A case with massive hemobilia long-term after internal drainage surgery for congenital biliary dilation

Background Currently, there is an unwavering consensus that the standard surgery for congenital biliary dilation (CBD) is extrahepatic bile duct resection and choledochojejunostomy. However, decades prior, choledochocyst–gastrointestinal anastomosis without extrahepatic bile duct resection (internal drainage surgery, IDS) was preferred for CBD because of its simplicity. Currently, there is almost no chance of a surgeon encountering a patient who has undergone old-fashioned IDS, which has been completely obsolete due to the risk of carcinogenesis from the remaining bile duct. Moreover, the pathological condition long after IDS is unclear. Herein, we report a case of life-threatening bile duct bleeding as well as carcinoma of the bile duct 62 years after IDS in a patient with CBD. Case presentation An 82-year-old Japanese woman with hemorrhagic shock due to gastrointestinal bleeding was transferred to our hospital. She had a medical history of unspecified surgery for CBD at the age of 20. Based on imaging findings and an understanding of the historical transition of the surgical procedure for CBD, the cause of gastrointestinal bleeding was determined to be rupture of the pseudoaneurysm of the dilated bile duct that remained after IDS. Hemostasis was successfully performed by transcatheter arterial embolization (TAE) in an emergency setting. Then, elective surgery for extrahepatic bile duct resection and choledochojejunostomy was performed to prevent rebleeding. Pathological examination revealed severely and chronically inflamed mucosa of the bile duct. Additionally, cholangiocarcinoma (Tis, N0, M0, pStage 0) was incidentally revealed. Conclusion It has been indicated that not only carcinogenesis, but also a risk of life-threatening bleeding exists due to long-lasting chronic inflammation to the remnant bile duct after IDS for CBD. Additionally, both knowledge of which CBD operation was performed, and an accurate clinical history are important for the diagnosis of hemobilia.

Biliary Schwannoma That Required Differentiation from Bile Duct Cancer

Schwannomas are benign tumors originating from Schwann cells, which are the main component of the neural sheath. Biliary schwannomas are extremely rare. We report the case of a 78-year-old man who presented with no abdominal symptoms or jaundice. CT imaging showed a hyperdense mass extending along the extrahepatic bile duct, and the upstream bile ducts were dilated. We performed extrahepatic bile duct resection under a preoperative diagnosis of the extrahepatic bile duct cancer. A histopathological examination of the resected specimen revealed that the tumor consisted of spindle cells which exhibited a palisading arrangement. Immunohistochemical staining was positive for protein S-100 and vimentin. Based on these pathological findings, we diagnosed the patient with schwannoma of the extrahepatic bile duct. Our search of the relevant literature revealed 19 case studies of biliary schwannomas. In our case, the surgical findings showed that the tumor was noninvasive and mobile. During surgery, a fast frozen section analysis was performed, and no malignant findings were observed. These results enabled us to avoid extrahepatic bile duct resection with major hepatectomy. We experienced a case of biliary schwannoma that was difficult to distinguish from bile duct cancer.

A case of resected hepatocellular carcinoma with gallbladder metastasis

Background Advanced hepatocellular carcinoma (HCC) can often spread as intrahepatic metastases. Extrahepatic metastasis (e.g., lung, lymph nodes, and bones) is rare, and gallbladder metastasis from HCC is extremely rare. Case presentation A 66-year-old woman who presented with right hypochondrial pain was referred to our hospital for further examination of a liver tumor. The blood chemistry data showed elevated levels of serum α-fetoprotein (AFP) (3730 ng/mL), protein induced by vitamin K absence or antagonist II (PIVKA-II) (130 mAU/mL), and carcinoembryonic antigen (CEA) (358.6 ng/mL). Hepatitis B surface antigen and hepatitis C virus antibody were negative. Dynamic computed tomography (CT) showed a tumor measuring 12 × 7 cm in the right lobe of the liver. This tumor was contrast-enhanced in the hepatic arterial phase and then became less dense than the liver parenchyma in the portal phase. A well-enhanced tumor was found in the gallbladder. No regional lymph nodes were enlarged. Contrast-enhanced magnetic resonance imaging (MRI) demonstrated that the liver tumor showed a pattern of early enhancement and washout. The gallbladder tumor was also detected as an enhanced mass. Endoscopic retrograde cholangiography (ERC) showed compression of the left hepatic duct due to the liver tumor. The patient was diagnosed with simultaneous HCC and gallbladder cancer. Right hepatic trisectionectomy and caudate lobectomy with extrahepatic bile duct resection were performed. Histopathological examination of the resected liver specimen showed a poorly differentiated HCC cell component with a trabecular and solid growth, and diffuse invasion of the portal vein. The same tumor cells were found in the gallbladder, but no continuity with the liver tumor was identified. Immunohistochemistry of the liver tumor and gallbladder was positive for AFP, Glypican 3, and CK7, and negative for CK19. The final pathological diagnosis was the gallbladder metastasis from HCC. A follow-up diagnostic image 33 months after surgery showed a mass in the upper lobe of the left lung. The patient underwent left upper lobectomy. Postoperative pathology revealed that the lung lesion was a metastasis of HCC. The patient was still alive with lung metastasis and was being treated with a molecular-targeting drug in good health 42 months after the initial surgery. Conclusions The standard treatment for advanced HCC with extrahepatic metastases is molecularly targeted drugs, but surgery is also an option if the lesion can be resected en bloc without remnants.

Probiotic-related bacteremia after major hepatectomy for biliary cancer: a report of two cases

Background Probiotics have been reported to be beneficial for the prevention of postoperative complications and are often used during the perioperative period. Among the probiotic-related adverse events, bacteremia is rare. Here, we report two cases of probiotic-related bacteremia after major hepatectomy for biliary cancer. Case presentation 1 A 74-year-old man was referred to our hospital to be treated for gallbladder cancer. Neoadjuvant chemotherapy, two courses of gemcitabine plus S-1 combination therapy, was administered. Extended right hepatectomy with caudate lobectomy, extrahepatic bile duct resection and biliary reconstruction were performed 3 weeks after chemotherapy. Probiotics, Clostridium butyricum (C. butyricum) MIYAIRI 588, were administered 6 days before surgery and continued after surgery. Sepsis of unknown origin occurred 17 days after surgery and developed into septic shock. C. butyricum was detected in blood cultures at postoperative day 26 and 45. After stopping the probiotic agent, C. butyricum was undetectable in the blood cultures. The patient died due to an uncontrollable sepsis 66 days after surgery. Case presentation 2 A 63-year-old man with diabetes mellitus whose past history included total colectomy, papillectomy, and Frey’s operation at the age of 19, 34 and 48, respectively, was referred to our hospital to be treated for perihilar cholangiocarcinoma. Extended left hepatectomy with caudate lobectomy, extrahepatic bile duct resection and reconstruction of bile duct were performed. Probiotics were administered during the perioperative period. Combined probiotics that included lactomin, amylolytic bacillus and C. butyricum, were given before surgery. C. butyricum MIYAIRI 588 was given after surgery. Sepsis occurred 16 days after surgery and developed to respiratory failure 8 days later. Blood culture at postoperative day 25 revealed Enterococcus faecalis and C. butyricum. After the probiotics were stopped at postoperative day 27, C. butyricum was not detected in the blood culture. The general condition improved with intensive care. The patient was transferred to another hospital for rehabilitation at postoperative day 156. Conclusion It should be noted that the administration of probiotics in severe postoperative complications can lead to probiotic-related bacteremia.

Utility of remnant liver volume for predicting posthepatectomy liver failure after hepatectomy with extrahepatic bile duct resection

Background Hepatectomy with extrahepatic bile duct resection is associated with a high risk of posthepatectomy liver failure (PHLF). However, the utility of the remnant liver volume (RLV) in cholangiocarcinoma has not been studied intensively. Methods Patients who underwent major hepatectomy with extrahepatic bile duct resection between 2002 and 2018 were reviewed. The RLV was divided by body surface area (BSA) to normalize individual physical differences. Risk factors for clinically relevant PHLF were evaluated with special reference to the RLV/BSA. Results A total of 289 patients were included. The optimal cut-off value for RLV/BSA was determined to be 300 ml/m2. Thirty-two patients (11.1 per cent) developed PHLF. PHLF was more frequent in patients with an RLV/BSA below 300 ml/m2 than in those with a value of 300 ml/m2 or greater: 19 of 87 (22 per cent) versus 13 of 202 (6.4 per cent) (P < 0.001). In multivariable analysis, RLV/BSA below 300 ml/m2 (P = 0.013), future liver remnant plasma clearance rate of indocyanine green less than 0.075 (P = 0.031), and serum albumin level below 3.5 g/dl (P = 0.015) were identified as independent risk factors for PHLF. Based on these risk factors, patients were classified into three subgroups with low (no factors), moderate (1–2 factors), and high (3 factors) risk of PHLF, with PHLF rates of 1.8, 14.8 and 63 per cent respectively (P < 0.001). Conclusion An RLV/BSA of 300 ml/m2 is a simple predictor of PHLF in patients undergoing hepatectomy with extrahepatic bile duct resection.

Robot-assisted and open resections for perihilar cholangiocarcinoma. Comparative analysis of the immediate outcomes

Background. Minimally invasive radical surgery for perihilar cholangiocarcinoma is in its early stages. Aim. A comparative analysis of the initial experience of robot-assisted and open resections for perihilar cholangiocarcinoma. Material and methods. The single-center experience, accumulated over the period from 2014 to 2018, is analyzed. Robot-assisted procedures included major liver resection and caudate lobectomy with extrahepatic bile duct resection and lymphadenectomy. The need for vascular reconstruction was considered a contraindication to surgery. Results. Thirteen robot-assisted resections were performed. The perihilar cholangiocarcinoma was confirmed by pathologic examination in 10 patients. The immediate outcomes were compared with that in 88 open procedures. There were no significant differences in blood loss, the rate of severe morbidity, mortality, and hospital stay. The duration of the robot-assisted surgical resections was significantly longer. Conclusion. Analysis of initial experience justifies the robotic approach for radical resection in highly selected patients.