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

Sharma M, Mally R, Velho V, Agarwal V - Asian J Neurosurg (2014)

Bottom Line: In this case, sphenoid dysplasia was not associated with type 1 Neurofibromatosis.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, 4 floor, Main Hospital Building, Grant Medical College and Sir J.J Group of Hospitals, Byculla, Mumbai, Maharashtra, India.

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.

No MeSH data available.


Related in: MedlinePlus

CT scan (3D reconstruction) showing dysplastic greater wing of sphenoid on right side with herniation of temporal lobe and CSF in right orbital space causing displacement of right eye globe anteriorly
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Figure 2: CT scan (3D reconstruction) showing dysplastic greater wing of sphenoid on right side with herniation of temporal lobe and CSF in right orbital space causing displacement of right eye globe anteriorly

Mentions: CT [Figures 2 and 3] and MRI scans [Figures 4 and 5] were suggestive of dysplastic greater wing of sphenoid on the right side with herniation of temporal lobe and CSF in to right orbital space causing displacement of right eyeball anteriorly. Diffuse atrophy of right cerebral hemisphere was seen. Multiple sutural defects were also seen in the right occipital bone and lambda. Right-sided extra ocular muscles and optic nerve were displaced and appeared indistinct.


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

Sharma M, Mally R, Velho V, Agarwal V - Asian J Neurosurg (2014)

CT scan (3D reconstruction) showing dysplastic greater wing of sphenoid on right side with herniation of temporal lobe and CSF in right orbital space causing displacement of right eye globe anteriorly
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4129575&req=5

Figure 2: CT scan (3D reconstruction) showing dysplastic greater wing of sphenoid on right side with herniation of temporal lobe and CSF in right orbital space causing displacement of right eye globe anteriorly
Mentions: CT [Figures 2 and 3] and MRI scans [Figures 4 and 5] were suggestive of dysplastic greater wing of sphenoid on the right side with herniation of temporal lobe and CSF in to right orbital space causing displacement of right eyeball anteriorly. Diffuse atrophy of right cerebral hemisphere was seen. Multiple sutural defects were also seen in the right occipital bone and lambda. Right-sided extra ocular muscles and optic nerve were displaced and appeared indistinct.

Bottom Line: In this case, sphenoid dysplasia was not associated with type 1 Neurofibromatosis.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, 4 floor, Main Hospital Building, Grant Medical College and Sir J.J Group of Hospitals, Byculla, Mumbai, Maharashtra, India.

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.

No MeSH data available.


Related in: MedlinePlus