What is Empty Sella Syndrome?
Empty Sella Syndrome is an extremely rare pathological condition of the brain characterized by enlargement of a structure located in the brain called the sella turcica. This structure is located in the bone at the base of the skull adjacent of the pituitary gland and is in the form of a slight depression.
What happens with Empty Sella Syndrome is that either it is partially filled with CSF with a small portion of the adjoining pituitary gland present on the floor of the structure or it is completely filled with CSF without any portion of the pituitary gland visible within the sella turcica.
In majority of the cases of Empty Sella Syndrome, the affected individual will have not symptoms associated with it and the condition is basically an incidental finding found when imaging is done of the brain to rule out certain other medical conditions associated with the brain.
Empty Sella Syndrome may occur as a single entity or primary disorder or may occur as a result of certain underlying medical conditions of the brain like a pituitary tumor. A medical condition called as intracranial hypertension is also found to be one of the causes of Empty Sella Syndrome.
What are the Causes of Empty Sella Syndrome?
The root cause of Empty Sella Syndrome has still not been identified as is believed to occur due to unknown reasons termed as idiopathic Empty Sella Syndrome.
Some researchers believe that Empty Sella Syndrome may occur as a result of a defect in the outermost lining of the layer of membranes in the brain and spinal cord. This outermost layer is termed as diaphragma sellae. This in most cases is a congenital abnormality.
This layer covers the sphenoid bone which contains the sella turcica and the pituitary gland. Some researchers believe that a tear in the diaphragma sellae causes the underlying membranes to bulge allowing the CSF to infiltrate within the sella turcica causing Empty Sella Syndrome.
The infiltration of the CSF into the sella turcica causes pressure to be exerted on the empty sella which tends to flatten or enlarge the sella turcica which may also cause the pituitary gland to flatten or enlarge.
While the diaphragma sellae may be absent in some individuals at birth but whether this is a definite cause for Empty Sella Syndrome has not yet been proved as of yet.
In cases of secondary Empty Sella Syndrome, it is usually caused due to a brain tumor or a pituitary gland tumor which may cause the empty sella to be filled with CSF. Certain infections, direct trauma or blow to the head, surgical procedures, radiation therapy for certain cancers may also be the causes of secondary Empty Sella Syndrome.
What are the Symptoms of Empty Sella Syndrome?
The symptoms of Empty Sella Syndrome are extremely variable. While in majority of cases of primary Empty Sella Syndrome there are no distinct symptoms and most of the affected individuals remain asymptomatic.
The condition is usually identified incidentally when radiographic studies are being done to rule out other conditions of the brain. The only symptoms which can be caused due to Empty Sella Syndrome are chronic headache.
Hypertension is yet another symptom that is seen in most of the affected individual with Empty Sella Syndrome. In some cases, increased intracranial pressure is seen in individuals with Empty Sella Syndrome. Papilledema and decreased visual acuity is also believed to be associated with Empty Sella Syndrome
How is Empty Sella Syndrome Diagnosed?
In majority of the cases as stated above, a diagnosis of Empty Sella Syndrome is made only due to an incidental finding when a radiological study is done to rule in or rule out some other medical condition of the brain.
Other than this, if an individual presents with complaints of headaches and has hypertension and further investigations reveals increased intracranial pressure and increased pressure in the optic nerves in the eyes then a diagnosis of Empty Sella Syndrome may be possible.
Advanced studies of the brain in the form of MRI or CT scan will clearly show the presence of CSF within the sella turcica confirming the diagnosis of Empty Sella Syndrome.
How is Empty Sella Syndrome Treated?
In majority of the cases of Empty Sella Syndrome, the treatment for Empty Sella Syndrome is purely symptomatic. In case if pituitary gland gets affected, then treatment is directed towards replacing the hormones that get affected as a result of Empty Sella Syndrome. Surgical intervention may be required in cases of CSF rhinorrhea which can at times occur due to Empty Sella Syndrome.