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

      case-report

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          Abstract

          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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          Most cited references12

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          Sonographic prenatal diagnosis of central nervous system abnormalities.

          Over the past 20 years, the spectrum of neonatal neurological malformations has changed due to the diffusion of ultrasound, performed either routinely or as required by maternal alpha-fetoprotein screening or history.
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            Basal encephalocele and morning glory syndrome.

            Basal encephaloceles are often associated with other midline anomalies such as hypertelorism, broad nasal root, cleft lip, and cleft palate. Optic disc anomalies such as pallor, dysplasia, optic pit, coLoboma, and megalopapilla have been reported to occur in patients with basal encephalocele We report a case of a child with a sphenoethmoidal encephalocele and morning glory syndrome of the optic nerve. The presence of such optic nerve anomalies with facial midline anomalies should alert the clinician to the possible presence of a basal encephalocele.
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              Syndromes identified in fetuses with prenatally diagnosed cephaloceles.

              An evaluation of all fetuses from our institution with prenatally diagnosed cephaloceles was conducted to determine the frequency and spectrum of genetic syndromes associated with this abnormality. Review of the sonographic images, postnatal autopsy or pathology reports, delivery room records, paediatric neurosurgical findings, radiographs, and cytogenetic data were collected. Fifteen postnatally confirmed cases of prenatally diagnosed cephaloceles and two misdiagnosed cases were identified. Thirteen were midline occipital lesions, one was frontoparietal, and one was frontoethmoidal. Excluding microcephaly, hydrocephaly, and distortion of intracranial anatomy, 9 of 15 (60 per cent) had other associated anomalies, including two fetuses with aneuploidy. The prenatal diagnosis of a cephalocele should initiate a thorough search for other abnormalities. In this series, there were three multifactorial, two chromosomal, two sporadic, and two autosomal recessive syndromes identified. An accurate diagnosis is critical in determining the prognosis and providing appropriate genetic counselling.
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                Author and article information

                Journal
                Asian J Neurosurg
                Asian J Neurosurg
                AJNS
                Asian Journal of Neurosurgery
                Medknow Publications & Media Pvt Ltd (India )
                1793-5482
                2248-9614
                Apr-Jun 2014
                : 9
                : 2
                : 108-111
                Affiliations
                [1]Department of Neurosurgery, 4 th floor, Main Hospital Building, Grant Medical College and Sir J.J Group of Hospitals, Byculla, Mumbai, Maharashtra, India
                Author notes
                Address for correspondence: Dr. Mayur Sharma, Department of Neurosurgery, Grant Medical College and Sir J. J. Group of Hospitals, Byculla, Mumbai - 400 008, India. E-mail: drmayur_sharma@ 123456yahoo.co.in
                Article
                AJNS-9-108
                10.4103/1793-5482.136728
                4129575
                25126130
                a08e0e2a-098b-4309-a9a7-a922c06c2f46
                Copyright: © Asian Journal of Neurosurgery

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Case Report

                Surgery
                basal encephalocele,sphenoid dysplasia,spheno-orbital encephalocele
                Surgery
                basal encephalocele, sphenoid dysplasia, spheno-orbital encephalocele

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