Empty Sella Syndrome

What is Empty Sella Syndrome?

Sella Tursica is a depression situated at the human skull. The exact location of the sella is in the upper surface area of the sphenoid bone, where pituitary gland is sited.

The empty sella syndrome is associated with excessive pressure provided by the cerbrospinal fluid on the pituitary gland and the membrane of the sella turnica. This causes enlargement of sella turnica and pituitary gland shrinkage and flattened.

The pressure of the Cerebrospinal fluid is increased due to the defective tissue barrier, which separate the CSF from the sella turnica and hold it around the brain.

The empty sella syndrome does not produce any serious health condition, but the hormonal imbalance is common in severe condition. The incidence rate is more in obese female, who is having hypertension. ⁽¹⁾

Symptoms

Empty sella syndrome is usually asymptomatic and mostly detected during other clinical investigations. Some individual may complain the following symptom:

• Chronic headache
• High Blood pressure

In rare conditions, following symptoms

• Benign intracranial pressure or increased pressure inside the skull
• Cerebrospinal rhinnorhea means outflow of cerebrospinal fluid through the nose
• Papilledema or inflammation of the optic disc, which causes visual disturbance

Usually pituitary gland shrinkage does not affect physiological functioning, but in severe cases, it can cause hypopituitarism (insufficient pituitary hormone secretion). Hypopituitarism may affect other hormonal secretion also, as the pituitary gland is master gland and affects other gland secretion also. Hypopituitarism may provide following symptoms:

• Impotence due to erectile dysfunction
• Absent or irregular menstruation cycle
• Low libido or reduce sexual urge
• Lethargy due to low energy level
• Secretion from nipple due to hyperprolactemia

Onset of empty sella syndrome in the early childhood can cause growth retardation of the child due to deficiency of growth hormone. ^(1,2,3)

Causes

Ideopathic condition (unknwon reason) may responsible for development of the empty sella syndrome.  the possible causes which may provide empty sella syndrome are as follows:

Congenital defect

The is a crease of dura matter is the outmost maninges present in the central nervous system and it has many folds. Diaphragma sellae is one of the folding of dura matter. The faulty development of the diaphragma sellae at the time of birth can cause empty sella syndrome. The faulty diaphragma sellae causes leakage of the CSF and its store into the sella turcica.  The CSF fluid then provide pressure to the wall of the sella turcica and shrinkage of the pituitary gland.

Other Reasons

• Head injury or accidental truma in the head.
• Tumor formation in the pituitary gland
• Radiation therapy
• Pituitary gland surgery
• Infection in cerebrospinal fluid or any infection spread to CNS
• Sheehan syndrome: It is rare symptom, where a woman loses excessive blood during childbirth due to (1,2)

Diagnosis

The following diagnostic tests help to detect the disease:

Skull X-Ray

The image obtains from skull x-ray is similar with pituitary mass. The enlarged sella with thin barrier structure, but unable to the deformity is not clearly viewed.

CT Scan

CT scan helps to provide the image of enlarged sella filled with cerebrospinal fluid.

MRI

MRI usually conducts for confirmation of enlargement of the sella and CSF is filled in the sella.

• To investigate the intracranial pressure the following test also may order:
• Retinal examination conducted by an ophthalmologist.
• Lumber puncture is conducted for collection and further testing of the CSF. ⁽⁴⁾

Treatment

Usually no treatment is required to empty sella syndrome, as this is an asymptomatic process.

• Medication is prescribed to control the chronic headache.
• Specific hormonal replacement therapy is required to minimize the abnormality associated with severe hormonal deficiency. ^(2,3)

Surgery

Surgical intervention is rarely needed. The usual cause of surgical intervention is CSF rhinorrhea or visual disturbances. Before surgical intervention commencement all the necessary test, including X-ray, CT scan and MRI should conduct. Depending upon the radiological findings the options of the surgical intervention is chosen for stoppage of CSF:

• Transsellar fistulous tract
• Subfrontal craniotomy with intradural restoration of the anterior cranial fossa with fascia.

The surgical effect with empty sella syndrome is provided favorable result, most of the patient becomes improved or stabilize their symptoms. ⁽⁵⁾

References

1. Ian M. Chapman (2015); Empty Sella Syndrome; Retrieve from: http://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/pituitary-gland-disorders/empty-sella-syndrome
2. NIH Clinical Center (2013); Empty Sella Syndrome; Retrieve from: http://rarediseases.org/rare-diseases/empty-sella-syndrome/
3. Brent Wisse (2013); Empty sella syndrome; Retrieve from: https://www.nlm.nih.gov/medlineplus/ency/article/000349.htm
4. A.Prof Frank Gaillard; Empty sella; Retrieve from: http://radiopaedia.org/articles/empty-sella
5. Wael Fouad (2011); Review of empty sella syndrome and its surgical management; Retrieve from: http://www.sciencedirect.com/science/article/pii/S2090506811000406