A watch, wait, and rescan approach for incidental benign-appearing notochordal lesions of the skull base.

Objective: The aim of this study was to describe the natural history of incidental benign-appearing notochordal lesions of the skull base with specific attention to features that can make differentiation from low-grade chordoma more difficult, namely contrast uptake and bone erosion.

Methods: In this retrospective case series, the authors describe the clinical outcomes of 58 patients with incidental benign-appearing notochordal lesions of the clivus, including those with minor radiological features of bone erosion or contrast uptake.

Results: All lesions remained stable during a median follow-up of almost 3 years.

Intraoperative diagnosis of facial schwannomas: a multicenter summation of clinical experience, preoperative avoidance, and intraoperative management protocol.

Objective: Preoperative differentiation of facial nerve schwannoma (FNS) from vestibular schwannoma (VS) can be challenging, and failure to differentiate between these two pathologies can result in potentially avoidable facial nerve injury. This study presents the combined experience of two high-volume centers in the management of intraoperatively diagnosed FNSs. The authors highlight clinical and imaging features that can distinguish FNS from VS and provide an algorithm to help manage intraoperatively diagnosed FNS.

Prevalence and natural history of schwannomas in neurofibromatosis type 2 (NF2): the influence of pathogenic variants.

This study explores the natural history of vestibular, trigeminal and lower cranial nerve schwannomas (VS, TS, LCNS) in patients with Neurofibromatosis type 2 (NF2), to understand how pathogenic variants (PVs) of the NF2 gene affect tumour burden and growth rate, via a retrospective analysis of a UK NF2 centre database and imaging. VS, TS and LCNS location and size were measured in accordance with a standardised protocol. PVs were categorised in accordance with the UK NF2 Genetic Severity Score (GSS).

Disease course of neurofibromatosis type 2: a 30-year follow-up study of 353 patients seen at a single institution.

Background: Limited data exist on the disease course of neurofibromatosis type 2 (NF2) to guide clinical trial design.

Methods: A prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990 and 2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred, and inheritance type.

Familial unilateral vestibular schwannoma is rarely caused by inherited variants in the NF2 gene.

Objectives/hypothesis: Unilateral vestibular schwannoma (VS) occurs with a lifetime risk of around 1 in 1,000 and is due to inactivation of the NF2 gene, either somatically or from a constitutional mutation. It has been postulated that familial occurrence of unilateral VS occurs more frequently than by chance, but no causal mechanism has been confirmed.

Study Design: Retrospective database analysis.

Schwannomatosis: a genetic and epidemiological study.

Objectives: Schwannomatosis is a dominantly inherited condition predisposing to schwannomas of mainly spinal and peripheral nerves with some diagnostic overlap with neurofibromatosis-2 (NF2), but the underlying epidemiology is poorly understood. We present the birth incidence and prevalence allowing for overlap with NF2.

Methods: Schwannomatosis and NF2 cases were ascertained from the Manchester region of England (population=4.

High-Grade Glioma is not a Feature of Neurofibromatosis Type 2 in the Unirradiated Patient.

Background: The Manchester criteria for neurofibromatosis type 2 (NF2) include a range of tumors, and gliomas were incorporated in the original description. The gliomas are now widely accepted to be predominantly spinal cord ependymomas.

Objective: To determine whether these gliomas include any cases of malignant glioma (WHO grade III and IV) through a database review.

Calcifying pseudoneoplasm of the neuraxis (CaPNoN): an unusual cause of third nerve palsy in a teenager.

An 18-year-old part-time teacher presented with headache and diplopia. Physical examination showed partial left oculomotor palsy. Neurology examination was otherwise unremarkable.

Isolated Pupil-Involving Third Nerve Palsy as the First Presentation of Sarcoidosis.

Neurosarcoidosis is seen in 5-15% of patients with systemic sarcoidosis. The most common cranial nerve presentations are optic neuropathy and facial nerve palsy. The authors present a case of sarcoidosis presenting with a pupil-involving third nerve palsy.

Bisphosphonate-induced osteonecrosis of the maxilla presenting as a cicatricial ectropion.

A 63-year-old gentleman, who was being treated with bisphosphonates for multiple myeloma, presented with a cicatricial ectropion of the lower eyelid, without exposure keratopathy. A CT scan demonstrated extensive destruction of bone with an infraorbital fracture surrounded by sclerotic bony changes. The patient was managed conservatively with discontinuation of bisphosphonate therapy and topical ocular lubricants.

Osteoradionecrosis of the exenterated orbit.

Osteoradionecrosis (ORN) occurs in an estimated 2% of head and neck-irradiated patients. It is seen most commonly in the mandible with other reported sites including the maxilla, temporal bone, clavicle, and vertebrae. It is defined as an area of exposed devitalised irradiated bone, with failure to heal during a period of at least 3 months, in the absence of local neoplastic disease.

A case of a spontaneous intraorbital arteriovenous fistula: clinico-radiological findings and treatment by transvenous embolisation via the superior ophthalmic vein.

A 72-year-old male presented with progressive right axial proptosis and red eye. Catheter angiography demonstrated an intraorbital arteriovenous fistula (IAVF) distal to the central retinal artery (CRA). Transvenous embolisation following direct surgical exposure of the superior ophthalmic vein (SOV) resulted in rapid resolution of his symptoms and signs.

Rate and clinical impact of intra-procedural complications during coil embolisation of ruptured small (3 mm or less) cerebral aneurysms.

Objective: Coiling of small (≤3 mm) cerebral aneurysms can be technically challenging and is associated with increased procedural-related morbidity and mortality. The authors report the clinical and radiological results following coiling of ruptured small cerebral aneurysms in a single-institution, and define the rates of intra-procedural rupture and thromboembolism.

Methods: A retrospective analysis was conducted on consecutive patients from 01/01/2008 to 31/12/2010 with subarachnoid haemorrhage (SAH) from ruptured cerebral aneurysms (≤3 mm) managed in a tertiary neurosurgical institution in the United Kingdom.

Double origin of the posterior inferior cerebellar artery with findings on conventional and CT angiography.

A case of double origin of the posterior inferior cerebellar artery (PICA) is presented, with appearances on both cross-sectional imaging and conventional angiography. An associated aneurysm was found, strengthening the belief that this variation is associated with intracranial aneurysms. Furthermore, this is the first report of a right-sided double-origin PICA in a female patient, which calls into question the previously proposed male and left-sided preponderance of this variation.

Coincident Giant Cavernous Angioma and Large Middle Cerebral Artery Aneurysm.

Cavernous angiomas although relatively common lesions rarely reach a large size. They have a well documented association with AVMs, capillary telangiectases and venous angiomas but are not specifically associated with intracerebral aneurysms. We present a case of what we believe to be the 4th largest reported giant cavernous angioma to present in adulthood.

Visual pathway compromise after hydrocoil treatment of large ophthalmic aneurysms.

Objective: Hydrogel-coated coils (MicroVention, Inc., Aliso Viejo, CA) for endovascular aneurysm treatment offer the theoretical advantages of increased volumetric occlusion, thrombus stabilization, and improved neointimal healing. Reports of local inflammation and hydrocephalus after coiling of unruptured aneurysms have raised questions about the safety profile or appropriate usage of these new devices.

Hypercellularity of the mastoid as a cause of spontaneous pneumocephalus.

In this paper two cases are reported in which spontaneous entry of air into the head appears to have occurred through a hypercellular mastoid air cell system. In both these cases forceful sneezing and nose blowing were considered contributory factors. They underwent surgical repair of the bony defects which, combined with less vigorous nose blowing, has affected a successful repair.