The Management of Orbital Roof Fractures and Defects: A Review.

PURPOSE: To explore the anatomy, etiopathogenesis, diagnosis and classification, current evidence on intervention and the surgical management of orbital roof fractures and defects (ORFD) for oculoplastic surgeons presented with such cases. METHODS: A review of the current literature through the MEDLINE database using the following search terms: "orbital roof fracture (+treatment/management)," "orbital roof defect (+treatment/management)," "orbital roof erosion (+treatment/management)," "orbital roof repair," "orbital roof," "orbital fracture," "pediatric orbital roof (defect/fracture/erosion)," "orbital anatomy," and "orbital roof anatomy" was conducted. As relatively little has been published on this topic, inclusion criteria were broad and peer-reviewed articles judged to be of clinical importance, relevant to the aims of this review, were included. Non-English abstracts were also included if relevant. Year of publication was not a strict exclusion criterion, and older articles were judged for their suitability based on clinical importance and relevance to current practice. Additional references were obtained from citations in key articles and recommendations from the coauthors based on their areas of expertise. RESULTS: The etiopathogenesis of ORFD varies. Classification systems have been formulated to guide management decisions and can range from conservative management to complex neurosurgery. Eyelid approaches have also been described. This review provides a summary of the evidence for each and a management framework oculoplastic surgeons can use when presented with ORFD. CONCLUSION: Oculoplastic surgeons can be involved, either alone or as part of a multidisciplinary team, in the management of ORFD, and for some, conduct orbital approach reconstructive surgery.

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.

Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia?

Multinodular and vacuolating neuronal tumor (MVNT) is a new pattern of neuronal tumour included in the recently revised WHO 2016 classification of tumors of the CNS. There are 15 reports in the literature to date. They are typically associated with late onset epilepsy and a neoplastic vs. malformative biology has been questioned. We present a series of ten cases and compare their pathological and genetic features to better characterized epilepsy-associated malformations including focal cortical dysplasia type II (FCDII) and low-grade epilepsy-associated tumors (LEAT). Clinical and neuroradiology data were reviewed and a broad immunohistochemistry panel was applied to explore neuronal and glial differentiation, interneuronal populations, mTOR pathway activation and neurodegenerative changes. Next generation sequencing was performed for targeted multi-gene analysis to identify mutations common to epilepsy lesions including FCDII and LEAT. All of the surgical cases in this series presented with seizures, and were located in the temporal lobe. There was a lack of any progressive changes on serial pre-operative MRI and a mean age at surgery of 45 years. The vacuolated cells of the lesion expressed mature neuronal markers (neurofilament/SMI32, MAP2, synaptophysin). Prominent labelling of the lesional cells for developmentally regulated proteins (OTX1, TBR1, SOX2, MAP1b, CD34, GFAPδ) and oligodendroglial lineage markers (OLIG2, SMI94) was observed. No mutations were detected in the mTOR pathway genes, BRAF, FGFR1 or MYB. Clinical, pathological and genetic data could indicate that MVNT aligns more with a malformative lesion than a true neoplasm with origin from a progenitor neuro-glial cell type showing aberrant maturation.

Frontal sinus mucocele in association with fibrous dysplasia: review and report of two cases.

We present two paediatric cases of fibrous dysplasia (FD) who presented to the craniofacial neurosurgical clinic with ophthalmological symptoms associated with sinus mucoceles. The first patient presented with a history of orbital cellulitis and an increasing bony swelling around the orbit associated with proptosis. Radiological imaging revealed monostotic FD associated with an obstructive mucocele in the frontal sinus with extension into the orbit. The second patient presented with recurrent conjunctivitis, painful proptosis, rhinitis and a bony peri-orbital swelling. Both patients had histological diagnoses of frontal mucoceles invading the orbit in association with FD. They both underwent frontal craniotomies and excision of the mucocele/fibrous dysplastic complex. In summary, mucocele development is an unusual complication of FD, likely to occur secondary to occlusion of the sinus drainage system. Orbital involvement may lead to visual disturbance caused by pressure effects. A multi-disciplinary approach including maxillofacial surgeons, plastic surgeons and neurosurgeons is advocated.