Empty sella syndrome

Overview

Empty sella syndrome (ESS) is a condition in which the sella turcica—a bony structure at the base of the brain that houses the pituitary gland—appears empty on imaging studies. This appearance is due to the flattening or shrinkage of the pituitary gland, which may be compressed against the walls of the sella. Despite the name, the sella is not truly empty; it is usually filled with cerebrospinal fluid (CSF). ESS can be classified as either primary (occurring without an obvious cause) or secondary (due to injury, surgery, or disease affecting the pituitary gland). It can be asymptomatic or associated with hormonal imbalances, vision problems, and headaches.

Causes

ESS is classified based on its origin:

• Primary Empty Sella Syndrome:

  • Occurs when a defect in the diaphragma sellae (the membrane covering the sella turcica) allows CSF to enter and compress the pituitary gland

  • More common in women and in individuals who are obese or have high blood pressure

• Secondary Empty Sella Syndrome:

  • Results from damage to the pituitary gland due to surgery, radiation therapy, head trauma, pituitary apoplexy (bleeding), or a pituitary tumor

  • The gland shrinks or flattens, leading to an “empty” appearance on imaging

Symptoms

Many people with ESS, especially the primary form, have no symptoms and the condition is discovered incidentally during brain imaging for other reasons. When symptoms are present, they may include:

• Headaches: The most common symptom, though the cause-effect relationship is unclear

• Visual disturbances: Blurred or double vision, or loss of peripheral vision (especially if the optic chiasm is affected)

• Hormonal dysfunction:

  • Fatigue

  • Irregular menstrual periods or infertility

  • Low libido or erectile dysfunction

  • Weight changes, cold intolerance, or other symptoms of pituitary hormone deficiency

• In rare cases: CSF rhinorrhea (leakage of cerebrospinal fluid from the nose)

Diagnosis

Diagnosis of ESS is typically made through imaging and hormone evaluation. The diagnostic process includes:

• Magnetic resonance imaging (MRI): The preferred method for visualizing an empty or partially empty sella; shows the pituitary gland flattened or displaced

• Computed tomography (CT) scan: May also detect an empty sella, though less detailed than MRI

• Endocrine evaluation: Blood tests to assess levels of pituitary hormones (e.g., ACTH, TSH, LH, FSH, GH, and prolactin)

• Ophthalmologic exam: To evaluate vision if optic nerve involvement is suspected

Treatment

Treatment depends on whether the patient has symptoms or hormonal deficiencies. In many asymptomatic cases, no treatment is necessary. Management strategies may include:

• Observation: Regular follow-up with imaging and hormone testing for asymptomatic individuals

• Hormone replacement therapy: For patients with pituitary hormone deficiencies (e.g., corticosteroids, thyroid hormones, sex hormones, or growth hormone)

• Treatment of underlying cause: For secondary ESS, addressing the primary issue (e.g., tumor, trauma)

• Vision care: If visual disturbances are present, neurosurgical consultation may be needed to relieve pressure on the optic chiasm

• CSF leak repair: In rare cases of CSF rhinorrhea, surgical repair may be necessary

Prognosis

The prognosis for most individuals with empty sella syndrome is favorable, especially in primary ESS where symptoms may be minimal or absent. When hormone deficiencies are present, they can usually be managed effectively with hormone replacement therapy. Long-term outlook depends on the extent of pituitary dysfunction and the presence of other neurological or systemic conditions. Regular monitoring by an endocrinologist is often recommended to ensure hormonal balance and prevent complications.