Hepatobiliary stone disease is one of the common diseases in China, and its condition is complex and difficult to treat. In order to meet the needs of clinical work and standardize the diagnosis and treatment of hepatobiliary duct stone disease in China, the Biliary Surgery Group of the Chinese Medical Association, Branch of Surgery, organized relevant experts in China, and based on the summary of more than 40 years of experience in the diagnosis and treatment of hepatobiliary duct stone disease in China and the latest clinical research results at home and abroad, formulated the first “Guidelines for the Diagnosis and Treatment of Hepatobiliary Duct Stone Disease”. It should be noted that the essence of clinical medicine lies in taking the most reasonable diagnostic and therapeutic measures according to the specific condition of the patient and the available medical resources. Therefore, the guidelines are intended to guide physicians in making rational decisions about the diagnosis and treatment of hepatobiliary stone disease, and are not mandatory standards. In the future, this guideline will be revised and updated periodically as research on hepatobiliary duct stone disease progresses and evidence-based medical evidence accumulates. The term hepatolithiasis (primary intrahepatic stone) refers to stones that originate in the intrahepatic bile duct system, excluding stones that drain from the gallbladder and migrate up to the intrahepatic bile duct, and excluding stones secondary to other biliary diseases, such as injury bile duct stricture, bile duct cyst, bile duct anatomical variation, etc. It also does not include stones formed in the hepatic bile ducts secondary to other biliary diseases such as biliary stasis and biliary inflammation. Hepatobiliary stone disease is a common disease in China, especially in the vast regions of South China, Southwest China, Yangtze River basin and Southeast coast. Because of its complex lesions, high recurrence rate and often serious complications, this disease has become an important cause of death from benign biliary diseases in China. Most hepatobiliary stone diseases are bile pigment stones. Cholesterol stones originating from the intrahepatic bile ducts can also be seen clinically. Since their stone formation mechanism and clinicopathological features are different from those of bile pigment intrahepatic bile duct stones, they should be treated as a separate disease. This guideline is for the diagnosis and treatment of pigmented hepatobiliary stones. 2. Etiology and basic pathological changes of hepatobiliary stone disease The etiology of hepatobiliary stone disease is not fully understood. The formation of intrahepatic stones is related to chronic inflammation of the biliary tract, bacterial infection, biliary ascariasis, biliary stasis, malnutrition and other factors. Chronic inflammation in the bile ducts is an important factor in stone formation, and bile stasis is a necessary condition for stone formation. Stagnant bile flow and chronic inflammation of the biliary tract are most likely to form intrahepatic bile duct stones. The basic pathological changes of hepatobiliary stone disease are biliary obstruction, biliary tract infection and destruction of the liver parenchyma. The hepatic bile ducts in the affected area are dilated, with circumferential or segmental narrowing of the bile ducts; thickening of the duct walls, hyperplasia of the bile duct walls and surrounding fibrous tissues, and chronic inflammatory cell infiltration; massive inflammatory cell infiltration and fibroblast proliferation in the confluent area, accompanied by damage to the liver parenchyma and, in severe cases, fibrotic atrophy and loss of function of liver segments or lobes. The combination of biliary tract infection can cause a series of serious complications such as biliary sepsis, liver abscess, subphrenic abscess, biliary bronchial fistula and biliary hemorrhage. About 2.0% to 9.0% of cases of hepatobiliary stones can be complicated by hepatobiliary cancer in the later stages of the disease. The important clinicopathological features of hepatobiliary stone disease are: (1) Stones are distributed segmentally along the diseased bile duct tree in the liver. (2) Stones often coexist with varying degrees of hepatic bile duct stenosis, which is an important factor in stone formation and recurrence. Biliary stenosis is an important factor in stone formation and recurrence. When hepatic bile duct stones are combined with stenosis of the hepatic ducts above the first level of branching, it is easy to lead to atrophy of the affected hepatic segments or subhepatic segments. (3) Long-term recurrent biliary obstruction and/or infection can lead to diffuse and irreversible damage to the biliary tree, associated vessels and liver parenchyma in the area of hepatobiliary stone lesions, including structural destruction of the bile duct wall, multiple bile duct stenosis and irregular bile duct dilatation, bile duct pus accumulation, narrowing of the portal vein and small branches of the hepatic artery, fibrosis and atrophy of the liver parenchyma, chronic liver abscess, secondary intrahepatic bile duct cancer and other destructive lesions. These lesions can be effectively treated only by surgical resection. (4) Atrophy of liver tissue occurs within the lesion area of hepatobiliary stone disease, while normal liver tissue proliferates and hypertrophies, forming liver atrophy.