This syndrome involves the bony structure known as sella turcica that is located at the base of the brain and protects the pituitary gland. Although most of the cases don’t present any evident symptoms but can develop them from the partial or complete loss of pituitary function. There are two types of this syndrome, the primary and the secondary. The primary form allows the spinal fluid to partially or completely fill the sella turcica which may present high levels of prolactin hormone. The secondary form is the regression of the pituitary gland within the cavity as the result of an injury or radiation therapy. The symptoms of the secondary form develop from the loss of pituitary functions such as infertility, intolerance to stress and infection. This condition is not a life-threatening disease and generally, the primary form does not present any complications or health problems.
Causes
The exact cause of this syndrome is not known. Research indicates that the congenital defect of the diaphragm sellae (the outer layer of the membrane that lines the brain and the spinal cord) may cause the development of this condition. In some cases, a tear in the diaphragm sellae can create the underlying membranes to penetrate through (herniate) which can result in the cerebrospinal fluid to leak and accumulate in the sella turcica. The pressure of the accumulated fluid can consequently compress the pituitary. The secondary form of this syndrome is caused by various conditions such as trauma to the head, pituitary tumors, radiation therapy or as the result of an injury.
Symptoms
In most cases of this syndrome, the affected individuals remain asymptomatic, with no symptoms apparent. However, when symptoms develop, it is based on the age of the individual and the cause of the syndrome. This condition is usually detected on an x-ray when evaluated for other health ailments. However, one of the common symptoms is a chronic headache along with high blood pressure (hypertension). In rare cases, symptoms include intracranial pressure (pressure within the skull), leakage of cerebrospinal fluid from the nose, swelling of the optic disc and visual problems. Although the pituitary gland is usually not affected, the rare occasion can develop abnormal pituitary function and growth hormone deficiency. Additional symptoms can include impotence in men, lacking interest for sex and irregular menstrual periods in women.
Diagnosis
The diagnosis is based on the evaluation of the individual’s medical history and a physical examination. Additional tests may include CT scan to detect abnormalities and MRI scan particularly of the brain and the spinal cord.
Treatment
The treatment may not be required if no symptoms are evident and the pituitary gland is not swollen. However, the treatment for the pituitary malfunction is supportive and symptomatic. Individuals with the high level of prolactin may be administered medication and those presenting spinal fluid drainage may require surgery.
References
https://rarediseases.org/rare-diseases/empty-sella-syndrome/
http://pituitary.org/knowledge-base/disorders/empty-sella-syndrome
http://www.ninds.nih.gov/disorders/emptysella/emptysella.htm
