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

• Autor(a) principal: Andrade,Guilherme Cabral de
• Data de Publicação: 2003
• Outros Autores: Braga,Otávio Piccirillo, Hisatugo,Marcelo Ken-Iti, Paiva Neto,Manuel Antônio de, Succi,Ernesto, Braga,Fernando Menezes
• Tipo de documento: Relatório
• Idioma: eng
• Título da fonte: Arquivos de neuro-psiquiatria (Online)
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2003000400029
• topic: intrathoracic meningocele; neurofibromatosis type I

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.