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

2003; Thieme Medical Publishers (Germany); Volume: 61; Issue: 3A Linguagem: Inglês

10.1590/s0004-282x2003000400029

1678-4227

Guilherme Cabral de Andrade, Otávio Piccirillo Braga, Marcelo Ken-Iti Hisatugo, Manuel Antônio de Paiva Neto, Ernesto Succi, Fernando Menezes Braga,

Neuroblastoma Research and Treatments

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.