An Excerpt of 2022 European Association for the Study of the Liver Clinical Practice Guidelines on the Management of Cystic Liver Diseases / 胃肠病学

The advent of enhanced radiological imaging techniques has facilitated the diagnosis of cystic liver lesions. Concomitantly, the evidence base supporting the management of these diseases has matured over the last decades. As a result, comprehensive clinical guidance on the subject matter is warranted. This guideline covers the diagnosis and management of hepatic cysts, mucinous cystic neoplasms of the liver, polycystic liver disease, caroli disease, caroli syndrome, biliary hamartomas and peribiliary cysts. On the basis of in⁃depth review of the relevant literature, this guideline provides recommendations to navigate clinical dilemmas followed by supporting text. The recommendations are graded according to the Oxford Centre for Evidence⁃based Medicine system and categorized as "weak" or "strong". This guideline aims to provide the best available evidence to aid the clinical decision⁃making process in the diagnosis and treatment of patients with cystic liver diseases, and presents the readers with translations and summarizations of the above mentioned recommendations.

Biliary Duct Hamartomas: A Systematic Review.

Biliary duct hamartomas are benign intrahepatic bile duct lesions. Despite being primarily incidental findings on imaging, these lesions can provide a diagnostic conundrum due to their shared characteristics with malignant tumors. The goal of this systematic review is to offer a thorough clinical profile of biliary duct hamartomas. There were 139 cases of biliary duct hamartomas identified in a structured systematic review of the literature. Patient demographics, clinical presentation, significant laboratory and imaging data, diagnostic modalities, treatment choices, and outcomes were all studied and reported. Biliary duct hamartomas present with mild symptoms and laboratory abnormalities, and while being visible on imaging, the results are non-specific and may require biopsy in case of red flag signs such as weight loss and a progressive increase in the size of the lesion. Furthermore, there are currently no published guidelines for the treatment of biliary duct hamartomas, and many people have had surgery despite the clinically benign nature of these abnormalities. As per the findings of the study, individuals who exhibit signs of malignancy should be investigated further. Eyeballing for red flag symptoms, followed by a specialized imaging scan and invasive treatment, is the three-step approach to biliary duct hamartomas. Since our recommendations include a shift in strategy and do not contradict existing rules, there are likely to be few roadblocks to improvement; the key barriers being technological equipment and image quality. In this study, we intended to pave the way for future research in the field. In our opinion, the next decade will bring a better understanding of the characteristics of biliary hamartomas, disease symptoms, and better recognition of any suspicious features. These indications will aid in reducing the number of unneeded surgical or invasive operations. Finally, the findings of these future studies will allow the medical community to improve and provide the best care possible.

An excerpt of 2022 European Association for the Study of the Liver clinical practice guidelines on the management of cystic liver diseases / 中华消化杂志

The advent of enhanced radiological imaging techniques has facilitated the diagnosis of cystic liver lesions. Concomitantly, the evidence base supporting the management of these diseases has matured over the last decades. As a result, comprehensive clinical guidance on the subject matter is warranted. This guideline covers the diagnosis and management of hepatic cysts, mucinous cystic neoplasms of the liver, biliary hamartomas, polycystic liver disease, caroli disease, caroli syndrome, biliary hamartomas and peribiliary cysts. On the basis of in-depth review of the relevant literature, this guideline provide recommendations to navigate clinical dilemmas followed by supporting text. The recommendations are graded according to the Oxford Centre for Evidence-Based Medicine system and categorized as "weak" or "strong" . This guideline aims to provide the best available evidence to aid the clinical decision-making process in the management of patients with cystic liver disease, and presents the readers with translations and summarizations of the above mentioned recommendations.

MR imaging features of multiple biliary hamartomas (Von Meyenburg Complex): A pictorial review and differential diagnosis.

Biliary hamartomas (BHs) are rare malformative cystic/cystic-like lesions of the liver affecting the biliary tree, named after Hanns von Meyenburg who described them for the first time and still known with this eponym to this day. They usually lack clinical symptoms, and abnormalities in liver function tests are unusual; thus, it is typically an incidental finding of liver imaging. Despite being benign lesions, BHs can pose clinical challenges; the first one is differential diagnosis with other more relevant pathological conditions. Therefore, knowledge of MR imaging findings of BHs is helpful for a prompt and correct diagnosis, avoiding unnecessary invasive procedures and/or an excessive number of radiological investigations. This pictorial review is aimed to depict the most typical MR imaging features of multiple biliary hamartomas (von Meyenburg Complex), in order to familiarize the diagnosis and facilitate the differentiation from other hepato-biliary cystic diseases.

Portal hypertension in a patient with biliary hamartomas: A case report.

BACKGROUND: Biliary hamartomas (BH) are a rare benign disease caused by malformation of the intrahepatic bile ducts. BH are occasionally diagnosed, but often lack obvious clinical symptoms. They are usually diagnosed by biopsy and imaging tests in clinical practice. Few studies have reported the association of BH with portal hypertension. CASE SUMMARY: A 40-year-old man was repeatedly admitted to our hospital due to hematochezia. The source of bleeding was considered to be gastroesophageal varices and portal hypertensive gastropathy by endoscopy. He had no history of hepatitis virus infection, alcohol abuse, drug-induced liver injury, or autoimmune liver disease. He underwent magnetic resonance imaging, which showed rounded, irregular, low-signal-T1 and high-signal-T2 lesions diffusely distributed on the liver, that were not communicated with the biliary system on magnetic resonance cholangiopancreatography. According to the imaging examination, the patient was considered to have a diagnosis of BH with portal hypertension. CONCLUSION: Based on the present case report, BH may be a potential etiology of portal hypertension.

MR imaging and MR Cholangiopancreatography of multiple biliary hamartomas.

Multiple biliary hamartomas (MBH), also known as von Meyenberg complexes, are rare benign malformations of the intrahepatic bile ducts. It is thought that MBH result from ductal plate malformations involving the small interlobular bile ducts. Proliferation of bile ducts' cuboidal epithelium in fibrous stroma subsequently leads to duct dilation. Macroscopically, MBH appear as well-defined subcapsular or parenchymal grayish-white nodules less than 0.5 cm in diameter without true capsulation in an abundant fibrous stroma. MR imaging is considered the best imaging modality for demonstrating MBH. We herein reported MR imaging and MR cholangiopancreatography of a patient with typical MBH appearance. Multiple tiny T1 hypointense and T2 hyperintense lesions of uniform distribution in the hepatic parenchyma were revealed. At MR cholangiopancreatography, the lesions were also hyperintense. Maximum intense projection (MIP) of MR cholangiopancreatography showed uniformly distributed hyperintense lesions appearing as ''starry sky'' configuration.

Imaging findings of biliary hamartomas (von Meyenburg complexes).

To evaluate the imaging findings of biliary hamartomas (von Meyenburg complexes, vMCs) and discuss their differential diagnosis from other related diseases, imaging findings of biliary hamartomas on ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), and hepatobiliary scintigraphy were prospectively analyzed in six patients. On ultrasound images, five of the six cases showed multiple small hyper- and hypoechoic lesions with comet-tail echoes, especially when magnified using the zoom function. In all six cases, multiple tiny hypodense lesions less than 10 mm in diameter were scattered throughout the liver with no enhancement on CT. These tiny lesions were demonstrated to be hyper- and hypointense on T2- and TI-weighted images, respectively, in three patients who underwent MRI examinations. MRCP was performed in two patients, and it clearly showed multiple tiny round and irregular-shaped hyperintense lesions. MRCP and hepatobiliary scintigraphy showed normal appearances of intra- and extrahepatic bile ducts in two and one patients, respectively. Imaging modalities are useful in the diagnosis and differential diagnosis of vMCs. A correct diagnosis might be obtained when typical imaging findings present even without histological confirmation.