Isolated true anterior thoracic meningocele (original) (raw)
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Neurosurgical Review, 1995
We report two cases of ventrolateral thoracic and lumbar meningoceles associated with neurofibromatosis type 1, and present a review of the literature. Thoracic and lumbar meningoceles are rare lesions which are associated with neurofibromatosis in 60% to 85% of cases. Large meningoceles may cause pain, neurological signs, or respirtory complaints when located intrathora-ciealLly. Surgery is indicated in such cases, as well as when small meningoceles enlarge with time. The etiology of meningoceles in neurofibromatosis remains controversial. The authors postulate that ventrolateral lesions of the thoracic and lumbar spine are secondary to congenital mesodermal dysplasia and hypoplastic bone changes. MRI is the diagnostic method of choice, since it most adequately visualizes the pathological anatomy of the spinal cord, meninges, and adjacent structures in every sectional plane. CT scans are particularly useful in assessing the spinal bony changes.
Thoracic Meningoceles in NF1: Case Report and Considerations for Surgical Management
2019
Thoracic meningoceles are rare entities seen in association with Neurofibromatosis type 1 (NF1). Although they uncommonly require resection, surgical techniques and postoperative management for these patients are highly varied and complex. Here, we report our experience with the first successful thoracotomy performed from a posterior-only approach for resection of a large thoracic meningocele. Additionally, we describe surgical and medical considerations for intra operative and postoperative management as well as potential complications. YouRong Sophie Su1*, Belinda Shao2, Taine TV Pechet3 and William C Welch1 1Department of Neurosurgery, Hospital of the University of Pennsylvania, USA 2Rutgers New Jersey Medical School, USA 3Department of Thoracic Surgery, University of Pennsylvania, USA
Giant intrthoracic meningoceles associated with cutaneous neurofibromatosis type I: case report
Arquivos de Neuro-Psiquiatria, 2003
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 findin...
Isolated giant intrathoracic meningocele associated with vertebral corpus deformity
Interactive cardiovascular and thoracic surgery, 2004
Published reports of intrathoracic meningocele with vertebral corpus defects in the absence of neurofibromatosis are very rare. We report a 9-year-old male with intrathoracic meningocele. We believe that vertebral corpus defects may play a certain role in the etiology of intrathoracic meningocele.
Giant true dorsal thoracic meningocele in a school-age child
Journal of Neurosurgery: Pediatrics, 2008
✓A meningocele is a common form of spinal dysraphic lesions, but it is extremely uncommon in children in the upper thoracic region. The authors describe a rare case in which they found a giant true dorsal meningocele in the upper thoracic region in a symptomatic child, which, to their knowledge, is the first such reported case. A school-age child, who harbored a giant dorsal mass, complained of restriction of function. He underwent successful surgery in which the meningocele sac was totally removed.
Case Report: A Large Intrathoracic Mass in A Patient With Neurofibromatosis Type 1
IRJNS Journal, 2021
Background and Importance: Dural ectasia is circumferential extension or dilatation of the dural sac, it is commonly associated with Neurofibromatosis type 1 (NF1). Because NF1 is associated with a high likelihood of tumor formation, it is critical to distinguish it from other posterior mediastinal cancers such as neurofibroma, neuroblastoma, and ganglioneuroma. Scoliosis is the most frequently observed feature in NF1 patients with spinal deformities. Moreover, there are several distinctive radiographic features, including dural ectasia, defective pedicles, and spondylolisthesis, which are relatively less common in these patients. Surgery may be performed for stabilization of the spine. Case Presentation: The present study reports on a 62-year-old female with neurofibromatosis type 1 who was referred for an unusual chest X-ray and chest Computed Tomography (CT) revealing a thoracic mass while she was hospitalized and under observation for dyspnea. The chest X-ray film showed homogenous opacity with a well-defined margin in the right apex. The chest CT showed an enlarged intervertebral foramen and defected vertebral arch around the mass and scoliosis. Although the pre-operative diagnosis was dumbbell type neurinoma, the tumor was found to be a protrusion of dura mater with spinal fluid out of the spinal canal. Part of the wall was excised, the residual opening was repaired, and surgical stabilization was performed. Conclusion: Retrospectively, magnetic resonance imaging showed the characteristics of thoracic dural ectasia and anterior meningocele, which, in an asymptomatic case, require regular radiographic follow-ups. Surgical intervention is an alternative for patients with spinal deformities or symptomatic patients due to the adverse effects of spinal cord compression and mediastinal structures.
Surgical Neurology, 2008
Background: Thoracic meningoceles are associated with neurofibromatosis 1 in 60% to 85% of all cases. Usually, these meningoceles remain asymptomatic, but back pain, headache, cough, and dyspnea are possible manifestations. Often, there is an associated kyphoscoliotic deformity of the thoracic spine. Case Description: A 60-year-old woman known in our department after a fossa posterior decompression for an Arnold-Chiari malformation was admitted through the emergency department because of progressive dyspnea. A giant intrathoracic meningocele was already diagnosed earlier but was left untreated because the patient was asymptomatic at that time. She now had dyspnea, and on chest x-ray and CT scan, there was an obvious shift of the mediastinum to the right.Because of the long-existing hemithoracic meningocele, we assumed that this patient actually had only 1 functional lung, and so, left-sided thoracotomy with resection of the meningocele and closure of the defect included a high operative mortality. Instead, we chose to obtain a permanent drainage of the meningocele by putting a shunt between the meningocele and the peritoneum. Postoperatively, the patient recovered well and became oxygen-independent. Conclusion: Treatment of giant intrathoracic meningoceles in patients with progressive dyspnea can be challenging, and different options can be found in the literature. Treatment with a cystoperVtoneal shunt, as in our case, can be a less invasive alternative in patients with a high operative mortality risk. To our knowledge, this is the first report of a patient with neurofibromatosis 1 treated in this way. D
Journal of Surgical Technique and Case Report, 2014
Neurofibromatosis type 1 (NF-1) is a heterogeneous autosomal dominant disease with an incidence ranging from 1 in 2500 to 1 in 3000. Rare intrathoracic vascular disorders resulting in massive spontaneous hemothorax with fatal consequences may occur in these patients, so also are various types of skeletal dysplasia which may result in dramatic presentations, posing management challenges to the attending physicians. We report the case of a 43-year-old woman with NF-1 who developed spontaneous massive hemothorax and was discovered to have a concurrent giant intrathoracic meningocele and thoracic kyphoscoliosis with severe vertebral dysplastic changes. Surgical treatment via a right thoracotomy with primary repair of the meningocele and spinal fusion with fibula graft resulted in good outcome. This case represents an extreme manifestation of this otherwise indolent disease in clinical practice.
Anterior thoracic myelomeningocele presenting as a retromediastinal mass
Journal of …, 2005
An 11-year-old boy with a completely normal systemic and neurological examination suffering pulmonary problems such as effort dyspnea and severe kyphosis was evaluated and an anterior thoracic myelomeningocele was diagnosed. The boy underwent surgery for excision of ...