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      Bilateral calcification of the optic nerve sheath: A diagnostic dilemma

      case-report

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          Abstract

          Purpose

          To present a case of symptomatic optic nerve sheath calcification and highlight clues and pitfalls for the final diagnosis: bilateral optic nerve sheath meningioma.

          Observations

          A 48-year-old man presented with painless vision loss in his left eye and findings consistent with left optic nerve atrophy. Magnetic resonance imaging (MRI) displayed thinning of the left optic nerve without contrast-enhancement or evidence of compressive lesions. A supplementary computed tomography angiography (CTA) exposed scattered dural calcification, which included the optic nerves. This was regarded as an incidental finding. The initial diagnosis was ischemic optic neuropathy. Over the next two years, the vision loss in the left eye progressed. A CT of the orbits revealed extensive calcification surrounding both optic nerves. A second MRI was unchanged in comparison to the first MRI. The diagnosis was changed to idiopathic duro-optic calcification. The vision in the left eye further declined over another two years. Consecutive optical coherence tomography measurements of the peripapillary retinal nerve fiber layer suggested bilateral progressive thinning. A third MRI displayed progression of tubular contrast-enhancement surrounding the optic nerves. On the basis of this finding, the patient was finally diagnosed with a bilateral optic nerve sheath meningioma and received external beam radiotherapy.

          Conclusion and importance

          It is crucial to differentiate an optic nerve sheath meningioma from idiopathic calcification of the optic nerve. In the present case the initial MRI did not detect optic nerve sheath abnormalities. To better demonstrate characteristic calcification, additional CT imaging should be considered when a bilateral optic nerve sheath meningioma is suspected.

          Highlights

          • Harmless dural calcification can sporadically affect the optic nerve.

          • Concurrent optic neuropathy should raise the suspicion of underlying pathology.

          • Bilateral optic nerve sheath meningiomas commonly exhibit calcification.

          • If a bilateral meningioma is suspected, CT better shows calcification than MRI.

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          Most cited references12

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          Diagnosis and management of optic nerve sheath meningiomas.

          Optic nerve sheath meningiomas account for a third of all intrinsic tumours of the optic nerve. Despite their classification as histologically benign tumours they cause progressive visual loss that often leads to blindness if left untreated. Recent therapeutic advances have increased the treatment options available to clinicians but patient management remains controversial. We systematically review the progress made in the diagnosis and management of optic nerve sheath meningiomas, clarify current best practice, and suggest future avenues for research.
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            Is Open Access

            Optic nerve sheath meningiomas: prevalence, impact, and management strategies

            Optic nerve sheath meningiomas are rare benign neoplasms of the meninges surrounding the optic nerve. They are a significant cause of morbidity. While the mortality rate is practically zero, these tumors can blind or disfigure patients. Given that the clinical course can be variable, and treatment has the capacity to cause morbidity itself, the management of these patients can be difficult. We review the literature to discuss the prevalence of optic nerve sheath meningiomas, the association with neurofibromatosis type 2, natural history, and management options and strategies.
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              From variome to phenome: Pathogenesis, diagnosis and management of ectopic mineralization disorders.

              Ectopic mineralization - inappropriate biomineralization in soft tissues - is a frequent finding in physiological aging processes and several common disorders, which can be associated with significant morbidity and mortality. Further, pathologic mineralization is seen in several rare genetic disorders, which often present life-threatening phenotypes. These disorders are classified based on the mechanisms through which the mineralization occurs: metastatic or dystrophic calcification or ectopic ossification. Underlying mechanisms have been extensively studied, which resulted in several hypotheses regarding the etiology of mineralization in the extracellular matrix of soft tissue. These hypotheses include intracellular and extracellular mechanisms, such as the formation of matrix vesicles, aberrant osteogenic and chondrogenic signaling, apoptosis and oxidative stress. Though coherence between the different findings is not always clear, current insights have led to improvement of the diagnosis and management of ectopic mineralization patients, thus translating pathogenetic knowledge (variome) to the phenotype (phenome). In this review, we will focus on the clinical presentation, pathogenesis and management of primary genetic soft tissue mineralization disorders. As examples of dystrophic calcification disorders Pseudoxanthoma elasticum, Generalized arterial calcification of infancy, Keutel syndrome, Idiopathic basal ganglia calcification and Arterial calcification due to CD73 (NT5E) deficiency will be discussed. Hyperphosphatemic familial tumoral calcinosis will be reviewed as an example of mineralization disorders caused by metastatic calcification.
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                Author and article information

                Contributors
                Journal
                Am J Ophthalmol Case Rep
                Am J Ophthalmol Case Rep
                American Journal of Ophthalmology Case Reports
                Elsevier
                2451-9936
                22 April 2021
                June 2021
                22 April 2021
                : 22
                : 101106
                Affiliations
                [a ]Department of Ophthalmology, Oslo University Hospital, Norway
                [b ]Department of Radiology and Nuclear Medicine, Oslo University Hospital, Norway
                [c ]Department of Oncology, Oslo University Hospital, Norway
                [d ]Institute for Cancer Genetics and Informatics, Oslo University Hospital, Norway
                [e ]Department of Neurosurgery, Oslo University Hospital, Norway
                [f ]Section of Specialized Endocrinology, Oslo University Hospital, Norway
                [g ]Faculty of Medicine, University of Oslo, Norway
                Author notes
                []Corresponding author. Department of Ophthalmology, Oslo University Hospital, Postboks, 4950, Nydalen, 0424, Oslo, Norway. ynghus@ 123456ous-hf.no
                Article
                S2451-9936(21)00115-8 101106
                10.1016/j.ajoc.2021.101106
                8102398
                8cd923cf-5731-49e6-8528-0c8af0d7dcb6
                © 2021 The Authors

                This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

                History
                : 24 March 2020
                : 13 March 2021
                : 11 April 2021
                Categories
                Case Report

                optic nerve sheath calcification,optic nerve atrophy,bilateral optic nerve sheath meningioma,idiopathic duro-optic calcification

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