Giant intrathoracic meningoceles associated with cutaneous neurofibromatosis type I: case report

Giant intrathoracic meningoceles associated with cutaneous neurofibromatosis type I: case report

Título alternativo Meningoceles intratorácicas gigantes associadas a neurofibromatose tipo I: relato de caso
Autor Andrade, Guilherme Cabral de Autor UNIFESP Google Scholar
Braga, Otávio Piccirillo Autor UNIFESP Google Scholar
Hisatugo, Marcelo Ken-Iti Autor UNIFESP Google Scholar
Paiva Neto, Manoel Antonio de Autor UNIFESP Google Scholar
Succi, Ernesto Autor UNIFESP Google Scholar
Braga, Fernando Menezes Autor UNIFESP Google Scholar
Instituição Universidade Federal de São Paulo (UNIFESP)
Resumo BACKGROUND: Intrathoracic meningocele is a rare pathology, almost always associated with neurofibromatosis type I and with a few cases related in the literature. In the majority of cases cysts are small or asymptomatic, and the surgery is indicated when big or symptomatic cysts are present. We report a case of giant intrathoracic cysts surgically extirpated through out thoracotomy. CASE: A 59-year-old male with familiar Von Recklinghausen's disease which developed thoracic radicular pain after a fall. On examination he presented some difficulty in walking fast and dyspneia on small efforts. The chest plain x-ray showed the presence of 3 huge left side intrathoracic cysts (10 to 15cm). The patient was submitted to a surgical treatment and complete extirpation of the cysts was performed through a left side thoracotomy. During the surgery a fourth smaller cyst was detected and also extirpated. Evolution was uneventful and the patient remains well in these last 12 years. This finding of intrathoracic cysts related to neurofibromatosis type I is rare and is probably unique in the literature the presence of 4 huge cysts in one side of the thorax.

Meningocele intratorácica é patologia rara e, quando presente, está quase sempre associada a neurofibromatose tipo I. Há poucos casos descritos na literatura e em sua maioria destes são de cistos pequenos ou assintomáticos, sendo a cirurgia indicada quando são grandes ou sintomáticos. Relatamos o caso de um homem de 59 anos com a doença de Von Recklinghausen familiar, com cistos gigantes extirpados cirurgicamente através de toracotomia.
Palavra-chave intrathoracic meningocele
neurofibromatosis type I
meningocele intratorácica
neurofibromatose tipo I
Idioma Inglês
Data de publicação 2003-09-01
Publicado em Arquivos de Neuro-Psiquiatria. Academia Brasileira de Neurologia - ABNEURO, v. 61, n. 3A, p. 677-681, 2003.
ISSN 0004-282X (Sherpa/Romeo, fator de impacto)
Publicador Academia Brasileira de Neurologia - ABNEURO
Extensão 677-681
Fonte http://dx.doi.org/10.1590/S0004-282X2003000400029
Direito de acesso Acesso aberto Open Access
Tipo Artigo
Web of Science WOS:000186186300029
SciELO S0004-282X2003000400029 (estatísticas na SciELO)
Endereço permanente http://repositorio.unifesp.br/handle/11600/1825

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