Survival after decompressive hemicraniectomy was better than previously reported using medical management alone. A vegetative state was avoided and functional independence was possible, especially in younger patients. Increasing age was a statistically significant predictor of disability and long-term functional dependence.

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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.

The authors present the case of an 11-year-old boy with an intraventricular chordoid meningioma, which is a rare presentation of prolonged fever of unknown origin due to a rare tumor in a rare location. The fever resolved after excision of the lesion. Subsequent imaging revealed recurrence at 1 year. After a repeat excision and fractionated radiotherapy, the patient has remained disease free 5 years after the first surgery. Very few cases of intraventricular chordoid meningioma have been reported to date. The pathological features and clinical course are described. A review of the literature describing management options for this tumor type, recently found to have a higher recurrence rate, is described herein.

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