Successful Treatment with Steroids in a Patient with Vanishing Bile Duct Syndrome and Acute Tubular Necrosis.

Vanishing bile duct syndrome (VBDS) is a rare but potentially serious cholestatic liver disease caused by various etiologies, including drugs. We herein report a complicated case of VBDS with acute tubular necrosis (ATN) that improved significantly with steroid treatment. An Asian man in his 30s was admitted with the acute onset of severe jaundice and a decline in the renal function. Although initial treatment with ursodeoxycholic acid did not reduce jaundice or renal dysfunction, steroid treatment remarkably improved the VBDS and ATN to within the respective normal ranges. Steroid treatment can be considered in cases of VBDS that appear to have an immune-mediated cause.

Biliary microhamartomas (von Meyenburg complexes), liver metastasis simulators: a series of eight cases.

BACKGROUND: Von Meyenburg complexes are benign hamartomatous lesions, they are part of the spectrum of ductal plate malformations. They are rare, reported in 0.35-5.6% of the general population, predominantly in adults, with no clear predilection for sex. OBJECTIVE: To present the clinical characteristics of Von Meyenburg complexes in our region. METHOD: We searched all cases with diagnosis of Von Meyenburg complexes in a period from 2012 to 2022, in our institutions. RESULTS: We identified eight cases, with an average age of 59.25 years, with a predominance of females and with one case associated with gastric carcinoma. CONCLUSIONS: It is important to adequately recognize this entity, since due to its multifocal nature it can easily simulate metastasis, additionally, and its presence does not rule out other synchronous neoplasms.

ANTECEDENTES: Los complejos de Von Meyenburg son lesiones hamartomatosas benignas que forman parte del espectro de las malformaciones de la placa ductal. Son poco frecuentes, se reportan en un 0.35-5.6% de la población general, predominantemente en adultos, sin clara predilección por un sexo. OBJETIVO: Presentar las características clínicas de los complejos de Von Meyenburg en nuestro medio. MÉTODO: Se buscaron todos los casos con diagnóstico de complejos de Von Meyenburg en nuestras instituciones entre 2012 y 2022. RESULTADOS: Identificamos ocho casos, con un promedio de edad de 59.25 años, con predominio por el sexo femenino y con un caso asociado a carcinoma gástrico. CONCLUSIONES: Es importante reconocer y diagnosticar adecuadamente esta afección, ya que por su naturaleza multifocal fácilmente puede simular metástasis, y además su presencia no descarta otros procesos neoplásicos sincrónicos.

[Implementation of the «safe cholecystectomy¼ program in the Leningrad Region]. / Realizatsiya programmy «bezopasnaya kholetsistektomiya¼ v Leningradskoi oblasti.

OBJECTIVE: To present a treatment program for patients with cholelithiasis in the region in accordance with modern requirements for the quality of medical care in the realities of a three-level system of surgical care. MATERIAL AND METHODS: The results of treatment of patients with cholelithiasis at various levels of medical care were analyzed with an assessment of the indicators of operational activity of performing cholecystectomy by laparoscopic and open methods, the development of complications of surgery and inpatient mortality. RESULTS: A programmatic approach has been developed to assist patients with cholelithiasis in the conditions of regional healthcare at different levels of surgical care. CONCLUSION: The implementation of this program minimizes the number of postoperative complications and mortality at the second and third levels of surgical care. It is determined that a rational approach to reduce the number of bile duct injuries is their prevention by impeccable compliance with the technique of surgical intervention on the organs of the upper floor of the abdominal cavity, and to reduce the number of negative consequences - compliance with the proposed algorithm of diagnosis and treatment.

Improvement of vanishing bile duct syndrome with hyperlipidemia.

A 75-year-old man was admitted to our hospital with acute onset of marked jaundice, elevated liver enzymes, and hyperlipidemia. He had been taking clopidogrel and pemafibrate for 3 months. He tested negative for autoantibodies and hepatitis-causing viruses. Gadoxetate-enhanced magnetic resonance imaging showed diffusely hypointense liver parenchyma in the hepatobiliary phase, with no appreciable excretion of gadoxetate into the biliary system. Histological examination of a liver specimen revealed disappearance of the bile ducts in the portal area and decreased expression of organic transporting polypeptide 1B3 on immunostaining. The patient was diagnosed with drug-induced vanishing bile duct syndrome and treated with ursodeoxycholic acid. The signs of liver dysfunction shown on blood chemistry tests improved spontaneously. After the acute hepatitis and lipid abnormalities had improved, repeat liver biopsy and gadoxetate-enhanced magnetic resonance imaging revealed improvement of the vanishing bile duct syndrome and recovery of the expression of organic transporting polypeptide 1B3. The reduction of OATP1B3 expression might be involved in the development of vanishing bile duct syndrome.

Benign Finding or Malignancy: von Meyenburg Complexes Found during Laparoscopic Cholecystectomy.

Von Meyenburg complexes are benign bile duct hamartomas that arise as cystic nodules of the liver. Von Meyenburg complexes are often asymptomatic and thus typically discovered incidentally on imaging or autopsy. They can also be encountered at the time of surgery where they often appear as scattered white liver lesions concerning for malignancy. Here, we present a case in which white hepatic nodules were found incidentally during laparoscopic cholecystectomy in a 36 -year-old female. Pathologic analysis confirmed the diagnosis of von Meyenburg complexes. The operating surgeon proceeded with laparoscopic cholecystectomy without complication. We report this case to encourage awareness of this benign entity. The finding of scattered hepatic lesions found intra-operatively can create concern for metastatic neoplastic processes. An awareness of von Meyenburg complexes and their gross appearance can better guide surgeons' intraoperative decision-making when encountering these characteristic hepatic lesions.

The prognostic value of sarcopenia combined with hepatolithiasis in intrahepatic cholangiocarcinoma patients after surgery: A prospective cohort study.

INTRODUCTION: Intrahepatic Cholangiocarcinoma (ICC) is the second most common primary liver cancer with dismal survival rates. This study aimed to explore the prognostic value of sarcopenia combine with hepatolithiasis in surgically treated ICC patients and develop a prognostic nomogram to help make clinical decisions. MATERIALS AND METHODS: A prospective cohort study was conducted including patients who underwent hepatectomy for ICC between August 2012 and October 2019. The association between the sarcopenia combined with hepatolithiasis and survival, including overall survival (OS) and recurrence-free survival (RFS) was investigated using the Kaplan-Meier (K-M) method. Univariable and multivariable Cox regression analysis was performed to determine the independent prognostic factors and a nomogram establishment was undertaken based on the multivariable analysis. RESULTS: A total of 121 ICC patients were included in the study. K-M analysis revealed that ICC patients with sarcopenia and hepatolithiasis have worse OS and RFS than those without sarcopenias and/or hepatolithiasis (p < 0.01). Multivariable analysis showed that age, serum CEA, hepatolithiasis, sarcopenia and diabetes were independent prognostic factors for OS(p < 0.05). Finally, a nomogram with good performance in survival prediction was established (C-index was 0.721; the area under the curve of OS was 0.837). The stratified analysis based on the nomogram disclosed that the median OS was 11.9 months in high-risk patients and 51.2 months in low-risk patients (p < 0.001). CONCLUSIONS: ICC patients with sarcopenia and hepatolithiasis have worse OS and RFS. The nomogram we developed is a practical tool that can provide a more individualized risk assessment for surgically treated ICC patients.

[Hybrid minimally invasive interventions in the treatment of solitary cholangiogenic liver abscesses]. / Gibridnye miniinvazivnye vmeshatel'stva pri lechenii solitarnykh kholangiogennykh abstsessov pecheni.

Treatment of solitary cholangiogenic liver abscesses is considered in the article. Successful treatment of cholangiogenic liver abscess with hybrid technologies is reported. The authors conclude that restoration of bile outflow is essential for fast elimination of the abscess.

Hepatic subcapsular or capsular flow in biliary atresia: is it useful imaging feature after the Kasai operation?

OBJECTIVES: To evaluate the implications of hepatic subcapsular and capsular flows using ultrasonography (US) in children after Kasai operation. METHODS: Children who underwent liver US including color Doppler US and microvascular imaging (MVI) from May 2017 to October 2017 were retrospectively included. Children who underwent the Kasai operation for biliary atresia were included in the Kasai group and children with normal liver were included in the control group. Using US results, the number of intrahepatic biliary cysts and the maximum diameter of the spleen were evaluated in the Kasai group. Liver stiffness values were included when patients in the Kasai group had transient elastography (TE) or shear wave elastography (SWE) results. Hepatic subcapsular and capsular flows on color Doppler US and MVI were compared between the two groups using the following scores: 0, no flow reaching the hepatic capsule; 1, any flow reaching the hepatic capsule; and 2, contiguous hepatic capsular flow. The logistic regression test was used to identify associations between age, intrahepatic biliary cysts, spleen size, SWV, TE results, and flow scores measured on Doppler US and MVI in the Kasai group using the odds ratio (OR) and 95% confidence interval (CI). RESULT: A total of 65 children (mean 7.6 ± 5.3 years), 44 in the Kasai group and 21 in the control group, were included. In the control group, one child had score 1 on MVI and others had score 0 on both Doppler US and MVI. Among the Kasai group, 28 children (63.6%) had score 1, while others had score 0 using Doppler US. Using MVI, 24 children (54.5%) had score 2, 18 children had score 1, and one child had score 0. In the Kasai group, increased liver stiffness on TE was the only factor significantly associated with the presence of subcapsular flow on color Doppler US (OR 1.225, 95% CI 1.020-1.470) and increased spleen size was the only factor significantly associated with increased flow scores on MVI (OR 1.397, 95% CI 1.002-2.724). CONCLUSION: Detection of hepatic subcapsular, capsular flows on US would be meaningful for children after receiving the Kasai operation. KEY POINTS: • Hepatic subcapsular or capsular flows can be useful not only for the diagnosis but also for the postoperative follow-up in patients with biliary atresia. • Increased liver stiffness and splenomegaly after the Kasai operation were associated with presence of subcapsular or capsular flow on ultrasonography. • Evaluation of hepatic subcapsular and capsular flows could be needed to assess disease progression after receiving the Kasai operation.

Mirizzi syndrome: when the gallbladder meets bile ducts.

A 64-year-old female presented to the Emergency Department with jaundice, choluria, fever and abdominal pain over the last few days. The abdomen was tender with epigastric pain on palpation.

A novel prognostic model for diagnosing atypical bile duct hyperplasia in patients with intrahepatic lithiasis.

There is no specific method for the preoperative diagnosis of atypical bile duct hyperplasia, which is a precursor of cholangiocarcinoma. This study aimed to create a new model for diagnosing atypical bile duct hyperplasia based on routine laboratory tests in patients with intrahepatic lithiasis.The new diagnostic model was developed with a derivation cohort that included 375 patients with intrahepatic lithiasis. Clinical and pathological data were retrospectively collected. Prognostic factors were evaluated with univariate and logistic regression analyses. The validation cohort included 136 patients who were retrospectively screened to quantify the model's predictive value.Age and Carbohydrate Antigen 19-9 (CA-199) were revealed to be diagnostic indicators of atypical bile duct hyperplasia in patients with intrahepatic lithiasis. The new diagnostic model was created with the formula: -6.612 + (0.002 × CA-199) + (0.072 × Age). The area under the receiver operating curve of the model was 0.721. With 0.25 as the cutoff point, the sensitivity and specificity of this model in the derivation cohort were 13.9% and 95.9%, respectively. In the validation cohort, these values were 28.5% and 88.7%, respectively. The novel model has an acceptable and stable ability to predict atypical hyperplasia in the intrahepatic bile duct.This novel model provides a simple system for diagnosing atypical bile duct hyperplasia before surgery in patients with intrahepatic lithiasis.