A case of spontaneously ruptured hepatic angiosarcoma resected after transcatheter arterial embolization.

Hepatic angiosarcoma is a very rare disease, but it has a poor prognosis. Here, we report the case of a 77-year-old man who was referred to our hospital for suspicion of hepatocellular carcinoma and cancerous peritonitis. Based on the imaging findings, a diagnosis of spontaneously ruptured hepatic hemangioma was made. Six days later, the patient was transported to the hospital in a state of shock and an emergency transarterial embolization was performed. He underwent lateral hepatic segmentectomy 7 days later. Histopathologically, he was diagnosed with hepatic angiosarcoma. Fever was observed 21 days after surgery, and computed tomography was performed. Multiple liver masses, which ware increasing rapidly, were found, and hepatic angiosarcoma recurrence was confirmed. He requested home medical care and died at home 36 days after surgery. When a tumor diagnosed as a hepatic hemangioma by imaging has ruptured, the possibility of hepatic angiosarcoma should be considered.

Primary mucoepidermoid carcinoma of the intrahepatic bile duct: A case report.

Mucoepidermoid carcinoma (MEC) is the most common salivary gland carcinoma; however, hepatobiliary MEC is extremely rare. A 74-year-old patient was diagnosed with hepatobiliary MEC after hepatectomy. We considered its origin could be the peribiliary glands. Its genome profile was similar to salivary MEC rather than standard biliary tract carcinoma.

Usefulness of Preoperative Controlling Nutritional Status in Predicting Prolonged Hospitalization and Incidence of Postoperative Delirium for Elderly Hepatectomy with Hepatocellular Carcinoma.

The number of elderly patients with hepatocellular carcinoma (HCC) has been increasing, but it remains unclear whether hepatectomy can be performed using the same criteria as in younger patients. Furthermore, the appropriate preoperative evaluation for hepatectomy in elderly patients is not yet clearly defined. Here, we investigated the applicability of preoperative assessment using the Controlling Nutritional Status (CONUT) score to help improve hepatectomy outcomes in elderly patients with HCC. This was a single-center retrospective analysis, and the study population comprised 64 consecutive patients who underwent hepatectomy for HCC between January 2012 and August 2016. We compared the preoperative assessment and perioperative outcomes between elderly (≥ 75 years old) and younger (< 75 years old) patients. A total of 61 patients were reviewed. Poor preoperative CONUT score was associated with a longer postoperative hospital stay in elderly patients undergoing hepatectomy for HCC. In addition, although elderly patients had similar perioperative outcomes to younger patients, the incidence of delirium was significantly higher, and univariate analysis confirmed that old age is a risk factor for delirium among the preoperative factors. Hepatectomy for HCC in the elderly can be safely performed with appropriate preoperative nutritional assessment using CONUT score and prevention of postoperative delirium. Preoperative nutritional assessment using the CONUT score was useful in predicting prolonged hospitalization for elderly hepatectomy with HCC.

Recurrence of Adult Granulosa Cell Tumor in the Greater Omentum 11 Years after Surgery.

Adult-type ovarian granulosa cell tumors (AGCTs) are very rare tumors that account for <5% of all ovarian carcinomas. AGCTs have low malignancy potential and rarely metastasize 5-30 years after the initial diagnosis. Because time has passed from the first surgery and because recurrence develops in various locations, the differential diagnosis is difficult. In particular, tumors developing in the greater omentum are encountered rarely, and it is necessary to carefully consider the differential diagnosis, including primary and secondary neoplasms. Although CT is useful to detect omental tumors, the diagnosis requires invasive procedures. We report a case of AGCT recurrence in the greater omentum that was resected during laparoscopic cholecystectomy. A patient visited our hospital with right-sided abdominal pain. The CT revealed gallbladder stones, a ureteral stone, and a right abdominal mass. The diagnosis of the abdominal tumor was difficult on the basis of blood biochemical testing, gastrointestinal endoscopy, or image inspection. Although the patient underwent several previous surgeries and there were no findings of malignancy with positron emission tomography, we chose to resect the tumor for combined diagnosis and treatment during laparoscopic cholecystectomy. Intraoperative findings showed that the tumor originated from the greater omentum, and the tumor was diagnosed as AGCT recurrence by pathology. A recurrence of AGCT in the greater omentum is very rare, and laparoscopic surgery was safe and useful for resection, in our case.

[A case of inflammatory cerebral amyloid angiopathy with white matter lesions appearing after brain biopsy].

A 76-year-old woman with a 7-year history of dementia presented to our hospital with generalized convulsive seizure for the first time. Contrast-enhanced brain magnetic resonance imaging revealed leptomeningeal enhancement mainly in the right occipital lobe and multiple lobar microbleeds in the bilateral cerebral and cerebellar subcortex. No white matter lesions were observed. A brain biopsy of the right parieto-occipital lobe revealed cerebral amyloid angiopathy (CAA). White matter lesions appeared in the right parieto-occipital lobe three days after the biopsy, and we considered inflammatory CAA. Three courses of methylprednisolone pulse followed by oral prednisolone therapy gradually reduced leptomeningeal and white matter lesions. An apolipoprotein E genotype investigation identified the ε2/ε3 genotype. In patients with inflammatory CAA, a risk of exacerbation should be considered after brain biopsy, in which the ε2 allele might play a role.

Possible Cerebral Vasculitis in a Case with Rheumatoid Arthritis.

Cerebral rheumatoid vasculitis (CRV) is a rare, fatal, and diagnostically challenging disorder. We herein report an 81-year-old woman with a 4-year history of rheumatoid arthritis who presented with a fever, progressive disturbance of consciousness, high level of rheumatoid factor, and hypocomplementemia. The enhancement of the perforating branches in the left middle cerebral artery led us to suspect CRV. A brain biopsy could not be performed. After we intensified steroid therapy, the size of the cerebral lesions temporarily decreased. However, recurrence in the left frontal lobe occurred one month later, and the patient subsequently died. Early intensive treatments may be needed for CRV.

[A case of cerebral venous sinus thrombosis mimicking transient ischemic attack].

A 48-year-old woman with a right-sided headache beginning a month prior to admission presented with sudden-onset right hemiparesis. On admission, she had weakness of the right lower extremity, which disappeared 3 hours after onset. Contrast enhanced brain MRI revealed no parenchymal lesion, while indicated thrombi in the superior sagittal sinus and the right side of the transverse sinus, sigmoid sinus, and internal jugular vein, leading to the diagnosis of cerebral venous sinus thrombosis. Brain perfusion single photon emission computed tomography presented slightly decreased blood flow in the bilateral frontal lobes (left-sided dominant) and the right occipitotemporal lobe. Electroencephalogram showed no abnormal finding. After anticoagulant therapy, thrombi in the venous sinuses decreased and brain blood flow improved. We should consider cerebral venous sinus thrombosis in the case of a patient presenting with symptoms of a transient ischemic attack accompanied with headache. Moreover, the etiology of transient neurological deficits remains controversial.

Trousseau syndrome with intrahepatic cholangiocarcinoma that could be removed radically after endovascular treatment: Report of a case.

BACKGROUND: Trousseau syndrome is a poor prognosis. We report a case of Trousseau syndrome treated by radical resection after endovascular treatment. CASE: A 59-year-old woman presented to our department reporting spontaneous dizziness and pain of the upper abdomen. Magnetic resolution imaging (MRI) showed shower embolization of Brain. Contrast-enhanced computer tomography (CT) showed renal infarction and splenic infarction, and a tumor was observed in the retrohepatic area. On day 9, sudden right side joint prejudice, neglect of left half space, and left hemiplegia were observed. MRI revealed obstruction of the right middle cerebral artery (MCA) perfusion zone. On the same day, endovascular treatment was performed and reperfusion was obtained. We decided on a radical surgery policy because there were a primary lesion and a high risk of new embolism, and no metastasis was seen. DISCUSSION: Trousseau syndrome generally has a poor prognosis, but active treatment should be considered as an option when we can expect the recovery of function.

Effects of Short-Term Normothermic and Subnormothermic Perfusion After Cold Preservation on Liver Transplantation From Donors After Cardiac Death.

BACKGROUND: Liver transplantation from donors after cardiac death (DCD) could increase the pool of organs. We previously reported that oxygenated subnormothermic (20°C-25°C) ex vivo liver perfusion (SELP) improved the graft viability in rats. This study aimed to compare the effectiveness of SELP and normothermic (37°C) ex vivo liver perfusion (NELP) after cold storage (CS) in DCD liver grafts. METHODS: Male Wistar rats were used, and grafts were retrieved 30 minutes after cardiac arrest. We performed oxygenated NELP and SELP with a Krebs-Henseleit buffer for different time points and durations: Group 0, donation performed from heart-beating donors (control); Group 1 (DCD group), donation performed from DCD donors with no treatments; Group 2, NELP performed before CS (30 minutes); Group 3, NELP performed after CS (30 minutes); Group 4, SELP performed after CS (30 minutes); Group 5, SELP performed after CS (60 minutes); and Group 6, SELP performed after CS (90 minutes). After 15 minutes of incubation at room temperature, the grafts were reperfused under normothermic conditions for 60 minutes as a model of liver transplantation. RESULTS: No significant differences in body and liver weight were observed between all groups. In the SELP after CS groups, even 30 minutes of perfusion improved bile production, tumor necrosis factor-α, and interleukin-1ß significantly compared with the DCD group (P < .05), comparable with NELP groups. CONCLUSION: SELP rescued DCD livers from ischemia-reperfusion injury the same as the normothermic perfusion before or after CS groups. SELP after CS is more convenient than normothermic perfusion; hence, this technique may increase the organ pool.

Intraoperative modulation of arterial blood flow in a hybrid operating room: A report of three cases.

The preoperative modulation of arterial blood flow is widely performed to prevent massive intraoperative hemorrhage and unstable circulatory dynamics; however, this may cause complications. The intraoperative modulation of arterial blood flow can be performed with operation to reduce the physical and psychological stresses on the patients and improve intraoperative safety.

Association of post-transplant donor-specific HLA antibody with liver graft fibrosis during long-term follow-up after pediatric liver transplantation.

The aim of this study was to evaluate the significance of post-transplant DSA as a predictor of liver fibrosis during long-term follow-up after pediatric LT. We evaluated the histological findings in 18 LT recipients who underwent liver biopsy after DSA screening. Liver fibrosis was scored based on the METAVIR fibrosis staging. Patients were divided into 2 groups based on histological findings, and clinical characteristics among patients with liver fibrosis were assessed. Of 18 patients, 7 were included in the fibrosis group. No significant between-group differences were found regarding peritransplant characteristics, including age, sex, primary disease, ABO incompatibility, and immunosuppressive regimen. Episodes of acute rejection and non-adherence to immunosuppressive drugs were comparable between both groups. The MFI for anti-DR DSA and positive rate were significantly higher in the fibrosis group (1655 vs 216; P = .019, 86% vs 27%; P = .012, respectively). MFI for anti-DQ DSA was higher in the fibrosis group, but non-significantly (2052 vs 384; P = .46). Post-transplant anti-DR DSA is associated with graft fibrosis during long-term follow-up. This finding seems useful for the implementation of valid histological examinations of liver grafts for patients with higher MFI, especially for anti-DR DSA, after pediatric LT.

The Effects of Short-term Subnormothermic Perfusion After Cold Preservation on Liver Grafts From Donors After Circulatory Death: An Ex Vivo Rat Model.

BACKGROUND: We previously reported that short oxygenated warm perfusion before cold storage (CS) had improved the graft viability of rat livers from donors after circulatory death (DCD). In this study, we investigated the effectiveness of short-term oxygenated subnormothermic perfusion for different durations after CS in a rat DCD model. METHODS: We used an isolated perfused rat liver system. In study 1: the grafts were retrieved from Wistar rats 30 minutes after cardiac arrest (thoracotomy), preserved in CS for 6 hours, and perfused with oxygenated subnormothermic (20-25°C) Krebs-Henseleit buffer for different durations (0, 15, 30, 60, and 90 minutes groups; n = 5 in each). In study 2: in addition to subnormothermic ex vivo liver perfusion (SELP), after 15-minute incubation at room temperature, the grafts were reperfused under normothermic condition for 60 minutes as a model of liver transplantation (0, 30, 60, and 90 minutes groups; n = 5 in each). RESULTS: In study 1, portal flow, bile production and tissue adenosine triphosphate increased with perfusion duration. In study 2, SELP significantly improved portal flow volume (P <0.05), and bile production (P <0.05), decreased liver enzymes (P <0.05) and cytokines (P <0.0001), and increased tissue adenosine triphosphate (P <0.01). Histological examinations showed that additional SELP ameliorated tissue deterioration, preserved the parenchymal structure, and decreased apoptosis (P <0.01). Furthermore, scanning electron microscopy revealed that additional SELP alleviated sinusoidal endothelial cells and hepatic microvasculature. CONCLUSIONS: Even 30 minutes of SELP after CS rescued DCD livers from ischemia-reperfusion injury, which may help the viability of the grafts.

Arterial and biliary complications after living donor liver transplantation: a single-center retrospective study and literature review.

AIM: The mortality of patients on the waiting list for deceased donor liver transplantation (DDLT) is high, especially in countries where donation rates are low. Thus, living donor liver transplantation (LDLT) is an attractive option. However, compared with DDLT, LDLT is associated with increased rates of arterial and biliary complications. We examined the rates of complications and risk factors following LDLT. METHODS: We retrospectively investigated and compared the rates of complications of DDLT and LDLT in our institute. We also performed univariate and multivariate analyses to identify the independent risk factors for these complications. The complications and specific disadvantages of LDLT were reviewed and discussed. RESULTS: The incidence rate of arterial complications in LDLT was 6.0%, compared with 3.2% (13/441) in DDLT. A multivariate analysis identified low body weight (P = 0.032) as the only independent risk factor for hepatic artery thrombosis. The rate of all biliary complications in LDLT was 17.3%, compared with 18.7% in DDLT. The risk factors for biliary stricture identified by the multivariate analysis were recurrent cholangitis and the number of bile ducts. The durations of hospital stay and overall survival rates were similar between the two groups. CONCLUSION: Given the shortage of deceased donor organs, we believe that LDLT is acceptable in an attempt to meet demand.

The utility of superb microvascular imaging for monitoring low-velocity venous flow following pancreas transplantation: report of a case.

Vascular thrombosis is a major complication after pancreas transplantation. Because delays in detecting thrombosis often result in graft failure, monitoring blood flow is crucial. Periodic evaluation using color Doppler ultrasonography is mostly performed for monitoring blood flow in the grafted pancreas. However, conventional color Doppler imaging has limited capability to visualize low-velocity blood flow. Superb microvascular imaging (SMI) is a novel ultrasound Doppler technique that is especially sensitive in detecting low-velocity flow. Herein, the authors describe the utility of SMI for monitoring splenic venous blood flow not detected by conventional color Doppler ultrasonography after pancreas transplantation. Ultrasonographic evaluation was performed every 4-6 h for 2 weeks after pancreas transplantation. SMI was used for detecting venous blood flow that was not clearly visible by conventional color Doppler imaging. The greater part of venous blood flow was undetectable by conventional color Doppler imaging, especially in the horizontal regions of the splenic vein. However, SMI clearly described venous flow at all points, even immediately after transplantation and when the patient developed hypotension due to massive bleeding complications. SMI is an extremely useful tool for monitoring venous flow after pancreas transplantation and does not require contrast agents.

The extra-anatomical jump graft reconstruction of the right hepatic artery after resection of a biliary tract malignancy with a common hepatic artery aneurysm: a case report.

Performing resection of a biliary tract malignancy with a hepatic artery aneurysm is very challenging. Resection of the extrahepatic bile duct and extra-anatomical reconstruction can be successfully performed using free radial artery autografts from the aorta to the right hepatic artery. Hepatic artery thrombosis can be prevented with intimal preservation.

Renoportal Anastomosis in Left Lateral Lobe Living Donor Liver Transplantation: A Pediatric Case.

In adult liver transplantation, renoportal anastomosis (RPA) has been introduced as a useful technique for patients with grade 4 portal vein thrombosis and a splenorenal shunt. Here, we report a pediatric case in which RPA allowed a left lateral lobe living donor liver transplantation (LDLT) despite portal vein thrombosis and a large splenorenal shunt. At 36 days old, the patient underwent a Kasai operation for biliary atresia. At 17 months old, she underwent LDLT because of repetitive cholangitis. Pretransplant examinations revealed a large splenorenal shunt and portal vein thrombosis. Simple end-to-end portal reconstruction and clamping of the collateral route after removing the thrombosis were unsuccessful. Thus, RPA was performed using a donor superficial femoral vein as an interpositional graft. The portal vein pressure was 20 mm Hg after arterial reperfusion. Ligation of the splenic artery reduced the portal vein pressure. Although she developed severe acute cellular rejection and chylous ascites, there were no signs of portal vein complications. She was discharged 73 days after transplantation without any signs of renal dysfunction. The patient's condition was good at her last follow-up, 22 months after transplantation. To our knowledge, this is the youngest case of RPA in pediatric left lateral lobe LDLT. Additionally, this is the first case of RPA with splenic artery ligation and using the donor's superficial femoral vein as the venous graft for RPA. Although long-term follow-up is necessary, RPA could be a salvage option in LDLT in infants if other methods are unsuccessful.

Impact of the Trough Level of Calcineurin Inhibitor on the Prevalence of Donor-Specific Human Leukocyte Antigen Antibodies During Long-Term Follow-Up After Pediatric Liver Transplantation: Antibody Strength and Complement-Binding Ability.

BACKGROUND: In pediatric patients, long-term immunosuppression after liver transplantation (LT) is typically minimal. However, posttransplant donor-specific HLA antibodies (DSAs) may be prevalent under these conditions. Here, we evaluated the effects of minimized calcineurin inhibitor (CNI) on DSA development to assess the validity of minimized/withdrawn immunosuppression. METHODS: We retrospectively examined 66 patients who underwent pediatric LT at our institution between July 1991 and October 2013. Patients were divided into 2 groups based on the CNI trough level. The cutoff trough levels were 3 and 30 ng/mL for tacrolimus and cyclosporine, respectively. Luminex single-antigen bead assays were performed, and the cutoff for a positive reaction was set at a mean fluorescence intensity (MFI) of at least 1000. RESULTS: The mean recipient ages at the time of LT were 29.1 and 77.2 months for the low and regular CNI groups, respectively (P = 0.0007). Univariate logistic regression analysis revealed that recipient age at LT younger than 3 years (P = 0.0099) and low CNI (P < 0.0001) were significantly associated with DSA development. In multivariate analysis, low CNI was an independent risk factor of DSA development (P = 0.0011). Of 15 high-MFI DSAs, 3 were anti-DR, and 12 were anti-DQ. Two of 3 anti-DR DSAs and 11 of 12 anti-DQ DSAs had complement-binding ability and high MFIs. CONCLUSIONS: CNI minimization was an independent risk factor for posttransplant DSA during long-term follow-up after pediatric LT. Adjusting CNI to appropriate levels is a safe first step to prevent the immunological effects of DSA.

Effect of Recipient Age at Liver Transplantation on Prevalence of Post-Transplant Donor-Specific HLA Antibody.

BACKGROUND Post-transplant donor-specific HLA antibodies (DSA) may have a detrimental effect on long-term outcomes of organ transplantation. The aim of this study was to specifically evaluate the effect of recipient age on the prevalence of DSA over a long-term follow-up after living donor liver transplantation (LDLT). MATERIAL AND METHODS A retrospective analysis of DSA evaluations was performed in 50 pediatric patients with HLA data available. Patients were divided into 2 groups based on their age at the time of LDLT: younger (Y) group, age <3 years; older (O) group, age ≥3 years. DSA evaluation was performed using Luminex single-antigen bead assays, with a mean fluorescence intensity ≥1000 used as a cut-off for positive results. RESULTS There were no between-group differences in terms of sex, ABO incompatibility or acute rejection. Only one of our 50 patients tested positive for class I DSA. Significantly more patients tested positive for HLA-DR DSA in group Y (40.6%) than in group O (11.1%; p=0.02). Recipients <3 years of age at the time of LDLT may be at a higher risk of testing positive for class II DSA. CONCLUSIONS These findings can inform the implementation of cost-effective screening of post-transplant DSA in pediatric LDLT recipients.

Hassab's operation for Joubert syndrome with congenital hepatic fibrosis: A case report.

INTRODUCTION: Joubert syndrome is characterized by psychomotor developmental delay, hypotonia, oculomotor abnormalities, occasional retinal dystrophy and cystic kidneys, and frequent and often, striking breathing abnormalities, especially in the neonatal period, with panting tachypnea followed by apnea. We report a case of Joubert syndrome with hepatic fibrosis, portal hypertension, and pancytopenia treated by Hassab's operation. PRESENTATION OF CASE: Our patient was a 27-year-old woman with a history of tachypnea, muscle hypotonia, and psychomotor retardation shortly after birth and a diagnosis of Joubert syndrome at 2 years of age. At 19 years of age, she was diagnosed with progressive pancytopenia. At 27 years of age, she visited her local doctor for sudden-onset hematemesis. Endoscopy revealed esophageal varices exhibiting the red color sign and no evidence of recent bleeding. Splenomegaly and development of portal collateral circulation were observed on computed tomography scans. The patient was referred to our hospital, where she was diagnosed with Joubert syndrome and hepatic fibrosis, portal hypertension, and hypersplenism. After performing Hassab's operation, the pancytopenia improved, but anticoagulant therapy was required for splenic vein thrombosis. The patient was discharged on postoperative day 25. Two years following surgery, the gastroesophageal varices were controlled, and no progression of the splenic vein thrombosis or hepatic failure was evident. CONCLUSION: This is the first case report of Hassab's operation for congenital hepatic fibrosis in a patient with Joubert syndrome, a rare congenital condition. We achieved a favorable clinical outcome.

Extrahepatic bile duct hepatocellular carcinoma due to recurrence of hematogenous metastasis 50 months after hepatectomy.

BACKGROUND: Recurrent hepatocellular carcinoma (HCC) in the extrahepatic bile duct is rare with most cases diagnosed after manifesting sudden obstructive jaundice. Here, we report an extremely rare case of recurrent HCC in the common bile duct due to hematogenous metastasis. CASE PRESENTATION: A 66-year-old man underwent an extended left hepatectomy for HCC in the medial segment of the liver. Fifty months later, he presented with sudden obstructive jaundice. Endoscopic retrograde cholangiography showed a space-occupying lesion in the common bile duct, which was suspected as cholangiocarcinoma. Therefore, he underwent extrahepatic bile duct resection and choledochojejunostomy with lymph node dissection. Macroscopically, a polypoid tumor and several nodular tumors were found in the common bile duct, which was obstructed by a tumor thrombus. Histopathologically, the tumors were diagnosed as metastases from the HCC resected 50 months before. Several distinct, nodular tumors were observed in the subepithelium of the common bile duct and had invaded some blood vessels. These findings support the conclusion that the HCC metastasized hematogenously to the extrahepatic bile duct. CONCLUSIONS: Recurrent HCC in the extrahepatic bile duct due to hematogenous metastasis is rare, and it is difficult to diagnose. Further similar cases should be accumulated for clarifying the pathological mechanism.