Vascular Malformation and Common Keratinocytic Nevus of the Soft Type: Phacomatosis Pigmentovascularis Revisited

Phacomatosis pigmentovascularis is a rare syndrome characterized by the coexistence of a pigmented nevus and a cutaneous vascular malformation. We report a 5-year-old boy with all the typical findings of phacomatosis pigmentovascularis type Ia. Although its existence according to the traditional classification has been questioned, this case represents a very rare association of a capillary vascular malformation and a common keratinocytic nevus of the soft type.

Case for Diagnosis

A 27 year-old-man, with no known personal or familial history of disease, mentioned a 10-year history of asymptomatic groups of telangiectasias, with a Blaschko lines distribution on the right lateral aspect of the neck and asymptomatic. He denied any episodes of disease or drug intake that could be associated with the disease. Blood work had no changes, namely of liver enzymes or infectious serologies. The clinical diagnosis of Idiopathic Acquired Unilateral Nevoid Telangiectasia was made, an uncommon, benign vascular malformation. The patient declined doing a cutaneous biopsy or treatment with a cosmetic intent.

Treatment of Vascular Lesions of the Tongue with Nd: YAG Laser

The treatment of vascular lesions of the tongue is a very challenging procedure since the maintenance of the lingual tissue is of critical importance. Numerous treatment options have been described in literature but the Nd:YAG Laser appears to be one of the safest therapeutic options. We described a successful treatment of vascular lesions of the tongue with an excellent clinical result after only one treatment session with the Nd:YAG laser, with conservation of the lingual tissue and its functionality.

Prevalência de Doença Carotídea na Patologia Cérebro-Vascular Isquémica. O Papel do Eco-Doppler

BACKGROUND: Atherosclerotic carotid disease represents approximately 20% of the causes of ischemic stroke. Effective treatment options, such as endovascular or surgical revascularization procedures, are available. Doppler Ultrasound (DUS) is a non-invasive, inexpensive, routine exam used to evaluate the presence of internal carotid artery (ICA) stenosis. We retrospectively analysed the prevalence of severe atherosclerotic carotid disease in a population of patients with acute ischemic stroke/transitory ischemic attacks (TIAs), and the role of DUS in the detection of ICA stenosis and treatment decisions in these patients. METHODS: A total of 318 patients with ischemic stroke or TIAs was admitted to our stroke unit, and 260 patients were studied by DUS. ICA stenosis was evaluated by DUS according to peak systolic velocity. All DUS exams were performed by the same operator. ICA stenosis was further assessed in 43 patients by digital subtraction angiography (DSA) using NASCET criteria. RESULTS: Of the total 318 patients, 260 (82%) had DUS evaluation. Of the total 520 ICAs studied by DUS, degrees of ICA stenosis were: 0-29% n= 438 (84%); 30-49% n= 8 (2%); 50-69% n= 27 (5%); 70-89% n= 15 (3%); 90-99% n= 20 (4%); oclusão n= 14 (2%). Of the total 260 patients studied, 43 (16.5%) underwent DSA. Sensibility and specificity of DUS in the diagnosis of carotid stenosis over 70% were, respectively, 91% e 84%. Of the total 31 patients with significant carotid stenosis (70-99%), 23 (74%) underwent subsequent carotid revascularization procedures. DISCUSSION: DUS is an important screening test in our stroke unit, justifying its use as a routine exam for all patients with ischemic stroke/TIAs. Moreover, our results show the relevance of severe carotid disease in a population with acute ischemic stroke/TIAs (16.5%), with a total of 9% of patients being submitted to carotid revascularization procedures.

Da Disfunção Endotelial à Oclusão Vascular Papel do Sistema Renina - Angiotensina

There is a body of evidence that supports the important role of the renin-angiotensin system (RAS) in atherosclerotic disease and in the cardiovascular disease continuum: from endothelial dysfunction to vascular occlusion. In the earlier stages of vascular disease, the RAS promotes functional changes, of which endothelial dysfunction is the best example. The deposition of atherogenic lipoproteins in the intima, their oxidative modification and the onset and amplification of the inflammatory response strengthens the atherogenic role of the RAS. Inflammatory cells are one of the main sources of angiotensin-converting enzyme (ACE) and angiotensin II (Ang II) in the vascular wall, in a process that leads to structural changes in the artery and progression of atherosclerotic disease. Ang II promotes the migration of vascular smooth muscle cells and their phenotypic differentiation in synthesis that accelerates vascular disease. By modulating the inflammatory response and, in general, all the elements of the plaque, Ang II plays a part in its instability, in the onset of acute events and in the promotion of the local prothrombotic state that leads to infarction.

Three-Dimensional Contrast-Enhanced Magnetic Resonance Pulmonary Angiography in the Study of Pulmonary Vascular Pathology

OBJECTIVES: To assess the feasibility of performing pulmonary angiography using MRI with contrast enhancement in patients with pulmonary vascular disease. METHODS: We present our experience in ten individuals, two controls and eight patients who underwent the exam after injection of a gadolinium-based contrast agent on a 1 Tesla MR scanner using a time-of-flight sequence and breath-holding during injection of contrast. RESULTS: Pathology in the main pulmonary artery and its major branches was detected easily while resolution at the segmental and subsegmental levels was inadequate. CONCLUSION: Contrast-enhanced magnetic resonance pulmonary angiography is feasible on a 1 Tesla MR scanner for the study of pathology of the main pulmonary artery and its major branches, like massive pulmonary embolism. However its ability to detect and define distal vessel pathology as found in chronic thromboembolic pulmonary hypertension and small pulmonary emboli is limited.

Reabilitação no Acidente Vascular Cerebral: do Hospital à Comunidade

The field of action for rehabilitation is that of making use of the patient's maximum functional capacity with the purpose of adapting to life in relation to the environment. Rehabilitation must commence immediately, although it may be in different forms from the acute phase to sequelae. It is considered appropriate to call the physiatrist as soon as the neurologic condition has stabilised. A list is made of the measures to be taken for rehabilitation in the acute phase and sequelae, and the composition of the rehabilitation team is described. In what concerns location, where to rehabilitate the patient? The group of ambulatory patients should have their rehabilitation as outpatients. Our experience with house calls is briefly described. The group of patients who cannot walk, those that present an eminently motor condition, with the possibility of being able to walk, should be with their families, with transport provided to health and rehabilitation centres. The second group, with the capacity of walking within a reasonable time, especially if with multiple associated problems such as impaired communication, should be hospitalised in a rehabilitation department. The third group consists of severely handicapped patients, for whom a solution must be found that provides life with a minimum of dignity in centres or homes. From among the measures to be introduced, we point out following: acquisition of transport for patients who must travel, as outpatients, to the department; providing family doctors with complete freedom to refer their patients to rehabilitation centres.

Na Busca da Excelência: Qualidades e Requisitos das Modernas Cirurgias Cárdio-Torácica e Vascular

In this article the author evokes the figures of the past-presidents of the Portuguese Society for Cardio-Thoracic and Vascular Surgery, since its foundation, and enhances their qualities, regarded as examples for the new generations of cardio-thoracic and vascular surgeons in training. He approaches and describes the skills and personal requirements considered as essentials to be developed nowadays, by all those who dedicate their lives to those exciting and passionate surgical specialities.

Avaliação Preoperatória do Doente Vascular

Periferal vascular disease usually results from a systemic entity in which atherothrombosis develops in different vascular territories, having common risk factors. It is hence usual to find coexistent, often subclinical, coronary artery disease, which is responsible for most of perioperatory morbidity and mortality in patients submitted to vascular surgery. An adequate preoperatory risk stratification must be accomplished, having in mind the clinical manifestations, risk factors, comorbidities, functional capacity and global left ventricular systolic function of the patient. He should be included in one of three different subgroups: low, high or intermediate risk, which might reinforce the need for further testing, most often aiming at the detection of coronary artery disease and foresee the short, medium and long term outcome. This strategy is very important and it is in part due to it and to better medical/surgical and anesthetic care that the surgical results have markedly improved in recent years. In this paper a state of the art is done of the guidelines to follow and the results of several studies performed on this subject. The role of methods to detect coronary ischemia is remarked, using either nuclear or echocardiographic techniques for this purpose.

Tratamento Cirúrgico dos Defeitos do Septo Auriculo-Ventricular. Experiência Uni-Institucional

OBJECTIVES: Atrio-ventricular septal (AVSD) defects include a variable spectrum of congenital malformations with different forms of clinical presentation. We report the surgical results, from a single institution, with this type of congenital cardiac malformation. Patients with hypoplasia of one of the ventricles were excluded from this analysis. POPULATION: Between November of 1998 and June of 2005, 49 patients with AVSD were operated on by the same team and in the same department. The average age was 37.3 months (medium 6 months) and 31 patients were female. In 38 patients (78%) an inter-ventricular communication was present (AVSD-complete) and of these, 26 were of the type A of Rastelli, being 13 of type B or C. The age for defect correction of the complete form was of 5.5 months, palliative surgery was not carried out on any of the patients. Associated lesions included: Down's syndrome in 22 patients (45%), patent arterial duct in 17 patients (35%), severe AV regurgitation in 4 patients (8%), tetralogy of Fallot in two (4%) and sub-aortic stenosis in one patient (2%). Pre-operatively 10 patients presented severe congestive heart failure and two were mechanically ventilated. RESULTS: Complete biventricular correction was carried out in all patients. The average time on bypass (ECC) was 74.1+/-17.5 min. and time of aortic clamping was 52.0+/-12.9 min. The complete defects were corrected by the double patch technique, and in all patients the mitral cleft was closed, except in two with single papillary muscle. There was no intra-operative mortality, but hospital mortality was 8%(4 patients), due to pulmonary hypertension crises, in the first 15 post-operative days. The mean ventilation time was of 36.5+/-93 hours (medium 7 h) and the average ICU stay was of 4.3+/-4.8 days (medium 3 days). The minimum follow-up period is 1 month and the maximum is 84 months (medium 29.5 months), during which time 4 re-operations (8%) took place: two for residual VSD's and two for mitral regurgitation. There was no mortality at re-do surgery. At follow up there was residual mitral regurgitation, mild in 17 patients and moderate in two. Four other patients presented with minor residual defects. CONCLUSIONS: The complete correction of AVSD can be carried out with acceptable results, in a varied spectrum of anatomic forms and of clinical severity. Despite the age of correction, for the complete forms, predominantly below 12 months, pulmonary hypertension was the constant cause for post operative mortality. Earlier timing of surgery and stricter peri-operative control might still improve results.

Implementação de uma Unidade de Dor Vascular

A preocupação em tratar adequadamente a dor dos doentes do Serviço de Angiologia e Cirurgia Vascular (SACV) do Hospital de Santa Marta levou ao desenvolvimento de uma estrutura organizada, destinada à optimização da analgesia peri-operatória. Pretendemos descrever neste artigo o processo de implementação da Unidade de Dor Vascular (UDV) do Hospital de Santa Marta, cujo projecto se iniciou em 2003 e que viria a culminar na sua inauguração em Janeiro de 2005.

Acidente Vascular Cerebral Hemorrágico Associado ao Consumo de Cocaína

Os autores apresentam o caso clínico de um homem de 37 anos, fumador de cocaína, que foi admitido no serviço por acidente vascular cerebral hemorrágico. Faz-se a discussão dos diagnósticos diferenciais e uma referência aos tipos, formas de administração, metabolismo, características clínicas e toxicidade da cocaína.

A Angiografia no Diagnóstico da Patologia Vascular Cerebral. Indicações Actuais e Controvérsias

The role of cerebral angiography in the diagnosis of cerebrovascular disease is currently being questioned due to both the increasing availability of carotid sonography and the recent introduction of Magnetic Resonance Angiography (MRA). After a technical foreword about the different modalities available today in Cerebral Angiography, we discuss its present indications (Conventional or Digital subtraction by intra-arterial route), in patients with extra and intra cranial atherosclerotic cerebro vascular disease, subarachnoid hemorrhage and arterial aneurysms, in vascular malformations, particularly arterio-venous malformations (AVM's), in occlusive non-atherosclerotic non hypertensive arteriopathies and in occlusive venous pathology. Although it is possible that the future will show us the progressive replacement of the invasive technologies by MRA, at the present stage of Magnetic Resonance development there is still an important role, if not crucial, for catheter angiography in the diagnosis of most of the diseases producing stroke syndromes.

Perfil Hipertensivo dos Doentes com Doença Vascular Cerebral

A HTA é uma das situações de risco mais referenciada para o ocorrência de doença vascular cerebral. Estão documentados antecedentes de HTA em 25 a 40% dos doentes que sofreram AVC. Outros estudos apontam para uma frequência de 80% de HTA na altura do acidente. Por isso, é importante o conhecimento da história evolutiva da HTA, bem como a sua repercursão num doente com AVC. Os autores apresentam um estudo prospectivo de 470 doentes que sofreram um primeiro AVC ou AIT. Pretendem analisar o perfil hipertensivo desses doentes, relacioná-lo com o tipo de doença vascular cerebral, e caracterizar a evolução da HTA 6 meses após o acidente. Após a análise estatística dos resultados obtidos concluíram: - na população estudada a prevalência de HTA foi de 67,2%; - 68% dos doentes com antecedentes de HTA não tinham a HTA controlada na altura do acidente; - a duração média de HTA conhecida antes do AVC ou AIT foi de 104 meses; - a HTA foi de início mais precoce e de estádio mais grave nos doentes com AVC hemorrágico, comparativamente com os doentes que sofreram doença vascular isquémica; - seis meses após AVC/AIT, 65% dos doentes não tinham a HTA controlada.

Factores Preditivos de Depressão Pós-Acidente Vascular Cerebral. Estudo Retrospectivo numa Unidade de Reabilitação

INTRODUÇÃO/OBJECTIVOS: O Acidente Vascular Cerebral (AVC) pode limitar de forma importante a funcionalidade. As complicações psiquiátricas têm sido identificadas como factores determinantes na reabilitação pós-AVC, sendo a Depressão a complicação psiquiátrica mais frequente e a que está associada a pior prognóstico. Subsiste ainda incerteza quanto à sua etiologia e factores de risco. Na revisão sistemática mais recente, reconhecem-se como factores preditivos a gravidade do AVC, o grau de incapacidade do doente e o défice cognitivo. Questões metodológicas impediram a determinação de outros factores. Assim, urge definir novos factores que facilitem um diagnóstico atempado, que possa diminuir os efeitos negativos sobre o processo de reabilitação. Objectivos: determinação da incidência da Depressão de novo pós-AVC (DPA) e o estudo das variáveis descritas na literatura como possíveis factores preditivos de DPA: sexo, idade, tipo de AVC, lateralidade, território vascular e presença de afasia. Foi definido como endpoint secundário o estudo do tipo de afasia. MATERIAIS E MÉTODOS: Estudo longitudinal retrospectivo, envolvendo todos os doentes admitidos no internamento de um Serviço de MFR com o diagnóstico de AVC de novo, entre 1-1-2009 e 31-12-2009. Foram colhidos elementos demográficos e clínicos dos processos clínicos, num total de 74 doentes. Critérios de exclusão: ‘AVC prévio’, ‘Doença psiquiátrica com perturbação do humor prévia’ e ‘Medicação antidepressiva à data do AVC’. Para o tratamento estatístico usou-se o SPSS 11.5. RESULTADOS: A incidência da DPA observada foi de 44,6%. Dos possíveis factores preditivos testados, apenas a presença de afasia apresentou uma relação estatisticamente significativa com a depressão (p=0.02). Não se encontrou relação com o tipo de afasia. Os restantes factores preditivos testados não mostraram correlação estatística significativa. Parece existir uma relação entre o sexo masculino e a DPA (p=0.07), que não atingiu significância no tamanho da amostra conseguido (n). CONCLUSÕES: Este estudo estabelece a afasia como factor preditivo da DPA. A elevada incidência de DPA nesta população particular concorda com os estudos existentes, sendo necessário outro tipo de estudo que permita justificar o valor encontrado. São necessários mais estudos não só para aumentar o conhecimento dos factores de risco para a DPA, como para melhorar os resultados dos programas de reabilitação.