Empty Sella Syndrome: Diagnosis and Management
Empty sella is a radiographic finding characterized by cerebrospinal fluid filling the sella turcica with compression of the pituitary gland against the sellar floor. 1 It can be classified as primary (spontaneous) or secondary (due to identifiable causes like surgery or radiotherapy).
Types and Pathophysiology
Primary Empty Sella (PES): Occurs when cerebrospinal fluid enters the sella through a defect in the sellar diaphragm
- Partial PES: <50% CSF filling with pituitary height >2mm
- Complete PES: ≥50% CSF filling with pituitary height <2mm 2
Secondary Empty Sella: Results from pituitary injury (apoplexy), surgery, or radiation treatment 3
Diagnostic Evaluation
Imaging
- MRI with high-resolution pituitary protocols: Gold standard showing flattened pituitary against sellar floor and CSF-filled sella turcica 1
- CT of the sella: Less sensitive but may show sellar enlargement; can miss cystic tumors 1
Hormonal Assessment
- Essential for all patients with empty sella, regardless of type, as affected-axis rates often exceed 10% and may reach 50% 2
- Complete PES has significantly higher rates of secondary adrenal insufficiency and hypogonadism compared to partial PES 2
Recommended Testing
- Morning ACTH and cortisol
- TSH and free T4 (secondary hypothyroidism more common in males)
- FSH, LH, estradiol (females) or testosterone (males)
- Prolactin
- Growth hormone and IGF-1 2, 4
Additional Evaluations
- Ophthalmologic examination: Mandatory to assess for papilledema 1
- Lumbar puncture: If symptoms suggest increased intracranial pressure (diagnostic criterion: >250 mm CSF in adults) 1
Clinical Implications
- Up to 30-40% of patients with empty sella demonstrate some degree of hypopituitarism 1, 5
- Complete PES patients have higher rates of multiple hormone deficiencies (two or more affected axes) compared to partial PES 2
- Most patients with partial empty sella remain asymptomatic with good prognosis 1, 6
Management Approach
- Comprehensive hormonal evaluation for all patients with empty sella, even if asymptomatic 6, 4
- Hormone replacement therapy based on specific deficiencies:
- Levothyroxine for hypothyroidism (only after steroid replacement in cases with concurrent adrenal insufficiency)
- Hydrocortisone 15-25 mg daily in divided doses for adrenal insufficiency
- Sex hormone replacement for hypogonadism
- Growth hormone if deficient 1
- Regular follow-up with endocrine evaluation
- If no hormonal abnormalities are detected initially, reevaluation at 24-36 months is suggested 5
Important Considerations
- Patients with both adrenal insufficiency and hypothyroidism should start steroids before thyroid hormone replacement to avoid adrenal crisis 1
- Patients with adrenal insufficiency should obtain and carry a medical alert bracelet 1
- In atypical presentations, secondary causes of increased intracranial pressure should be thoroughly investigated 1
Prognosis
The majority of patients with radiographic findings of empty sella have a good prognosis and never become symptomatic. If symptoms do arise, they usually have minimal impact on quality of life 6. However, regular monitoring is essential due to the potential for developing hormonal deficiencies over time.