Pancreaticobiliary maljunction (PBM) is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall. Since the duodenal papillary sphincter fails to exert any influence on the pancreaticobiliary junction, reciprocal reflux between pancreatic juice and bile occurs, resulting in various pathologic conditions in the biliary tract and pancreas. For diagnosis of PBM, an abnormally long common channel and/or an abnormal union between the pancreatic and bile ducts must be evident on direct cholangiography, such as endoscopic retrograde cholangiopancreatography, percutaneous transpehatic cholangiography, or intraoperative cholangiography; magnetic resonance cholangiopancreatography; or three-dimensional drip infusion cholangiography computed tomography. Elevated amylase levels in bile and extrahepatic bile duct dilatation strongly suggest the existence of PBM.

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We conducted a nation-wide survey to elucidate the characteristics of hepatolithiasis by sending questionnaires. Two-hundred and ten patients with hepatolithiasis were identified for current 2 years. Compared to the previous elucidation, the cases underwent a surgical operation in biliary tract increased and the cases treated by endoscopic retrograde technique via duodenal papilla of Vater increased. According to the guideline regarding gallstone disease edited the Japanese Society of Gastroenterology, percutaneous transhepatic cholangioscopy is recommended to a patient underwent with surgery. However, ERCP technique by using double balloon endoscopy may make possible extraction of intrahepatic biliary stones. Furthermore, the increase of endoscopic treatment brings the occurrence of biliary carcinoma, because the morbid lesion of biliary tract was preserved. It will be demanded to follow up carefully.

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Aims: In this study, we investigated an effective strategy of treatment for unresectable gallbladder cancer (GBC) by the retrospective analysis of prognostic factors and anti-tumor therapies, especially combination therapy of arterial infusion chemotherapy and radiation therapy (AI+RT). Material and methods: Forty-three patients with unresectable GBC were enrolled, and prognostic factors were investigated by multivariate analysis using a proportional hazard model. In addition, we examined the indication and after-therapy by analyzing the each factor cumulative survival rates and anti-tumor effect about the AI+RT group (n=24). Results: AI+RT and the responders to the first-line therapy were significant prognostic factors. In AI+RT group, median survival time, progression-free survival and the 1-year survival rate, the response and disease control rates was 15.5 months, 7.1 months, 62.5%, 54.2%and 95.8%, respectively; which suggested prolonged survival and high anti-tumor effect. Cumulative survival rate was significantly shorter in cases with distant metastasis except liver metastases, and has been tendency to extend in the group who underwent systemic chemotherapy as after-therapy. Conclusion: The treatment strategy, using the AI+RT as first-line with the systemic chemotherapy as after-therapy, suggested contribute to the prolonged survival in locally advanced and liver metastases cases of GBC.

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The patients with biliary cancer sometimes present obstructive jaundice or biliary infection by the stricture or obstruction of the biliary tract. Biliary infection often induces severe sepsis or endotoxinemia. If surgery is performed without enough control of biliary infection, severe infectious complications or hepatic failure may follow. In this article, we first mention the consensus about the preoperative biliary drainage that plays important role in perioperative management for patients with biliary cancer, and explain the indication, methodology, and problems of preoperative biliary drainage. Then, we explain the management of preoperative biliary infection, the effectiveness of bile replacement during external biliary drainage, and perioperative nutrition support as promising strategies for preventing perioperative infectious complications. Finally, we comment on preoperative chemotherapy or chemoradiotherapy and coagulopathy associated with biliary cancer as current topics around this field.

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A 80-year-old male with liver dysfunction was referred to our hospital because of an ampullary tumor measuring 30 mm in diameter with common bile duct and pancreatic duct dilatation as well as para-aortic lymph node swelling demonstrated by CT and MRI. The accumulation of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) was observed in ampullary tumor, but not in para-aortic lymph nodes. Intraductal ultrasonography (IDUS) revealed slightly cancer invasion beyond the sphincter of Oddi. Pancreatoduodenectomy was performed and histopathological examination of the resected specimen revealed the most of the tumor extended within the sphincter of Oddi. In spite of slight cancer invasion beyond the sphincter of Oddi, this case was thought to be rare due to accompanied extensive lymph node metastases.

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Porphyria is a disease caused by a genetic abnormality of heme synthesis enzymes. This disease is known to cause acute attacks and has many drug contraindications. Case: 59 year-old man. Post history: Porphyria was diagnosed before. Medical history: Gallstone attack occurred and he was hospitalized immediately in diagnosis of acute cholecystitis. Because he was a porphyria patient, drainage and emergency surgery were considered risky and he was treated conservatively. He was later readmitted for surgery. Image: Gallbladder was enlarged, and its wall was thick. Stones were observed in the cystic duct and common bile duct. This is a porphyric patients with photosensitivity, photosensitivity occurs in shadowless lamp and laparoscopic light. In addition, it is necessary to choose safe drugs because many drugs are contraindicated. Surgery: Interior light was off, shadowless lamp was attached to the filter which cut the harmful light. Headlamps of the surgeon and monitor of the anesthesiologist were also attached to the same filter. In addition, the wavelength of light was measured during surgery. Cholecystectomy and choledocholithotomy under laparotomy were performed. He was discharged on postoperative day 8 with no acute attacks. Surgery could be performed safely in the complete measures.

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The patient was a 61-year-old female who was admitted to our hospital because of abdominal pain. Initial blood investigations revealed an increased white cell count of 16200 /μl and a C-reactive protein level of 31.8 mg/dl. Based on the findings of diagnostic imaging, we diagnosed the patient to have cholecystitis and thus started conservative therapy. Three days after admission, the fever and abdominal pain still remained, and serum CA19-9 level had also markedly increased (>12000 U/ml). Abdominal computed tomography showed the presence of ascites around the liver, and performing a needle biopsy, we confirmed the presence of biliary fluid. As a result, the patient was diagnosed to have a perforation of the gallbladder and immediately underwent emergency cholecystectomy and intraadbominal drainage. The pathologic examination revealed chronic cholecystitis, but no malignancy was identified. The mucosa and stroma of the gallbladder were both positive for the immunohistochemical staining of CA19-9, and the serum CA19-9 levels also normalized to 19 U/ml postoperatively. CA19-9 has been widely used as a useful marker for diagnosing malignant tumors, especially for biliary malignancies. However, the serum CA19-9 level also sometimes increases in benign diseases, such as cholecystitis.

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Intraoperative biliary injury is a severe complication of cholecystectomy which causes bile leakage or biliary stricture although this injury seldom occurs. This paper deals with clinical characteristics and therapies of eight cases of biliary injury in cholecystectomy, including two cases of amputation, and six cases of laceration. The diagnosis of injury was made in seven cases during surgery, and one case on the 7th postoperative day. Cholecystitis was accompanied in six and aberrant bile duct existed in four cases. For these injuries, operative repairs were performed, including primary suture in seven cases, sacrificing the aberrant bile duct in one case. Biliary tube, such as RTBD tube, T-tube, or C-tube was indwelt for prevention of narrowing repaired bile duct, although severe late biliary stricture was occurred in one case.

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The formation of biliary sludge after ceftriaxone (CTRX) therapy is widely known, especially in children. Two cases of elderly adults with cholecystolithiasis caused by high-dose, long-term CTRX administration, which resolved completely after discontinuation of CTRX therapy, are reported. One case required biliary drainage because of acute occlusive cholangitis. It took 21 and 38 days, respectively, for the biliary stones to disappear. Neither patient suffered recurrence of biliary sludge during follow-up. We should be aware of the fact that administration of CTRX sometimes results in the formation of biliary sludge and can cause severe adverse events, not only in children, but also in elderly patients.

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We have experienced gallbladder torsion which is a rare entity as a disease causing acute abdomen and have gained a favorable therapeutic outcome by making early preoperative diagnosis. An 88-year-old woman consulted our hospital because of right hypochondralgia with local tenderness and Murphy sign. Abdominal CT showed an enlarged free-floating gallbladder with thickening of the wall but without enhancement. We diagnosed as having gallbladder torsion. Following untwisting via single incision approach, laparoscopic cholecystectomy was performed completely. The patient was discharged four days after operation. In this case, a laparoscopic cholecystectomy with early proper diagnosis might lead to a minimally invasive surgery, resulting in a good outcome. Gallbladder torsion has similar symptoms to those of acute cholecystitis, so it is difficult to diagnose it preoperatively. In addition, single incision approach can be possible as one of option for gallbladder torsion only if untwisting is safely-performed.

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An upper gastrointestinal endoscopy revealed a swelling of the papilla of Vater for a 79 year-old man. Abdominal computed tomography showed an enhanced, well-demarcated mass, and endoscopic ultrasonography showed a low echoic mass (11×10 mm) in the papilla of Vater. Endoscopic retrograde cholangiopancreatography showed dilatation of the papilla of Vater, the common bile duct, and the main pancreatic duct. Based on these findings, an ampullary cancer was suspected, and a pylorus preserving pancreaticoduodenectomy was performed. Macroscopically, a hard, yellowish-white tumor of 1.8 cm in diameter was identified. The tumor was well-demarcated, but not encapsulated, occupying the papilla of Vater and the duodenal submucosal layer, and involving the sphincter of Oddi and duodenal epithelia. Microscopically, the tumor was composed of three components: the major component showing a paraganglioma-like appearance, and spindle cell proliferation similar to Schwannoma and isolated ganglion-like cells as minor components. Based on the histological appearances and immunohistochemical findings, the tumor was diagnosed as a gangliocytic paraganglioma.

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A 77-year-old man was admitted to our hospital with melena 13 months after hepatectomy with bile duct resection for benign gallbladder tumor. Abdominal CT and angiography revealed a complete obstruction of the extrahepatic portal vein (PV) with dilated hepatopetal collaterals through the jejunal loop. Obstruction of the PV was relieved with metallic stenting through percutaneous transhepatic PV angioplasty and coil embolization of the collaterals of the jejunum. The PV pressure remarkably decreased from 35 to 21 cmH₂O after metallic stenting of the PV and coil embolization of the collaterals. He has recovered well 7 years after these interventional procedures without any episode of gastrointestinal hemorrhage and with good patency of the PV. These interventional approaches with metallic stenting and coil embolization might be useful for the patients with benign PV obstruction.

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A 60-year-old man presented with dark urine and had a diagnosis of distal bile duct cancer and was referred to our hospital. Preoperative abdominal CT findings were an anomaly of the hepatomesenteric trunk as the common hepatic artery (CHA) replaced from the superior mesenteric artery, CHA running through the parenchyma of the pancreatic head and right-sided round ligament. As the location of the tumor was distant from the CHA, we performed a pylorus preserving pancreatoduodenectomy while carefully preserving this artery. The histopathological diagnosis was ampullary cancer. In surgery on pancreatic head area, we must consider to maintain the hepatic blood flow and to keep the radical curability. Detailed understanding of the hepatic arterial anatomy and accurate recognition of anatomy are important to perform safe and curative surgery.

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Biliary cancer causes distinctive symptoms dependent on the location of the tumor. Understanding of these cancer-related symptoms is helpful for optimal palliative care. In this article, common problems and management are described; cancer-related pain, duodenal obstruction, obstructive jaundice, biliary infection, pruritus and vitamin K deficiency. The choice of opioids for biliary cancer is also stated as a topic, though they should be used cautiously in patients with liver dysfunction. Sphincter of Oddi dysfunction related to opioids is discussed as a noteworthy issue. In palliative care, setting some procedures such as celiac plexus block, duodenal stent placement and PEG for decompression would be effective. These interventions would provide more palliative care options for clinicians. It is important for healthcare workers to pay much attention not only to physical suffering but also to other kinds of sufferings known as total pain. In decision-making at clinical settings, QOL and satisfaction of patients' and their families should be considered.

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