A Unique Presentation of Solid Pseudopapillary Neoplasm of the Pancreas Requiring Pancreaticoduodenectomy Without Pancreatojejunostomy: A Case Report and Literature Review.

Solid pseudopapillary neoplasms (SPNs) of the pancreas are rare tumors that predominantly affect young females and are typically located in the body and tail of the pancreas. Here, we present the case of a 59-year-old male with a large, heavily calcified SPN in the pancreatic head. His surgical history includes an aborted pancreaticoduodenectomy due to vascular involvement, followed by a gastrojejunostomy. Twenty years after the initial discovery, a pancreaticoduodenectomy was performed - the first of its kind - where the pancreas was completely atrophied, and no pancreaticojejunostomy was performed. Histological examination revealed typical features of SPN. This case demonstrates that even with relatively large lesions in a male patient over an extended duration, SPNs can still exhibit favorable features, highlighting the absence of specific preoperative markers for aggressive tumors. Therefore, unless there is an absolute contraindication, complete resection of all SPNs remains advisable.

Gastric Schwannoma in an Octogenarian: A Case Report and Review of the Literature.

Gastric schwannomas are an exceedingly rare tumor arising from the myenteric plexus of the gastrointestinal enteric nervous system. These schwannomas are most commonly benign and reported to occur in female patients with a mean age of 58 at presentation. They are most often discovered incidentally, but can occasionally present with abdominal discomfort, obstructive symptoms, or GI bleeding. Frequently, the initial clinical consideration is for a gastrointestinal stromal tumor, which is much more common. A definitive diagnosis is made with microscopic imaging and immunohistochemical staining. Complete surgical resection, typically performed laparoscopically, is the most definitive and usually curative treatment, requiring no further follow-up. Herein, we present the first and only case of gastric schwannoma in an octogenarian and discuss an update on current diagnostic and therapeutic options.

Atypical Hemolytic Uremic Syndrome After Kidney Transplantation: Lessons Learned From the Good, the Bad, and the Ugly. A Case Series With Literature Review.

Atypical hemolytic uremic syndrome (aHUS) after kidney transplantation is rare and carries a grave outcome. We present a single-center experience of all aHUS cases since the program's inception. Six patients were diagnosed with aHUS, all after kidney transplants, except for 1 patient. All had nonreactive crossmatches. Delayed graft function (DGF) occurred in 2 patients. Five patients developed aHUS after transplant; 4 (80%) of these patients manifested aHUS ≤ 14 days. All were confirmed by allograft biopsy. Genetic testing was abnormal in all patients except for 1 patient. Actual patient and graft survival during the first year was 100% and 83.3%, respectively. A single graft was lost early in the study secondary to aHUS (eculizumab was not used in the treatment process). Prophylactic and therapeutic use of eculizumab salvaged all other cases. At 1 year, mean creatinine level was 1.9 mg/dL (range, 1.3-2.5). After 6 months of eculizumab treatment (halted in 2 cases) 1 patient had recurrence 2 months later and eculizumab was restarted. However, graft function continued to worsen, and the graft was ultimately lost at 20 months after kidney transplantation. High index of suspicion, prompt diagnosis, and utilization of eculizumab are key to successful salvage of allografts in cases of aHUS after kidney transplantation. aHUS can be prevented by prophylactic use of eculizumab. It still needs to be determined when and if eculizumab therapy can be safely discontinued.

Successful Transplantation of Pediatric Kidneys Despite Vascular Injuries.

The gap between the kidney transplant recipient list and the number of organs available for transplantation continues to grow. Pediatric donors help fill a small and valuable portion of that gap. Normally these organs are transplanted en-bloc by closing the proximal vascular caps and using the distal aorta and distal inferior vena cava (IVC) for inflow. They are however commonly injured during the donor operation making the standard operation for pediatric en-bloc transplantation not possible. This case report presents two cases in which injured small pediatric kidneys were transplanted successfully in adult patients. We are presenting two examples of common vascular injuries to small pediatric kidneys, one venous and one arterial. In both scenarios, the kidneys were transplanted using a modification to the standard technique. The two kidneys were separated and the technique of implantation was modified to allow safe transplantation. This way we were able to transplant both kidneys successfully and using a reproducible methodology. Both recipients were young adults. There were no surgical complications.

Renal Cell Carcinoma Metastases to the Pancreas and the Thyroid Gland: A Case Report and Review of the Literature.

Renal cell carcinoma (RCC) is known for having unpredictable clinical behavior. Metastases can occur in unusual locations with a long-time lag after the treatment of the primary cancer. Despite being a sign of poor prognosis, aggressive metastasectomy may prolong survival. Presented is a case of delayed sequential metastases of RCC to the pancreas and the thyroid gland that occurred eight years after the radical nephrectomy. Both were resected. A history of remote nephrectomy for RCC is important and may be suggestive of metastatic disease. Ultrasound-guided fine needle aspiration can be diagnostic and helps in decision-making. Aggressive surgical intervention when possible is recommended.

Attitudes and barriers to the use of donation after cardiac death livers: Comparison of a United States transplant center survey to the united network for organ sharing data.

Transplantation of liver grafts from donation after cardiac death (DCD) is limited. To identify barriers of DCD liver utilization, all active US liver transplant centers (n = 138) were surveyed, and the responses were compared with the United Network for Organ Sharing (UNOS) data. In total, 74 (54%) centers responded, and diversity in attitudes was observed, with many not using organ and/or recipient prognostic variables defined in prior studies and UNOS data analysis. Most centers (74%) believed lack of a system allowing a timely retransplant is a barrier to utilization. UNOS data demonstrated worse 1- and 5-year patient survival (PS) and graft survival (GS) in DCD (PS, 86% and 64%; GS, 82% and 59%, respectively) versus donation after brain death (DBD) recipients (PS, 90% and 71%; GS, 88% and 69%, respectively). Donor alanine aminotransferase (ALT), recipient Model for End-Stage Liver Disease (MELD), and cold ischemia time (CIT) significantly impacted DCD outcomes to a greater extent than DBD outcomes. At 3 years, relisting and retransplant rates were 7.9% and 4.6% higher in DCD recipients. To optimize outcome, our data support the use of DCD liver grafts with CIT <6-8 hours in patients with MELD ≤ 20. In conclusion, standardization of donor and recipient criteria, defining the impact of ischemic cholangiopathy, addressing donor hospital policies, and developing a strategy for timely retransplant may help to expand the use of these organs. Liver Transplantation 23 1372-1383 2017 AASLD.

Outcomes after combined liver-kidney transplant vs. kidney transplant followed by liver transplant.

INTRODUCTION: The decision for isolated kidney transplant (KT) vs. combined liver-kidney transplant (CLKT) in patients with end-stage renal disease (ESRD) with compensated cirrhosis remains controversial. We sought to determine outcomes of patients requiring listing for a liver transplant (LT) following either a cadaveric or living donor KT and compare these outcomes to similar patients receiving a CLKT. METHODS: Our dataset included the United Network for Organ Sharing (UNOS)/Standard Transplant and Analysis and Research (STAR) kidney files from 1987 to 2012 after being joined with the liver files from 2002 to 2012. Outcomes of patients who received a CLKT with an international normalized ratio (INR) ≤1 and total bilirubin ≤1 were compared to patients who received a primary KT and subsequently required listing for LT between zero and five yr or after five yr. RESULTS: For the three groups, 244 patients had a CLKT, 216 were wait-listed for LT between zero and five yr after KT (0-5 WL), and 320 were wait-listed five yr after KT (+5 WL). From the time of KT, the 0-5 WL group had significantly worse survival than the CLKT group and the +5 WL group. The +5 WL had the best survival of all groups. For the 0-5 WL group, 45% underwent LT and 40% died while waiting compared to the +5 WL group with 53% having LT and 26% died while waiting. At the time of LT, the 0-5 WL group had a higher model for end-stage liver disease (MELD) score, higher incidence of being in the ICU at the time of transplant, and higher incidence of requiring life support. From the time of LT, the CLKT trended toward better survival (p = 0.0549) than both the 0-5 WL and +5 WL groups, which had equivalent survival. CONCLUSION: The 0-5 WL group is a higher risk group with poorer survival due to a higher incidence of dying on the waitlist. Better identification of patients with a high risk for hepatic decompensation following KT and agreement for regional exception for LT in the event of decompensation may improve utilization of organs and better survival for those patients.

Biliary complications adversely affect patient and graft survival after liver retransplantation.

Inferior outcomes are consistently observed for recipients of liver retransplantation (re-LT) versus recipients of primary transplants. Few studies have examined the incidence and impact of biliary complications (BCs) on outcomes after re-LT. The aim of this study was to compare patient and graft survival for re-LT recipients with BCs (BC(+) ) and re-LT recipients without BCs (BC(-) ). Additional aims were to determine the impact of biliary reconstruction on the incidence of BCs and to identify risk factors for BCs after re-LT. A single-center, retrospective analysis of all re-LT recipients over a decade was performed. Univariate analyses were performed, and survival was compared with the log-rank method. A multivariate Cox regression analysis was performed to determine independent predictors of death and graft failure. The BC rate was 20.9% (n = 23) for 110 re-LT cases. The average follow-up was 55 months. The survival rates for BC(-) recipients at 3 months and 1, 3, and 5 years were 95.3%, 91.7%, 85.4%, and 80.9%, respectively, whereas BC(+) patients had survival rates of 64.3%, 49.7%, 34.8%, and 29.8%, respectively (P < 0.001, log-rank). The graft survival rates at 3 months and 1, 3, and 5 years were 92.0%, 88.5%, 82.4%, and 78.0%, respectively, for the BC(-) group and 60.9%, 43.5%, 30.4%, and 26.1%, respectively, for the BC(+) group (P < 0.001, log-rank). BCs, a length of stay ≥ 12 days, and donor age were strongly associated with death and graft failure in a regression analysis, whereas retransplant indications other than chronic rejection and recurrent disease also affected graft failure. In conclusion, BCs significantly affected both patient and graft survival, with an increased risk of death and graft loss among BC(+) recipients. Early recognition, appropriate interventions, and preventative measures for BCs are critical in the clinical management of re-LT recipients.

The value of C4d deposit in post liver transplant liver biopsies.

BACKGROUND: Presence of C4d in renal and cardiac allografts is a sign of antibody-mediated rejection and is associated with worse outcomes. The value of C4d in liver specimens is controversial. We aimed to determine the association of C4d deposition with acute cellular rejection (ACR), hepatitis C (HCV) recurrence, and clinical outcome after ABO compatible liver transplants (OLT). METHODS: Using immunohistochemical stain, 70 liver biopsies (44 study and 26 control groups) were evaluated for C4d deposition. Study group included for-cause post OLT biopsies. Staining of endothelial cells was considered positive. RESULTS: In the study group C4d was positive in 22.7% versus 3.8% in controls (P=0.03), all had portal vein deposits. In 17 biopsies with ACR, 3 had positive C4d (17.6%) versus 7/27 with HCV recurrence (25.9%) (P=0.4). In HCV recurrence, 3/7 biopsies with fibrosing cholestatic hepatitis had positive C4d (42.9%) versus 4/20 without these features (20%) (P=0.24). Out of 10 recipients with positive C4d 4 had poor outcomes versus 3/22 with negative C4d (P=0.12). CONCLUSIONS: C4d staining was significantly more frequent in post OLT biopsies compared with controls. C4d is not specifically associated with ACR and does not differentiate it from HCV recurrence but is associated with a trend toward poorer outcome.

Ureteral stents are associated with reduced risk of ureteral complications after kidney transplantation: a large single center experience.

BACKGROUND: Controversy exists regarding the benefit of ureteral stents in kidney transplantation. We aimed to examine the association of stents with risk of ureteral complications, particularly in relationship with donor type. METHODS: Kidney transplants from 2005 to 2009 were evaluated (n=1224). Patients with previous or simultaneous nonkidney transplants, death, or lost to follow-up within 90 days were excluded, unless already developed a ureteral complication. Only cases with a single extravesical ureteroneocystostomy were included. The cohort (n=961) was divided into stent (32.2%) and no-stent (67.7%) groups. Poisson regression was used to examine the association of stent with ureteral complications (leak or stricture) and urinary tract infections (UTI). RESULTS: Ureteral complication rate was 1.9% in stent versus 5.8% in no-stent group (P=0.007). UTI rate was 14.2% with stent versus 7.9% without stent (P=0.003). Stent use was independently associated with reduction in ureteral complications (incidence rate ratios [IRR], 0.40; P=0.04; 95% confidence interval [CI], 0.17-0.96) and an increase in UTI risk (IRR, 1.79; P=0.006; 95% CI, 1.18-2.74). Stent protective effect was primarily related to reduction in stricture risk (IRR, 0.23; P<0.05; 95% CI, 0.05-0.99). Stents were associated with a decrease in ureteral complications in deceased donor recipients (IRR, 0.34; P=0.03; 95% CI, 0.13-0.88), but not living donors (IRR, 1.24; P=0.84; 95% CI, 0.15-10.2). CONCLUSIONS: Ureteral stents are associated with a significant reduction in ureteral complications but increases UTI risk. Routine stenting in deceased donor transplants is recommended as its protective effect was observed in this group. The value of stents in living donor transplants warrants further investigation.

Central pontine myelinolysis: a case report and clinical-pathological review.

An 11-yr-old child presented with acute mental status changes and spastic quadriplegia after orthotopic liver transplantation. Magnetic resonance (MR) imaging findings were consistent with central pontine and EPM. Initial immunosuppression included tacrolimus, mycophenolate mofetil, and corticosteroids. Given that neurotoxicity is a well-established side effect of CNI, the patient was converted to rapamycin and subsequently experienced significant neurologic recovery. The temporal resolution of the patient's symptoms suggests that prompt recognition of central pontine and EPM and conversion from tacrolimus to rapamycin during the early post-operative course may have therapeutic benefits for patients undergoing pediatric transplant with CNI-related neurotoxicity.

Valganciclovir is an effective prophylaxis for cytomegalovirus disease in liver transplant recipients.

OBJECTIVES: Cytomegalovirus (CMV) infection is responsible for significant morbidity and mortality among solid organ transplant recipients. Prophylaxis using valganciclovir (VGCV) in orthotopic liver transplant (OLT) recipients is not approved by the Food and Drug Administration and its use is controversial. This study aimed to evaluate the effectiveness of VGCV in CMV prophylaxis in OLT recipients. METHODS: We carried out a retrospective, single-centre study including all OLT procedures performed during 2005-2008. Patients with early death (at ≤ 30 days), without CMV serology or prophylaxis, or with follow-up of <1 year were excluded. RESULTS: The overall incidence of CMV disease was 6% (n= 9). The ganciclovir (GCV) and VGCV groups had similar incidences of CMV disease (4.6% vs. 7.0%; P= 0.4) and similar distributions of disease presentation (CMV syndrome vs. tissue-invasive CMV; P= 0.4). Incidences of CMV infection, as well as disease presentation, were similar between the high-risk (CMV D+/R-) and non-high-risk groups (P= 0.16). Although acute cellular rejection occurred more frequently in patients who developed CMV disease (P= 0.005), overall survival in these patients did not differ from that in patients who did not develop CMV infection (P= 0.5). CONCLUSIONS: Valganciclovir is an effective antiviral for the prevention of CMV disease in liver transplant recipients. Our data support its use in high-risk OLT patients.