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

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      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.


      Grant Medical College and Sir J.J Group of Hospitals, Byculla, Mumbai, Maharashtra, India.


      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.


      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.


      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 references 12

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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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        A rare case of upper airway obstruction in an infant caused by basal encephalocele complicating facial midline deformity.

        A four-month-old male infant with basal encephalocele of the transsphenoidal type presented with upper airway obstruction and facial midline deformity, including cleft lip, cleft palate, hypertelorism and exophthalmos. Basal encephalocele is a rare disease, and usually not detectable from the outside. In this case, initially the cause of an upper airway obstruction was considered to be posterior rhinostenosis, and posterior rhinoplasty with inferior nasal conchectomy was scheduled. However, in preoperative examination, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a bony defect in the sphenoidal bone and a cystic mass in communication with cerebrospinal fluid, herniating into the nasal cavity through the bony defect. The mass was diagnosed as a transsphenoidal encephalocele, the scheduled operation cancelled, and tracheostomy performed for airway management. The possibility of basal encephalocele should be considered in the case of upper airway obstruction with facial midline deformity.
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          Caution in treating transsphenoidal encephalocele with concomitant moyamoya disease.

          A basal encephalocele is a rare congenital malformation involving a cranial bone defect and cystic-like herniation through the defect. Moyamoya is a rare cerebrovascular disease of unclear etiology involving occlusion of the distal internal carotids and formation of collateral vascular networks. Both diseases have been correlated with optic disc anomalies, hypopituitarism, and midfacial defects. We present a case of a 2-year-old boy with a midline facial cleft who underwent surgical correction of a basal encephalocele. His moyamoya disease may have contributed to a vascular complication. There is growing evidence indicating an overlap in disease profiles for these two rare diseases. In addition, molecular evidence indicates elevated levels of fibroblast growth factor and transforming growth factor in both diseases, suggesting common molecular pathways.

            Author and article information

            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@
            Asian J Neurosurg
            Asian J Neurosurg
            Asian Journal of Neurosurgery
            Medknow Publications & Media Pvt Ltd (India )
            Apr-Jun 2014
            : 9
            : 2
            : 108-111
            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.

            Case Report


            spheno-orbital encephalocele, sphenoid dysplasia, basal encephalocele


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