Introduction

The Empty sella refers to the radiological appearance of an enlarged or deformed sella turcica that is partially or
completely filled with cerebrospinal fluid. In the earlier days, the radiographic appearance of an enlarged sella
turcica was frequently equated with the presence of an intra or perisellar neoplasm. It was found, however, in some
of these patients on pneumoencephalography, that the enlarged sella was not occupied by a tumour mass, but a
significant amount of air was reaching the sella indicating that the subarachnoid space was extending well below the
clinoid processes. To describe this finding, several different terms were used such as intrasellar arachnoid
diverticulum, intrasellar cysts, deficient sellar diaphragm, intrasellar cistern1. Busch2, while reporting the presence
of empty sella in 5% of normal subjects on autopsy studies, used the term “empty sella” to describe this condition.
However, the term empty sella in fact, is a misnomer as the sella is not completely empty, but the pituitary is always
present both anatomically and functionally, though often it is displaced downwards and compressed by CSF
pressure. An empty sella thus is usually an incidental anatomic finding and occasionally it results in abnormal
pituitary function. Regardless of the size, the sella is completely or partially filled with CSF. It can occur at any age
and in either sex. However, it is more common in women and increases in frequency with age.

Definition The empty sella turcica is defined as an intrasellar herniation of the suprasellar subarachnoid space with
compression of the pituitary gland producing in many cases a remodelling of sella which results from a combination
of incomplete diaphragma sellae and an increased CSF fluid pressure.

Classification of empty sella

An empty sella may be classified as primary when this occurs in persons who have not received pituitary radiation or
pituitary surgery, while an empty sella discovered following such procedures is classified as secondary empty sella.

Pathogenesis

An incomplete sellar diaphragm is an essential pre-requisite for the development of the empty sella. All other factors
are only predisposing to the development of intrasellar subarachnoid herniation, whether by causing increased
pressure in the suprasellar subarachnoid space or by reduction in the size of the pituitary gland.

1. Congenital deficiency of the diaphragma sellae : Total absence of the diaphragma sellae has been reported to
occur in 20.5% of normal subjects5. Empty sella is also reported to occur in 5.5% of normal population.

2. Suprasellar promoting factors : When the diaphragma sellae is incomplete, cerebrospinal fluid pulsations act
directly on the upper aspect of the pituitary gland. The suprasellar cistern pressure is accentuated when the
intracranial pressure is raised. In addition, a posteriorly placed optic chiasma may also expose the upper surface of
the pituitary gland and thereby increase the cerebrospinal fluid pressure on it.

3. Pituitary promoting factors: Any reduction in size of the pituitary gland favours intrasellar extension of the
suprasellar subarachnoid space. Such reductions may be due to:

i) Physiological involution: These occur often in women. Pregnancies bring about a large variation in the size of the
pituitary gland and after delivery there is an involution. Similarly, after menopause there is a reduction in the
pituitary volume. These changes explain the very clear predominance of primary empty sella in women, especially
after the age of 40 years.

A similar situation occurs in cases of primary end-organ failure (thyroid, adrenal, gonad) wherein pituitary
hyperplasia occurs due to loss of feedback control. Replacement of the deficient hormone results in feedback
suppression of the pituitary tropic hormone secretion and involution of the hyperplastic pituitary gland resulting in
an ‘empty sella’.

ii) Pathological involution: Shrinkage of the pituitary gland may occur after post-partum pituitary necrosis (Sheehan’s
syndrome) or pituitary infarction in patients with vascular diseases, diabetes, increased intracranial pressure, head
injury, meningitis, or cavernous sinus thrombosis.

4. Rupture of an intrasellar or parasellar cyst: Fluid filled cysts of the sellar region are well known and may cause
visual or endocrine symptoms as well as changes in the contour of the sella. Rupture of such a cyst allows intrasellar
extension of the subarachnoid space. However, such rupture probably occurs only rarely.
Clinical features

Primary empty sella

Most persons with primary empty sella are asymptomatic and the detection of this abnormality may be incidental.
Typically primary empty sella syndrome occurs in obese, multiparous women. These patients sometimes may have
headache and hypertension. The headache has no characteristic pattern or localisation and hence may lead to an x-
ray examination which leads to the finding of this incidental abnormality. Infrequently, visual abnormalities have
been reported in the form of diminished visual acuity, peripheral field constriction, bitemporal hemianopia, or
quadrantinopia.

Endocrine abnormalities are not a common occurrence. Hyper-prolactinaemia occurs occasionally, possibly due to
stalk stretching or coincidental micro-prolactinomas. Growth hormone secretory reserve is often abnormal in these
patients, probably as a result of obesity. Occasionally, thyrotropin and gonadotropin deficiency is also noted. Rarely,
empty sella is associated with hormone excess possibly due to coexisting micro-adenoma within the compressed
gland. Spontaneous CSF rhinorrhoea and pseudotumour cerebri are two syndromes occasionally associated with an
empty sella.

Children with an empty sella most commonly have GH deficiency, although other pituitary hormone dysfunction may
occur. In children with isolated GH deficiency or multiple pituitary deficiencies, empty sella was observed in 48% but
was present in only 2% of children with normal pituitary function9.

Secondary empty sella

Secondary empty sella turcica is one which is associated with an iatrogenic event such as surgery, radiation, or both;
or with non-iatrogenic disease such as infarction and infection of the pituitary gland.

Post-surgical empty sella is related to the removal of pituitary tumours either from the transcranial or
transphenoidal route, with the diaphragma sellae incompetent at the end of the procedure. This subsequently leads
to herniation of the third ventricle and optic apparatus into the empty sella.

Secondary empty sella occurring in patients with non-surgical complicating events such as irradiation, infection, or
infarction in contrast does not occur due to a fresh defect created in the diaphragma sella, but have a diaphragma
sellae which is similar to that in the general population.

The predominant clinical finding in these patients is visual abnormality, occurring due to arachnoidal adhesions and
traction on the optic apparatus. They may have initial improvement in visual symptoms with surgery followed by the
recurrence of symptoms due to the development of empty sella. While some patients who do not have visual
symptoms initially may present with a fresh onset of these symptoms.

Diagnosis

Imaging

The lateral radiograph of the skull may reveal a normal sized sella or it may be enlarged. The primary empty sella is
frequently confused with intrasellar neoplasm when lateral skull radiographs reveal an enlarged sella. Certain
features, however, can serve to distinguish the primary empty sella in many instances. The typical empty sella shows
“symmetrical ballooning”, that is, a smoothly curving regular cavity of the sella.

There is maintenance of the “closed” configuration in contrast to intrasellar masses which tend to straighten the
curve of the dorsum and further separate the clinoid processes towards an “open” configuration. There is limited
evidence of thinning or fragmentation of the cortical bone margins, no attenuation of the clinoid processes and
symmetry of the sellar floor on frontal projection. However, a few empty sellae do not conform to this picture.

The confirmation of empty sella with x-rays used to be done by performing the pneumoencephalography or
metrizamide cisternography, which are no longer required with the advent of better imaging modalities.

Computerised tomography scans will show the pituitary fossa to be occupied largely by substance of CSF or water
density rather than a normal gland. Magnetic resonance imaging (MRI) can readily confirm the diagnosis of an empty
sella. On T1 sagittal MR images, extension of CSF into the sella is easily identified and remaining gland is compressed
along the floor. Typical central position of the infundibulum is a useful sign in an empty sella which helps to rule out
a cystic lesion in the suprasellar region.

Hormonal profile

To establish the diagnosis of hypopituitarism (partial or complete) and to determine the hypophyseal cause of
dysfunction, measurement of following hormones are required – serum thyrotropin and T4, corticotropin and
cortisol, LH and testosterone, basal and stimulated growth hormone and prolactin level. Usually, the hormonal
profile is normal in patients with empty sella. However, mild hyper-prolactinaemia (usually <100 µg/l) with or
without galactorrhoea occurs in approximately 15% of patients.

Complications

i) In view of the increased intracranial pressure and traction over the optic chiasma caused by the post-
surgical adhesions, visual field defects are known to occur and often require treatment.
ii) Cerebrospinal fluid rhinorrhoea can occur presenting as a non-traumatic and persistent nasal discharge.
This can also increase the chance of spread of infection to the meninges.
iii) Long standing increase in the intrasellar pressure can also lead to pituitary dysfunction, which, if present
needs to be corrected with appropriate hormone replacement therapy.

Treatment of empty sella

The primary empty sella is generally asymptomatic and incidentally detected, it requires no specific treatment.
However, when it is accompanied by endocrine dysfunction, replacement therapy of the appropriate target gland
hormones would be required. Presence of CSF rhinorrhoea may require surgical correction.

In secondary empty sella, adhesions are formed between the diaphragma sellae and the chiasma. Retraction of
these adhesions due to reduction in tumour size may pull the chiasma into the empty sella resulting in visual field
defects. This needs to be corrected by chiasmopexy. Packing the sellar cavity after transphenoidal removal of the
pituitary tumour can also be done to avoid herniation of the chiasma into the surgical empty sella.