Management of Intrathoracic Huge Meningocele in a Case of Neurofibromatosis Type-1, Report of a Case and Review of the Literature

Background: Meningocele is the extension of the dural sac into the pleural or peritoneal cavity. Thoracic meningoceles mainly occur in neurofibromatosis type-1 (NF-1). It always coincides with kyphosis/scoliosis. Because the initial symptoms are respiratory problems, they are usually misdiagnosed as a massive pleural effusion at first. Case Presentation: This is a 60-year-old man, a known case of NF-1, who presented with flank pain 8 months before surgery. As an incidental finding in the sonographic evaluation of the urinary system, he was diagnosed and managed as a massive pleural effusion for months until referred to our tertiary center. After further evaluations, a right thoracic huge meningocele was diagnosed. Posterolateral approach for laminectomy, costotransversectomy, ligation of the ectatic sleeves of the spinal nerve roots, cyst resection, dural repair, and spinal fusion was utilized. A pleural and lumbar draining tube was applied for 8 days. The patient recovered completely from his respiratory problems, without any neurological deficit or catastrophic events. Conclusion: Thoracic meningoceles limit the expansion of the lungs. The diagnosis and management are always challenging. Management of this pathology should include resection of the cyst to decompress the neighboring organs, repair of the dural sac, and reconstruction of the spinal canal. A two-step posterolateral approach was utilized: first, laminectomy and costotransversectomy with ligation of ectatic nerve roots; second, definitive ligation of the fistula, cyst resection, dural repair, and spinal fusion. Although the posterolateral approach typically allows single-stage management, complex anatomy may necessitate a planned second surgery after reassessment with cisternography. Keywords: neurofibromatosis-1, meningocele, scoliosis, massive pleural effusion