[A case of neurofibromatosis with intrathoracic meningocele presenting respiratory failure caused by pleural effusion]

Abstract

We reported a 58-year-old man with neurofibromatosis and an intrathoracic meningocele. He was admitted to our hospital because of left-sided chest pain and dyspnea on exertion. He presented with severe kyphoscoliosis and showed a round, well circumscribed mass lesion in the paravertebral region of the left upper lung on a chest roentgenogram. Just before admission, pleural effusion accumulated in the left thoracic cavity, which had caused the respiratory symptoms. The mass was diagnosed as an intrathoracic meningocele by MRI and iotrolan CT myelography. The pleural effusion was transudate fluid and no leakage from the meningocele to pleural cavity was demonstrated. Posterolateral extradural approach with laminectomy was done and dural plasty to close the connection between the meningocele and the subarachnoidal space was carried out. After the operation, both the intrathoracic meningocele and the pleural effusion disappeared with remarkable improvement in the respiratory function. Intrathoracic meningocele is known to be seen in association with neurofibromatosis and scoliosis, but it is very rare to see an intrathoracic meningocele which causes respiratory failure due to massive pleural effusion like this report.