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Pilocytic Astrocytoma Presenting as an Orbital Encephalocele: A Case Report

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      Abstract

      We describe the case of a 29-year-old male who presented with new-onset seizures. He was subsequently found to have an orbital encephalocele containing a focus of pilocytic astrocytoma. We believe that this is the first report of a pilocytic astrocytoma located within the orbit that did not originate from the optic pathway. It is also the first case of a pilocytic astrocytoma completely contained within an encephalocele. This case suggests a close pathological examination of encephaloceles for underlying diseases.

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      Most cited references 13

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      Frequent recurrence and progression in pilocytic astrocytoma in adults.

      Most pilocytic astrocytomas (piloA) are benign growths (World Health Organization [WHO] grade 1) of the deep midline structures, the brainstem, and the cerebellum. To the authors' knowledge, the literature contains only scarce data regarding piloA in adults. Between 1995 and 2005, 44 patients (26 women and 18 men) underwent surgery for a primary or recurrent piloA at the authors' institution. All patients were aged > 16 years (mean +/- standard deviation: 31 +/- 14 years) at the time of their first surgery. The histopathologic diagnoses were reviewed, and relevant clinical information was obtained through a chart review and telephone interviews. The mean follow-up was 76 +/- 59 months (range, 1-227 months). There were 20 patients (45%) with supratentorial lobar piloA (including 10 temporal/temporomesial tumors, 5 parietal tumors, 3 insular tumors, 1 frontal tumor, and 1 occipital tumors), 12 patients with cerebellar piloA, 7 patients with brainstem piloA, 2 patients with opticochiasmatic PiloA, 1 patient with intramedullary piloA, and 2 patients with piloA of the basal ganglia. All but 1 patient with a lobar tumor presented with epilepsy. In 6 of 44 patients (14%), increased proliferative activity was revealed. WHO grade 3 primary anaplastic piloA was diagnosed in 2 patients (5%), and WHO grade 3 secondary anaplastic piloA was diagnosed in 4 patients (9%). Tumor recurrence or disease progression was observed in 13 of 44 patients (30%). Eight of 44 patients (18%) died from their disease. Histologic grading and extent of surgical resection proved to be important predictors of survival. PiloA in adult patients, surprisingly, often was not a benign disease. The degree of surgical resection was found to be of major importance for the patient's further clinical course; therefore, an aggressive surgical resection should be attempted whenever possible.
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        Traumatic encephalocele related to orbital roof fractures: report of six cases and literature review.

        Orbital roof fractures after blunt injury are rare. Traumatic encephaloceles in the orbital cavity are even rarer, with only 15 cases published to date. The clinical, radiological, and surgical findings of 6 cases of traumatic encephalocele treated at our institution from June 1998 to January 2000 are presented. They are also compared with previously published series. In contrast to other published cases, 5 out of 6 patients in our series were adults. The most common cause of trauma was road traffic accident. Ecchymosis and preoperative exophthalmos/proptosis were frequent. In all of our patients a coronal CT scan (3 mm increments with bone windows) was obtained. It demonstrated the extension of the orbital roof fractures and a possible encephalocele in 4 cases. Associated frontal brain contusions were seen in 5 cases. An MRI was performed in 3 patients (and only in 2 previously published cases); it showed the extension of the brain herniation into the orbital cavity. Surgical treatment via a fronto-basal approach with evacuation of the contused herniated brain tissue and orbital roof reconstruction was performed. The outcome at 6 months was good recovery in five patients with one patient still in a persistent vegetative state. Postoperatively the ocular disturbances improved in 5 cases. A review of the other published cases confirmed recovery of normal ocular function in the vast majority of the cases. Whenever orbital roof fractures associated with frontal contusions are identified in an acute brain injured patient, an orbital encephalocele should be suspected. In our opinion MRI is the investigation of choice in such patients. If the encephalocele is confirmed, a surgical approach via the subfrontal route is indicated with resection of herniated contused brain tissue, dural closure, and orbital roof reconstruction. Good results in regard to the orbital symptoms (mainly exophthalmos) can be expected.
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          Spheno-orbital encephalocele: A rare entity – A case report and review of literature

          Objective: To report a rare case of basal encephalocele (spheno-orbital encephalocele), managed successfully in our institute. This is one of the rarest type of encephaloceles with very little literature available. In this case, sphenoid dysplasia was not associated with type 1 Neurofibromatosis. Settings: Grant Medical College and Sir J.J Group of Hospitals, Byculla, Mumbai, Maharashtra, India. Methods: A 22 year old male presented with a history of protrusion of right eye since the age of 7 years followed by progressive diminution of vision in the same eye for 5 months. MRI scan was suggestive of sphenoid dysplasia with herniation of right fronto-temporal lobe. The patient was operated upon with right frontal craniotomy with excision of encephalocele and repair of skull base. Subsequently, the patient underwent plastic surgery for facial skin remodeling. Results: Patient's encephalocele reduced completely after excision and repair with good cosmetic results and no neurological deficits. There is no evidence of recurrence till recent follow-up. Conclusion: Basal encephaloceles are very rare. Complete excision of encephalocele with repair of the defect should be the aim to achieve cure.
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            Author and article information

            Affiliations
            aVirginia Commonwealth University, Richmond, Va., USA
            bMayo Clinic, Mayo Clinic, Rochester, Minn., USA
            cDepartment of Neurosurgery, Mayo Clinic, Rochester, Minn., USA
            dDepartment of Anatomic Pathology, Mayo Clinic, Rochester, Minn., USA
            Author notes
            *Jamie J. Van Gompel, Department of Neurosurgery, Mayo Clinic, 200 First Street SW, Rochester, MN 55905 (USA), E-Mail vangompel.jamie@ 123456mayo.edu
            Journal
            Case Rep Neurol
            Case Rep Neurol
            CRN
            Case Reports in Neurology
            S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )
            1662-680X
            Jan-Apr 2015
            23 April 2015
            23 April 2015
            : 7
            : 1
            : 90-94
            4448056
            10.1159/000381943
            crn-0007-0090
            Copyright © 2015 by S. Karger AG, Basel

            This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (www.karger.com/OA-license), applicable to the online version of the article only. Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

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            Figures: 3, References: 10, Pages: 5
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            Published online: April, 2015

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