Caso Clínico/Clinical Case
Pneumotórax espontâneo – A pista para outro diagnóstico
Spontaneous pneumothorax – a clue to another diagnosis
1 Interna do Internato Complementar de Pediatria, Unidade de Pneumologia Pediátrica, Serviço de Pediatria do Hospital de Santa Maria
2 Assistente Hospitalar Graduada de Pediatria, Unidade de Pneumologia Pediátrica, Serviço de Pediatria do Hospital de Santa Maria
3 Chefe de Serviço de Pediatria, Unidade de Pneumologia Pediátrica, Serviço de Pediatria do Hospital de Santa Maria
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"titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Spontaneous pneumothorax – a clue to another diagnosis" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2008-04-01" "fechaAceptado" => "2008-05-12" "PalabrasClave" => array:2 [ "pt" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palavras-chave" "identificador" => "xpalclavsec570144" "palabras" => array:4 [ 0 => "Pneumotórax espontâneo" 1 => "síndroma de Marfan" 2 => "diagnóstico" 3 => "terapêutica" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key-words" "identificador" => "xpalclavsec570145" "palabras" => array:4 [ 0 => "Spontaneous pneumothorax" 1 => "Marfan syndrome" 2 => "diagnosis" 3 => "management" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<span id="as0005" class="elsevierStyleSection elsevierViewall"><p id="sp0005" class="elsevierStyleSimplePara elsevierViewall">A síndroma de Marfan é uma doença do tecido conjuntivo de transmissão autossómica dominante, com manifestações esqueléticas, oculares e cardiovasculares típicas. Com menor frequência, verifica-se envolvimento pulmonar, nomeadamente pneumotórax espontâneo. Este é habitualmente recorrente, surgindo em 4-11% dos casos. Descreve-se um adolescente de 14 anos, sem diagnóstico anterior de doença, internado por pneumotórax espontâneo unilateral, com fenótipo de síndroma de Marfan. A investigação subsequente conduziu ao diagnóstico de prolapso da válvula mitral e dilatação da raiz da aorta. Houve resolução do pneumotórax através de drenagem activa e excluíram-se, por tomografia computorizada, lesões bolhosas subpleurais. O doente foi reinternado, dois meses depois, por novo pneumotórax espontâneo, com idêntica localização, procedendo-se a ressecção cirúrgica de bolha enfisematosa então identificada e pleurodese. Dois anos depois encontra-se assintomático.</p><p id="sp0010" class="elsevierStyleSimplePara elsevierViewall">Realça-se a importância do diagnóstico precoce e acompanhamento multidisciplinar destes doentes. A monitorização da progressão da doença e a prevenção de complicações graves, nomeadamente cardiovasculares, são imprescindíveis.</p><p id="sp0015" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2008; XIV (5): 699-704</span></p></span>" ] "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="as0010" class="elsevierStyleSection elsevierViewall"><p id="sp0020" class="elsevierStyleSimplePara elsevierViewall">Marfan syndrome is an autosomal dominant illness of the connective tissue, with typical skeletal, ocular and cardiovascular manifestations. Less frequently, pulmonary involvement occurs, namely spontaneous pneumothorax, which generally is recurrent and occurs in 4-11% of cases.</p><p id="sp0025" class="elsevierStyleSimplePara elsevierViewall">The authors describe a 14-year-old boy, previously healthy, admitted with a unilateral spontaneous pneumothorax, and clinical phenotype of Marfan syndrome. The subsequent investigation led to the diagnosis of mitral valve prolapse and dilatation of the root of aorta. Pneumothorax resolved through active drainage and a computerised tomography scan excluded subpleural blebs. The patient was readmitted two months later with a new spontaneous pneumothorax, with an identical localization. Surgical resection of a newly identified enfisematous bleb and pleurodesis were performed. Two years later he is asymptomatic.</p><p id="sp0030" class="elsevierStyleSimplePara elsevierViewall">We highlight the importance of an early diagnosis of and a multidisciplinary approach to these patients. Monitoring illness progression and prevention of serious complications, namely cardiovascular, are essential.</p><p id="sp0035" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Rev Port Pneumol 2008; XIV (5): 699-704</span></p></span>" ] ] "lecturaRecomendada" => array:1 [ 0 => array:3 [ "vista" => "all" "titulo" => "<span class="elsevierStyleSectionTitle" id="st0025">Bibliografia</span>" "seccion" => array:1 [ 0 => array:2 [ "vista" => "all" "bibliografiaReferencia" => array:17 [ 0 => array:3 [ "identificador" => "bb0005" "etiqueta" => "1." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Marfan syndrome: clinical diagnosis and management" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "J.C.S. 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Sonnappa" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Pediatric Respiratory Reviews" "fecha" => "2005" "volumen" => "6" "paginaInicial" => "181" "paginaFinal" => "189" ] ] ] ] ] ] 15 => array:3 [ "identificador" => "bb0080" "etiqueta" => "16." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "BTS guidelines for the management of spontaneous pneumothorax" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "M. Henry" 1 => "T. Arnold" 2 => "J. Harvey" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Thorax" "fecha" => "2003" "volumen" => "58" "paginaInicial" => "39" "paginaFinal" => "52" ] ] ] ] ] ] 16 => array:3 [ "identificador" => "bb0085" "etiqueta" => "17." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Persistent spontaneous pneumothorax in an adolescent with Marfan’s Syndrome and Pulmonary Bullous Dysplasia" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "D. Rigante" 1 => "G. Segni" 2 => "A. 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Article information
ISSN: 08732159Original language: Portuguese
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