What are the symptoms and clinical manifestations of empty sella syndrome?

Empty Sella Syndrome: Clinical Manifestations

Empty sella syndrome (ESS) presents with a constellation of endocrine, neurological, and ophthalmologic symptoms, though many patients remain asymptomatic with only an incidental radiographic finding. 1, 2

Primary Symptom Categories

Endocrine Manifestations

• Pituitary hormone deficiencies occur in 19-51% of patients, with thyroid-stimulating hormone, T3, and T4 deficiencies seen in up to 48% of cases 1, 3, 4
• Hyperprolactinemia is present in approximately 28% of cases 1
• Clinical manifestations include:
  • Fatigue and cold intolerance (hypothyroidism) 1, 2
  • Sexual dysfunction and hypogonadism 1, 2
  • Growth hormone deficiency 1, 4
  • Less commonly, hypersecretion syndromes including acromegaly and Cushing's syndrome 5

Neurological Symptoms

• Headache is the most common symptom, occurring in approximately 70% of symptomatic patients 5
• Headaches may have features of increased intracranial pressure, particularly when ESS is associated with idiopathic intracranial hypertension (IIH) 2
• CSF rhinorrhea occurs in 11.8% of cases and represents a critical complication requiring specific management 5
• Pulsatile tinnitus may indicate elevated intracranial pressure 6

Ophthalmologic Manifestations

• Visual disturbances occur in approximately 34% of symptomatic patients 5
• Specific findings include:
  • Visual field defects 5, 4
  • Papilledema (when associated with increased intracranial pressure) 2, 7
  • Declining visual acuity 6
  • Optic chiasm compression in severe cases 1, 2

Important Clinical Context

Asymptomatic Presentation

• The majority of patients with empty sella remain asymptomatic, with the finding discovered incidentally on imaging performed for unrelated reasons 7, 3
• Approximately 30% of patients with empty sella demonstrate some degree of hypopituitarism upon testing, though most never develop clinical symptoms 8, 1, 2

Association with Idiopathic Intracranial Hypertension

• Partially empty sella is a typical neuroimaging feature of raised intracranial pressure and may indicate underlying IIH, particularly in patients presenting with headache and papilledema 2
• This represents a distinct clinical entity requiring specific management focused on weight loss and CSF pressure reduction 2

Critical Pitfalls to Avoid

• Do not attribute headache symptoms directly to empty sella without considering more common headache etiologies, as empty sella is usually an incidental finding unrelated to headache 1, 2
• Do not overlook the possibility of IIH in patients presenting with headache and partially empty sella, as this requires distinct management strategies 2
• Do not miss rare but important hormonal deficiencies by failing to perform appropriate screening, as endocrine dysfunction rates often exceed 10% and may reach 50% 1, 2
• Be aware that some cases may result from spontaneous pituitary adenoma necrosis, explaining the presence of both hypopituitarism and occasional hypersecretion 9