Empty sella syndrome
|Empty sella syndrome|
|Classification and external resources|
Empty sella syndrome (abbreviated ESS) is where the pituitary gland shrinks or becomes flattened, filling the sella turcica with cerebrospinal fluid on imaging instead of the normal pituitary. ESS can be found in the diagnostic workup of pituitary disorders, or as an incidental finding when imaging the brain.
There are two types of ESS: primary and secondary.
- Primary ESS happens when a small anatomical defect above the pituitary gland increases pressure in the sella turcica and causes the gland to flatten out along the interior walls of the sella turcica cavity. Primary ESS is associated with obesity and increase in intracranial pressure in women.
- Secondary ESS is the result of the pituitary gland regressing within the cavity after an injury, surgery, or radiation therapy. Individuals with secondary ESS due to destruction of the pituitary gland have symptoms that reflect the loss of pituitary functions, such as intolerance to stress and infection.[medical citation needed]
- Abnormality (middle ear ossicles)
- Arnold-Chiari type I malformation
- Patent ductus arteriosus
- Muscular hypotonia
The cause of this condition is divided into primary and secondary, as follows:
- The cause of this condition in terms of secondary empty sella syndrome happens when a tumor or surgery damages the gland, this is an acquired manner of the condition.
- ~70% of patients with Idiopathic intracranial hypertension will have empty sella on MRI
- The cause of primary empty sella syndrome is a congenital defect(diaphragma sellae)
The normal mechanism of the pituitary gland sees that it controls the hormonal system, which therefore have an effect on growth, sexual development and adrenocortical function. The gland is divided into anterior and posterior.
The major differential to consider in empty sella syndrome is intracranial hypertension, of both unknown and secondary causes, and an epidermoid cyst, which can mimic cerebrospinal fluid due to its low density on CT scans, although MRI can usually distinguish the latter diagnosis.
In terms of management, unless the syndrome results in other medical problems, treatment for endocrine dysfunction associated with pituitary malfunction is symptomatic and thus supportive;however, in some cases, surgery may be needed.
- "Empty Sella Syndrome Information Page | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Retrieved 2017-03-05.
- "Empty sella syndrome: MedlinePlus Medical Encyclopedia". medlineplus.gov. Retrieved 6 March 2017.
- "Empty sella syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2017-03-05.
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- Horton, Arthur MacNeill (2012-01-01). The Encyclopedia of Neuropsychological Disorders. Springer Publishing Company. p. 282. ISBN 9780826198549.
- González-Tortosa, J (2009). "Primary empty sella: Symptoms, physiopathology, diagnosis and treatment". Neurocirugia (Asturias, Spain). 20 (2): 132–51. PMID 19448958.