A case of conservatively managed idiopathic spinal cord herniation presenting with low-pressure headache.

Idiopathic spinal cord herniation presenting with low-pressure headache is extremely rare. We present a case of thoracic ventral spinal cord herniation in a 35-year-old lady who presented with low-pressure headaches. To our knowledge, this is only the fourth case described in the literature of spontaneous ventral cord herniation presenting in this way. The patient was managed conservatively with no manifestation of focal neurological symptoms at 12-month follow-up. The proposed aetiology of spontaneous ventral cord herniation is an initial CSF leak via a dural defect, through which the cord subsequently also enters blocking the CSF leak. We endorse a conservative approach for patients who present similarly, secondary to the above pathophysiology.

Generating Operative Workflows for Vestibular Schwannoma Resection: A Two-Stage Delphi's Consensus in Collaboration with the British Skull Base Society. Part 2: The Translabyrinthine Approach.

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 2, we present a codified operative workflow for the translabyrinthine approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Seventeen consultant skull base surgeons (nine neurosurgeons and eight ENT [ear, nose, and throat]) with median of 13.9 years of experience (interquartile range: 18.1 years) of independent practice participated. There was a 100% response rate across both the Delphi rounds. The translabyrinthine approach had the following five phases and 57 unique steps: Phase 1, approach and exposure; Phase 2, mastoidectomy; Phase 3, internal auditory canal and dural opening; Phase 4, tumor debulking and excision; and Phase 5, closure. Conclusion We present Part 2 of a national, multicenter, consensus-derived, codified operative workflow for the translabyrinthine approach to vestibular schwannomas. The five phases contain the operative, steps, instruments, technique errors, and event errors. The codified translabyrinthine approach presented in this manuscript can serve as foundational research for future work, such as the application of artificial intelligence to vestibular schwannoma resection and comparative surgical research.

Generating Operative Workflows for Vestibular Schwannoma Resection: A Two-Stage Delphi's Consensus in Collaboration with the British Skull Base Society. Part 1: The Retrosigmoid Approach.

Objective An operative workflow systematically compartmentalizes operations into hierarchal components of phases, steps, instrument, technique errors, and event errors. Operative workflow provides a foundation for education, training, and understanding of surgical variation. In this Part 1, we present a codified operative workflow for the retrosigmoid approach to vestibular schwannoma resection. Methods A mixed-method consensus process of literature review, small-group Delphi's consensus, followed by a national Delphi's consensus, was performed in collaboration with British Skull Base Society (BSBS). Each Delphi's round was repeated until data saturation and over 90% consensus was reached. Results Eighteen consultant skull base surgeons (10 neurosurgeons and 8 ENT [ear, nose, and throat]) with median 17.9 years of experience (interquartile range: 17.5 years) of independent practice participated. There was a 100% response rate across both Delphi's rounds. The operative workflow for the retrosigmoid approach contained three phases and 40 unique steps as follows: phase 1, approach and exposure; phase 2, tumor debulking and excision; phase 3, closure. For the retrosigmoid approach, technique, and event error for each operative step was also described. Conclusion We present Part 1 of a national, multicenter, consensus-derived, codified operative workflow for the retrosigmoid approach to vestibular schwannomas that encompasses phases, steps, instruments, technique errors, and event errors. The codified retrosigmoid approach presented in this manuscript can serve as foundational research for future work, such as operative workflow analysis or neurosurgical simulation and education.

New Onset Alacrima as a Presenting Feature of a Skull Base Chondrosarcoma.

Acquired unilateral alacrima as a presenting sign of an intracranial tumor is exceptionally rare, and only described once previously in a case of nasopharyngeal carcinoma. The authors present a 32-year-old female patient who presents with a year-long history of alacrima and arhinorrhea. She was subsequently diagnosed with a petroclival chondrosarcoma extending into Meckel's cave and the cavernous sinus and underwent surgical debulking. To the authors' knowledge, this is the first reported case of acquired unilateral alacrima as a presenting feature of a skull base chondrosarcoma. This case serves to remind general ophthalmologists and oculoplastic surgeons alike that acquired alacrima may be the presenting feature of serious intracranial disease.

A thoracic extradural chordoid meningioma: a unique case report and literature review.

We describe the unique case of a patient being diagnosed with a thoracic extradural chordoid meningioma following her presentation with mild lower limb pyramidal weakness and a T8 sensory level. This is the first report of an extradural chordoid meningioma being identified in the thoracic spine. The tumour was successfully resected through a posterior thoracic laminectomy approach. Post-operatively, her neurological deficit resolved and to date she has not experienced a radiological recurrence. In this report, we review the literature and discuss this unusual tumour's characteristics and prognostic significance.

Are internet sites providing evidence-based information for patients suffering with Trigeminal Neuralgia?

Trigeminal neuralgia has a variety of treatments with variable efficacy. Sufferers present to a spectrum of disciplines. While traditional delivery of medical information has been by oral/printed communication, up to 50-80% patients access the internet for information. Confusion, therefore, may arise when seeking treatment for trigeminal neuralgia. We evaluated the quality of information on the internet for trigeminal neuralgia using the DISCERN© instrument. Only 54% websites had clear objectives; 42% delivered on these. A total of 71% provided relevant information on trigeminal neuralgia, 54% being biased/unbalanced; 71% not providing clear sources of information. No website detailed the side-effect profile of treatments; 79% did not inform patients of the consequences/natural history if no treatment was undertaken; it was unclear if patients could anticipate symptoms settling or when treatment would be indicated. Internet information on trigeminal neuralgia is of variable quality; 83% of sites assessed were of low-to-moderate quality, 29% having 'serious shortcomings.' Only two sites scored highly, only one being in the top 10 search results. Websites on trigeminal neuralgia need to appreciate areas highlighted in the DISCERN© instrument, in order to provide balanced, reliable, evidence-based information. To advise patients who may be misguided from such sources, neurosurgeons should be aware of the quality of information on the internet.

Malignant transformation of acoustic neuroma/vestibular schwannoma 10 years after gamma knife stereotactic radiosurgery.

Only a handful of cases of de-novo malignancies of the vestibulocochlear nerve have been reported. Even rarer is the malignant transformation of a previously histologically diagnosed benign vestibular schwannoma. We present the case of a young adult who had combined operative/Gamma knife treatment for a benign vestibular schwannoma, followed by further surgery 2 years later. He represented 10 years after original diagnosis with facial numbness and ataxia, MRI showing gross tumor recurrence. After radical resection, histology showed malignant transformation to a malignant peripheral nerve sheath tumor. Within 3 months there was rapid, aggressive recurrence with brainstem compression, requiring further surgery for brainstem decompression. Histology confirmed further de-differentiation to an anaplastic sarcoma. While awaiting radiotherapy the tumor recurred again, the patient succumbing. The patient had no features of neurofibromatosis type 2. In the literature there are 13 other cases of malignant vestibular schwannomata. Only six had radiotherapy and of these only two had histological confirmation of a benign lesion preradiotherapy. Neither of these had neurofibromatosis. Three other cases had histological proof of malignancy postradiosurgery, but with no preradiotherapy histology; of these, two were positive for neurofibromatosis. The tumor biology of vestibular schwannomata as well as the radiobiology in the context of malignant transformation is discussed.

Penetrating civilian craniocerebral gunshot wounds: a protocol of delayed surgery.

OBJECTIVE: Several factors have led to our unique approach of delayed definitive débridement. We wanted to evaluate the effectiveness of our management and compare it with the existing data in the literature. METHODS: We retrospectively reviewed the records of 194 patients presenting between January 1996 and October 2003 with penetrating craniocerebral gunshot wounds. After exclusion criteria, 125 patients qualified. RESULTS: Of the patients, 88.8% were male. The mean age was 24.9 +/- 10.9 years. In 70.4% of patients, the presenting Glasgow Coma Scale (GCS) score was 3 to 8. Only 38 (30.4%) of the 125 patients survived, with poor outcome in 2 and good outcome in 36. Bilaterally fixed and dilated pupils and bihemispheric tract on computed tomographic scan were significantly related to poor outcome. There were 49 surgical procedures performed on 27 of the patients, with a mortality rate of 7.4%. Of the 38 survivors, 13 underwent no surgery. Average time to surgery was 11.04 days. Total rate of infection was 8%, and it did not influence outcome. No patient presenting with a GCS score of 3 or 4 survived. Seventeen patients attended follow-up, for a total of 3609 days (average, 212 d) and very few late complications. CONCLUSION: Our supportive care of patients is not optimal. We should have saved more of our patients who presented with GCS scores of 14 and 15 who subsequently died. We have been able to report unconventionally late surgical management of two-thirds of survivors, with no surgery in one-third of survivors. Despite a high rate of infectious complications, infection did not lead to death or disability. Our protocol rarely leads to patients surviving in a permanently vegetative state. In the future, we would perform early surgery for patients who present awake and continue our current management for poor-grade patients. In this way, we will improve the number of good outcomes without increasing the population of severely damaged and dependent survivors.

Penetrating civilian craniocerebral gunshot wounds: a protocol of delayed surgery.

OBJECTIVE: Several factors have led to our unique approach of delayed definitive débridement. We wanted to evaluate the effectiveness of our management and compare it with the existing data in the literature. METHODS: We retrospectively reviewed the records of 194 patients presenting between January 1996 and October 2003 with penetrating craniocerebral gunshot wounds. After exclusion criteria, 125 patients qualified. RESULTS: Of the patients, 88.8% were male. The mean age was 24.9 +/- 10.9 years. In 70.4% of patients, the presenting Glasgow Coma Scale (GCS) score was 3 to 8. Only 38 (30.4%) of the 125 patients survived, with poor outcome in 2 and good outcome in 36. Bilaterally fixed and dilated pupils and bihemispheric tract on computed tomographic scan were significantly related to poor outcome. There were 49 surgical procedures performed on 27 of the patients, with a mortality rate of 7.4%. Of the 38 survivors, 13 underwent no surgery. Average time to surgery was 11.04 days. Total rate of infection was 8%, and it did not influence outcome. No patient presenting with a GCS score of 3 or 4 survived. Seventeen patients attended follow-up, for a total of 3609 days (average, 212 d) and very few late complications. CONCLUSION: Our supportive care of patients is not optimal. We should have saved more of our patients who presented with GCS scores of 14 and 15 who subsequently died. We have been able to report unconventionally late surgical management of two-thirds of survivors, with no surgery in one-third of survivors. Despite a high rate of infectious complications, infection did not lead to death or disability. Our protocol rarely leads to patients surviving in a permanently vegetative state. In the future, we would perform early surgery for patients who present awake and continue our current management for poor-grade patients. In this way, we will improve the number of good outcomes without increasing the population of severely damaged and dependent survivors.

Thoracic idiopathic spinal cord herniation at the vertebral body level: a subgroup with a poor prognosis? Case reports and review of the literature.

The authors present two cases of thoracic idiopathic spinal cord herniation (TISCH) occurring at the vertebral body (VB) level in whom adequate surgical reduction failed to reverse symptoms. In the second case, in which TISCH occurred into a VB cavity, presentation was atypical (subacute spinal cord syndrome) and there was persistent postoperative deterioration. In both cases, adequate surgical reduction was achieved via a posterior midthoracic laminectomy, and reduction was maintained by closure of the anterior dural defect by using prosthetic material. Thoracic idiopathic spinal cord herniation occurring at a VB level may be technically well treated by surgical reduction, but the outcome appears less predictable. Herniation that occurs directly into a VB cavity may form a distinct subgroup in which the presentation is atypical and the prognosis worse.