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Case Report

Complex Unilateral Orbital Dysplasia with Plexiform Neurofibroma, Presented as Unilateral Pulsating Proptosis: Staged Surgery and our Learning

Bipin Chaurasia , Dhiman Chowdhury1 , Nazmin Ahmed2 , Bipin Chaurasia3 , Kanak Kanti Barua4 , Md. Ziaul Hoq5

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International Journal of Neurology and Neurosurgery 11(3):p 234-240, July-September 2019. | DOI: DOI: http://dx.doi.org/10.21088/ijnns.0975.0223.11319.11

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Dhiman Chowdhury, Nazmin Ahmed, Bipin Chaurasia et al. Complex Unilateral Orbital Dysplasia with Plexiform Neurofibroma, Presented as Unilateral Pulsating Proptosis: Staged Surgery and our Learning. Int J Neurol Neurosurg. 2019;11(3):234-240.

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Abstract

Presence of sphenoid wing dysplasia fulfills one of the diagnostic criterias of neurofibromatosis type 1 (NF1). This defect of the skull base can manifests as pulsating proptosis. Associated calvarial defects, especially away from the lambdoid suture are a much rarer presentation. This report describes the successful removal of plexiform neurofibroma followed by reconstruction of the dysplastic sphenoid wing in an NF1 patient. Later on, he underwent blepharoplasty for scarring of the upper eyelid. After successful management of the overall procedure in three stages, the patient achieved improved visual outcome along with acceptable cosmetic appearance.

Key words: Sphenoid wing dysplasia; neurofibromatosis; proptosis.

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Dhiman Chowdhury, Nazmin Ahmed, Bipin Chaurasia et al. Complex Unilateral Orbital Dysplasia with Plexiform Neurofibroma, Presented as Unilateral Pulsating Proptosis: Staged Surgery and our Learning. Int J Neurol Neurosurg. 2019;11(3):234-240.

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DOI: DOI: http://dx.doi.org/10.21088/ijnns.0975.0223.11319.11

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