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      Intraorbital Encephalocele Presenting with Exophthalmos and Orbital Dystopia : CT and MRI Findings

      case-report

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          Abstract

          A 15-year-old female patient with progressive pulsatile exophthalmos caused by intraorbital encephalocele was evaluated with computed tomography (CT) and magnetic resonance imaging (MRI) in our clinic. She had no history of trauma or reconstructive surgery. When she was a little girl, she had undergone surgery for congenital glaucoma on the right eye. On the three-dimensional image of CT, a hypoplasic bone defect was observed in the greater wing of the right sphenoid bone. MRI and CT scan showed herniation through this defect of the arachnoid membrane and protruded cerebral tissue into the right orbita. Intraorbital encephalocele is an important entity that can cause pulsatile exophthalmos and blindness.

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          Spheno-orbital encephalocele: A rare entity – A case report and review of literature

          Mayur Sharma, Rahul Mally, Vernon Velho … (2014)
          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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            Intraorbital encephalocele in an adult patient presenting with pulsatile exophthalmos. Case report.

            Hirofumi Morihara, Kiichiro Zenke, Daisuke Shoda … (2010)
            A 25-year-old man presented with an intraorbital encephalocele manifesting as progressive left pulsatile exophthalmos. He had a history of frontal lobe contusion from a motorbike accident 10 years before the onset of the symptom. Computed tomography and magnetic resonance imaging revealed an oval-shaped defect in the left orbital roof with an underlying intracranial cystic lesion, herniated into the orbit. Intraoperative findings included disruption of the dura mater around the bony defect. The loculated arachnoid membrane and protruding brain tissue were excised with primary dural closure and reconstructive cranioplasty with a titanium mesh. The postoperative course was uneventful, and the pulsatile proptosis disappeared immediately after the procedure. Intracranial cyst may be important in the development of progressive pulsatile exophthalmos and intraorbital encephalocele.
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              A non-midline spheno-orbital encephalocele in a newborn.

              U Knopp, A. Knopp, F. Stellmacher … (2009)
              Basal encephaloceles in western countries occur in 1 of every 35 000-40 000 live births; with an incidence of less than 10% they are the least common of all encephaloceles. Certain subtypes such as transsphenoidal variants may be as rare as 1 in 700 000 live births. These rare encephaloceles are classified into five anatomic types: spheno-ethmodial, transsphenoidal, spheno-orbital, transethmoidal, and spheno-maxillary. Here we present an exceedingly rare variant of a non-midline basal encephalocele of the spheno-orbital type, which was treated by resection of the encephalocele, which contained dysplastic central nervous system tissue, on day four post partum. The patient had no neurological deficits and a six year follow-up showed a normal intellect and a good cosmetic result.
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                Author and article information

                Journal
                Journal ID (nlm-ta): J Korean Neurosurg Soc
                Journal ID (iso-abbrev): J Korean Neurosurg Soc
                Journal ID (publisher-id): JKNS
                Title: Journal of Korean Neurosurgical Society
                Publisher: The Korean Neurosurgical Society
                ISSN (Print): 2005-3711
                ISSN (Electronic): 1598-7876
                Publication date (Print): January 2015
                Publication date (Electronic): 31 January 2015
                Volume: 57
                Issue: 1
                Pages: 58-60
                Affiliations
                [1 ]Department of Radiology, Sakarya University Medical Faculty, Sakarya, Turkey.
                [2 ]Department of Neurosurgery, Sakarya University Medical Faculty, Sakarya, Turkey.
                Author notes
                Address for reprints: Kiyasettin Asil, M.D. Department of Radiology, Sakarya University Medical Faculty, 54100 Korucuk, Sakarya, Turkey. Tel: +90-2642751010, Fax: +90-2642552466, drkiyasettin@ 123456gmail.com
                Article
                DOI: 10.3340/jkns.2015.57.1.58
                PMC ID: 4323507
                SO-VID: cd7820ae-5348-4d54-bd30-8f2faca5c7de
                Copyright © Copyright © 2015 The Korean Neurosurgical Society
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 17 March 2014
                Date revision received : 21 August 2014
                Date accepted : 04 September 2014
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Surgery
                Keywords: intraorbital encephalocele,3-dimensional ct,mri
                Data availability:
                ScienceOpen disciplines: Surgery
                Keywords: intraorbital encephalocele, 3-dimensional ct, mri

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