Introduction: The neurofibromatosis (NF) (Neurofibromatosis types 1 and 2, and shwannomatosis) are rare, but they give rise to a greater tumor burden for the nervous system than any other neoplastic disease. NF1 has an incidence of 1 in 3000, inherited in autosomal dominant pattern, with an approximately 50 % de novo mutations in each, and is among the group of tumor-suppressor syndromes. Overall, it is an uncommon pathology, but 69 % of thoracic meningoceles are associated with NF1. Meningoceles have been reported in the cervical and lumbar spine as well; however, they are fairly uncommon.
Methods: This is a 60-year-old male with a medical history of Neurofibromatosis 1. He has been experiencing progressive dyspnea for several years and sought help at an outside hospital. A chest CT scan was performed, revealing a left chest mass lesion with a diameter of approximately 17 to 20 cm. Additionally, a spinal MRI showed multiple paraspinal cystic lesions at the T2-T9 level bilaterally, with the largest one located at the left T8-9 level.
Results: The patient underwent left posterolateral thoracotomy for excision of the left T8-9 huge paraspinal cystic lesion. The patient was discharged and follow-up in the Neurosurgery Outpatient Department. Post operation follow up at outpatient department, chest MRI revealed greater resolved of meningocele and his clinical condition was improved.
Conclusion : Large intrathoracic meningoceles are rare pathologies, and the optimal intra- and perioperative management is not well defined. We have revisited its formation mechanism, presentation, diagnosis, the choice of surgical approach, and intraoperative and perioperative management. We share our experience on using a combined intradural and thoracoscopic approach for definitive treatment of a large hemithoracic meningocele.
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