Abstract

Empty sella syndrome (abbreviated ESS) is where the pituitary gland shrinks or becomes flattened, filling the sella turcica with cerebrospinal fluid on imaging instead of the normal pituitary. ESS can be found in the diagnostic workup of pituitary disorders, or as an incidental finding when imaging the brain.

Classification

There are two types of ESS: "primary" and "secondary".

- Primary ESS happens when a small anatomical defect above the pituitary gland increases pressure in the sella turcica and causes the gland to flatten out along the interior walls of the sella turcica cavity. Primary ESS is associated with obesity and increase in intracranial pressure in women.

- Secondary ESS is the result of the pituitary gland regressing within the cavity after an injury, surgery, or radiation therapy. Individuals with secondary ESS due to destruction of the pituitary gland have symptoms that reflect the loss of pituitary functions, such as intolerance to stress and infection.

Signs/symptoms

If there are symptoms, people with empty sella syndrome can have headaches, as symptoms, which subsides when lying down. Additional symptoms are as follows:

- Abnormality (middle ear ossicles)

- Cryptorchidism

- Dolichocephaly

- Arnold-Chiari type I malformation

- Meningocele

- Patent ductus arteriosus

- Muscular hypotonia

- Platybasia

Cause

The cause of this condition is divided into primary and secondary, as follows:

- The cause of this condition in terms of "secondary empty sella syndrome" happens when a tumor or surgery damages the gland, this is an acquired manner of the condition.

- ~70% of patients with Idiopathic intracranial hypertension will have empty sella on MRI

- The cause of "primary empty sella syndrome" is a congenital defect(diaphragma sellae)

Mechanism

The normal mechanism of the pituitary gland sees that it controls the hormonal system, which therefore have an effect on growth, sexual development and adrenocortical function. The gland is divided into "anterior" and "posterior".

Its pathophysiology is such that individuals affected with the condition can have cerebrospinal fluid build-up, which in turn causes intracranial pressure leading to "headaches" for the individual.

Diagnosis

The diagnosis of ESS, done via examination (and test), may be linked to early onset of puberty, growth hormone deficiency or pituitary gland dysfunction(at an early age). Additionally there is:

Diagnosis | Differential diagnosis

The major differential to consider in empty sella syndrome is intracranial hypertension, of both unknown and secondary causes, and an epidermoid cyst, which can mimic cerebrospinal fluid due to its low density on CT scans, although MRI can usually distinguish the latter diagnosis.

Treatment

In terms of management, unless the syndrome results in other medical problems, treatment for endocrine dysfunction associated with pituitary malfunction is symptomatic and thus supportive;however, in some cases, surgery may be needed.