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Vol. 15. Issue 5.
Pages 937-941 (September - October 2009)
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Vol. 15. Issue 5.
Pages 937-941 (September - October 2009)
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Hemotórax espontâneo em doente com neurofibromatose tipo I – A propósito de um caso clínico
Spontaneous hemothorax in a neurofibromatosis type I patient – A case report
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Alexandra Bento1,
Corresponding author
alexitbento@gmail.com

Unidade de Saúde Local EPE, Guarda, Serviço de Pneumologia, Avenida Rainha D. Amélia, 6300 Guarda.
, Ana Paula Gonçalves2
1. Interna do Internato Complementar de Pneumologia
2. Assistente Graduada de Pneumologia
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Resumo

O hemotórax espontâneo é uma complicação rara e potencialmente fatal da neurofibromatose. Vários mecanismos patológicos são descritos para a vasculopatia associada à doença: a) invasão dos vasos por tumores adjacentes, como shwannoma, neurofibroma ou neurofibrosarcoma; b) displasia vascular com formação de aneurismas e estenoses6,7,9. Outros mecanismos envolvidos podem incluir patologias pleuropulmonares (infecções com necrose, embolia pulmonar, endometriose, neoplasias) e discrasia sanguínea. Os autores relatam o caso de uma jovem de 33 anos, que recorreu ao serviço de urgência por toracalgia superior direita, contínua, irradiando para o ombro e escápula homolaterais, sem factores de agravamento nem de alívio e com oito dias de evolução.

Palavras-chave:
Neurofibromatose
hemotórax espontâneo
Abstract

Spontaneous hemothorax is a rare and potentially lethal neurofibromatosis’ complication. Several pathological mechanisms may explain the associated vasculopathy: a) direct vascular invasion from adjacent tumors such as Shwannoma, neurofibroma or neurofibrosarcoma; b) vascular dysplasia with stenotic or aneurysm formation6,7,9. Other mechanisms involved may include pleuropulmonar pathologies (necrotizing infections, pulmonary embolism, endometriosis, neoplasms) and blood dyscrasias. The authors describe a case of a 33 years old female, who went to the ER Service complaining with right persistent hemithoracic pain, extending to the ipsilateral shoulder and shoulder blade, without aggravation or relieving factors, since the last 8 days.

Key-words:
Neurofibromatosis
spontaneous hemothorax
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Copyright © 2009. Sociedade Portuguesa de Pneumologia
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