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

Arq Neuropsiquiatr. 2003 Sep;61(3A):677-81. doi: 10.1590/s0004-282x2003000400029. Epub 2003 Sep 16.

Abstract

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Meningocele / complications*
  • Meningocele / surgery
  • Middle Aged
  • Neurofibromatosis 1 / complications*
  • Neurofibromatosis 1 / surgery
  • Thorax
  • Tomography, X-Ray Computed