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MR image of the sellar and parasellar region in the patient with transsphenoidal encephalocele (coronary section, T1).
Source publication
A case of a 32-year-old woman with transsphenoidal encephalocele is presented. Encephaloceles are congenital defects in the skull through which meninges and brain tissues herniate. Basal encephaloceles account for 1.5% of all encephaloceles and are found in 1:35,000 live births. Transsphenoidal basal encephaloceles are very rare, accounting for onl...
Context in source publication
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Citations
... Trans-sphenoidal is the rarest of all, constituting 5% of all basal encephaloceles and is associated with visual and hormonal disturbances.[3] ...
... Encephaloceles occur most commonly in females than in males.[3] They are often associated with midline craniofacial dysraphism,[3] which is not seen in the present case. When it protrudes into the nasal cavity, it can lead to upper airway obstruction. ...
... Posterior encephaloceles are most common (75%) and basal ones are least frequent (1.5%).[3] ...
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.
... Encephaloceles are divided into two types: anterior and posterior. Posterior encephaloceles account for three-fourths of those described and anterior for only about 1.5% of all encephaloceles [3]. On the basis of the location of the bone defect, basal encephaloceles are further subdivided into transphenoidal, transethmoidal, spheno-ethmoidal and spheno-orbital [4]. ...
... Though intrasphenoidal encephaloceles are classified as a type of transphenoidal encephalocele, they should be considered a completely separate entity [5]. Intrasphenoidal encephaloceles (extending into but not through the sphenoid sinus) and true transphenoidal encephaloceles have different morphological and clinical features as well as different etiologies [3,6]. The etiology of the ISE can be attributed to "trauma, tumor, intracranial infection, or a surgical procedure involving the sellar region, the parasellar region, or the paranasal sinus" or a small defect in the middle cranial fossa [7]. ...
Basal encephaloceles are rare clinical entities. Intrasphenoidal encephalocele (ISE) is a rare form of basal encephalocele. The clinical presentation is often subtle and may remain undetected. Only a limited number of cases of ISE have been reported, mostly in middle-aged patients who presented with symptoms. Clinical diagnosis and management remain a challenge. Our case reports ISE as an incidental finding in a very elderly male patient.
We present a case of ISE discovered as an incidental finding in a 99-year-old male who presented to the emergency department (ED) after a fall from bed. The encephalocele was discovered in the evaluation of the computed tomography (CT) scan of the head.
This is an incidental manifestation of a rare disease. It demonstrates that these encephaloceles may not manifest clinically or symptomatically as other reports have suggested.
