Spontaneous Aortoesophageal Fistula And Ruptured Aortic Aneurysm - A Case Report On Combined Aortic And Esophageal Prosrhesis Palliative Treatment.

Aortoesophageal fistulas are uncommon, dreadful vascular events, most frequently found in the setting of thoracic aorta aneurysms. Patients usually present with thoracic pain, dysphagia and sentinel hematemesis - the Chiari triad - followed by life threatening hematemesis. Emergent open surgery with debridement of necrotic tissue and in situ aortic graft repair is currently the best strategy. However, in patients which cannot withstand surgery, endovascular repair is currently gaining acceptance as a palliative treatment or as a bridge to surgery. We present a case of a 55-year-old female with a past of heavy alcohol abuse and a previously unknown massive aortic aneurysm, who presented to the emergency department complai- ning of acute dysphagia and epigastric pain. An abdominal ultrasound revealed left pleural effusion and suspected clots in the pleural space. A thoracic CTA was promptly done, where a spontaneous ruptured aortic aneurysm with aortoesophageal fistula was discovered. The team, fearing open surgery due to poor cardiac function, opted for a thoracic endovascular aortic repair. The aortoesophageal fistula dissected the esophageal wall in all of its thickness without rupture into the lumen. This was complicated with esophageal ischemia, aneurysmal sac infection and mediastinitis. Because the patient was in shock, in order to help control the infection, an esophageal prosthesis was placed, followed by proximal esophagostomy, distal esophageal closure and gastrostomy. Six months after initial presentation, the patient died at the emergency room, shortly after reentering with massive hematemesis and hypovolemic shock of undetermined origin.

[Mycotic aneurysms: a case report, clinical review, imaging appearances and management].

To report a clinical case of mycotic thoracic aortic aneurysm. To describe and illustrate the spectrum of MDCT findings, analysing the pathophysiology, diagnostic evaluation and therapeutic management. To emphasis the early non-invasive diagnosis as a way to overall survival improvement. A mycotic aneurysm is an uncommon vascular lesion resulting from arterial wall destruction by infection, generally involving the peripheral arteries or aorta and rarely the cerebral and visceral arteries. Defined as a lobulated saccular outpouching of the wall communicating with the lumen, it is surrounded by oedema, hematoma and/or fibro-inflammatory tissue. Due the non-specific and delayed - or even absent - manifestations, a high degree of clinical suspicion is necessary to achieve a timely treatment and prognosis improvement. In this setting, radiologic evaluation - mainly by MDCT angiography - is essential, allowing detection, characterization and complications assessment before treatment planning.

Pretende-se reportar um caso clínico de aneurisma micótico da aorta torácica; descrever e ilustrar o espectro de achados tomodensitométricos (TDM); rever a fisiopatologia, a avaliação diagnóstica e o tratamento; e enfatizar o diagnóstico não-invasivo precoce como forma de aumentar a sobrevivência. Um aneurisma micótico é uma lesão vascular infrequente que resulta da destruição da parede arterial por um processo infecioso, que geralmente afeta as artérias periféricas ou a aorta, e, raramente, as artérias cerebrais ou viscerais. É definida como uma protrusão sacular e lobulada da parede em comunicação com o lúmen arterial, rodeada por edema, hematoma e/ou tecido inflamatório. Atendendo às manifestações inespecíficas e tardias que a caracterizam, é necessário um elevado grau de suspeição para que se obtenha um tratamento atempado e se possa melhorar o prognóstico. Consequentemente, a avaliação imagiológica ­ particularmente o estudo angiográfico TDM ­ é essencial, permitindo a deteção, caracterização e avaliação de complicações, necessárias ao planeamento terapêutico.

Acute aortic syndrome: Spectrum of imaging appearances.

OBJECTIVES: The main objective of this paper is to illustrate and analyse the spectrum of MDCT findings of the entities that compose acute aortic syndrome (AAS) - namely acute aortic dissection (AD), intramural hematoma (IMH) and penetrating atherosclerotic ulcer (PAU) - discussing their pathophysiology. These related entities have a similar nonspecific clinical presentation mimicking other conditions such as myocardial infarction. Therefore, a high degree of clinical suspicion should prompt immediate confirmation by non-invasive imaging techniques which can modify its natural history, being MDCT the technique of choice, allowing accurate detection, characterization and complications evaluation in the Emergency Department. METHODS: A detailed analysis of a series of clinical case of acute aortic syndrome seen at our Department is done, counselling with a review of the state of the art regarding the pathologic mechanisms, diagnostic evaluation and therapeutic management. RESULTS: They share a common feature of separation of the aortic walls. In AD there is an inward intimal displacement or tear, creating a true and a false lumen (double lumen). Detailing the extent, configuration - including true and false lumina distinction - and complications, are essential to proper treatment planning. IMH is defined by the presence of acute haemorrhage within wall layers without no visible intimal flap or tear on conventional techniques. It can lead to wall infarction and consequent dissection. PAU is due to erosion of internal elastic lamina - reaching the media - by an atherosclerotic plaque in the setting of advanced atherosclerosis or, less commonly, after rupture of a mycotic plaque or due to connective tissue disorders, appearing as an irregular focal contrasted outpouching outside the lumen. It can precipitate IMH or a pseudoaneurysm and progress, frequently, to dissection or rupture, coursing with high mortality, if not treated. Well known complications are aortic rupture with massive haemorrhage, pericardial tamponade by hemopericardium, mediastinal hematoma or hemothorax, pulmonary artery stenosis and/or pulmonary haemorrhage or organ ischemia. CONCLUSION: MDCT findings knowledge allows a correct diagnosis. These life-threatening entities have an undistinguishable clinical presentation but different physiopathology mechanisms - reflected in differing radiologic findings - prognosis and management.

Objetivos: O presente artigo pretende ilustrar e analisar o espectro de achados tomodensitométricos (TDM) das três entidades que compõe a síndrome aórtico agudo (SAA) ­ designadamente dissecção aórtica aguda (DA), hematoma intramural (HIM) e úlcera aterosclerótica penetrante (UAP) ­ e discutir a sua fisiopatologia. Estas entidades, estreitamente relacionadas, manifestam-se, habitualmente, de forma inespecífica, mimetizando outras condições clínicas, como o enfarte agudo do miocárdio (EAM). Assim sendo, um elevado grau de suspeição clínica impõe uma confirmação imediata por técnicas de imagem não invasivas que podem modificar a história natural da doença, sendo, num contexto emergente, o estudo dinâmico multidetectores por tomografia computorizada (TC), a técnica de eleição. Permite, com elevada acuidade diagnóstica, a deteção, caracterização e a avaliação de complicações. Métodos: É feita uma análise detalhada de uma série de casos clínicos de SAA observada no nosso Departamento, conciliando com a revisão do estado da arte no que diz respeito aos mecanismos patológicos, avaliação diagnóstica e opções terapêuticas. Resultados: As três entidades partilham de uma característica comum, a separação anómala das camadas da parede aórtica. Na DA observa-se uma descontinuidade focal com destacamento da íntima em direção ao lúmen, criando um duplo lúmen (verdadeiro e falso). Detalhar a extensão, morfologia ­ incluindo a distinção dos dois lumens ­ e caracterizar possíveis complicações, são aspetos essenciais à decisão e planeamento da terapêutica. O HIM é definido pela presença de uma hemorragia aguda ou recente na espessura da parede vascular, sem que seja possível objetivar uma descontinuidade da íntima, utilizando técnicas de imagem convencionais. Poderá progredir com enfarte parietal e, consequentemente, disseção. A UAP deve-se à erosão a lâmina elástica interna ­ com atingimento da média ­ geralmente, por uma placa aterosclerótica num contexto de aterosclerose avançada; ou, menos frequentemente, é consequência da rotura de uma placa micótica, ou de doenças do tecido conjuntivo; manifestando-se por uma irregularidade com protusão focal da parede, em continuidade com o lúmen. Pode precipitar um HIM ou progredir com a formação de um pseudoaneurisma e/ou disseção, ou mesmo rotura, de elevada mortalidade se não tratados. Constituem complicações a rotura aórtica com hemorragia massiva, tamponamento pericárdico por hemopericárdio, hemotórax ou hematoma mediastínico, estenose da artéria pulmonar e/ou hemorragia pulmonar, ou isquemia de órgãos. Conclusão: O reconhecimento dos achados TDM permite um diagnóstico atempado e correto. Estas entidades ­ potencialmente fatais ­ são, muitas vezes, indistinguíveis clinicamente, embora apresentem mecanismos fisiopatológicos distintos, o que se traduz em diferentes achados imagiológicos, prognósticos e abordagens terapêuticas.

[Thymic lesions: A 4 years-review]. / Patologia do Timo: Revisão de 4 Anos.

INTRODUCTION: The difficulty in differentiating invasive from non-invasive thymic tumours solely by histological features, leads to the decision of resecting suspected lesions except in the presence of: (a) diffuse metastasis; or (b) typical imaging findings of thymic hyperplasia or after recovering from some recent stress. Therefore, the role of radiology is of the utmost importance in the initial evaluation and staging of thymic mediastinal masses. METHODS: The objective of this study was to elaborate a retrospective analysis of the cases of thymic lesions that underwent surgery at the Cardiothoracic Department of Centro Hospitalar Vila Nova de Gaia/Espinho (CHVNG/E), from January 2013 to December 2016, illustrating their main radiologic findings with computed tomography (CT) images. RESULTS: During the studied time period, 35 patients were diagnosed with thymic lesions including: 14 thymomas (40%), 9 thymic cysts (26%), 4 thymic hyperplasias (11%), 3 thymic carcinomas (9%), 2 thymolipomas (6%), 2 lymphomas (6%) and 1 neuroendocrine tumour (3%). The mean age of the sample was 60 years, ranging from 23 to 88 years, with a predomi- nance of the female sex (69%). In this article, 11 CT images and 1 thoracic x-ray are presented to illustrate the common imaging features of each thymic disease. Thymoma was the most frequent thymic lesion found in our 4-year case sample, similarly to what is described in literature for the adult age. CONCLUSION: Knowledge of the main radiologic findings of each pathological entity of the thymus is vital for the correct therapeutic management and disease staging, frequently allowing the differentiation of non-surgical from surgical lesions.

Introdução: A dificuldade na diferenciação de neoplasias tímicas invasivas de não invasivas apenas pelas suas caracte- rísticas histológicas, leva a que se preconize a resseção cirúrgica destas lesões, exceto na presença de: (a) metastização difusa; e (b) manifestações imagiológicas típicas de hiperplasia tímica ou após um stress corporal conhecido. Dessa forma, o papel da radiologia torna-se de suma importância na avaliação da suspeita inicial e no seu estadiamento. Métodos: O objetivo do presente trabalho foi estudar retrospetivamente os casos de patologia tímica submetidos a cirurgia pelo Serviço de Cirurgia Cardiotorácica do Centro Hospitalar Vila Nova de Gaia/Espinho (CHVNG/E) entre janeiro 2013 e dezembro 2016, ilustrando, com as respetivas imagens de tomografia computorizada (TC), as principais manifestações ima- giológicas deste espectro de lesões. Resultados: Neste período foram operados 35 doentes com patologia tímica com os seguintes diagnósticos: 14 timomas (40%), 9 quistos (26%), 4 hiperplasias tímicas (11%), 3 carcinomas tímicos (9%), 2 timolipomas (6%), 2 linfomas (6%), e 1 tumor neuroendócrino (3%). A média de idades foi de 60 anos, com idade mínima de 23 anos e máxima de 88 anos, e predomínio do sexo feminino (69%). São apresentadas 11 imagens de cortes de TC e 1 radiograma torácico, representativas das manifestações imagiológicas de cada patologia obtida. Na amostra estudada, a patologia tímica mais frequente foi o timoma, de forma semelhante ao descrito na literatura para a idade adulta. Conclusão: O conhecimento das principais características imagiológicas das diferentes entidades patológicas do timo é fulcral na orientação terapêutica, permitindo por vezes diferenciar a hiperplasia de "verdadeiras" neoplasias, e possibilitar o estadiamento das lesões.

BONE RECONSTRUCTION IN THE TREATMENT OF TIBIAL HEMIMELIA: AN ALTERNATIVE TO AMPUTATION?

Objective: To evaluate the advantages and disadvantages of bone reconstruction and lengthening compared to amputation in the treatment of tibial hemimelia for patients and their families. Methods: Systematic review of articles published in English and Portuguese between 1982 and 2022 in the MEDLINE, PubMed, Cochrane and SciELO databases. The variables of interest were: year of publication, sample characteristics, classification of tibial hemimelia according to Jones, treatment outcome and follow-up time. Results: A total of eleven articles were included in the scope of this review. The studies involved 131 patients, 53.4% male and 46.6% female. The age of the patients who underwent a surgical procedure ranged from 1 year and 10 months to 15 years. The most common type was Jones' I (40.9%). The most recurrent complications in the reconstruction treatment were: infection of the external fixator path, flexion contracture (mainly of the knee), reduction in the range of motion of the knee and ankle. Conclusion: We did not find enough relevant studies in the literature to prove the superiority of reconstruction. Amputation remains the gold standard treatment for tibial hemimelia to this day. Level of Evidence III, systematic review of level III studies.

Objetivo: Avaliar as vantagens e desvantagens da reconstrução óssea e alongamento comparada à amputação no tratamento da hemimelia tibial para pacientes e familiares. Métodos: Revisão sistemática, com análise de artigos publicados nas línguas inglesa e portuguesa entre 1982 e 2022, nas bases de dados MEDLINE, PubMed, Cochrane e SciELO. As variáveis de interesse foram: ano de publicação, característica da amostra, classificação da hemimelia tibial segundo Jones, desfecho do tratamento e tempo de seguimento. Resultados: Fizeram parte do escopo desta revisão onze artigos. Os estudos envolveram 131 pacientes, 53,4% do sexo masculino e 46,6% do feminino. A idade dos pacientes submetidos a algum procedimento cirúrgico variou de 1 ano e 10 meses a 15 anos. O tipo mais comum foi o I de Jones (40,9%). As complicações mais recorrentes no tratamento pela reconstrução foram: infecção do trajeto de pinos do fixador externo, contratura em flexão (principalmente do joelho), redução do arco de movimento de joelho e tornozelo. Conclusão: Não encontramos na literatura estudos suficientemente relevantes para comprovar a superioridade da reconstrução. A amputação se mantém até os dias de hoje o tratamento padrão-ouro para hemimelia tibial. Nível de evidência III; revisão sistemática de estudos de nível III.

Drug decriminalization and the price of illicit drugs.

BACKGROUND: This study is an empirical assessment of the impact of the drug decriminalization policy followed by Portugal in July 2001, on the price of illicit drugs. METHODS: The analysis is performed using a difference-in-differences approach and the Synthetic Control Method in order to construct a synthetic control unit from a convex combination of countries. RESULTS: The results suggest that the prices of opiates and cocaine in the post-treatment period did not decrease in the sequence of the policy change. CONCLUSION: We conclude that the drug decriminalization policy seems to have caused no harm through lower illicit drugs prices, which would lead to higher drug usage and dependence.

Brain magnetic resonance imaging: perception and expectations of neurologists, neurosurgeons and psychiatrists.

Magnetic resonance imaging (MRI) has rapidly become an essential diagnostic tool in modern medicine. Understanding the objectives, perception and expectations of the different medical specialties towards MRI is therefore important to improve the quality of the examinations. Our aim was to better comprehend the reasons and expectations of neurologists, neurosurgeons and psychiatrists when requesting brain MRI scans for their patients, and also to perceive the degree of confidence of these specialists in the images and respective reports. Sixty-three specialists were recruited from two tertiary hospitals and answered a tailored questionnaire. Neurosurgeons were more concerned with the images themselves; neurologists lacked confidence in both MRI images and reports, and one third of the psychiatrists only read the report and were the most confident of the specialties in MRI findings. These results possibly reflect the idiosyncrasies of each of these medical specialties. This knowledge, driven by efficient communication between neuroradiologists and neurosurgeons, neurologists and psychiatrists, may contribute to improve the quality of MRI examinations and consequently patient care and management of health resources.

Cardiac magnetic resonance myocardial perfusion imaging for detection of functionally significant obstructive coronary artery disease: a prospective study.

BACKGROUND: Cardiac magnetic resonance myocardial perfusion imaging (CMR-MPI) is considered a state of the art non-invasive modality for the detection of reversible ischemia. Recent studies have shown its utility in the diagnosis of coronary artery disease (CAD) and superiority over other established techniques. However, only a few studies compared CMR-MPI against the invasive standard including fractional flow reserve (FFR) and clinical validation in non-specialized centers is scarce. The aim of this study was to validate CMR-MPI in a real-world clinical environment and to test its diagnostic accuracy in symptomatic patients with suspected CAD versus FFR as the reference standard of functionally significant disease. METHODS AND RESULTS: 103 symptomatic consecutive patients (62 ± 8.0 years, 66% males) with suspected CAD and intermediate or high probability of disease underwent sequential CMR and invasive coronary angiography (XA). The CMR protocol included stress-rest adenosine perfusion, SSFP cine imaging and late-enhancement imaging. Functionally significant CAD was defined as occlusive/sub-occlusive stenoses on XA or non-occlusive stenoses with a FFR measurement of <0.80 in vessels >2mm. On a patient-based model, CMR-MPI had sensitivity, specificity, positive and negative predictive values of 89%, 88%, 85%, and 91%, respectively, with a global accuracy of 88%. On a vessel-based analysis, these values were 80%, 93%, 79% and 94%, respectively, with a global accuracy of 90%. CONCLUSIONS: CMR-MPI had a very high accuracy for detection of functionally significant CAD as assessed by FFR in patients with intermediate to high pretest probability.

Additive value of magnetic resonance coronary angiography in a comprehensive cardiac magnetic resonance stress-rest protocol for detection of functionally significant coronary artery disease: a pilot study.

BACKGROUND: Cardiovascular magnetic resonance (CMR) myocardial perfusion imaging (MPI) is a state-of-the-art noninvasive modality for detection of myocardial ischemia and coronary artery disease. Magnetic resonance coronary angiography (MRCA) allows visualization of the coronary tree, but its incremental value as part of a CMR protocol including MPI and late gadolinium enhancement (LGE) is not well established. We aimed to evaluate the additive diagnostic value of a 3-dimensional whole-heart MRCA integration into a 1.5T CMR-MPI/LGE protocol for the detection of functionally significant coronary artery disease. METHODS AND RESULTS: Forty-three symptomatic patients (61 ± 8.3 years; 65% men) with suspected coronary artery disease and intermediate/high-pretest probability underwent CMR (including CMR-MPI, MRCA, and LGE) and x-ray invasive coronary angiography (XA) with fractional flow reserve evaluation. Diagnostic performances of MRCA, CMR-MPI/LGE, and MRCA+CMR-MPI/LGE integration were determined having XA+fractional flow reserve as standard for coronary artery disease (≥90% stenosis/occlusion or fractional flow reserve ≤ 0.80 in vessels>2 mm). MRCA inclusion into the CMR protocol was associated with a mean increase of 7.9 ± 4.69 (0-17.7) minutes in total examination duration (14%). On patient-based analysis, MRCA had 96% sensitivity, 68% specificity, positive predictive value of 79%, and negative predictive value of 93%. CMR-MPI/LGE had 79% sensitivity, 95% specificity, positive predictive value of 95%, and negative predictive value of 78%. Integration of MRCA with CMR-MPI/LGE further improved CMR performance to 96% sensitivity, 89% specificity, positive predictive value of 92%, and negative predictive value of 94%, with a global accuracy of 93%. CONCLUSIONS: In this intermediate/high-pretest population, integration of noncontrast-enhanced whole-heart MRCA nonsignificantly improved per-patient diagnostic accuracy of a comprehensive 1.5-T stress-rest CMR-MPI/LGE protocol at a cost of longer scanning times.

Assessment of left ventricular diastolic function with cardiovascular MRI: what radiologists should know.

Diastolic dysfunction is a common entity and the predominant cause of heart failure in 40%-50% of patients. Diagnosis of diastolic dysfunction is clinically relevant and associated with a poor prognosis. The aim of this essay was to review the pathophysiology and different grades of diastolic dysfunction and to provide an overview on the role of cardiovascular magnetic resonance imaging in the assessment of diastolic function.