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DESMOID TUMORS are histologically benign but locally aggressive neoplasms of fibroblast origin. They are sporadic or associated with familial adenomatous polyposis and may arise from any musculoaponeurotic structure in the body. The treatment of choice for advanced and symptomatic intra-abdominal desmoid tumors is operative excision, which is challenging because of their propensity to involve major vascular structures such as the root of the mesentery. Most current imaging modalities, including computed tomography and magnetic resonance imaging, offer limited information on the true extent of the tumor, making operative planning a complex task for patients with substantial tumor burden and/or poorly defined margins. Threedimensional (3D) imaging has been used successfully in the last decade in a number of operative procedures to understand spatial relationships between vascular and other anatomic structures and thereby to minimize intraoperative complications. We describe 2 patients with advanced intraabdominal desmoid tumors in which 3D imaging technology (MeVis Technology, Bremen, Germany) allowed for precise preoperative planning and optimization of the surgical outcome.

The effects of normothermic machine perfusion (NMP) on the postreperfusion hemodynamics and extrahepatic biliary duct histology of donation after cardiac death (DCD) livers after transplantation have not been addressed thoroughly and represent the objective of this study. Ten livers (5 per group) with 60 minutes of warm ischemia were preserved via cold storage (CS) or sanguineous NMP for 10 hours, and then they were reperfused for 24 hours with whole blood in an isolated perfusion system to simulate transplantation. In our experiment, the arterial and portal vein flows were stable in the NMP group during the entire reperfusion simulation, whereas they decreased dramatically in the CS group after 16 hours of reperfusion (P0.05); these findings were consistent with severe parenchymal injury. Similarly, significant differences existed between the CS and NMP groups with respect to the release of hepatocellular enzymes, the volume of bile produced, and the levels of enzymes released into bile (P0.05). According to histology, CS livers presented with diffuse hepatocyte congestion, necrosis, intraparenchymal hemorrhaging, denudated biliary epithelium, and submucosal bile duct necrosis, whereas NMP livers showed very mild injury to the liver parenchyma and biliary architecture. Most importantly, Ki-67 staining in extrahepatic bile ducts showed biliary epithelial regeneration. In conclusion, our findings advance the knowledge of the postreperfusion events that characterize DCD livers and suggest NMP as a beneficial preservation modality that is able to improve biliary regeneration after a major ischemic event and may prevent the development of ischemic cholangiopathy in the setting of clinical transplantation.

The presence of portal vein thrombosis (PVT) is still considered by many transplantation centers to be an absolute contraindication to liver transplantation because of the technical difficulties that it can present and its association with a higher rate of patient morbidity and mortality. Renoportal bypass (RPB) can help to remove these barriers. This study describes our institution's experience with RPB through the description of a new and successful simplified surgical strategy, a patient and graft outcome analysis, intraoperative vascular flow measurements, and the use of splenic artery embolization (SAE) as an effective adjunct for treating sporadic cases of unrelieved portal hypertension. Between January 2004 and January 2013, 10 patients with grade 4 PVT underwent RPB. At the last follow-up (42.2 ± 21.1 months), the patient and graft survival rates were 100%. Five patients (50%) experienced posttransplant ascites, and 2 of those underwent proximal SAE to modulate the liver inflow and overcome the ascites. Three patients (30%) experienced transient kidney injury in the early posttransplant period and were treated efficiently with medical therapy. The renoportal flows were close to the desirable 100 mL/100 g of liver tissue in all cases. The experience and data support RPB as a feasible and easily reproducible technique without the risks and technical challenges associated with the tedious dissection of a cavernous hilum. Liver Transpl 21:344–352, 2015. © 2015 AASLD.

Ductal adenocarcinoma of pancreas represents one of the most aggressive tumor as demonstrated by 3- and 5-year survival rates. Involvement of mesenteric pedicle affects both the possibility to perform a tumor-free margin resection and accounts for most exploratory laparotomy for locally advanced disease. The ex vivo resection of the tumor (autotransplantation) after total exenteration and perfusion of the intestine might have a role to overcome some technical obstacles. So far, only 5 patients have been reported to have undergone small-bowel autotransplantation for tumor involving the mesenteric root. We describe 2 cases of adenocarcinoma of pancreas involving mesenteric root treated by small-bowel autotransplantation. Both patients survived from the procedure and were discharged home on postoperative days 16 and 29, respectively. The tumor was resected with free surgical margins, and both patients underwent adjuvant treatment. Intestinal autotransplantation can represent a significant technical advance for increasing the resectability rate and, ultimately, the survival rate for advanced adenocarcinoma of the pancreas in highly selected patients.

Introduction One of the critical factors that influence graft function after live donor liver transplantation is the presence or absence of global or sectorial liver congestion. Many authors advocate for routine middle hepatic vein (MHV) reconstruction because it is often difficult to determine when the MHV or one of its major branches have functional significance. Predictive tests to assess hemodynamic and functional significance of the MHV and its tributaries are still under study. Case Report We have described a novel intraoperative manipulation and Doppler ultrasonographic evaluation that led to the decision to include the MHV with the right lobe graft.

Abdominal closure is a complex surgical problem in intestinal transplant recipients with loss of abdominal domain, as graft exposure results in profound morbidity. Although intraoperative coverage techniques have been described, this is the first report of preoperative abdominal wall augmentation using tissue expanders in patients awaiting intestinal transplantation. We report on five patients who received a total of twelve tissue expanders as a means to increase abdominal surface area. Each patient had a compromised abdominal wall (multiple prior operations, enterocutaneous fistulae, subcutaneous abscesses, stomas) with loss of domain and was identified as high risk for an open abdomen post-transplant. Cross-sectional imaging and dimensional analysis were performed to quantify the effect of the expanders on total abdominal and intraperitoneal cavity volumes. The overall mean increase in total abdominal volume was 958 cm(3) with a mean expander volume of 896.5 cc. Two expanders were removed in the first patient due to infection, but after protocol modification, there were no further infections. Three patients eventually underwent small bowel transplantation with complete graft coverage. In our preliminary experience, abdominal tissue expander placement is a safe, feasible, and well-tolerated method to increase subcutaneous domain and facilitate graft coverage in patients undergoing intestinal transplantation.

Living donor liver transplantation (LDLT) is associated with a low but finite and well-documented risk of donor morbidity and mortality, so organizations and individuals involved in this activity must accept the fact that a donor death is a question of when and not if. Studies in the field of crisis management show that preparing for the inevitable not only is critical in preparing institutions to better respond to catastrophic events but more importantly plays a crucial role in preventing them. This article describes the background of crisis management with specific reference to the death of a living liver donor and proposes a general framework that can be adopted by LDLT programs around the world.

Living donor liver transplantation (LDLT) is a well-established strategy to decrease the mortality in the waiting list and recent studies have demonstrated its value even in patients with low MELD score. However, LDLT is still under a high level of scrutiny because of its technical complexity and ethical challenges as demonstrated by a decline in the number of procedures performed in the last decade in Western Countries. Many aspects make LDLT different from deceased donor liver transplantation, including timing of transplantation, procedure-related complications as well as immunological factors that may affect graft outcomes. Our review suggests that in selected cases, LDLT offers significant advantages over deceased donor liver transplantation and should be used more liberally.

Most solid-organ transplants performed in the Western world are from deceased donors. In the last decade, deceased donation rates have reached a plateau as the number of patients with end-stage organ disease has steadily increased, resulting in a large discrepancy between organ supply and demand. Living donor transplantation is one way to decrease this discrepancy. However, living donation is not universally accepted. For instance, living donation rates vary geographically (eg, living donation is more accepted in Asia than in the Western world) and depend on the organ donated (eg, kidney versus liver donation). In this article we will review the ethical principles guiding living donor liver transplantation, with emphasis on justification and safeguards that support the practice of adult-to-adult living donor liver transplantation, the most clinically and ethically challenging type of living organ donation. Our ethical justification will include a presentation of triangular or tripartite equipoise, a framework that aims to balance donor safety, expected recipient outcomes, and need.

This review describes our program and its outcomes and then provides an in-depth focuses into many of the unique aspects of our practice that have been important to the success of the program. These include a global appreciation for the impact and various presentations of chronic portal hypertension. We have sought to better understand and describe the various effects it can have on local allograft hemodynamics and graft survival. Intraoperative blood flow measurements of the hepatic artery and portal vein are important. Postoperative follow-up with Doppler ultrasound has been essential for both partial and whole grafts. A better understanding of systemic and graft hemodynamics has changed our clinical practice with regards to the intra- and post-operative management of the hepatic artery and portal vein. We have also focused on the issue of hepatocellular carcinoma, one of the major indications for liver transplantation. We have sought to better understand the heterogeneous clinical presentations of this disease and how to best approach them in a multidisciplinary fashion. Finally, we describe the various methods we have utilized to increase the number of hepatic grafts available for our patients. We have aggressively utilized all forms of grafts; living and deceased; partial and whole; and extended and standard criteria donors. We have done this with the focus on living donor safety and then concentrated on finding the best graft for the individual patient in the context of the national allocation systems in which we all work.

Recurrence of hepatitis C virus (HCV) can be difficult to distinguish from acute cellular rejection (ACR) following liver transplantation. The Cylex Immune Function Assay (ImmuKnow) provides objective measure of recipient's immune function. The goal is to assess the ability of this assay to distinguish these similar conditions. A retrospective review was performed in 54 recipients with HCV. ImmuKnow assays were measured with allograft biopsies. Levels of adenosine triphosphate (ATP) release from CD4+ T cells (ng/mL) were compared with the following biopsy result classifications: 365 ± 130 with ACR (n = 11), 152 ± 100 with recurrent HCV (n = 26), 240 ± 71 with normal biopsies (n = 12), and 157 ± 130 with overlapping features of ACR and recurrent HCV (n = 5). Recipients with recurrent HCV had lower immune response than those with ACR (p0.0001).Using a cutoff level of 220, the sensitivity and specificity for distinguishing two conditions were 88.5% and 90.9%, respectively. When recipients with overlapping features had low immune response, three of four recipients' subsequent biopsies showed recurrent HCV. In conclusion, the ImmuKnow assay can be a sensitive and specific additional test for distinguishing recurrent HCV from ACR and may be useful for predicting which recipients may be most vulnerable to recurrent HCV.

The aim of the study is to define the role of the HABR in the pathophysiology of the SFS liver graft and to demonstrate that restoration of hepatic artery flow (HAF) has a significant impact on outcome and improves survival. Nine pigs received partial liver allografts of 60% liver volume, Group 1; 8 animals received 20% LV grafts, Group 2; 9 animals received 20% LV grafts with adenosine infusion, Group 3. HAF and portal vein flow (PVF) were recorded at 10 min, 60 min and 90 min post reperfusion, on POD 3 and POD 7 in Group 1, and daily in Group 2 and 3 up to POD 14. Baseline HAF and PVF (ml/100 g/min) were 29 +/- 12 (mean +/- SD) and 74 +/- 8 respectively, with 28% of total liver blood flow (TLBF) from the HA and 72% from the PV. PVF peaked at 10 mins in all groups, increasing by a factor of 3.8 in the 20% group compared to an increase of 1.9 in the 60% group. By POD 7-14 PVF rates approached baseline values in all groups. The HABR was intact immediately following reperfusion in all groups with a reciprocal decrease in HAF corresponding to the peak PVF at 10 min. However in the 20% group HAF decreased to 12 +/- 8 ml/100 g/min at 90 min and remained low out to POD 7-14 despite restoration of normal PVF rates. Histopathology confirmed evidence of HA vasospasm and its consequences, cholestasis, centrilobular necrosis and biliary ischemia in Group 2. HA infusion of adenosine significantly improved HAF (p.0001), reversed pathological changes and significantly improved survival (p = .05). An impaired HABR is important in the pathophysiology of the SFSS. Reversal of the vasospasm significantly improves outcome.

Purpose To correlate fundus autofluorescence (FAF) in choroidal metastases with clinical changes overlying the lesions using confocal scanning laser ophthalmoscope. Methods Retrospective chart review of 15 metastatic tumors in 14 eyes of 11 consecutive patients. The patients underwent confocal scanning laser ophthalmoscope to compare the clinical findings and FAF. Correlation between increased FAF and clinical changes was defined as complete, partial, or no correlation. Results Of 11 patients, 8 (73%) were women, and the average age was 56 years. All 15 metastases were amelanotic, focal hyperpigmentation was present in 8 (53%), subretinal fluid in eleven (73%), whereas drusen and orange pigment were absent. Of 8 tumors that were visualized by scanning laser ophthalmoscope, 7 tumors (87%) showed correlation between FAF and hyperpigmentation, no correlation in 1 (13%). A complete correlation between hyperpigmentation and FAF was found in 5 (70%) tumors and a partial correlation in 2 (30%). There was partial correlation between subretinal fluid and FAF in 6 metastases (46%), no correlation in 5 (39%). Three metastases without hyperpigmentation had FAF, one showed the presence of subretinal fluid. Conclusion Amelanotic choroidal metastases with hyperpigmentation are associated with increased FAF in most cases. Subretinal fluid overlying choroidal metastases may cause FAF as well.

Splenic artery embolization (SAE) improves hepatic artery (HA) flow in liver transplant (OLT) recipients with so-called splenic artery steal syndrome. We propose that SAE actually improves HA flow by reducing the HA buffer response (HABR). Patient 1: On postoperative day (POD) 1, Doppler ultrasonography (US) showed patent vasculature with HA resistive index (RI) of 0.8. On POD 4, aminotransferases rose dramatically; his RI was 1.0 with no diastolic flow. Octreotide was begun, but on POD 5 US showed reverse diastolic HA flow with no signal in distal HA branches. After SAE, US showed markedly improved flow, RI was 0.6, diastolic flow in the main artery, and complete visualization of all distal branches. By POD 6, liver function had normalized. RI in the main HA is 0.76 at 2 months postsurgery. Patient 2: On POD 1, RI was 1.0. US showed worsening intrahepatic signal, with no signal in the intrahepatic branches and reversed diastolic flow despite good graft function. On POD 7, SAE improved the intrahepatic waveform and RI (from 1.0 to 0.72). Patient 3: Intraoperative reverse diastolic arterial flow persisted on PODs 1, 2, and 3, with progressive loss of US signal in peripheral HA branches. SAE on POD 4 improved the RI (0.86) and peripheral arterial branch signals. Patient 4: US on POD 1 showed good HA flow with a normal RI (0.7). A sudden waveform change on POD 2 with increasing RI (0.83) prompted SAE, after which the wave form normalized, with reconstitution of a normal diastolic flow (RI 0.68). In conclusion, these reports confirm the usefulness of SAE for poor HA flow but suggest that inflow steal was not the problem. Rather than producing an increase in arterial inflow, SAE worked by reducing portal flow and HABR, thereby reducing end-organ outflow resistance. Evidence of this effect is the marked reduction of the RI after the SAE to 0.6, 0.72, 0.86, and 0.68, in patients 1-4, respectively. SAE reduces excessive portal vein flow and thereby ameliorates an overactive HABR that can cause graft dysfunction and ultimately HA thrombosis.

Backgrounds/aims De-novo autoimmune hepatitis (AIH) after liver transplantation (LT) is rising. Yet there is scarcity of data on the characteristics and the long-term outcomes of this condition. The aim of this study is to investigate the clinical characteristics and long-term outcomes of patients with de novo AIH following LT.Methods Using transplant liver biopsy database, we identified all patients with de-novo AIH following LT at our institution between 2008 and 2013. Patients with hepatitis C virus infection were excluded. The diagnosis of de-novo AIH was made according to the classical and simplified criteria defined by the International Autoimmune Hepatitis Group. Clinical information was gathered from electronic medical records. H&E stained sections and histochemical stains from the liver biopsies revealed findings compatible with AIH. Results A total of nineteen patients with de-novo AIH were identified (58% female, median age of 46 years), with mean international autoimmune hepatitis score of 12.1. Underlying liver disease were primary sclerosing cholangitis (n=4), primary biliary cirrhosis (n=3), biliary atresia (n=3), drug induced liver failure (n=2), alcoholic hepatitis (n=2) and others (n=5). The interval period from the LT to diagnosis of de-novo AIH was 19.6 months (1.6-197.8), during which 11 (58%) patients developed at least one episode of acute cellular rejection prior to the diagnosis of de-novo AIH. All patients were successfully treated with corticosteroids and incremental dose in immunosuppression. All showed complete biochemical response to treatment but 9 (47.4%) patients relapsed upon tapering down corticosteroids. Patients were followed over 6.7 year (1.5-17) years post-LT. Eight (42%) patients progressed to cirrhosis of whom 3 (15%) patients expired and 2 (10%) required second LT due to complications of end stage liver disease. Conclusion The present study shows the long term clinical outcomes of the patients with de-novo AIH post-LT. Although most patients exhibit a good initial response to medical therapy, de-novo AIH post-LT is likely to recur and progress to liver cirrhosis. Therefore, we should consider denovo AIH in patients who show abnormal liver tests or graft dysfunction after LT.

BackgroundReconstruction of biliary drainage after liver transplantation (LTx) in patients with primary sclerosing cholangitis (PSC) has been a matter of controversy. Over recent years, the traditional method of Roux-en-Y hepaticojejunostomy (RY) has been challenged by duct-to-duct (DD) biliary reconstruction.MethodsThis study represents a retrospective review of biliary complications, patient and graft survival after LTx in PSC patients based on type of biliary reconstruction. Outcomes of DD reconstruction in this group of patients and non-PSC patients are compared.ResultsA total of 53 primary LTx procedures were performed for PSC between August 2005 and July 2010. Seven patients were excluded because unexpected cholangiocarcinoma was found in the explants (n= 3) or because they received partial livers (n= 4). Biliary reconstruction was performed as DD in 18 patients and RY in 28 patients. There were no bile leaks. Anastomotic stricture occurred in two (11%) patients in the DD group and one (4%) in the RY group. Two (7%) patients in the RY group developed non-PSC intrahepatic strictures and one had recurrence of PSC. Rates of 1- and 3-year patient and graft survival in the RY and DD groups were 96.7% and 96.7%, and 100% and 94.5%, respectively. In a group of 34 randomly selected patients transplanted for a non-PSC diagnosis with DD reconstruction during the same period, the anastomotic stricture rate was 9% and 1- and 3-year patient and graft survival rates were 97.0% and 88.5%; differences were not significant.ConclusionsDuct-to-duct biliary reconstruction at the time of LTx in selected PSC patients is both effective and safe, and shows outcomes comparable with those of RY reconstruction in these patients and those of DD reconstruction in non-PSC patients.