hepatolithiasis is asymptomatic. When patients do present with symptoms, these symptoms are usually features of cholangitis (fever, jaundice and right upper-quadrant abdominal pain). Other symptoms may include chronic stomachache, abscesses and liver cirrhosis. The natural history of hepatolithiasis involves recurrent episodes of cholangitis that lead to chronic inflammatory changes and further stricture formation in the bile ducts, causing bile stasis. Hepatolithiasis is one of the known risk factors for cholangiocarcinoma. Based on several studies, the prevalence of cholangiocarcinoma in patients with hepatolithiasis ranges between 5% and 13%.3,4

Case presentation

A 43-year-old Bengali man presented to the emergency department with right upper-quadrant abdominal pain, fever, chills and jaundice of 2 days’ duration. His surgical history revealed recurrent cholangitis, pancreatitis, liver abscess, cholecystectomy more than 10 years previously and multiple endoscopic retrograde cholangiopancreatographies (ERCPs), one of which was accompanied a sphincterotomy. On presentation, he was conscious, his temperature was 38°C and his blood pressure was 125/78 mmHg. He had scleral icterus and right upper-quadrant abdominal tenderness with a positive Murphy’s sign. Blood analysis revealed a white blood cell count of 19.5 × 109/l, alkaline phosphatase (ALP) of 200 IU/l, total bilirubin of 3.8 mg/dl and C-reactive protein of 405 mg/l. Lipase (11.7 U/l) and alanine aminotransferase (90 U/l) were within normal limits. Computed tomography (CT) of the abdomen revealed multiple intrahepatic stones, with the largest occluding the left hepatic biliary duct and causing proximal dilation of the hepatic duct (Figures 1 and 2).


Axial pre-contrast image showing a hypodense stone in the left main hepatic duct with proximal dilation of the duct.


Coronal pre-contrast image showing a hypodense stone in the left main hepatic duct with proximal dilation of the duct.


The patient was admitted and acute recurrent ascending cholangitis was diagnosed. ERCP showed proximal left hepatic duct occlusion with the proximal duct dilated and filled with pus. A stent was inserted for draining the pus. Left hepatectomy was later carried out because of the presence of multiple large stones, the location of these stones and recurrent cholangitis (Figure 3). The postoperative period was uneventful; the patient’s vital signs were stable and he was doing well. There was no longer abdominal pain or jaundice and he was discharged 1 week later.


The resected left liver lobe with the stones extracted in the kidney dish.



The aetiology of hepatic stones is suggested to be multifactorial with diet possibly playing a larger role than ethnicity.5 Parasitic causes have also been implicated, but the evidence is inconclusive and these causes have been suggested to be incidental.6 Considering that the bile of patients with hepatolithiasis has an almost 100% prevalence of bacteria as opposed to the sterile bile of patients without hepatolithiasis, bacteria have also been implicated.7 Most of these bacteria show β-glucuronidase activity (responsible for catalysing the hydrolysis of direct to indirect bilirubin). This unconjugated bilirubin is water insoluble and combines with calcium bilirubinate to form calcium bilirubinate stones, which are the most prevalent type of stones in hepatolithiasis (80%).8

Bile stasis caused by many underlying hepatic diseases (such as sclerosing cholangitis or secondary to benign strictures) is another possible cause. This is proposed as the main cause of hepatolithiasis in the West.5

Stones are more common in the left lobe of the liver and the reason for this is thought to be the merging of the left hepatic duct at an acute angle with the common bile duct, which tends to induce bile stasis in an already narrow biliary tree with strictures.

The diagnosis of hepatolithiasis is multimodal and similar to the process of diagnosing choledocholithiasis or choleliathiasis. Ultrasonography, contrast-enhanced CT and magnetic resonance cholangiopancreatography may all be used. However, the gold standard for therapy and diagnosis is ERCP and percutaneous transhepatic cholangiography (PTC). Carcinoembryonic antigen levels may be checked to determine the presence of concomitant cholangiocarcinoma.3

To decide the approach to the surgical management of hepatolithiasis, Dong’s classification system is used (Table 1). This classifies the disease based on location of the stones and the presence of intrahepatic bile duct strictures or hepatic parenchymal atrophy.1


Dong’s classification, which aids the approach to surgical management of hepatolithiasis

Hepatolithiasis type Definition
1 Localized stone disease: unilobar or bilobar
2 Diffuse stone disease
2a No atrophy of the hepatic parenchyma or stricture of the intrahepatic bile duct
2b Segmental atrophy and/or stricture of the intrahepatic bile ducts
2c Biliary cirrhosis and portal hypertension
Additional type E Extrahepatic stones

The goal of managing hepatolithiasis is to break the vicious pathophysiological circle by treating ongoing infections, preventing the recurrence of cholangitis and consequent hepatic fibrosis, reducing repeated instrumentation, and preventing progression to cholangiocarcinoma.

The current options for treating hepatolithiasis include non-surgical endoscopic approaches and hepatectomy. Endoscopic management, including PTC, peroral cholangioscopy and ERCP, can be combined with extracorporeal shockwave lithotripsy – for cholesterol stones – or holmium:YAG laser surgery. Although less invasive and easier to implement in most cases, a limitation to endoscopic techniques is that around 40% of patients with hepatolithiasis have strictures in the intrahepatic bile duct. This not only makes extraction of stones difficult, but does not resolve these strictures, which, in turn, causes disease recurrence. In addition, already limited access to intrahepatic stones is made more difficult by the location of these strictures, angulation of the ducts and extent of stone impaction.2 Newer procedures include endoscopic resection of peripheral bile duct stenoses to facilitate the evacuation of intrahepatic stones and chemical bile duct embolization. However, as the latter technique has been successfully demonstrated in only two patients to date, these newer approaches need to be studied for efficacy in larger numbers.9 According to Öztürk et al.,10 in patients with a high load of calculi, percutaneous intervention through a minimally dilated access tract is a safe and successful method of eliminating the stones.

Until 1998, surgery was the primary method for managing hepatolithiasis; however, management has since shifted to primarily non-surgical techniques. In 2011, 66.7% of hepatolithiasis cases were treated using non-operative approaches, with the most common procedures being ERCP and stone extraction.11 However, when comparing hepatectomy with percutaneous transhepatic cholangioscopic lithotripsy as management options for hepatolithiasis, hepatectomy was associated with a higher rate of survival (77%), a lower rate of bile duct strictures (18% vs. 58%) and a lower rate of recurrence of stones and/or cholangitis.2,12,13 A lower rate of recurrence is an important risk factor as the prevalence of cholangiocarcinoma increases with recurrent hepatolithiasis.14

Indications for surgical management include unilobar disease, fibrosis due to recurrent cholangitis, multiple abscesses, suspicion of concurrent intrahepatic cholangiocarcinoma and disease that cannot be treated endoscopically.15


Hepatolithiasis is a rare disease found mostly in East Asia. The gold standard for its diagnosis is ERCP and PTC, but the presence of concomitant cholangiocarcinoma adds considerable risk. Indications for its surgical management include the presence of stones in one liver lobe (especially the left lobe), multiple large stones that cannot be treated by other methods, complications secondary to cholangitis and suspected cholangiocarcinoma.


Written consent was obtained from both patients for the publication of this case report and accompanying images.

Conflict of interest

No conflict of interest.



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