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[A case of intrathoracic meningocele associated with neurofibromatosis]
T Sakamoto1, N Tsubota, M Yoshimura
1Department of General Thoracic Surgery, Hyogo Medical Center for Adult, Japan.
Abstract:
A rare case of intrathoracic meningocele associated with neurofibromatosis was reported. A 47-year-old female was admitted to our institute because of an abnormal shadow on chest X-ray. The chest X-ray film showed homogenous opacity with a well defined margin in the right apex. Chest CT showed an enlarged intervertebral foramen and defect of the vertebral arch around the tumor. There was no scoliosis. Though preoperative diagnosis was dumbbell type neurinoma, the tumor was found to be a protrusion of dura mater with spinal fluid out of the spinal canal, covered by thickening tumorous tissue. Part of the wall was excised and the residual opening was carefully repaired and wrapped by the pedicled parietal pleura. Retrospectively, MRI showed characteristic findings of intrathoracic meningocele. The tumorous part of it was diagnosed as neurofibroma on histological examination.
Insights
A rare intrathoracic meningocele, a protrusion of dura mater, was found in a neurofibromatosis patient. Surgical repair was performed, confirming the diagnosis via histology.
Area of Science:
- Neurosurgery
- Radiology
- Pathology
Background:
- Intrathoracic meningoceles are rare congenital abnormalities.
- Neurofibromatosis is a genetic disorder that affects nerve growth.
Observation:
- A 47-year-old female presented with an abnormal chest X-ray finding.
- Chest CT revealed an enlarged intervertebral foramen and vertebral arch defect.
- Preoperative diagnosis suggested a dumbbell-type neurinoma.
Findings:
- The tumor was identified as an intrathoracic meningocele, a protrusion of dura mater with cerebrospinal fluid.
- Histological examination of the tumorous tissue revealed neurofibroma.
- Surgical intervention involved excision and repair using pedicled parietal pleura.
Implications:
- This case highlights the importance of considering intrathoracic meningocele in the differential diagnosis of thoracic masses, especially in patients with neurofibromatosis.
- Characteristic MRI findings can aid in preoperative diagnosis.
- Successful surgical management is crucial for preventing complications.