Giant Renal Tumor with Inferior Vena Cava Thrombus – a Case Report

INTRODUCTION: Vascular migration and venous tumor thrombus are infrequent but unique aspects of renal cell carcinoma, and these features have signifcant therapeutic and prognostic implications. We report a case of renal neoplasm with a vena cava tumor thrombus treated with surgical resection and adjuvant chemotherapy. CASE REPORT: A 53-year-old, otherwise healthy woman presented to the emergency department due to macroscopic hematuria and abdominal pain. A large abdominal mass was noted. A computed tomography angiography was performed, and a right renal tumor (105x207mm) with level II inferior vena cava tumor thrombus and local adenopathy was noted. After a multidisciplinary discussion, she was proposed for surgical resection. Through a bilateral subcostal incision (Chevron), a standard right radical nephrectomy and perivascular lymph node excision were performed. The IVC was exposed, and a thrombectomy was performed through a longitudinal cavotomy. Pathology revealed clear renal cell carcinoma and lymph node metastasis. The postoperative period was uneventful. However, the patient developed multiple liver and lung metastases at early follow-up and was treated with chemotherapy. DISCUSSION: Tumor thrombus can extend up to the right atrium and occurs in nearly 10–25% of renal cell carcinoma patients. The natural history of this condition is poor, with a median survival of 5 months and signifcant survival improvements following radical nephrectomy and IVC tumor thrombus removal are observed, with 40- 60% 5-year survival. Surgical treatment should, therefore, be considered in this group of patients. Such operations can be challenging, particularly when thrombus extent is signifcant, and the combination of efforts between oncologists, urologists, and vascular surgeons can improve patient safety and perioperative outcomes with signifcant improvements in overall prognosis

Diagnosis and Predictors of Post-Implantation Syndrome Following Endovascular Repair of Aortic Aneurysms – a Narrative Review

INTRODUCTION: After endovascular aortic repair (EVAR), many patients develop a systemic inflammatory response called post-implantation syndrome (PIS). AAA and procedure-related characteristics have been linked with increased odds of developing this syndrome. Similarly, some short- and long-term consequences have been associated with PIS. This study aims to review the literature on the diagnosis and predictors of post-implantation after endovascular repair of aortic aneurysms. RESULTS: A non-systematic review of the MEDLINE and Scopus databases was performed using the keywords "abdominal aortic aneurysm," "inflammation," and "endovascular techniques.” No time or language limitations were imposed. Manuscripts were considered irrespective of study design. Articles of interest were analyzed, and the relevant information was organized in tables. RESULTS: PIS is defined as a combination of constitutional symptoms, including fatigue and fever, and elevated inflammatory markers. There are several proposed diagnostic criteria, most including a combination of fever with leukocytosis and/or elevated C-reactive protein (CRP). These result in discrepant rates, as low as 2% and up to 100%. The typical evolution of this syndrome is spontaneous resolution, although pharmacologic measures for symptom relief may be needed. These symptoms often resolve within two weeks; no significant permanent complications remain. Most PIS cases will present up to the first 72 postoperative hours. Endograft material, particularly polyester-based stent grafts, has been consistently linked to increased odds of PIS, up to five-fold, compared to polytetrafluoroethylene (PTFE) grafts. Aneurysm thrombus load (both pre-existing and new-onset) has also been related to an increased odds of PIS. Bacterial translocation, contrast media, and other patient or procedure-related characteristics have not been linked to an increased risk of PIS. CONCLUSION: PIS is a common finding after EVAR. Universal diagnostic criteria for diagnosis are required. Polyester-based stent grafts present the highest risk of developing this syndrome. Aneurysm thrombus load may also relate to this increased risk. The impact of other clinical or anatomical factors remains undetermined

Mycotic Aneurysm in an Immunocompromised Patient with Pneumonia and Spondylodiscitis: Who’s Guilty?

BACKGROUND: Mycotic aneurysm is a rare entity with rapid progression, which can be fatal without adequate treatment. The incidence of rupture is greater than that of degenerative aneurysms and is associated with a high mortality rate. CASE REPORT: We report the case of a 58-year-old man with a known history of HIV infection with good immunovirological staging, treated squamous cell carcinoma of the anal canal and chronic gastritis, who presented with a six-day history of intense back pain, malaise, fever, and chills. After examination, he was hospitalized with a clinical suspicion of acute pyelonephritis. During hospitalization, he was diagnosed with pneumonia of the right pulmonary base, infectious spondylodiscitis, and mycotic aneurysm of the abdominal aorta, which involved the visceral plaque. The microbiological workup revealed only positive blood cultures for Klebsiella pneumoniae. After a multidisciplinary discussion of the case and six weeks of antibiogram-oriented antibiotic therapy, the patient underwent an aorto-aortic interposition via left thoracophrenolaparotomy without the need to reimplant visceral vessels due to the patch confguration of the proximal anastomosis. The procedure was performed under left heart bypass. The postoperative course was uneventful, and the patient was discharged four weeks later. At 18 months follow-up, she remained asymptomatic and free of recurrence. CONCLUSION: In this case, it remains to be defned whether the cause of the mycotic aneurysm was hematogenous dissemination from the identifed pneumonia or contiguity from the diagnosed spondylodiscitis. Given the morbidity and mortality associated with this entity, early diagnosis and adequate treatment with surgical correction and antibiotic therapy with suffcient duration and dose are important aspects for improving survival in these cases

COVID- 19 in Patients Affected by Red Blood Cell Disorders, Results From the European Registry ERN-EuroBloodNet.

Background: Despite several publications covering patients from multiple centers, no international registry covered all patients with red blood cell diseases (RBCD) affected by COVID- 19. The ERN-EuroBloodNet's registry provided real-time registration of SARS-CoV- 2 patients with RBCD, promoting timely disease-specific knowledge sharing during the pandemic's early stages. Procedures: The study evaluated patient distribution, the infection across different RBCDs, and severity risk factors across similar healthcare systems, using data collected from the ERN-EuroBloodNet's REDCap platform. Results: From April 2020 to April 2023, 681 infections were recorded among 663 patients, of which 373 had transfusion-dependent thalassemia or non-transfusion-dependent thalassemia (TDT/NTDT), and 269 had sickle cell disease (SCD). SCD patients had a higher incidence of COVID- 19 than those with TDT/NTDT (10.5 vs. 4.8 COVID/100 patients). Notably, 92% of the cases were mild, with neither age nor the specific RBCD affecting severity. The number of comorbidities, notably obesity and hypertension, that patients had prior to infection was associated with more severe COVID- 19. During the infection, the presence of vaso-occlusive crises, acute chest syndrome, kidney failure, and ground-glass opacities on chest tomography scans were associated with a more severe clinical picture. The vaccination rate (32%) mirrored that of the general population and showed a protective effect against severe COVID- 19. The observed mortality rate was 0.7%, aligning with Europe's general population. Conclusion: SARS-CoV- 2 infection in SCD and TDT/NTDT patients is mild and without higher mortality than the general population. The ERN-Eurobloodnet's registry collaborative structure exemplifies the power of international cooperation in tackling rare diseases, especially during health emergencies

A Giant Arteriovenous Malformation of the Abdominal Wall

INTRODUCTION: Arteriovenous Malformations (AVMs) are high-fow anomalous connections between the arterial and venous systems composed of dysplastic vessels resulting from aberrant angiogenesis. They are congenital and when symptomatic they rarely manifest before adolescence. Depending on the location, size, stage and severity of the symptoms, treatment options vary from conservative management to surgical resection. We report a case of a giant arteriovenous malformation of abdominal wall (tipe IIIb of Yakes Classifcation) treated with surgical resection after prior attempts of scleroembolization.. CLINICAL CASE: 54-year-old woman with known history of osteoarticular pathology and dyspepsia presented a mass on the left side of the abdominal wall with hard consistency, warm, slightly pulsating and tenderness to touch with several years of evolution. The mass showed infltration of the internal and external oblique muscles sparing the transverse muscle. Clinically she presented easy fatigue with efforts. Due to the risk of abdominal wall herniation after excision of the AVM, scleroembolization was considered frst-line treatment in this case. This strategy resulted in regression of the mass and symptoms improvement. Four years after the last intervention, the patient presented lesion growth, recurrence and worsening of symptoms with severe interference in the quality of life (QoL). After multidisciplinary discussion, she was proposed for complete resection of the AVM. She was frst submitted to scleroembolization with Onyx of identifed arterial afferents and sclerosis of the lesion nidus with 2% polidocanol. One month after she underwent successfully total resection of the AVM with the collaboration of General Surgery. CONCLUSION: No unifed agreement exists on the best treatment of these complex high fow lesions and it is diffcult to establish a comprehensive strategy given the pathology’s clinical variability, complex stratifcation and the risk of relapse. A case-by-case approach is needed in managing these types of lesions

A Reconciliação da Medicação nos Cuidados de Saúde Primários: Práticas, Conhecimentos e Atitudes na Região de Saúde de Lisboa e Vale do Tejo

Despite the importance of medication reconciliation for the continuity of care, there is currently no information on the practices, knowledge, and attitudes of Portuguese family doctors on this subject. This study aimed to characterize the formal medication reconciliation procedures in the Lisbon and Tagus Valley Health Region, as well as the perception of family doctors in this region about what they know, how they think and how they practice medication reconciliation.A total of 89 primary healthcare units and 208 family doctors participated in the study (31% and 12% response rates, respectively). Only one in four units (n = 22/89) had a formal medication reconciliation procedure. Among the units with a formal procedure, there was variability in some parameters, although all procedures included physicians. More than 70% (n = 150; 72.1%) of family physicians reported having previous contact with the term 'medication reconciliation', and a half (n = 104; 50.0%) reported carrying out medication reconciliation in more than 75% of consultations after hospital discharge. No differences were identified regarding the frequency with which family physicians practice medication reconciliation after hospital discharge depending on age, gender, type of unit where they work, and volume of consultations. Most family physicians (n = 155; 74.5%) included the three steps recommended by the Directorate-General for Health at the time and resolved discrepancies without contacting the hospital physician (n = 168; 88.8%). Family physicians recognize the importance of medication reconciliation (more than 95% agree/completely agree), although the level of agreement regarding their responsibility for this practice is lower.The proportion of primary health care units with a formal medication reconciliation procedure is low. Family doctors in the Lisbon and Tagus Valley Region value medication reconciliation, although they do not include it in all consultations after hospital admission. Communication between levels of care and the standardization of processes are areas with potential for improvement to promote the safe and patient-centered use of medication.We conducted an observational, cross-sectional and descriptive study, using two observation units: primary health care units (study 1) and family doctors (study 2) in the Lisbon and Tagus Valley Health Region. Data was collected through two self-completed questionnaires, which were made available online.Introdução: Apesar da relevância da reconciliação da medicação para a continuidade dos cuidados, atualmente, não existe informação relativa às práticas, conhecimentos e atitudes dos médicos de família nacionais sobre este assunto. Este estudo teve como objetivos caracterizar os procedimentos formais de reconciliação da medicação em vigor na Região de Saúde de Lisboa e Vale do Tejo, bem como a perceção dos médicos de família desta região sobre o que sabem, como pensam e como praticam a reconciliação da medicação. Métodos: Foi conduzido um estudo observacional, transversal e descritivo, utilizando duas unidades de observação: unidades de cuidados de saúde primários (estudo 1) e médicos de família (estudo 2) da Região de Saúde de Lisboa e Vale do Tejo. A recolha de dados realizou-se através de dois questionários de autopreenchimento, disponibilizados online. Resultados: Participaram 89 unidades de cuidados de saúde primários e 208 médicos de família (taxas de resposta de 31% e 12%, respetivamente). Verificou-se que apenas uma em cada quatro unidades (n = 22/89) tinha um procedimento formal de reconciliação da medicação em vigor. Entre as unidades com procedimento formal, existe variabilidade em alguns parâmetros, ainda que todos incluam os médicos. Mais de 70% (n = 150; 72,1%) dos médicos referiram ter contacto prévio com o termo ‘reconciliação da medicação’, e metade (n = 104; 50,0%) referiu realizar reconciliação da medicação em mais de 75% das consultas após alta hospitalar. Não se identificaram diferenças relativamente à frequência com que os médicos praticam reconciliação da medicação após alta hospitalar em função da sua idade, sexo, tipo de unidade onde trabalham e volume de consultas. A maioria dos médicos (n = 155; 74,5%) inclui no processo os três passos preconizados pela Direção-Geral da Saúde à data, e resolve as discrepâncias sem contactar o médico hospitalar (n = 168; 88,8%). Os médicos reconhecem a importância da reconciliação da medicação (mais de 95% estão de acordo/totalmente de acordo), ainda que o nível de concordância quanto à sua responsabilização sobre esta prática seja inferior. Conclusão: A proporção de unidades de cuidados de saúde primários com procedimento formal de reconciliação da medicação é baixa. Apesar dos médicos de família da Região de Saúde de Lisboa e Vale do Tejo valorizarem a reconciliação da medicação, não a incluem em todas as consultas após internamento hospitalar. A comunicação entre níveis de cuidados e a padronização dos processos são áreas com potencial de melhoria, com vista ao objetivo de promover a segurança da utilização dos medicamentos, centrada no doente

Chronic Limb-Threatening Ischemia Under the Age of 50 – a Single-Center 12-Year Retrospective Study

INTRODUCTION: Premature peripheral artery disease (PAD), defined as occurring before or at the age of 50, is a poorly studied subset of PAD due to its low incidence. It has been associated with a higher risk of progression to chronic limb-threatening ischemia (CLTI), major adverse limb events and mortality. Etiology is multifactorial, with genetics and environmental causes at play, with the most common risk factors being smoking, diabetes, and hypertension. METHODS: A single-center retrospective study was conducted in a Portuguese tertiary center, including all patients up to the age of 50 submitted to a revascularization procedure for CLTI from May 2011 to November 2023. The primary endpoint was a composite outcome of mortality, amputation and/or reintervention. The secondary endpoints were reintervention, amputation and mortality rates, and amputation-free survival (AFS). RESULTS: Ninety-one patients were included (74% male, median age 48). The most common risk factors were smoking (69%), diabetes (45%) and hypertension (44%). Most patients presented as Fontaine grade IV (79%). Thirtytwo patients (35%) had aortoiliac lesions, while 38 (42%) had femoropopliteal, and 21 (23%) had infra-popliteal disease. During the median follow-up period of 7.4 years (IQR 4.3-10.3), 57 patients (63%) underwent reintervention, amputation, or died. The reintervention rate was 40%, while amputation was performed on 25% of patients, and 29% of patients died. Median AFS was 4.7 years (IQR 0.8-7.8). Subgroup analysis comparing diabetic and non-diabetic patients and endovascular vs. open surgery were performed. The 30-day amputation rate was significantly higher in diabetics (12% vs. 2%, p=.05), but there were no other significant differences in subgroup analysis. CONCLUSION: Premature CTLI is associated with poor outcomes and high mortality rates, with most patients undergoing reintervention or amputation. Further studies are needed to identify non-traditional risk factors to improve outcomes in this young population

Hipoperfusão Abdominal e Lesão Renal Aguda no Doente Crítico com Cirrose Hepática – Estudo de Coorte Prospetivo

Reduced abdominal perfusion pressure (APP) is an underdiagnosed potential pathophysiological mechanism for acute kidney injury (AKI) in the patient with liver cirrhosis and ascites. This study aimed to analyze the prevalence of abdominal hypoperfusion (AhP) (APP <60 mm Hg) and the impact of APP on AKI in critically ill patients with liver cirrhosis. This was a post hoc analysis from a prospective cohort study set in a general ICU at a tertiary university hospital. Patients were recruited between October 2016 and December 2021. Acute renal failure (ARF) was defined by stage 3 AKI according to the International Club of Ascites. Fifty-eight patients where included, with a mean age of 57 (±8.4) years, 79% were male, and 93% had acute-on-chronic liver failure at admission. The prevalence of AhP reached 75%, and 29% of cases had persisting AhP during the first week of ICU stay. Patients with baseline AhP had a higher 28-day mortality compared to those without AhP (respectively, 76% vs. 49%, = 0.03). Acute renal failure developed in 48% of patients. Higher serum urea (aOR: 1.01, 95% CI: 1.00-1.02, = 0.04) and white blood cell count (aOR: 1.1, 95% CI: 1.01-1.2, = 0.02) at ICU admission, as well as low persisting APP (aOR: 0.9, 95% CI: 0.86-0.98, = 0.02) were independent risk factors for ARF. Critically ill patients with liver cirrhosis presented a high prevalence of ARF, independently associated with higher baseline serum urea and WBC, and lower persisting APP. A structured clinical approach to optimize APP may reduce renal dysfunction in high-risk patients with cirrhosis.A pressão de perfusão abdominal (PPA) é um possível mecanismo fisiopatológico para a lesão renal aguda (LRA) frequentemente sub-diagnosticado no paciente cirrótico com ascite. Este estudo teve como objetivo analisar a prevalência de hipoperfusão abdominal (hPA) (PPA <60 mm Hg) e o impacto da PPA na lesão renal aguda em doentes com cirrose e doença crítica. Esta foi uma análise pós-hoc de um estudo de coorte prospetivo de doentes críticos com cirrose hepática realizado numa unidade de cuidados intensivos (UCI) polivalente de um hospital universitário terciário. Os doentes foram recrutados entre outubro de 2016 e dezembro de 2021. A falência renal aguda (FRA) foi definida de acordo com o estadio 3 de LRA do International Club of Ascites. Cinquenta e oito doentes foram incluídos, com uma média de idade de 57 (±8.4) anos, 79.3% eram do sexo masculino e 93.1% apresentavam a síndrome . A prevalência de hPA foi de 75.3%, e 29.3% dos casos apresentaram hPA persistente durante a primeira semana na UCI. Os doentes com hPA basal apresentaram um aumento de mortalidade aos 28 dias em comparação com aqueles sem hPA (76.0% vs. 48.5%, = 0.03). Verificou-se FRA na admissão em 48.3% dos pacientes. O aumento da concentração da ureia sérica (aOR 1.01, IC95% 1.001–1.02, = 0.04) e da contagem de leucócitos (CL) (aOR 1.1, 95% IC: 1.01–1.2, = 0.02) na admissão à UCI, bem como a redução persistente da PPA (aOR 0.9, 95% IC: 0.86–0.98, = 0.02) foram fatores de risco independentes para o desenvolvimento de FRA. Os doentes críticos com cirrose hepática apresentaram uma alta prevalência de FRA, cujos fatores de risco independentes incluíram o aumento da ureia sérica e da CL basais, e a redução persistente da PPA. Uma abordagem clínica estruturada para otimizar a PPA poderá reduzir a lesão renal aguda nos doentes cirróticos de alto risco

Percutaneous Vertebral Biopsies – Case-Based Description of a Single Centre Experience

Introdução: A biópsia percutânea vertebral tem substituído a biópsia cirúrgica aberta nos últimos 50 anos. A biópsia não-invasiva é mais custo-efetiva e tem menos complicações quando comparada com procedimentos abertos. Realizou-se uma revisão das biópsias percutâneas vertebrais realizadas no nosso centro para avaliar o yield diagnóstico e a segurança. Métodos: Analisou-se retrospetivamente 240 biópsias vertebrais percutâneas realizadas no nosso centro terciário durante 4 anos. As variáveis adquiridas incluíram técnica diagnóstica de imagem, segmento vertebral, localização da biópsia, resultados histopatológicos, tratamento adjuvante e complicações. Resultados: 102 (42,5%) dos pacientes eram mulheres, com uma média de 68 anos. A técnica mais utilizada foi a fluoroscopia (99%, n=237). A maioria dos procedimentos foi realizado no segmento lombar, representando 47% (n=112), seguido do segmento torácico (42%, n=100). Obtivemos amostra suficiente para análise histológica em 93%. Das 240 biópsias, 18 (7,5%) tiveram de ser repetidas, tendo-se obtido um diagnóstico em 14 (78%). Histologicamente, em 28% (n=67) das amostras não se obteve alterações patológicas e em 27% (n=65) confirmou-se doença metastática. Realizou-se vertebroplastia em 19% (n=46) dos casos após a biópsia. Apenas um paciente teve uma complicação clinicamente significativa secundária ao procedimento. Conclusão: A biópsia vertebral percutânea é uma importante ferramenta na avaliação de lesões vertebrais e dos tecidos paravertebrais adjacentes, e pode ser realizada com baixa morbilidade e alta taxa de deteção como um procedimento de ambulatório. Em conformidade com a literatura, a maior parte das nossas amostras eram adequadas para análise histopatológica e o achado mais comum foi lesão metastática.Introduction: Percutaneous spine biopsy has widely replaced open surgical biopsy for the last 50 years. Non-invasive biopsy is more cost effective and has fewer complications than open procedures. We reviewed our single center experience in percutaneous spinal biopsies to evaluate their diagnostic yield and safety. Methods: We retrospectively analyzed 240 percutaneous vertebral biopsies performed at our tertiary center, in a 4-year period. Collected variables included diagnostic imaging techniques, vertebral segment, location of biopsy, histopathological results, adjunct treatment and complications. Results: 102 (42.5%) of patients were female, with an average age of 68. Fluoroscopy was the most used technique (99%, n=237). The majority of our procedures were performed on the lumbar spine, representing 47% (n=112) of the total biopsies. In 93% of biopsies (n=223) we attained sufficient samples for histological analysis. Out of the 240 biopsies, 18 (7.5%) had to be repeated, of which 14 (78%) had diagnostic yield. Histologically, 28% (n=67) of samples had no pathological changes and 27% (n=65) yielded the diagnosis of metastatic disease. We performed adjunct treatment with vertebroplasty in 19% (n=46) of cases following biopsy. Only one patient had a clinically significant complication following the procedure. Conclusion: Percutaneous vertebral biopsy is an important tool in the evaluation of vertebral body lesions, and adjacent paravertebral tissues, and can be performed with minimal morbidity and high diagnostic yield as an outpatient procedure. In line with the literature, most of the biopsy samples in our study were adequate for histopathological analysis, and metastatic lesions were the most common finding