Search

The first TAVI implantation was performed in 2002 by Cribier and colleagues. From then, we have seen significant progress in technology, with the appearance of new generations – lower-profile, longer-lasting and more stable during positioning TAVI prostheses. The present publication presents single-center results and experience with TAVI procedures over an eight-year period. During the period 2013 – November 2022 a total of 354 TAVI procedures were performed in Acibadem City Clinical Cardiovascular Center. Two operating strategies are used – standard and minimalist approach. From the first procedure in 2013 until mid-2018 the standard approach (surgical vascular access, general anesthesia, direct valve implantation where possible) was mostly used. Then, the minimalist approach (percutaneous access, no intubation anesthesia, mandatory valve predilation and use of rapid pacing) was introduced as a standard approach in the center.

Purpose Performing a systematic review and meta-analysis to assess the evidence of intra-arterial therapies in liver metastatic breast cancer (LMBC) patients. Methods A systemic literature search was performed in PubMed, EMBASE, SCOPUS for studies regarding intra-arterial therapies in LMBC patients. Full text studies of LMBC patients (n >= 10) published between January 2010 and December 2020 were included when at least one outcome among response rate, adverse events or survival was available. Response rates were pooled using generalized linear mixed models. A weighted estimate of the population median overall survival (OS) was obtained under the assumption of exponentially distributed survival times. Results A total of 26 studies (1266 patients) were included. Eleven articles reported on transarterial radioembolization (TARE), ten on transarterial chemoembolization (TACE) and four on chemo-infusion. One retrospective study compared TARE and TACE. Pooled response rates were 49% for TARE (95%CI 32-67%), 34% for TACE (95%CI 22-50%) and 19% for chemo-infusion (95%CI 14-25%). Pooled median survival was 9.2 months (range 6.1-35.4 months) for TARE, 17.8 months (range 4.6-47.0) for TACE and 7.9 months (range 7.0-14.2) for chemo-infusion. No comparison for OS was possible due to missing survival rates at specific time points (1 and 2 year OS) and the large heterogeneity. Conclusion Although results have to be interpreted with caution due to the large heterogeneity, the superior response rate of TARE and TACE compared to chemo-infusion suggests first choice of TARE or TACE in chemorefractory LMBC patients. Chemo-infusion could be considered in LMBC patients not suitable for TARE or TACE.

Background Insufficient preoperative work-up and consequent intralesional or marginal resection of soft-tissue sarcomas of the head and neck (STSHNs) is common. Methods This retrospective cohort study comprised 63 patients with STSHN treated at the Helsinki University Hospital between 2005 and 2017. We assessed the effect of pretreatment tumor sampling on surgical margin status and need for supplemental surgery, as well as prognostic factors and survival. Results The lack of representative pretreatment biopsy specimen was associated with unfavorable margin status. Primary surgery at a non-academic center was associated with need for supplemental surgery. The 3-year overall survival (OS) was 68%, disease-specific survival (DSS) 71%, and recurrence-free survival (RFS) 61%. Higher tumor grade and primary tumor size over 5 cm were associated with reduced DSS. Conclusions Diagnosis and management of STSHNs should be centralized to experienced academic centers. Decision-making between needle biopsy, open biopsy, or upfront radical surgery depends on tumor location and size.

Shortage of deceased donor organs for transplantation has led to the increased use of organs from donation after circulatory death (DCD) donors. There are currently no reports describing outcomes after multiorgan transplantation with DCD livers. The use of DCD organs for multiorgan transplantation can be enhanced if the detrimental effects of prolonged cold ischemia and subsequent ischemia-reperfusion injury are overcome. We present a case in which the liver and lungs of a DCD donor were preserved using ex situ machine perfusion for combined liver-lung transplantation. The recipient was a 19-year-old male patient requiring bilateral lung transplantation for severe progressive pleural parenchymal fibroelastosis and portal hypertension with portal vein thrombosis. The donor liver was preserved with dual hypothermic oxygenated machine perfusion, whereas the lungs were perfused using ex vivo lung perfusion. With ex vivo lung perfusion, total preservation time of right and left lung reached 17 and 21 h, respectively. Now, 2 y after transplantation, liver function is normal and lung function is improving. To conclude, we suggest that combined transplantation of DCD liver and lungs is feasible when cold ischemia is reduced with ex situ machine perfusion preservation.

Lung transplantation may be a final destination therapy in lung failure, but limited donor organ availability creates a need for alternative management, including artificial lung technology. This invited review discusses ongoing developments and future research pathways for respiratory assist devices and tissue engineering to treat advanced and refractory lung disease. An overview is also given on the aftermath of the coronavirus disease 2019 pandemic and lessons learned as the world comes out of this situation. The first order of business in the future of lung support is solving the problems with existing mechanical devices. Interestingly, challenges identified during the early days of development persist today. These challenges include device-related infection, bleeding, thrombosis, cost, and patient quality of life. The main approaches of the future directions are to repair, restore, replace, or regenerate the lungs. Engineering improvements to hollow fiber membrane gas exchangers are enabling longer term wearable systems and can be used to bridge lung failure patients to transplantation. Progress in the development of microchannel-based devices has provided the concept of biomimetic devices that may even enable intracorporeal implantation. Tissue engineering and cell-based technologies have provided the concept of bioartificial lungs with properties similar to the native organ. Recent progress in artificial lung technologies includes continued advances in both engineering and biology. The final goal is to achieve a truly implantable and durable artificial lung that is applicable to destination therapy.

Context Acute pulmonary embolism (PE) is a lethal sequela of venous thromboembolism (VTE). Surgical trauma injures the tissue directly, releasing a large number of tissue agent. The frequency of re-exploration owing to bleeding after lung surgery is between 1 and 3.7%, whereas the need of allogenic blood transfusion spans from 20 to 52%. Aims To assess the role of tranexamic acid (TXA) in reducing the need of allogenic blood transfusion in patients undergoing elective lung surgery. Patients and methods This retrospective study was conducted on 140 patients who underwent elective lung surgery. Patients were allocated into two groups. Group I patients received TXA at the end of procedure, and group II patients received blood and/or blood product transfusion. Statistical analysis Qualitative variables are expressed as mean±SD. Quantitative variables are compared by using the Student’s t test. Results The patients comprised 80 males and 60 females in our series. All cases in group II needed transfusion of one or more of the following: concentrated red blood cells, whole blood, fresh frozen plasma, and platelets. There was an obvious decrease in the postoperative hemoglobin level between groups, in favor of blood transfusion group (group II), and this was statistically significant. Conclusions Elective thoracic surgery patients have a low incidence of VTE and PE (2.85 and 2.14%, respectively). Hence, TXA helps in minimizing not only transfusion-related hazards but also operative cost.

Introduction Patients with Peyronie's disease may experience significat distress. The choice of treatment depends on a variety of factors, including the stage of the disease, the presence of pain, severity and direction of the curvature, penile length and the quality of erectile function. Aim To review the evidence associated with surgical treatment of Peyronie‘s Disease and provide clinical recommendations on behalf of the European Society for Sexual Medicine. 131 peer-reviewed studies and systematic reviews, which were published from 2009 to 2019 in the English language, were included. Methods MEDLINE, Google Scholar and EMBASE were searched for randomized clinical trials, meta-analyses, open-label prospective and retrospective studies. Main Outcome Measure The panel provided statements on clinically relevant questions including patient involvement in the decision process, indications for surgery, choice of the approach, and the management of patient expectations. A comparison of the different grafts used in patients who have undergone plaque incision/excision and grafting in order to identify an ideal graft, has been carried out. The prevalence of postoperative complications has been summarized. Levels of evidence were provided according to the Oxford 2011 criteria and Oxford Centre for Evidence-Based Medicine recommendations. Results In order to allow shared decision making, a patient preoperative counselling regarding the pros and cons of each intervention is recommended. In particular, adverse effects of surgical treatments should be discussed to set realistic understanding and expectations of surgical outcomes and ultimately improve postoperative satisfaction rates. Surgical treatment should be only offered in the chronic phase of the condition, when the deformity and/or degree of erectile dysfunction, prevent patients from engaging in satisfying sexual interaction, or if the deformity is the cause of severe bother. Conclusions Current European Society for Sexual Medicine recommendations cover several aspects of Peyronie's disease treatment. These recommendations aim both to ensure patients and partners have accurate and realistic expectations of their treatment options, as well as to formulate algorithms to guide clinician management pathways. Osmonov D, Ragheb A, Ward S et al, ESSM Position Statement on Surgical Treatment of Peyronie's Disease. Sex Med 2022;10:100459.

Despite therapeutic advances, high-risk neuroblastoma is still associated with a poor long-term prognosis. Immunotherapy with the anti-GD2 antibody dinutuximab beta has recently been added to the standard of care for patients with high-risk neuroblastoma in our center in Bratislava, and our initial experience with dinutuximab beta has been reported previously. Here we provide a follow-up on the outcomes of 7 patients who were treated with dinutuximab beta under clinical practice conditions at our center. Medical records of 31 patients diagnosed with neuroblastoma between 2017 and 2020 at the Children's Hematology and Oncology Clinic in Bratislava were retrospectively reviewed and 7 patients with high-risk neuroblastoma who were treated with dinutuximab beta were identified. All 7 patients received dinutuximab beta as continuous infusion over 10 days at a dose of 10 mg/m2/day for 5 cycles, following induction and consolidation therapy. Supportive therapy was administered to manage adverse events. Clinical outcomes and treatment tolerance were evaluated. Six of 7 patients treated with dinutuximab beta achieved complete remission, with a median duration of response of 21.5 months as of January 2022, and 1 displayed stable disease 21 months after treatment completion. Treatment was tolerable in most patients, with the majority of adverse events managed with supportive care. Dinutuximab beta is an effective immunotherapy for patients with high-risk neuroblastoma in routine clinical practice when coupled with optimal supportive management of adverse events.

Background This study aimed to examine fetomaternal outcomes in pregnant women in a large Turkish Takayasu arteritis (TAK) cohort and to evaluate the effects of pregnancy on the disease in those patients. Methods This is a cohort study involving 296 pregnancies of 112 TAK patients from 8 tertiary rheumatology centers in Turkey. Pregnancies were divided into 2 groups as pre-d (before disease onset) and post-d (after disease onset). In addition, post-d pregnancies were further divided into 2 subgroups according to fetomaternal complications (FMC) development status. Finally, patients were grouped into those with and without a history of pregnancy after disease onset. Results In post-d pregnancies, rates of worsening hypertension, new-onset hypertension, and preeclampsia were higher than in pre-d pregnancies (0.9% vs 16%, P < .001, 0.5% vs 5.3%, P = .012, and 0% vs 4%, P = .013, respectively). Patients with FMC were more likely to have renal artery involvement (65% vs 21%, P = .003). The patients who had post-d were younger, had longer disease duration, and had more relapses number than other patients (P < .001, P = .028, P = .016, respectively). Vasculitis Damage Index (VDI) results were similar in patients with or without post-d pregnancies. Conclusion Pregnancies after disease onset were found to be associated with HT and preeclampsia/eclampsia. HT-related FMCs are increased in TAK, and patients with renal artery involvement are at higher risk. The number of relapses increases in patients who become pregnant after disease onset, but pregnancy was not an independent risk factor for relapse. Pregnancy after the onset of disease had no negative effect on VDI.

Pacemakers, implantable cardiac defibrillators, and cardiac resynchronization therapy devices are potentially life-saving treatments for a number of cardiac conditions, but are not without risk. Most concerning is the risk of a cardiac implantable electronic device (CIED) infection, which is associated with significant morbidity, increased hospitalizations, reduced survival, and increased healthcare costs. Recommended preventive strategies such as administration of intravenous antibiotics before implantation are well recognized. Uncertainties have remained about the role of various preventive, diagnostic, and treatment measures such as skin antiseptics, pocket antibiotic solutions, anti-bacterial envelopes, prolonged antibiotics post-implantation, and others. Guidance on whether to use novel device alternatives expected to be less prone to infections and novel oral anticoagulants is also limited, as are definitions on minimum quality requirements for centres and operators and volumes. Moreover, an international consensus document on management of CIED infections is lacking. The recognition of these issues, the dissemination of results from important randomized trials focusing on prevention of CIED infections, and observed divergences in managing device-related infections as found in an European Heart Rhythm Association worldwide survey, provided a strong incentive for a 2019 International State-of-the-art Consensus document on risk assessment, prevention, diagnosis, and treatment of CIED infections. This article is simultaneously published also in Eur J Cardiothorac Surg. (https://doi.org/10.1093/ejcts/ezz296) and European Heart Journal (https://doi.org/10.1093/eurheartj/ehaa010). Minor differences in style may appear in each publication, but the article is substantially the same in each journal.

The European Society of Gastrointestinal Endoscopy (ESGE) together with the United European Gastroenterology (UEG) recently developed a short list of performance measures for small-bowel endoscopy (i.e. small-bowel capsule endoscopy and device-assisted enteroscopy) with the final goal of providing endoscopy services across Europe with a tool for quality improvement. Six key performance measures both for small-bowel capsule endoscopy and for device-assisted enteroscopy were selected for inclusion, with the intention being that practice at both a service and endoscopist level should be evaluated against them. Other performance measures were considered to be less relevant, based on an assessment of their overall importance, scientific acceptability, and feasibility. Unlike lower and upper gastrointestinal endoscopy, for which performance measures had already been identified, this is the first time small-bowel endoscopy quality measures have been proposed. ispartof: UNITED EUROPEAN GASTROENTEROLOGY JOURNAL vol:7 issue:5 pages:614-641 ispartof: location:England status: published

BACKGROUND: Orthotopic liver transplantation (OLT) has emerged as the mainstay of treatment for end-stage liver disease. However, technical aspects of OLT are still subject of ongoing debate and are widely based on personal experience and local institutional protocols. METHODS: An international online survey was sent out to all liver transplant centers (n = 52) within the Eurotransplant, Swisstransplant, Scandiatransplant, and British Transplant Society networks. The survey sought information on center-specific OLT caseload, vascular and biliary reconstruction, graft reperfusion, intraoperative control of hemodynamics, and drain policies. RESULTS: Forty-two centers gave a valid response (81%). Out of these, 50% reported piggy-back and 40.5% total caval replacement as their standard technique. While 48% of all centers generally do not apply veno-venous bypass (vvBP) or temporary portocaval shunt (PCS) during OLT, vvBP/PCS are routinely used in six centers (14%). Portal vein first reperfusion is used in 64%, followed by simultaneous (17%), and retrograde reperfusion (12%). End-to-end duct-to-duct anastomosis without biliary drain (67%) is the most frequently performed method of biliary reconstruction. No significant associations were found between the center caseload and the surgical approach used. The predominant part of the centers (88%) stated that techniques of OLT are not evidence-based and 98% would participate in multicenter clinical trials on these topics. CONCLUSION: Technical aspects of OLT vary widely among European centers. The extent to which center-specific variation of techniques affect transplant outcomes in Europe should be elucidated further in prospective multicenter trials.

Introduction To provide an overview of the experience of endovascular treatment for traumatic thoracic aortic lesions (TTAL). Thoracic aortic injury secondary to high-energy chest trauma constitutes an emergency situation with potentially devastating outcome. In the present time, the majority of patients are treated with thoracic endovascular aortic repair. The aim of this study was to provide a systematic overview of endovascular aortic repair in patients with TTAL with special attention to perioperative mortality, paraplegia, stroke, influence of left subclavian artery (LSA) coverage herein, and long-term stent-related complications. Evidence acquisition PubMed and Embase were searched for studies describing endovascular treatment of TTAL in the period between January 1st, 2000 and January 30th, 2016. The methodological quality of articles was assessed using the MINORS score and PRISMA guidelines. Data on early mortality, perioperative complications, and long-term stent-related complications were extracted. Evidence synthesis A total of 2005 reports were screened, and 74 publications were finally included in the analysis describing treatment of 1882 patients. Early mortality rate, paraplegia rate, and stroke rate were 7.5%, 0.4%, and 1.5%, respectively. LSA coverage did not increase the risk of stroke. The severity of injuries had a positive association on early mortality. Late stent related mortality and morbidity were uncommon. Conclusions TTAL can be treated endovascularly with low mortality and (neurological) morbidity. LSA coverage does not increase the risk of neurological complications but is associated with a moderate risk of left arm ischemia. Long-term stent-related complications are rare, but the available data is limited.

INTRODUCTION : To provide an overview of the experience of endovascular treatment for traumatic thoracic aortic lesions (TTAL). Thoracic aortic injury secondary to high-energy chest trauma constitutes an emergency situation with potentially devastating outcome. In the present time, the majority of patients are treated with thoracic endovascular aortic repair. The aim of this study was to provide a systematic overview of endovascular aortic repair in patients with TTAL with special attention to perioperative mortality, paraplegia, stroke, influence of left subclavian artery (LSA) coverage herein, and long-term stent-related complications.EVIDENCE ACQUISITION : PubMed and Embase were searched for studies describing endovascular treatment of TTAL in the period between January 1st, 2000 and January 30th, 2016. The methodological quality of articles was assessed using the MINORS score and PRISMA guidelines. Data on early mortality, perioperative complications, and long-term stent-related complications were extracted.EVIDENCE SYNTHESIS: A total of 2005 reports were screened, and 74 publications were finally included in the analysis describing treatment of 1882 patients. Early mortality rate, paraplegia rate, and stroke rate were 7.5%, 0.4%, and 1.5%, respectively. LSA coverage did not increase the risk of stroke. The severity of injuries had a positive association on early mortality. Late stent related mortality and morbidity were uncommon.CONCLUSIONS : TTAL can be treated endovascularly with low mortality and (neurological) morbidity. LSA coverage does not increase the risk of neurological complications but is associated with a moderate risk of left arm ischemia. Long-term stent-related complications are rare, but the available data is limited.

Background: Autologous stem cell transplantation (auto-SCT) is a widely used treatment option in multiple myeloma (MM) patients. The optimal graft cellular composition is not known. Study design and methods: Autograft cellular composition was analyzed after freezing by flow cytometry in 127 MM patients participating in a prospective multicenter study. The impact of graft cellular composition on hematologic recovery and outcome after auto-SCT was evaluated. Results: A higher graft CD34+ cell content predicted faster platelet recovery after auto-SCT in both the short and long term. In patients with standard-risk cytogenetics, a higher graft CD34+ count (>2.5 × 10/kg) was linked with shorter progression-free survival (PFS; 28 vs. 46 months, p = 0.04), but there was no difference in overall survival (OS) (p = 0.53). In a multivariate model, a higher graft CD34+CD133+CD38− (>0.065 × 10/kg, p = 0.009) and NK cell count (>2.5 × 10/kg, p = 0.026), lenalidomide maintenance and standard-risk cytogenetics predicted better PFS. In contrast, a higher CD34+ count (>2.5 × 10/kg, p = 0.015) predicted worse PFS. A very low CD3+ cell count (≤20 × 10/kg, p = 0.001) in the infused graft and high-risk cytogenetics remained predictive of worse OS. Conclusions: Autograft cellular composition may impact outcome in MM patients after auto-SCT. More studies are needed to define optimal graft composition. publishedVersion

Introduction The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the outcome of very low birth weight (VLBW) and low BW (LWB) infants with esophageal atresia (EA). Materials and Methods The data registered by 24 centers between 2014 and 2018 were evaluated for demographic features, prenatal findings, associated anomalies, surgical treatment, and outcome. Patients were enrolled in three groups according to their BWs (VLBW 2,500 g). Results Among the 389 cases, there were 37 patients (9.5%) in the VLBW group, 165 patients (42.4%) in the LBW group, and 187 patients (48.1%) in the NBW group. Prenatal diagnosis rates were similar among the three groups (29.7, 34.5, and 24.6%, respectively). The standard primary anastomosis was achieved at a significantly higher rate in NWB cases than in the other groups (p Conclusion The national data of TEAR demonstrates that the developmental and feeding parameters are better in NBW patients. Although VLBW patients have higher risk of developing fistula canalization than the LBW and NBW groups, long-term complications, such as anastomotic strictures, weight, and height values, after 1 year are similar in both groups. According to our results, associated anomalies and LBWs are still significant risk factors for mortality in cases with EA.

Lung transplantation may be a final destination therapy in lung failure, but limited donor organ availability creates a need for alternative management, including artificial lung technology. This invited review discusses ongoing developments and future research pathways for respiratory assist devices and tissue engineering to treat advanced and refractory lung disease. An overview is also given on the aftermath of the coronavirus disease 2019 pandemic and lessons learned as the world comes out of this situation. The first order of business in the future of lung support is solving the problems with existing mechanical devices. Interestingly, challenges identified during the early days of development persist today. These challenges include device-related infection, bleeding, thrombosis, cost, and patient quality of life. The main approaches of the future directions are to repair, restore, replace, or regenerate the lungs. Engineering improvements to hollow fiber membrane gas exchangers are enabling longer term wearable systems and can be used to bridge lung failure patients to transplantation. Progress in the development of microchannel-based devices has provided the concept of biomimetic devices that may even enable intracorporeal implantation. Tissue engineering and cell-based technologies have provided the concept of bioartificial lungs with properties similar to the native organ. Recent progress in artificial lung technologies includes continued advances in both engineering and biology. The final goal is to achieve a truly implantable and durable artificial lung that is applicable to destination therapy.

Background. We report procedural and early results in the Netherlands of the Relay Branch device (Terumo Aortic, Sunrise, FL) for total endovascular repair of the aortic arch.Methods. Between 2014 and 2018, all consecutive patients who received the Aortic Relay double-branched stent graft in the Netherlands were included in a multicenter, retrospective registry.Results. The Relay Branch device was used in 11 patients to treat saccular (n = 4), fusiform (n = 5), or false aneurysms (n = 2) in the aortic arch. Patients were deemed unfit or extreme high-risk for open (redo) surgery. The brachiocephalic trunk and left common carotid artery were branched using a retrograde approach in all cases. Additional surgical left subclavian artery revascularization was performed in 8 patients. The main device and the branches were successfully introduced, positioned, and deployed with complete exclusion of the aortic pathology in all patients (100% technical success). There was no retrograde type A dissection or conversion to open surgery. Two procedure-related deaths occurred, both caused by perioperative or postoperative strokes. There were 2 minor strokes with full recovery. One patient recovered from transient paraplegia after spinal fluid drainage. No permanent paraplegia was observed. Follow-up imaging showed persistent adequate exclusion of aortic arch pathology. Mean follow-up was 17 months (range, 3-42 months).Conclusions. Total endovascular aortic arch repair using the Relay Branch device is technically feasible and effective in excluding aortic arch pathology. The observed stroke rate in the initial experience, however, was considerable. Although appealing, this new less-invasive technique should be carefully introduced and its progress thoroughly evaluated. (C) 2020 by The Society of Thoracic Surgeons

Background: Short-term survival after orthotopic liver transplantation (OLT) has improved over the past decades, but long-term survival remains impaired. The effects of obesity on long-term survival after OLT are controversial. Because pre-transplant body mass index (BMI) can be confounded by ascites, we hypothesized that post-transplant BMI at 1 year could predict long-term survival. Methods: A post-hoc analysis was performed of an observational cohort study consisting of adult recipients of a first OLT between 1993 and 2010. Baseline BMI was measured at 1-year post-transplantation to represent a stable condition. Recipients were stratified into normal weight (BMI < 25 kg/m2), overweight (25 ≤ BMI ≤ 30 kg/m2), and obese (BMI > 30 kg/m2). Kaplan-Meier survival analyses were performed with log-rank testing, followed by multivariable Cox proportional hazards regression analysis. Results: Out of 370 included recipients, 184 had normal weight, 136 were overweight, and 50 were obese at 1-year post-transplantation. After median follow-up for 12.3 years, 107 recipients had died, of whom 46 (25%) had normal weight, 39 (29%) were overweight, and 22 (44%) were obese (log-rank P = 0.020). Obese recipients had a significantly increased mortality risk compared to normal weight recipients (HR 2.00, 95% CI 1.08–3.68, P = 0.027). BMI was inversely associated with 15 years patient survival (HR 1.08, 95% CI 1.03–1.14, P = 0.001 per kg/m2), independent of age, gender, muscle mass, transplant characteristics, cardiovascular risk factors, kidney- and liver function. Conclusion: Obesity at 1-year post-transplantation conveys a 2-fold increased mortality risk, which may offer potential for interventional strategies (i.e. dietary advice, lifestyle modification, or bariatric surgery) to improve long-term survival after OLT.

Introduction Paediatric traumatic brain injury (pTBI) is one of the most frequent neurological presentations encountered in emergency departments worldwide. Every year, more than 200,000 American children suffer pTBIs, many of which lead to long-term damage. Objectives We aim to review the existing evidence on the efficacy of the decompressive craniectomy (DC) in controlling intracranial pressure (ICP) and improving long-term outcomes in children with pTBI. Methods A comprehensive search of the MEDLINE and EMBASE databases led to the screening of 212 studies, 12 of which satisfied inclusion criteria. Data extracted included the number and ages of patients, Glasgow Coma Scale scores at presentation, treatment protocols and short- and long-term outcomes. Results Each of the nine studies including ICP as an outcome reported that it was successfully controlled by DC. The 6–12 month outcome scores of patients undergoing DC were positive, or superior to those of medically treated groups in nine of 11 studies. Mortality was compared in only two studies, and was lower in the DC group in both.Very few studies are currently available investigating short- and long-term outcomes in children with TBI undergoing DC. Conclusion The currently available evidence may support a beneficial role of DC in controlling ICP and improving long-term outcomes.

Intestinal failure (IF) is a condition characterized by the inability to maintain a state of adequate nutrition, or fluid and electrolyte balance due to an anatomical or a physiological disorder of the gastrointestinal system. IF can be an extremely debilitating condition, significantly affecting the quality of life of those affected. The surgical management of patients with acute and chronic IF requires a specialist team who has the expertise in terms of technical challenges and decision-making. A dedicated IF unit will have the expertise in patient selection for surgery, investigative workup and planning, operative risk assessment with relevant anesthetic expertise, and a multidisciplinary team with support such as nutritional expertise and interventional radiology. This article covers the details of IF management, including the classification of IF, etiology, prevention of IF, and initial management of IF, focusing on sepsis treatment and nutritional support. It also covers the surgical aspects of IF such as intestinal reconstruction, abdominal wall reconstruction, and intestinal transplantation.

Adenosine deaminase (ADA) deficiency is an autosomal recessive primary immunodeficiency. It results in the intracellular accumulation of toxic metabolites which have effects particularly on lymphocytes and the brain. The aim of this study was to evaluate the outcome of 13 ADA-deficient patients. We planned to evaluate their clinical and laboratory findings before and after enzyme replacement therapy (ERT), allogeneic hematopoietic stem cell transplantation (aHSCT), and hematopoietic stem cell gene therapy (HSCGT). Measurement of ADA enzyme activity and metabolites and sequencing of the ADA gene were performed in most of the patients with ADA deficiency. One of the patients with late-onset ADA deficiency was diagnosed by the help of primary immunodeficiency panel screening. Ten out of 13 patients were diagnosed as SCID, while 3 out of 13 were diagnosed as delayed-/late-onset ADA deficiency. Late-onset ADA deficiency patients had clinical and laboratory findings of combined immunodeficiency (CID). Eight patients with ADA-SCID were found to have higher levels of ADA metabolite (dAXP%) (62.1% (34.6-71.9)) than 3 patients with delayed-/late-onset ADA deficiency (6.9% (2.1-8.9). All but one patient with SCID had T-B-NK- phenotype, one had T-B-NK+ phenotype. Genetic defect was documented in 11 patients. Four out of 11 patients had compound heterozygous defects. Three out of 4 patients with compound heterozygous defects had delayed-onset/late-onset ADA deficiency. Seven out of 11 patients with SCID had homozygous defects. Five out of 7 had the same homozygous indel frameshift mutation (c.955-959delGAAGA) showing a founder effect. There were two novel splice site defects: one (IVS10+2T > C) was heterozygous in a patient with late-onset ADA deficiency, and the other was homozygous (IVS2delT+2) in a SCID patient. Other defects were missense defects. Nine out of 13 patients were put on pegylated ADA ERT. Four out of six patients were transplanted without using a conditioning regimen. HSCGT was performed to one of the patients. The genetic diagnosis of SCID is utmost important. There is a chance to give ERT before the definitive therapy if the patient with SCID/CID has ADA deficiency. Although ERT was insufficient to restore a normal immune function in ADA-SCID patients, it was useful to improve and stabilize the clinical status before curative therapy (aHSCT/HSCGT). Enzyme replacement therapy was successful in patients with late-/delayed-onset ADA deficiency who presented with the features of combined immunodeficiency. Gastrointestinal polyposis in a patient with late-onset ADA deficiency may be an association or a coincidental finding. Intermittent neurodevelopmental evaluation especially for hearing impairment should be performed in most of the ADA-deficient patients. This may alleviate the speech delay and cognitive abnormalities which may be observed in the follow-up.

AIM To collect data from joint replacement in inhibitor patients, evaluate haemostatic and patient outcomes, and analyse the costs. METHODS We report our 21-year, single-centre cumulative experience of 15 joint arthroplasties in six inhibitor patients. RESULTS Two low responder inhibitor patients were in the early days treated with FVIII, whereas bypassing agents were used in the rest of the high responder patients. The primary haemostatic outcome was good in 8/15, fair in 4/15 and poor in 3/15 operations. The overall patient outcome, including joint health and patient satisfaction, was good in 10/15, fair 4/15 and poor in 1/15. No deep infections were observed. Cost analysis was most beneficial in low responders and in two immune-tolerized, high responder patients. In all cases, factor replacement comprised the main treatment costs. CONCLUSION Our experience supports the initial use of bypassing agents as well as preoperative immune-tolerance induction when possible. Despite the challenges of haemostasis and severe joint disease, total joint arthroplasty can reach a good outcome, even in inhibitor patients. The risk for deep infection might be smaller than previously reported. Individual planning, intense multidisciplinary teamwork and execution of operations should be centralised in a professional unit.

BACKGROUND: Patients with indolent systemic mastocytosis (ISM) are at risk for severe anaphylactic reactions to yellow jacket (YJ) stings while demonstration of sensitization can be challenging because specific IgE (sIgE) levels are regularly below 0.35 kU(A)/L. The implication of missing YJ allergy is illustrated by a case of fatal anaphylaxis.OBJECTIVE: To explore the natural course of YJ venom allergy and the diagnostic accuracy and therapeutic consequence of YJ venom sIgE in patients with ISM.METHODS: All patients with ISM seen from 1981 to 2015 (n = 243) were evaluated on the number of YJ stings, reaction severity, and sensitivity and specificity of YJ venom sIgE. YJ venom allergic patients without mastocytosis served as control (n = 313).RESULTS: A total of 153 patients with ISM were stung during adult life. The first systemic reaction was more often severe in patients with ISM than in patients without mastocytosis (69.9% vs 22.0%) and reactions recurred in 40 of 41 re-stung patients with ISM. ISM reactors showed lower YJ venom sIgE levels than nonmastocytosis reactors (0.61 vs 4.83 kU(A)/L; P CONCLUSIONS: The high rate of severe reactions and the fatal case underscore the importance of adequate diagnostic sensitivity of sIgE in patients with ISM. The sensitivity of sIgE can be ameliorated by lowering the threshold to 0.17 kU(A)/L, retaining good specificity. We recommend sIgE screening in all patients with ISM and discussing immunotherapy when YJ venom sIgE exceeds 0.17 kU(A)/L.

Background: Hepatocellular adenoma (HCA) larger than 5cm in diameter is considered an indication for elective surgery, because of the risk of haemorrhage and malignant transformation. Transarterial embolization (TAE) is used to manage bleeding HCA and occasionally to reduce tumour size. TAE might have potential as an elective therapy, but its current role in this context is uncertain. This systematic review provides an overview of clinical outcomes after TAE, in bleeding and non-bleeding HCA.Methods: Two independent reviewers performed a systematic search of literature in PubMed and Embase. Outcomes were change in tumour size, avoidance of surgery, complications and malignant transformation after TAE in bleeding and non-bleeding HCA. The Critical Appraisal Skills Programme tool for cohort studies was used for quality assessment of included studies.Results: From 320 potential articles, 20 cohort studies and 20 case reports including 851 patients met the inclusion criteria. TAE was performed in 151 of 851 patients (17.7 per cent), involving 196 tumours, of which 95 (48.5 per cent) were non-bleeding. Surgical treatment was avoided in 68 of 151 patients (45.0 per cent). Elective TAE was performed in 49 patients involving 66 HCAs, with 41 of these patients (84 per cent) not requiring surgery. Major complications occurred in eight of 151 patients (5.3 per cent); no death was reported. Among cohort studies, complete tumour disappearance was observed in 10 per cent of patients, and regression in 75 per cent.Conclusion: Acute or elective TAE in the management of HCA is safe. In the elective setting, TAE provides a potential alternative to surgery.

To evaluate long-term outcome of GORE EXCLUDER AAA Endoprosthesis (W.L. GoreAssociates, Inc, Flagstaff, Arizona) for elective treatment of infrarenal aortic aneurysms and to evaluate performance of different generations of the device.A retrospective analysis was performed of 248 patients undergoing elective endovascular aneurysm repair with the GORE EXCLUDER between January 2000 and December 2015 in 2 hospitals. Primary endpoint was reintervention-free survival. Secondary endpoints were technical success, overall survival, rupture-free survival, endoleaks, sac diameter change (5 mm), limb occlusion, and migration (5 mm). Median follow-up time was 26 months (range, 1-190 months).Assisted primary technical success was 96.8%. Reintervention-free survival for 5 and 10 years was 85.2% and 75.6%, respectively. Independent risk factors for reintervention were technical success (P.001), type I endoleak (P.001), and type II endoleak (P = .003). Late adverse events requiring reintervention included rupture (0.4%), limb occlusion (0.4%), and stent migration (0.4%). Type Ia (4.8%), Ib (2.8%), II (35.9%), and V (6.5%) endoleaks were reported throughout follow-up. Sac growth was more prevalent with the original GORE EXCLUDER compared with the low permeability GORE EXCLUDER (P = .001) and in the presence of type I, II, and V endoleaks (P.05). Three conversions (1.2%) were performed. Overall survival at 5 and 10 years was 68.4% and 49.0%, with no reported aneurysm-related deaths.Treatment with the GORE EXCLUDER is effective with acceptable reintervention rates in the long-term and few device-related adverse events or ruptures up to 10 years. Observed late adverse events and new-onset endoleaks emphasize the need for long-term surveillance.

// Jessica A. Howell 1, 2, 3, * , David J. Pinato 1, * , Ramya Ramaswami 1 , Tadaaki Arizumi 4 , Carlotta Ferrari 5 , Antonello Gibbin 5 , Michela E. Burlone 5 , Giulia Guaschino 5 , Pierluigi Toniutto 6 , James Black 1 , Laura Sellers 1 , Masatoshi Kudo 4 , Mario Pirisi 5 and Rohini Sharma 1 1 Department of Surgery and Cancer, Imperial College London, Hammersmith Hospital, London, UK 2 Centre for Population Health, Burnet Institute, Melbourne, Australia 3 Department of Medicine, University of Melbourne, Melbourne, Australia 4 Department of Gastroenterology and Hepatology, Kinki University School of Medicine, Osaka-Sayama, Osaka, Japan 5 Department of Translational Medicine, Universita degli Studi del Piemonte Orientale “A. Avogadro”, Novara, Italy 6 Department of Experimental and Clinical Medicine, University of Udine, Udine, Italy * These authors have contributed equally to the work Correspondence to: Jessica A. Howell, email: jessica.howell@svha.org.au Keywords: liver cancer, VEGF inhibitor, CLIP score, BCLC, inflammation Received: March 30, 2016 Accepted: October 13, 2016 Published: February 14, 2017 ABSTRACT Background and Aims: Response to sorafenib is highly variable in hepatocellular carcinoma (HCC). Baseline inflammatory parameters and treatment toxicities may improve survival prediction in patients on sorafenib therapy. Results: 442 patients with advanced stage HCC on sorafenib were recruited (follow-up 5096 person-months at risk). 88% had BCLC stage B or greater HCC and 72.3% had Child-Pugh A cirrhosis. On Cox multivariate regression, previously-treated HCC (HR 0.579, 95% CI 0.385-0.872, p=0.009), Cancer of Liver Italian Program (CLIP) score (HR 1.723, 95% CI 1.462-2.047, p

This clinical report details the results of endovascular treatment of symptomatic superior vena cava syndrome due to central vein stenosis or obstruction (CVSO) by stent angioplasty in patients with dialysis-dependent end-stage renal disease. A 3-year retrospective review of two institutional registries identified 25 chronic hemodialysis patients (17 men, 8 women) affected by CVSO who received endovascular treatment. The majority of the patients (n = 19) presented with symptomatic arm, breast, and facial swelling; and 6 patients presented with dialysis-access dysfunction and venous-line hypertension. The etiology of CVSO was before central venous catheter in all but 2 patients. Venography showed 19 cases of stenosis (4 stenoses of superior vena cava, 3 brachiocephalic, 10 subclavian, and 2 axillary veins) and 6 occlusions of the superior vena cava. After percutaneous transluminal angioplasty and primary stent angioplasty, there was an immediate regression of symptoms and arteriovenous fistula preservation in 21 cases; 4 patients received a new arteriovenous fistula after interventional treatment. No procedural major complications or patient deaths occurred. During the follow-up period, we recorded a primary patency rate of 95%, 80%, and 70%, respectively, at 6, 12, and 18 months; and a secondary patency rate of 100%, 95%, and 90%, respectively, at 6, 12, and 18 months. In conclusion, endovascular treatment with primary stenting has proven to be a durable treatment option for hemodialysis patients with CVSO, and this treatment should be considered before dialysis access placement in patients with catheter-induced central vein obstruction.

In 2014 the International Endohernia Society (IEHS) published the first international "Guidelines for laparoscopic treatment of ventral and incisional abdominal wall hernias". Guidelines reflect the currently best available evidence in diagnostics and therapy and give recommendations to help surgeons to standardize their techniques and to improve their results. However, science is a dynamic field which is continuously developing. Therefore, guidelines require regular updates to keep pace with the evolving literature.METHODS: For the development of the original guidelines all relevant literature published up to year 2012 was analyzed using the ranking of the Oxford Centre for Evidence-Based-Medicine. For the present update all of the previous authors were asked to evaluate the literature published during the recent years from 2012 to 2017 and revise their statements and recommendations given in the initial guidelines accordingly. In two Consensus Conferences (October 2017 Beijing, March 2018 Cologne) the updates were presented, discussed, and confirmed. To avoid redundancy, only new statements or recommendations are included in this paper. Therefore, for full understanding both of the guidelines, the original and the current, must be read. In addition, the new developments in repair of abdominal wall hernias like surgical techniques within the abdominal wall, release operations (transversus muscle release, component separation), Botox application, and robot-assisted repair methods were included.RESULTS: Due to an increase of the number of patients and further development of surgical techniques, repair of primary and secondary abdominal wall hernias attracts increasing interests of many surgeons. Whereas up to three decades ago hernia-related publications did not exceed 20 per year, currently this number is about 10-fold higher. Recent years are characterized by the advent of new techniques-minimal invasive techniques using robotics and laparoscopy, totally extraperitoneal repairs, novel myofascial release techniques for optimal closure of large defects, and Botox for relaxing the abdominal wall. Furthermore, a concomitant rectus diastasis was recognized as a significant risk factor for recurrence. Despite still insufficient evidence with respect to these new techniques it seemed to us necessary to include them in the update to stimulate surgeons to do research in these fields.CONCLUSION: Guidelines are recommendations based on best available evidence intended to help the surgeon to improve the quality of his daily work. However, science is a continuously evolving process, and as such guidelines should be updated about every 3 years. For a comprehensive reference, however, it is suggested to read both the initially guidelines published in 2014 together with the update. Moreover, the presented update includes also techniques which were not known 3 years before.

Cerebrovascular disease involves various medical disorders that obstruct brain blood vessels or deteriorate cerebral circulation, resulting in ischemic or hemorrhagic stroke. Nowadays, platinum coils with or without biological modification have become routine embolization devices to reduce the risk of cerebral aneurysm bleeding. Additionally, many intracranial stents, flow diverters, and stent retrievers have been invented with uniquely designed structures. To accelerate the translation of these devices into clinical usage, an in‐depth understanding of the mechanical and material performance of these metal‐based devices is critical. However, considering the more distal location and tortuous anatomic characteristics of cerebral arteries, present devices still risk failing to arrive at target lesions. Consequently, more flexible endovascular devices and novel designs are under urgent demand to overcome the deficiencies of existing devices. Herein, the pros and cons of the current structural designs are discussed when these devices are applied to the treatment of diseases ranging broadly from hemorrhages to ischemic strokes, in order to encourage further development of such kind of devices and investigation of their use in the clinic. Moreover, novel biodegradable materials and drug elution techniques, and the design, safety, and efficacy of personalized devices for further clinical applications in cerebral vasculature are discussed.

Background: This study aims to evaluate the feasibility and safety of resection of sarcoma liver metastases, and to identify possible prognostic factors for long-term survival. Methods: All patients who underwent resection of liver metastases of sarcoma in the Netherlands from 1998 to 2014 were included. Study data was retrospectively collected from patient files. Survival rates were calculated using Kaplan-Meier survival analysis. Results: Some 38 patients treated in 16 hospitals were included (15 male, 23 female). The median age was 57 years (37–80 years). The most common histological subtype was leiomyosarcoma (63%). The predominant site of primary tumour was the abdomen (59%). R0 resection was achieved in 16 patients. Mortality was 3 and 16% of included patients had 1 or more complications. The median follow-up period was 18 months (range 1–161). After liver resection, 1-, 3-, and 5-year survival were 88, 54, and 42% respectively. Median overall survival was 46 months (1–161 months). One- and three-year progression-free survival (PFS) after liver resection were 54 and 19% respectively. Median PFS was 16 months (1–61 months). Conclusions: Liver surgery for sarcoma metastases is safe and leads to a relatively good survival. The choice for surgical treatment should always be discussed in a multidisciplinary sarcoma and liver team.

While small cell neuroendocrine carcinomas (SCNCs) most often arise in the lung, extrapulmonary SCNCs arise in a variety of locations-including the head and neck region. In particular, laryngeal SCNCs-while rare tumors-are nevertheless recognized as distinct lesions. The rarity of laryngeal SCNC gives rise to two distinct difficulties: first (particularly with small biopsy specimens), laryngeal SCNC can be difficult to diagnose by routine light microscopy; second, limited experience with these tumors can make the crafting of a treatment plan for individual patients difficult. As regards the first problem, pathologic diagnosis is greatly enhanced by the application of immunohistochemistry. The second problem-crafting a successful treatment strategy-presents a much larger difficulty. It is tempting to extrapolate from experience with the (more common) pulmonary SCNC in search of a strategy applicable to laryngeal SCNC; such an extrapolation, however, may not be uniformly successful. In particular, while a combination of radiation therapy and chemotherapy appears to be as valuable in the treatment of extrapulmonary as it is in the treatment of pulmonary SCNC, prophylactic cranial irradiation (PCI)-which has enjoyed some success in the treatment of some patients with pulmonary SCNC-does not appear to have similar utility in patients with laryngeal SCNC. Accordingly, the use of PCI does not appear to have a role to play at this point in time in the treatment of patients with laryngeal SCNC.

In an era where there is a shortage of lungs for transplantation is increased utilization of lungs from donation after circulatory death (DCD) donors. We review the reports of 11 controlled and 1 uncontrolled DCD programs focusing on donor criteria, procedural criteria, graft assessment, and preservation techniques including the use of ex vivo lung perfusion. We have formulated conclusions and recommendations for each of these areas, which were presented at the 6th International Conference on Organ Donation. A table of recommendations, the grade of recommendations, and references are provided.

Background— Chronic thromboembolic pulmonary hypertension results from incomplete resolution of pulmonary emboli. Pulmonary endarterectomy (PEA) is potentially curative, but residual pulmonary hypertension following surgery is common and its impact on long-term outcome is poorly understood. We wanted to identify factors correlated with poor long-term outcome after surgery and specifically define clinically relevant residual pulmonary hypertension post-PEA. Methods and Results— Eight hundred eighty consecutive patients (mean age, 57 years) underwent PEA for chronic thromboembolic pulmonary hypertension. Patients routinely underwent detailed reassessment with right heart catheterization and noninvasive testing at 3 to 6 months and annually thereafter with discharge if they were clinically stable at 3 to 5 years and did not require pulmonary vasodilator therapy. Cox regressions were used for survival (time-to-event) analyses. Overall survival was 86%, 84%, 79%, and 72% at 1, 3, 5, and 10 years for the whole cohort and 91% and 90% at 1 and 3 years for the recent half of the cohort. The majority of patient deaths after the perioperative period were not attributable to right ventricular failure (chronic thromboembolic pulmonary hypertension). At reassessment, a mean pulmonary artery pressure of ≥30 mm Hg correlated with the initiation of pulmonary vasodilator therapy post-PEA. A mean pulmonary artery pressure of ≥38 mm Hg and pulmonary vascular resistance ≥425 dynes·s −1 ·cm −5 at reassessment correlated with worse long-term survival. Conclusions— Our data confirm excellent long-term survival and maintenance of good functional status post-PEA. Hemodynamic assessment 3 to 6 months and 12 months post-PEA allows stratification of patients at higher risk of dying of chronic thromboembolic pulmonary hypertension and identifies a level of residual pulmonary hypertension that may guide the long-term management of patients postsurgery.

In an era where there is a shortage of lungs for transplantation is increased utilization of lungs from donation after circulatory death (DCD) donors. We review the reports of 11 controlled and 1 uncontrolled DCD programs focusing on donor criteria, procedural criteria, graft assessment, and preservation techniques including the use of ex vivo lung perfusion. We have formulated conclusions and recommendations for each of these areas, which were presented at the 6th International Conference on Organ Donation. A table of recommendations, the grade of recommendations, and references are provided. ispartof: Transplant International vol:29 issue:7 pages:790-797 ispartof: location:Switzerland status: published

To review outcomes following microwave ablation (MWA) of colorectal cancer pulmonary metastases and assess predictors of oncologic outcomes.Technical success, primary and secondary technique efficacy rates were evaluated for 50 patients with 90 colorectal cancer pulmonary metastases at immediate, 4-8 weeks post-MWA and subsequent follow-up CT and/or F-18-FDG PET/CT. Local tumor progression (LTP) rate, LTP-free survival (LTPFS), cancer-specific and overall survivals were assessed. Complications were recorded according to SIR classification.Median follow-up was 25.6 months. Median tumor size was 1 cm (0.3-3.2 cm). Technical success, primary and secondary technique efficacy rates were 99, 90 and 92%, respectively. LTP rate was 10%. One-, 2- and 3-year LTPFS were: 93, 86 and 86%, respectively, with median LTPFS not reached. Median overall survival was 58.6 months, and median cancer-specific survival (CSS) was not reached. One-, 2- and 3-year overall and CSS were 94% and 98, 82 and 90%, 61 and 70%, respectively. On univariate analysis, minimal ablation margin (p = 5 mm and/or tumor size 10 ng/ml (p = 0.046) and >= 3 prior chemotherapy lines predicted decreased CSS (p = 0.02). There was no 90-day death. Major complications rate was 13%.MWA with minimal ablation margin >= 5 mm is essential for local control of colorectal cancer pulmonary metastases. Pleural-based metastases and larger tumor size were associated with higher risk of LTP. CEA level and pre-MWA chemotherapy impacted CSS.