Neurofibromatosis type 1: evaluation by chest computed tomography.

OBJECTIVE: The aim of this study was to evaluate chest computed tomography (CT) findings in patients diagnosed with neurofibromatosis type 1 (NF1). MATERIAL AND METHODS: This was a retrospective study in which we reviewed the chest CT scans of 14 patients diagnosed with NF1 and neurofibromatosis-associated diffuse lung disease (NF-DLD). The sample comprised eight women and six men. The median age was 55 years (range, 11-75 years). The diagnosis of NF1 was made on the basis of the diagnostic criteria established by the U.S. National Institutes of Health. The images were analyzed by two chest radiologists, who reached decisions by consensus. RESULTS: The predominant CT finding of NF-DLD was multiple cysts, which were observed in 13 patients (92.9%), followed by emphysema, in eight (57.1%) and subpleural bullae, in six (42.9%). Other findings included subcutaneous neurofibromas, in 12 patients (85.7%), ground-glass opacities, in one (7.1%), and tracheobronchial neurofibromas, in one (7.1%). The pulmonary abnormalities were bilateral in 12 cases (85.7%). The abnormalities were predominantly in the upper lung fields in eight cases (57.1%), and their distribution was random in 11 (78.6%). CONCLUSION: Pulmonary cysts, emphysema, and subpleural bullae appear to be the chest CT findings that are most characteristic of NF-DLD.

OBJETIVO: O objetivo deste estudo foi avaliar os achados pulmonares na tomografia computadorizada do tórax de pacientes diagnosticados com neurofibromatose tipo 1 (NF1). MATERIAIS E MÉTODOS: Foram revisados, retrospectivamente, os achados tomográficos de 14 pacientes com doença pulmonar difusa associada à NF1 (NF-DPD). A amostra incluiu oito mulheres e seis homens, com idade entre 11 e 75 anos (mediana de idade de 55 anos). O diagnóstico foi estabelecido com base em critérios diagnósticos predeterminados pelo National Institutes of Health dos Estados Unidos. As imagens foram analisadas de forma independente por dois radiologistas, que chegaram a um consenso. RESULTADOS: Os achados tomográficos predominantes foram múltiplos cistos em 13 pacientes (92,9%), enfisema em oito (57,1%) e bolhas subpleurais em seis (42,9%). Achados associados incluíram neurofibromas cutâneos e subcutâneos em 12 pacientes (85,7%), opacidades em vidro fosco em um (7,1%) e neurofibromas traqueobrônquicos em um (7,1%). As anormalidades pulmonares foram bilaterais em 12 casos (85,7%). Houve predomínio nos terços superiores em oito (57,1%) pacientes e se distribuíram randomicamente pelos pulmões em 11 (78,6%). CONCLUSÃO: Os achados tomográficos pulmonares mais frequentes na NF-DPD foram os cistos pulmonares, o enfisema e as bolhas subpleurais.

Consensus statement on thoracic radiology terminology in Portuguese used in Brazil and in Portugal.

Effective communication among members of medical teams is an important factor for early and appropriate diagnosis. The terminology used in radiology reports appears in this context as an important link between radiologists and other members of the medical team. Therefore, heterogeneity in the use of terms in reports is an important but little discussed issue. This article is the result of an extensive review of nomenclature in thoracic radiology, including for the first time terms used in X-rays, CT, and MRI, conducted by radiologists from Brazil and Portugal. The objective of this review of medical terminology was to create a standardized language for medical professionals and multidisciplinary teams.

Tomographic findings in bronchial atresia.

OBJECTIVE: To evaluate computed tomography (CT) findings in 23 patients with bronchial atresia. MATERIALS AND METHODS: The CT images were reviewed by two radiologists who reached decisions by consensus. We included only patients who presented with abnormalities on CT and in whom the diagnosis had been confirmed by pathological examination of the surgical specimen (if the lesion was resected). The CT scans were assessed in order to identify the main findings and to map the distribution of the lesions (i.e., to determine whether the pulmonary involvement was unilateral or bilateral). RESULTS: The main CT finding was the combination of bronchocele and hyperinflation of the distal lung. That combination was observed in all of the patients. The lesions were unilateral in all 23 cases, being seen predominantly in the left upper lobe, followed by the right lower lobe, right upper lobe, middle lobe, and left lower lobe. CONCLUSION: The diagnosis of bronchial atresia can be reliably made on the basis of a finding of bronchocele accompanied by hyperinflation of the adjacent lung parenchyma.

OBJETIVO: Analisar os achados na tomografia computadorizada (TC) de tórax de 23 pacientes com atresia brônquica. MATERIAIS E MÉTODOS: As imagens de TC foram avaliadas por dois observadores e os casos discordantes foram resolvidos por consenso. Os critérios de inclusão foram a presença de anormalidades na TC compatíveis com atresia brônquica e/ou diagnóstico confirmado por exame anatomopatológico das peças cirúrgicas para os pacientes submetidos a ressecção cirúrgica. As TCs foram avaliadas quanto aos principais achados de imagem, à distribuição das lesões, ao envolvimento pulmonar unilateral ou bilateral. RESULTADOS: Os principais achados na TC foram a presença de broncocele, hiperinsuflação do parênquima pulmonar ou ambos. A combinação desses achados foi encontrada em todos os pacientes. Em relação à distribuição, o envolvimento foi unilateral nos 23 casos. Quando se consideraram os lobos mais acometidos, o lobo superior esquerdo foi o mais acometido, seguido do lobo inferior direito, lobo superior direito, lobo médio e lobo inferior esquerdo. CONCLUSÃO: O diagnóstico de atresia brônquica pode ser feito em presença de broncocele associada com hiperinsuflação do parênquima pulmonar adjacente.

Saccular Superior Vena Cava Aneurysm: Case Report and Comprehensive Review.

Saccular superior vena cava aneurysms (SVCAs) are one of the rarest causes of mediastinal masses. Seventy years after the first published case report, to the best of our knowledge, fewer than 30 cases have been described in the literature. This article provides an overview of the peculiarities regarding diagnosis and treatment performed in these cases and addresses the protocol adopted in our case report. We present a saccular aneurysm resected through median sternotomy, under cardiopulmonary bypass and superior vena cava reconstruction using a bovine pericardial patch.

Superior vena cava aneurysm: an unusual mediastinal mass.

Superior vena cava aneurysms are rare mediastinal vascular lesions. Aneurysms are classified as fusiform and saccular, with the latter being rarer than the former. We report the case of an asymptomatic 75-year-old woman who presented with a superior mediastinal mass. She underwent chest computed tomography angiography, which demonstrated a saccular aneurysm in the superior vena cava.

Lymphocytic interstitial pneumonia: computed tomography findings in 36 patients.

OBJECTIVE: To analyze the computed tomography (CT) findings of lymphocytic interstitial pneumonia (LIP). MATERIALS AND METHODS: We retrospectively reviewed the clinical and CT findings of 36 patients with LIP, including 25 women and 11 men, with a mean age of 52.5 years (age range, 22-78 years). RESULTS: The main associated diseases with LIP were Sjögren syndrome (42%), human immunodeficiency virus infection (17%), amyloidosis (17%), Sjögren syndrome associated with secondary amyloidosis (11%), idiopathic (8%), and systemic lupus erythematosus (5%). The predominant CT abnormalities were multiple cystic airspaces (n = 35), small nodules (n = 15), ground-glass opacities (n = 13), bronchiectasis and/or bronchiolectasis (n = 8), and thickening of the bronchovascular bundles (n = 8). Other CT findings included reticular opacities (n = 7), calcified nodules (n = 4), airspace consolidation (n = 4), emphysema (n = 3), honeycombing (n = 3), lymph node enlargement (n = 2), mosaic attenuation pattern (n = 1), and cavitated nodules (n = 1). CONCLUSION: The main CT findings of LIP were multiple cysts, small nodules, and ground-glass opacities.

OBJETIVO: Analisar os achados da tomografia computadorizada da pneumonia intersticial linfocítica. MATERIAIS E MÉTODOS: Trinta e seis pacientes com diagnóstico de pneumonia intersticial linfocítica (25 mulheres, 11 homens; faixa etária de 22 a 78 anos; idade média de 52,5 anos) tiveram os achados clínicos e tomográficos revisados retrospectivamente. RESULTADOS: As principais doenças associadas à pneumonia intersticial linfocítica foram síndrome de Sjögren (42%), infecção pelo HIV (17%), amiloidose (17%), síndrome de Sjögren associada a amiloidose secundária (11%), síndrome de Sjögren idiopática (8%) e lúpus eritematoso sistêmico (5%). As alterações predominantes na TC consistiram em múltiplos espaços aéreos císticos (n = 35), pequenos nódulos (n = 15), opacidades em vidro fosco (n = 13), bronquiectasias e bronquiolectasias (n = 8) e espessamento de feixes broncovasculares (n = 8). Outros achados da TC incluíram opacidades reticulares (n = 7), nódulos calcificados (n = 4), consolidação do espaço aéreo (n = 4), enfisema (n = 3), faveolamento (n = 3), linfonodomegalia linfática (n = 2), padrão de atenuação em mosaico (n = 1) e nódulos escavados (n = 1). CONCLUSÃO: Os principais achados tomográficos observados em pacientes com pneumonia intersticial linfocítica foram múltiplos cistos, pequenos nódulos e opacidades em vidro fosco.

Neurofibromatosis type 1: State-of-the-art review with emphasis on pulmonary involvement.

Neurofibromatosis type 1 (NF-1), also known as von Recklinghausen's disease, is an autosomal dominant dysplasia of the ectoderm and mesoderm with a variable clinical expression, but near-complete penetrance before the age of 5 years. The estimated incidence is 1 in 3000 births. NF-1 is characterized by collections of neurofibromas, café-au-lait spots, axillary and inguinal freckling, and pigmented hamartomas in the iris (Lisch nodules). Pulmonary manifestations of NF-1, which usually include bilateral basal reticulations and apical bullae and cysts, are reported in 10-20% of adult patients. Clinically, neurofibromatosis-associated diffuse lung disease (NF-DLD) usually presents with nonspecific respiratory symptoms, including dyspnea on exertion, shortness of breath, and chronic cough or chest pain, at the time of diagnosis. Computed tomography (CT) is highly accurate for the identification and characterization of NF-DLD; it is the most reliable method for the diagnosis of this lung involvement. Various CT findings of NF-DLD, including cysts, bullae, ground-glass opacities, bibasilar reticular opacities, and emphysema, have been described in patients with NF-1. The typical CT pattern, however, is characterized by upper-lobe cystic and bullous disease, and basilar interstitial lung disease. Currently, the goal of NF-DLD treatment is the earliest possible diagnosis, focusing on symptom relief and interventions that positively alter the course of the disease, such as smoking cessation. The aim of this review is to describe the main clinical, pathological, and imaging aspects of NF-1, with a focus on pulmonary involvement.

CT-guided Transthoracic Core-Needle Biopsies of Mediastinal and Lung Lesions in 235 Consecutive Patients: Factors Affecting the Risks of Complications and Occurrence of a Final Diagnosis of Malignancy.

OBJECTIVE: To assess the impact of patient-, lesion- and procedure-related factors on the risks of complications and final diagnosis of malignancy in PCNB of mediastinal and lung lesions. MATERIAL AND METHODS: We studied a large single-center cohort of 235 consecutive patients (66.8% men; 58.5±18.0 years) with a range of thoracic benign and malignant lesions, who underwent PCNB performed along 24 months by a single experienced radiologist. Diagnostic accuracy analyses of PCNB for malignancy were performed, as well as estimations of relative risk and logistic regression models in order to assess possible associations between such factors and malignancy/complications. RESULTS: 155 lesions (65.9%) were diagnosed as malignant. Overall accuracy was 91.1%, with sensitivity of 87.1%, specificity of 98.8%, positive predictive value of 99.3%, and negative predictive value of 79.8%. Pneumothorax (49/235; 20.8%) and hemorrhage (37/235; 15.7%) were the most common complications. Emphysema, smoking, older age, intrapulmonary location, deeper location, smaller size, presence of cavitations and irregular contours of the lesions, and smaller needle-pleural angles were the most consistent factors related to the occurrence of complications. Emphysema, older age, smoking, solid and deeper lesions were also significantly associated with a final diagnosis of malignancy after PCNB. CONCLUSION: CT-guided PCNB of mediastinal and lung lesions is a safe procedure with high diagnostic accuracy for malignancy.