Which are the causes of Empty Sella Syndrome?

Empty Sella Syndrome (ESS) occurs when the sella turcica—the bony structure at the base of the skull holding the pituitary gland—appears empty or flattened on imaging because it is filled with cerebrospinal fluid. The cause is generally categorized into primary Empty Sella Syndrome, often linked to structural weakness in the diaphragm sellae, and secondary Empty Sella Syndrome, which results from trauma, surgery, radiation, or pituitary conditions.

What causes Empty Sella Syndrome?

In most cases, Empty Sella Syndrome is not a single disease but a radiological finding. Primary Empty Sella Syndrome is thought to result from a defect in the diaphragm sellae, the membrane that covers the sella turcica. If this membrane is incomplete or thin, cerebrospinal fluid can pulsate into the sella, gradually flattening the pituitary gland against the bony walls. Secondary Empty Sella Syndrome is caused by known external factors that damage the pituitary gland or the surrounding tissue, leading to the collapse of the sella contents.

Is Empty Sella Syndrome genetic?

While Empty Sella Syndrome is not typically classified as a hereditary genetic disorder, researchers are investigating whether certain connective tissue predispositions might make individuals more susceptible to the structural changes associated with the condition. It is rarely linked to specific chromosomal mutations, and most cases appear sporadically rather than following a traditional pattern of inheritance.

What are the risk factors for Empty Sella Syndrome?

Understanding the difference between a cause and a risk factor is vital. A cause is the direct physiological mechanism (like a membrane defect), whereas risk factors increase the likelihood of developing the condition. Key factors include:

• Obesity and Hypertension: Often associated with primary cases in women.


• Pituitary Surgery: Can lead to secondary Empty Sella Syndrome.


• Radiation Therapy: May shrink the pituitary gland, allowing fluid to fill the space.


• Trauma: Head injuries that affect the base of the skull.


• Pituitary Apoplexy: Sudden bleeding or infarction within the gland.

Next steps

• Consult an endocrinologist to assess pituitary hormone function.


• Undergo an MRI scan if you experience persistent headaches or vision changes.


• Connect with the 15 members currently sharing their experiences with Empty Sella Syndrome at DiseaseMaps.org.


• Monitor for symptoms like fatigue or hormonal imbalances, which may require clinical management.

Medical disclaimer: This content is for informational purposes only and does not constitute professional medical advice, diagnosis, or treatment.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Empty Sella Syndrome.


• Orphanet: Primary Empty Sella Syndrome.


• National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK).


• The Pituitary Foundation: Understanding Sellar Region Conditions.