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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A s&#237;ndroma de Marfan &#233; uma doen&#231;a do tecido conjuntivo de transmiss&#227;o autoss&#243;mica dominante&#44; com manifesta&#231;&#245;es esquel&#233;ticas&#44; oculares e cardiovasculares t&#237;picas&#46; Com menor frequ&#234;ncia&#44; verifica-se envolvimento pulmonar&#44; nomeadamente pneumot&#243;rax espont&#226;neo&#46; Este &#233; habitualmente recorrente&#44; surgindo em 4&#8211;11&#37; dos casos&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Descreve-se um adolescente de 14 anos&#44; sem diagn&#243;stico anterior de doen&#231;a&#44; internado por pneumot&#243;rax espont&#226;neo unilateral&#44; com fen&#243;tipo de s&#237;ndroma de Marfan&#46; A investiga&#231;&#227;o subsequente conduziu ao diagn&#243;stico de prolapso da v&#225;lvula mitral e dilata&#231;&#227;o da raiz da aorta&#46; Houve resolu&#231;&#227;o do pneumot&#243;rax atrav&#233;s de drenagem activa e exclu&#237;ram-se&#44; por tomografia computorizada&#44; les&#245;es bolhosas subpleurais&#46; O doente foi reinternado&#44; dois meses depois&#44; por novo pneumot&#243;rax espont&#226;neo&#44; com id&#234;ntica localiza&#231;&#227;o&#44; procedendo-se a ressec&#231;&#227;o cir&#250;rgica de bolha enfisematosa ent&#227;o identificada e pleurodese&#46; Dois anos depois encontra-se assintom&#225;tico&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Real&#231;a-se a import&#226;ncia do diagn&#243;stico precoce e acompanhamento multidisciplinar destes doentes&#46; A monitoriza&#231;&#227;o da progress&#227;o da doen&#231;a e a preven&#231;&#227;o de complica&#231;&#245;es graves&#44; nomeadamente cardiovasculares&#44; s&#227;o imprescind&#237;veis&#46;</p>"
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Vol. 14. Issue 5.
Pages 699-704 (September - October 2008)
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Vol. 14. Issue 5.
Pages 699-704 (September - October 2008)
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Pneumotórax espontâneo – A pista para outro diagnóstico
Spontaneous pneumothorax – a clue to another diagnosis
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Susana Rocha1, Luísa Pereira2, Celeste Barreto3, Doutor Gomes-Pedro4
1 Interna do Internato Complementar de Pediatria
2 Assistente Hospitalar Graduada de Pediatria
3 Chefe de Serviço de Pediatria
4 Unidade de Pneumologia Pediátrica, Serviço de Pediatria do Hospital de Santa Maria, Avenida Professor Egas Moniz, 1649-035 Lisboa
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Resumo

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.

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.

Palavras-chave:
Pneumotórax espontâneo
síndroma de Marfan
diagnóstico
terapêutica
Abstract

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.

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.

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.

Key-words:
Spontaneous pneumothorax
Marfan syndrome
diagnosis
management
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Copyright © 2008. Sociedade Portuguesa de Pneumologia
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