From the Guidelines
Partial empty sella syndrome (PESS) in a 30-year-old female should be evaluated with a comprehensive clinical history, physical examination, laboratory testing of pituitary hormones, and imaging studies, with MRI being the gold standard to confirm partial empty sella and rule out other sellar pathologies, as recommended by the American College of Radiology 1. The diagnostic workup for PESS should include:
- A comprehensive clinical history and physical examination to identify signs and symptoms such as headaches, visual disturbances, menstrual irregularities, galactorrhea, fatigue, and symptoms of specific hormonal deficiencies
- Laboratory testing of pituitary hormones including:
- Prolactin
- Thyroid function tests (TSH, free T4)
- Gonadal hormones (FSH, LH, estradiol)
- Growth hormone (GH) with IGF-1
- Morning cortisol with ACTH
- Dynamic testing may be necessary to assess pituitary reserve, including:
- Insulin tolerance test or glucagon stimulation test for GH and cortisol reserve
- GHRH-arginine test for GH deficiency
- Imaging studies, with MRI being the preferred diagnostic imaging modality for evaluation of the pituitary and sellar regions, as it can reliably depict anatomy and pathologies involving the pituitary gland, infundibulum, optic chiasm, and vascular structures 1
- Visual field testing should be performed if the patient reports visual symptoms or if MRI shows compression of the optic chiasm Many patients with PESS have normal pituitary function and require no specific treatment beyond regular monitoring, however, hormone replacement therapy should be initiated for documented deficiencies, with specific regimens tailored to the affected hormonal axes 1. The underlying pathophysiology typically involves herniation of the subarachnoid space into the sella turcica through an incompetent diaphragma sellae, often associated with increased intracranial pressure or following regression of a pituitary adenoma. MRI sella without and with IV contrast or MRI sella without IV contrast is usually appropriate for the initial imaging of adults with suspected or known hypofunctioning pituitary gland, as recommended by the American College of Radiology 1.
From the Research
Signs and Symptoms of Partial Empty Sella Syndrome
- The signs and symptoms of partial empty sella syndrome can vary, but may include endocrine disorders such as growth hormone deficiency, hypogonadotropism, or multiple pituitary hormone deficiencies 2
- Some patients may be asymptomatic, while others may experience symptoms such as visual field defects, pituitary insufficiency, or pituitary hypersecretion 3
- In rare cases, partial empty sella syndrome may be associated with secondary adrenal insufficiency, as seen in a case report of a 24-year-old woman with primary antiphospholipid syndrome 4
Diagnostic Workup for Partial Empty Sella Syndrome
- Magnetic resonance imaging (MRI) is the gold standard for radiological diagnosis of empty sella syndrome, including partial empty sella syndrome 5
- Endocrine pituitary evaluation should be assessed at the moment of diagnosis, along with careful imaging using MRI 5
- A suggested management protocol for asymptomatic empty sella includes an endocrine panel for evaluation of pituitary function and a referral to ophthalmology for formal assessment of papilledema 6
- Tailored management and referrals should be based on the results of these evaluations, as well as treatment of any subsequent symptomatology 6
Epidemiology and Characteristics of Partial Empty Sella Syndrome
- The incidence of empty sella is about 12% on neuroimaging, while in clinical practice it can reach 35% 5
- Endocrine pituitary disorders are reported in 19% to 40% of patients with empty sella syndrome 5
- Partial empty sella syndrome is more likely to be asymptomatic, but may still require careful evaluation and management to prevent progression to symptomatic empty sella syndrome 6