What specialty follows empty sella syndrome?

Specialty Management for Empty Sella Syndrome

Empty sella syndrome should be primarily managed by an endocrinologist, with multidisciplinary involvement from neurology and ophthalmology as needed. 1

Primary Care Approach

When empty sella is discovered (usually incidentally on imaging):

1. Initial referral to endocrinology for comprehensive hormonal evaluation

   • Up to 30-40% of patients with empty sella demonstrate some degree of hypopituitarism 1, 2
   • Complete empty sella has significantly higher rates of secondary adrenal insufficiency and hypogonadism compared to partial empty sella 2

2. Ophthalmology referral for formal assessment of:

   • Papilledema
   • Visual field testing 1, 3
   • This is particularly important as empty sella may be associated with idiopathic intracranial hypertension 4

3. Neurology consultation when:

   • Neurological symptoms are present
   • Idiopathic intracranial hypertension is suspected 4

Endocrinological Management

The endocrinologist will typically:

• Perform comprehensive hormone testing including:

  • Morning ACTH and cortisol
  • TSH and free T4
  • FSH, LH, estradiol (females) or testosterone (males)
  • Prolactin
  • Growth hormone and IGF-1 1, 2, 5

• Initiate hormone replacement therapy based on specific deficiencies:

  • Hydrocortisone (15-25 mg daily in divided doses) for adrenal insufficiency
  • Levothyroxine (typically 1.6 mcg/kg/day) for hypothyroidism
  • Sex hormone replacement for hypogonadism
  • Growth hormone replacement if deficient 1

• Important: Steroid replacement must precede thyroid hormone replacement in patients with both adrenal insufficiency and hypothyroidism 1

Neurological and Ophthalmological Management

These specialists will focus on:

• Evaluation and management of increased intracranial pressure if present
• Assessment and monitoring of visual fields and papilledema
• MRI with high-resolution pituitary protocols is the gold standard for diagnosis 1
• Lumbar puncture may be necessary to measure opening pressure in suspected idiopathic intracranial hypertension 1

Follow-up Considerations

• Regular endocrine follow-up is essential even in initially asymptomatic patients
• Ophthalmological monitoring for those with visual symptoms or papilledema
• Patients with adrenal insufficiency should obtain and carry a medical alert bracelet 1

Clinical Pearls and Pitfalls

• Pitfall: Assuming empty sella is always asymptomatic - up to 52% of patients may have some form of pituitary insufficiency 5
• Pitfall: Failing to distinguish between primary and secondary empty sella, which requires different management approaches 4
• Pearl: Most patients with partial empty sella have good prognosis and remain asymptomatic 1
• Pearl: Complete empty sella (≥50% CSF filling, pituitary <2mm) has higher rates of hormonal abnormalities than partial empty sella 2

In rare cases with progressive visual symptoms not responding to medical management, neurosurgical intervention may be considered 6, but this is uncommon in modern practice.