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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.

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Abstract 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.

Resumo 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.

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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.

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Abstract 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.

Resumo 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.

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ABSTRACT To evaluate the findings on chest CTs in 16 patients (8 men and 8 women) with laryngotracheobronchial papillomatosis. Methods: This was a retrospective study involving patients ranging from 2 to 72 years of age. The evaluation of the CT scans was independently performed by two observers, and discordant results were resolved by consensus. The inclusion criteria were presence of abnormalities on the CT scans, and the diagnosis was confirmed by anatomopathological examination of the papillomatous lesions. Results: The most common symptoms were hoarseness, cough, dyspnea, and recurrent respiratory infections. The major CT findings were nodular formations in the trachea, solid or cavitated nodules in the lung parenchyma, air trapping, masses, and consolidation. Nodular formations in the trachea were observed in 14 patients (87.5%). Only 2 patients had lesions in lung parenchyma without tracheal involvement. Only 1 patient had no pulmonary dissemination of the disease, showing airway involvement only. Solid and cavitated lung nodules were observed in 14 patients (87.5%) and 13 (81.2%), respectively. Masses were observed in 6 patients (37.5%); air trapping, in 3 (18.7%); consolidation in 3 (18.7%); and pleural effusion, in 1 (6.3%). Pulmonary involvement was bilateral in all cases. Conclusions: The most common tomography findings were nodular formations in the trachea, as well as solid or cavitated nodules and masses in the lung parenchyma. Malignant transformation of the lesions was observed in 5 cases.

RESUMO Objetivo: Analisar os achados em TCs de tórax em 16 pacientes (8 homens e 8 mulheres) com papilomatose laringotraqueobrônquica. Métodos: Estudo retrospectivo que incluiu pacientes com idade variando de 2 a 72 anos. As imagens de TC foram avaliadas por dois observadores, de forma independente, e os casos discordantes foram resolvidos por consenso. Os critérios de inclusão foram presença de anormalidades na TC, e o diagnóstico foi confirmado por exame anatomopatológico das lesões papilomatosas. Resultados: Os sintomas mais frequentes foram rouquidão, tosse, dispneia e infecções respiratórias de repetição. Os principais achados na TC foram formações nodulares na traqueia, nódulos sólidos e/ou escavados no parênquima pulmonar, aprisionamento aéreo, massas e consolidação. Formações nodulares na traqueia foram observadas em 14 pacientes. Somente 2 pacientes apresentaram lesões parenquimatosas pulmonares na ausência de acometimento traqueal, e 1 paciente não apresentou disseminação pulmonar da doença, somente comprometimento de via aérea. Nódulos sólidos no parênquima pulmonar foram observados em 14 pacientes (87,5%) e nódulos escavados em 13 (81,2%). Massas foram observadas em 6 pacientes (37,5%); aprisionamento aéreo, em 3 (18,7%); consolidação, em 3 (18,7%); e derrame pleural, em 1 (6,3%). O comprometimento pulmonar foi bilateral em todos os pacientes. Conclusões: Os achados tomográficos mais frequentes foram formações nodulares na traqueia, nódulos sólidos e/ou escavados no parênquima pulmonar e massas parenquimatosas. A transformação maligna das lesões foi observada em 5 casos.

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To evaluate the findings on chest CTs in 16 patients (8 men and 8 women) with laryngotracheobronchial papillomatosis. This was a retrospective study involving patients ranging from 2 to 72 years of age. The evaluation of the CT scans was independently performed by two observers, and discordant results were resolved by consensus. The inclusion criteria were presence of abnormalities on the CT scans, and the diagnosis was confirmed by anatomopathological examination of the papillomatous lesions. The most common symptoms were hoarseness, cough, dyspnea, and recurrent respiratory infections. The major CT findings were nodular formations in the trachea, solid or cavitated nodules in the lung parenchyma, air trapping, masses, and consolidation. Nodular formations in the trachea were observed in 14 patients (87.5%). Only 2 patients had lesions in lung parenchyma without tracheal involvement. Only 1 patient had no pulmonary dissemination of the disease, showing airway involvement only. Solid and cavitated lung nodules were observed in 14 patients (87.5%) and 13 (81.2%), respectively. Masses were observed in 6 patients (37.5%); air trapping, in 3 (18.7%); consolidation in 3 (18.7%); and pleural effusion, in 1 (6.3%). Pulmonary involvement was bilateral in all cases. The most common tomography findings were nodular formations in the trachea, as well as solid or cavitated nodules and masses in the lung parenchyma. Malignant transformation of the lesions was observed in 5 cases.

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