From the Guidelines
Empty sella syndrome is best diagnosed using MRI of the sella, with or without IV contrast, as it provides detailed imaging of the pituitary gland and surrounding structures, allowing for accurate characterization of the condition 1. The diagnosis of empty sella syndrome involves a combination of clinical evaluation, hormone testing, and imaging studies.
Key Diagnostic Steps:
- MRI of the sella is the preferred diagnostic imaging modality, as it can reliably depict the anatomy and pathologies involving the pituitary gland, infundibulum, optic chiasm, and vascular structures 1.
- Comprehensive hormone testing is essential to identify any pituitary hormone deficiencies, including measurement of TSH, free T4, cortisol, ACTH, prolactin, LH, FSH, estrogen/testosterone, and growth hormone levels.
- The presence of an enlarged sella turcica filled with cerebrospinal fluid and a flattened pituitary gland on MRI is characteristic of empty sella syndrome.
Management:
- Asymptomatic patients with normal hormone levels require regular monitoring without specific treatment 1.
- If hormone deficiencies are detected, appropriate hormone replacement therapy is necessary, such as levothyroxine for hypothyroidism, hydrocortisone for adrenal insufficiency, sex hormone replacement, and growth hormone replacement if indicated.
- Visual disturbances or CSF leakage may require neurosurgical consultation, while headaches can be managed with standard analgesics, and weight gain should be addressed through lifestyle modifications.
- Regular follow-up with an endocrinologist is important to monitor hormone levels and adjust replacement therapy as needed, typically every 3-6 months initially and then annually once stable.
From the Research
Diagnosis of Empty Sella Syndrome
- Empty sella syndrome is characterized by the herniation of the subarachnoid space within the sella turcica, often associated with a variable degree of flattening of the pituitary gland 2.
- The syndrome can be primary or secondary, with primary empty sella (PES) being an idiopathic disease and secondary empty sella occurring after treatment of pituitary tumors or other pituitary pathologies 2, 3.
- Diagnosis of empty sella syndrome requires a multidisciplinary approach, including endocrine, neurological, and ophthalmological experts 2, 4, 5.
Clinical Presentation
- Empty sella syndrome can present with a range of clinical symptoms, including headache, visual disturbances, and endocrine abnormalities such as hypopituitarism 2, 3, 5.
- The syndrome can also be asymptomatic, with empty sella being an incidental finding on brain imaging scans 4.
- Children with empty sella syndrome are more likely to have clinical symptoms and endocrinopathies, such as growth hormone deficiency, hypogonadotropism, or multiple pituitary hormone deficiencies 3.
Management and Treatment
- Management of empty sella syndrome involves replacement of hormone deficiencies and occasionally surgical measures to relieve obstructive intracranial lesions 3.
- A multidisciplinary approach is essential for the proper diagnosis, management, and follow-up of empty sella syndrome, including endocrine, neurological, and ophthalmological experts 2, 4, 5.
- Early assessment and management are necessary to detect endocrine abnormalities and institute appropriate replacement therapy, ensuring a better quality of life for patients with empty sella syndrome 6.