Pustulose Generalizada Exantemática Aguda Associada ao Diltiazem

Acute generalized exanthematous pustulosis (AGEP) usually presents with the acute appearance of oedematous and erythematous lesions, on which multiple sterile pustules appear, associated with fever. Almost 90% of cases are associated with drugs, with antibiotics (penicillins and macrolides) being the most frequent causative agents. We describe a 36-year-old female patient, which started diltiazem 120mg/day for hypertension. After 6 days of therapy, multiple erythematous and oedematous lesions appeared, with associated multiple small non-follicular pustules. Oral corticosteroids were started, with progressive and complete improvement. Patch-tests were performed, which revealed positivity for diltiazem. Although a rare entity, AGEP must be considered in cases of acute eruptions with disseminated pustules and fever. The use of patch tests in this disease may be useful as positive reactions are frequent.

Pseudoaneurisma do Ventrículo Esquerdo com Duplo Orifício de Entrada após Enfarte Agudo do Miocárdio

Doente do sexo feminino, 78 anos de idade, internada previamente por EAM combinado. A evolução clínica subsequente decorreu sem complicações, tendo alta ao 10.º dia. Cerca de 12 horas depois foi readmitida no Serviço de Urgência, por dor retrosternal prolongada, hipotensa e prostrada. O ECG (Fig. 1) mostrou re-elevação do segmento ST nas derivações anteriores e inferiores, sendo admitida na Unidade de Cuidados Intensivos com o diagnóstico de provável re-enfarte. O Ecocardiograma realizado na admissão revelou função sistólica global comprometida, sendo possível definir um volumoso espaço anecogénico no ápex do ventrículo esquerdo (VE), com solução de continuidade na transição do terço médio/apical da parede inferior (Fig. 2) e uma segunda solução de continuidade na transição do terço médio/apical da parede lateral do VE (Fig. 3). Em ambos os orifícios foi confirmada a presença de fluxo bidireccional por Doppler Pulsado e Cor (Fig. 2), sugerindo a presença de um pseudoaneurisma do VE com duplo orifício de entrada. A doente foi transferida para um centro cirúrgico, onde, após confirmação intra-operatória do pseudoaneurisma com duplo orifício, foi submetida a encerramento do colo com patch de teflon. Teve alta estável, sendo seguida em consulta de Cardiologia desde há cerca de 16 meses, sem eventos.

Coarctação Isolada da Aorta: Experiência em 100 Doentes Consecutivos

INTRODUCTION: Coarctation of the aorta (CoA) is a stenosis usually located in the descending aorta. Treatment consists of surgical or percutaneous removal of the obstruction and presents excellent immediate results but significant residual problems often persist. OBJECTIVES: To describe the presentation, treatment and long-term evolution of a population of 100 unselected consecutive patients with isolated CoA in a single pediatric cardiology center. METHODS: This was a retrospective study of all patients with isolated CoA treated during4 the last 21 years (1987-2008). RESULTS: The patients (n=100, 68.3% male) were diagnosed at a median age of 94 days (1 day to 16 years). The clinical presentation differed between patients aged less or more than one year, the former presenting with heart failure and the latter being asymptomatic with evidence of hypertension (88 and 63%, respectively; p < 0.01). Treatment, a median of 8 days after diagnosis, was surgical in 79 cases (20 end-to-end anastomosis, 31 subclavian flap, 28 patch) and percutaneous in the remaining 21 (15 balloon angioplasty, 6 with stenting). The mean age of surgical patients was younger than in those treated percutaneously (3.4 vs. 7.5 years; p < 0.01). Immediate mortality was 2% and occurred in the surgical group. There was no late mortality, in a mean follow-up of 7.2 +/- 5.4 years. Recoarctation occurred in 8 patients (6 surgical, 2 percutaneous). There are 46 patients who currently have hypertension (19 at rest, 27 with effort), their median age at diagnosis being older than the others (23 vs. 995 days; p < 0.01). CONCLUSIONS: Isolated CoA has an excellent short-term prognosis but a significant incidence of long-term complications, and should thus no longer be seen as a simple obstruction in the descending aorta, but rather as a complex pathology that requires careful follow-up after treatment. Its potentially insidious presentation requires a high level of clinical suspicion, femoral pulse palpation during physical examination of newborns and older children being particularly important. Delay in treatment has an impact on late morbidity and mortality. Taking into account the data currently available on late and immediate results, the final choice of therapeutic technique depends on the patient's age, associated lesions and the experience of the medical-surgical team. Hypertension should be closely monitored in the follow-up of these patients, as well as its risk factors and complications.

Volumoso Pseudoaneurisma do Ventrículo Esquerdo após Cirurgia Cardíaca

A 75-year old female patient, with previous inferior acute myocardial infarction (AMI) in December 2000, was admitted in April 2001 with angina and heart failure. Transthoracic echocardiography (TTE) was suggestive of a postero-inferior pseudoaneurysm (PA) of the left ventricle (LV), with 61x49 mm. of size and mitral regurgitation. Cardiac catheterization was suspected of a PA of the LV and revealed a three vessels coronary artery disease. On 20th April she was submitted to cardiac surgery with resection of a large LV aneurysm (AN) and triple coronary artery bypass surgery. Afterwards, she was on NYHA class III and subsequent TTE and transesophagic echocardiography (TEE) were suggestive of a 90x60 mm LV posterior PA (confirmed by nuclear magnetic resonance) and severe mitral regurgitation, with good LV systolic function. She underwent a new cardiac surgery on 31st May 2002, with resuturing of the LV postero-inferior wall patch and removal of the PA. The patient is in good condition and on NYHA functional class I-II.

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.

Endocardite por Cândida Parapsilosis Após Correcção Total de Tetralogia de Fallot. Tratamento Médico-Cirúrgico

São apresentados três casos de endocardite por Cândida Parapsilosis que surgiram em crianças com idade entre os sete e os nove anos, após terem sido submetidas a correcção total de Tetralogia de Fallot. As três crianças foram reoperadas, tendo recebido previamente uma delas terapêutica médica com anfotericina B e duas exclusivamente com Ketoconazol oral. Após negativação das hemoculturas foi efectuada remoção cirúrgica das vegetações com substituição do patch septal de dacron. A terapêutica com Ketoconazol prosseguiu durante 24 meses, com follow-up de 30 a 42 meses, não se tendo verificado nem reinfecção nem efeitos secundários da terapêutica. A ecocardiografia bidimensional revelou-se um método eficaz no diagnóstico e seguimento a longo prazo. A terapêutica médico-cirúrgica combinada, com timing cirúrgico baseado em dados clínicos e laboratoriais foi fundamental para os bons resultados, estando as crianças actualmente curadas.

Melanoma and Satellite Blue Papule

The colors that are seen in dermoscopy depend on the anatomic level of the skin at which the chromophores are seen. Blue color can be found in a variety of melanocytic and nonmelanocytic lesions. An 89-year-old man presented with a 3-year history of a slow-growing, hyperpigmented patch located on the distal third of the right arm. Dermoscopy showed an atypical network, irregularly distributed globules, pigmented internal streaks and a milky-red area. Based on these findings a diagnosis of slow-growing malignant melanoma was made. Simultaneously, a well-defined blue papule was seen on the proximal third of the same arm. Dermoscopy disclosed a homogeneous blue pattern. After clinical and dermoscopic correlation our differential diagnosis for this blue lesion included cutaneous melanoma metastasis, blue nevus and foreign body reaction. The patient recalled its onset 75 years ago after a grenade explosion. We also discuss the blue lesion appearance under reflectance confocal microscopy and high-definition optical coherence tomography. Histopathological examination after excision of the hyperpigmented patch and blue papule revealed a melanoma in situ and a foreign body reaction, respectively. The diagnostic evaluation of a blue lesion should always rely on the integration of all data, especially clinical and dermoscopic features. Other non-invasive techniques, like reflectance confocal microscopy and high-definition optical coherence tomography can also be important aids for its differential diagnosis.

Provas Epicutâneas sob Imunossupressão Sistémica - Contra-Indicação Absoluta?

Introdução: As provas epicutâneas (PE) são o exame complementar de diagnóstico indicado para avaliação de suspeita de dermite de contacto alérgica. Idealmente, devem ser realizadas sem que o doente se encontre sob imunossupressores. Existem contudo situações clínicas em que tal não é possível, não havendo informação disponível acerca de como realizar e valorizar os resultados das PE nestes doentes. O objectivo do presente trabalho é rever a literatura no que concerne à realização de PE sob imunossupressão iatrogénica. Material e Métodos: Revisão da literatura relevante para o tema publicada até Janeiro de 2015 e indexada à Medline. Resultados: De acordo com o reportado na literatura, foram realizadas PE em 77 doentes sob corticóide sistémico, 78 doentes sob ciclosporina (CyA), 6 sob azatioprina, 10 sob metotrexato (MTX), 4 sob micofenolato de mofetil (MMF), 11 sob fármacos anti-factor de necrose tumoral e 7 sob fármaco anti-IL-12/23. Foram ainda descritos 15 casos de realização de PE sob associação de imunossupressores. Verificaram-se reacções positivas em todos os grupos. Conclusão: O tratamento concomitante com imunossupressores não deve ser uma contra-indicação para realização de PE, estando descritas reacções positivas em doentes sob prednisolona, azatioprina, CyA, MTX, MMF, infliximab, etanercept, adalimumab e ustecinumab. Os resultados negativos ou duvidosos devem, contudo, ser interpretados de forma cautelosa.