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Empty Sella Syndrome

Empty Sella Syndromes

Empty sella syndrome is a radiological feature of an extended or depressed sella turcica that is filled completely or partially with cerebrospinal fluid.


Presentation

The symptoms related to empty sella syndrome vary from person to person. Especially in cases of primary empty sella syndrome, affected individuals are frequently asymptomatic and the disorder is often found incidentally through X-ray examination set up for other purposes.

Previously it was thought that among potentially associated symptoms, the major one is chronic headaches and visual disturbances. It is now believed that many of these patients suffer from idiopathic intracranial hypertension and empty sella syndrome might be a consequence. Though the pituitary gland is not usually affected, in rare cases hypopituitarism is observed. Some of the related symptoms are:

However, in cases of secondary empty sella syndrome visual abnormalities and decreased pituitary function are most likely to appear.

Pneumonia
  • Our patient had pneumonia with respiratory failure. He was intubated and mechanically ventilated. Subsequent tracheostomy was done because of failure of weaning.[ncbi.nlm.nih.gov]
Asymptomatic
  • Asymptomatic cysts of Rathke's pouch are fairly frequent autopsy observations. They can cause optic-chiasmatic and endocrine symptomatology or, very rarely, an Empty Sella syndrome if the cyst is very large.[ncbi.nlm.nih.gov]
  • It is usually recognized incidentally during brain imaging studies performed for different indications, and in most cases this condition is asymptomatic.[ncbi.nlm.nih.gov]
  • In adults, ES is most commonly found in older, obese, hypertensive, multiparous women and may be asymptomatic.[ncbi.nlm.nih.gov]
  • Signs and symptoms of hormonal imbalance (for example, hypopituitarism) may be present, as may headache, but some patients are asymptomatic.[medical-dictionary.thefreedictionary.com]
  • Six complained of headaches and menstrual irregularities, and two were asymptomatic. Three had visual symptoms and four had papilledema at the time of examination.[neurology.org]
Hypertension
  • The primary empty sella syndrome (ESS) represents a heterogeneous clinical picture characterized by endocrine disturbances and signs of intracranial hypertension.[ncbi.nlm.nih.gov]
  • Otolaryngologists should be familiar with this disease entity and the implications intracranial hypertension has on patient management.[ncbi.nlm.nih.gov]
  • The empty sella syndrome commonly presents with headache and visual impairment and occasionally with endocrine disturbances in hypertensive middle aged women.[ncbi.nlm.nih.gov]
  • Benign intracranial hypertension (BIH) and empty sella syndrome occurred in a patient with Cushing syndrome resulting from adrenal adenoma, removal of which was followed by return of cerebrospinal fluid pressure to normal.[ncbi.nlm.nih.gov]
  • Primary empty sella syndrome (ESS) shares with idiopathic intracranial hypertension (IIH) high intracranial pressure (ICP) and association with obesity. Bariatric surgery is effective in IIH treatment; no data, however, exist about ESS.[ncbi.nlm.nih.gov]
Long Arm
  • A 2 8/12-year-old boy with severe growth failure and mental retardation was found to have a maternally derived tandem duplication of the long arm of X chromosome, dup(X) (q13.3----q21.2).[ncbi.nlm.nih.gov]
Headache
  • Otolaryngologists frequently encounter patients with complaints of headaches. On occasion, normal physical examination findings may be accompanied by a roentgenographic enlargement of the sella turcica.[ncbi.nlm.nih.gov]
  • Intrasellar extradural placement of a detachable vascular balloon via a transsphenoidal approach was performed successfully in a patient with primary empty sella syndrome, relieving headache and visual field defect.[ncbi.nlm.nih.gov]
  • Substantial visual disturbance was accompanied by minimal endocrine dysfunction in an 8-year-old girl whose only other complaint was headache.[ncbi.nlm.nih.gov]
  • The empty sella syndrome commonly presents with headache and visual impairment and occasionally with endocrine disturbances in hypertensive middle aged women.[ncbi.nlm.nih.gov]
  • The empty sella syndrome is common in middle-aged women, usually presenting with headache, and only occasionally associated with endocrine or visual abnormalities. It is rare in childhood.[ncbi.nlm.nih.gov]
Bitemporal Hemianopsia
  • A 48-year-old man with a prolactin-secreting macroadenoma treated with cabergoline presented with progressive bitemporal hemianopsia.[ncbi.nlm.nih.gov]
  • Decrease in visual acuity, generalized by narrowing of its peripheral fields, bitemporal hemianopsia are possible. Papilledema is rare, but there is such a description in literature.[iliveok.com]
  • Alternatively, Mortara and Norrell have described a lamina terminalisotomy which in theory vents the CSF pulsations and, in at least one case, resulted in successful treatment of a bitemporal hemianopsia.[pituitaryadenomas.com]
Sexual Dysfunction
  • Fifty-three of these patients reported originally headache, 12 reported visual alterations, and eight reported oligomenorrhea or sexual dysfunction without demonstrated hormonal alteration.[academic.oup.com]

Workup

Measurement of the following hormones is required to establish a diagnosis of hypopituitarism and to examine the hypophyseal cause of dysfunction [7] [8]:

  • Serum thyrotropin and T4
  • Corticotropin and cortisol 
  • Prolactin 
  • Luteinizing hormone and testosterone 
  • Basal and stimulated growth hormone

The hormonal profile is usually normal, however, approximately 15% of patients show mild hyperprolactinemia (usually <100 μg/l) with or without galactorrhoea. 

Normal or enlarge sized sella can be revealed through the lateral radiograph and is often confused with intrasellar neoplasm which is distinguishable by certain features. Normally an empty sella depicts "symmetrical ballooning", indicated by a smoothly bending regular sella turcica cavity. Straightening of the curve of the dorsum and further separation of the clinoid processes towards an "open" configuration is caused by maintenance of the "closed" configuration against intrasellar masses. There is limited evidence of thinning or fragmentation of the margins of cortical bone, no attenuation of the clinoid processes and uniformity of the floor of sella on frontal projection. 

The following are some different methods of imaging for empty sella syndrome:

  1. Plain film: Lateral skull x-ray appearances are indistinguishable from those of patients with a pituitary mass (eg. pituitary macroadenoma). Empty sella is confirmed with X-rays by performing pneumoencephalography or metrizamide cisternography, although they are nowadays no important imaging modalities.
  2. Magnetic resonance imaging (MRI) is the modality of choice for confirming the diagnosis of an empty sella. It will demonstrate the sella to be filled with CSF and the infundibulum can be seen to traverse the space, thereby excluding a cystic mass.
  3. Computed tomography (CT) generally shows a fossa filled with CSF, again of variable size. If thin section imaging is obtained the infundibulum may be seen coursing through the space.
Enlarged Sella
  • The authors treated 12 children, ranging in age between 2 and 8 years, in whom neuroradiological studies demonstrated an enlarged sella turcica filled with cerebrospinal fluid and herniation of suprasellar and arachnoid spaces.[ncbi.nlm.nih.gov]
  • A skull radiogram revealed an enlarged sella turcica. Computed tomography (CT) revealed a low density in the sella, and magnetic resonance imaging revealed equal intensity of the sella turcica and the cerebrospinal fluid.[ncbi.nlm.nih.gov]
  • The finding of a moderately enlarged sella turcica, due to a partial/complete absence of the sellar diaphragm.[medical-dictionary.thefreedictionary.com]
  • ESS may happen if you have an enlarged sella turcica. This is a bony structure where the pituitary gland sits at the base of the brain. Health experts don’t know what causes primary ESS.[elcaminohospital.org]
  • Key points ESS may happen if you have an enlarged sella turcica. This is a bony structure where the pituitary gland sits at the base of the brain. Health experts don’t know what causes primary ESS.[hopkinsmedicine.org]
Diffuse Encephalopathy
  • Postmortem examination revealed brain atrophy due to a diffuse encephalopathy, numerous calcified neurons in cerebral cortex, deep telencephalic and diencephalic nuclei, diffuse neuronal necrosis in hypothalamic nuclei, moderate atrophy of optic nerves[ncbi.nlm.nih.gov]

Treatment

As discussed, many patients affected by empty sella syndrome do not exhibit any symptoms and thus do not require any treatment. When symptoms start to appear the treatment is directed towards the specific symptom. For example, in case of affected pituitary, specific hormone replacement therapy is needed; whereas in case of liquorrhea [9] [10], surgery may be required.

Follow up

As empty sella is related to neuroradiological features and endocrine symptoms, patients suffering from primary empty sella syndrome should undergo neurological, ophthalmological and endocrine evaluation. 

Patients subjected to endocrine abnormalities must be treated medically and should be reevaluated according to standard guidelines. If any hormonal abnormalities are found, endocrine screening is needed followed by some defined endocrine testing. This procedure has a high detection rate and shows only a small number of unconfirmed diagnoses.

Post surgery reevaluation is required at least twice a year for examining side effects and long-term results. Some indications for surgical attention required for empty sella syndrome are given below:

Idiopathic intracranial hypertension patients should receive follow-up radiological study. Furthermore, affected persons diagnosed with empty sella syndrome with visual disturbance should also be analyzed for optic disc, visual field patterns and intraocular pressure measurement on follow up visits.

Prognosis

Primary empty sella syndrome may result in mild hyperprolactinemia [5]. Complications of secondary empty sella syndrome may be related to the underlying disorder [6].

Etiology

Regardless of the idiopathic nature of the primary empty sella syndrome, it is believed that a congenital defect in the diaphragma sellae contributes in the development of primary empty sella syndrome. The diaphragma sellae is a fold of dura mater which surrounds the brain and spinal cord and envelops the sphenoid bone, where the pituitary and sella turcica are situated. In some cases, a hernia of underlying membranes can occur due to a tear in the diaphragma sellae, that causes leakage and accumulation of CSF in sella turcica. The pressure produced by the fluid causes the sella turcica to flatten or enlarge which results in compressed and flattened pituitary.

Secondary empty sella syndrome occurs due to various types of conditions such as:

Epidemiology

Detection of epidemiology for empty sella syndrome is difficult without contamination by the patients with idiopathic intracranial hypertension [2] [3]. However, most of the researchers report that primary empty sella syndrome affects women more often than men. Most cases occur in middle-aged women who are obese and have hypertension

Sex distribution
Age distribution

Pathophysiology

An empty sella is assumed to result from herniation of the arachnoid space towards the pituitary fossa due to a weak diaphragm. This can happen in patients with normal CSF pressure, however, the likelihood is higher when intracranial hypertension is present. 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.

Prevention

Once diagnosed, the disorder cannot be precluded but a multidisciplinary approach and close examination of the affected individual is needed. Instructions should be given to the patient and the family about reporting new symptoms and routine follow-up visits. If affected individuals exhibit symptoms of unnatural pituitary function (e.g. impotence or disrupted menstrual cycle), tests and treatment must be provided in consultation with the physician. If the patient does not show new symptoms after five years from initiation of the treatment, the follow up visits can be reduced. As for visual disturbances, the patient is in higher risk of injury and relatives should be instructed in attending self-care and assisting in everyday life activities. However, patients suffering from liquorrhea should maintain bed rest in a semi-Fowler position. Also, sneezing and coughing should be avoided, as these increase the pressure within the skull and favor a continuity of CSF leak.

Summary

Empty sella syndrome is a rare disorder characterized by enlargement or malformation of the sella turcica, a saddle-shaped depression located in the sphenoid bone, in which resides the pituitary gland. In empty sella syndrome, the malformed sella turcica is either partially or completely filled with cerebrospinal fluid (CSF). As a result, the pituitary gland is often compressed and flattened so that the sella turcica appears empty. Most of the individuals with this syndrome do not have any associated symptoms.

In every case, the radiological feature does not provide a pathological situation. It is the pathological variant of a radiologically verified empty sella. There are two types of empty sella syndrome:

  • Primary empty sella syndrome
  • Secondary empty sella syndrome

Previously, an enlarged sella turcica was often detected radiographically accompanied by the presence of a perisellar neoplasm or an intrasellar neoplasm. It was found on pneumoencephalography. In this medical procedure most of the cerebrospinal fluid was drained from around the brain by means of a lumbar puncture and replaced with air, oxygen, or helium to allow the structure of the brain to show up more clearly on an X-ray image. If the expanded sella was not filled with a tumor mass but with a substantial volume of air, the extension of subarachnoid space well below the clinoid processes was indicated. Usually, these findings were described through several different terms like:

  • Intrasellar cistern
  • Intrasellar arachnoid diverticulum 
  • Deficient sellar diaphragm
  • Intrasellar cysts

Autopsy studies reported that empty sella is present in 5% of normal subjects. Usually an empty sella is an incidental anatomic finding that occasionally can result into abnormal pituitary function. Whatever small or big, the sella is either completely or relatively filled with cerebrospinal fluid. Though it is independent of age and sex, empty sella syndrome is commonly seen in women with increasing frequency with age [1] [2] [3] [4].

Patient Information

Empty sella syndrome is characterized by the enlargement of the head structure called sella turcica, a saddle-shaped depression located in the bone at the base of the skull where the pituitary gland is found. In this pathological condition, the sella turcica is partially or completely filled with cerebrospinal fluid, which in turn begins to compress and flatten the pituitary gland. Main clinical signs associated with empty sella syndrome include headache and pituitary malfunction, even though many patients show no signs at all (remain asymptomatic). Empty sella might come as primary disorder if the underlying cause is unknown (idiopathic), or as secondary disorder if it follows another pathology such as pituitary tumor or pituitary trauma.

Causes

The pituitary gland is a major endocrine gland, a pea-sized body attached to the base of the brain that is important in controlling growth and development as well as the functioning of the other endocrine glands. It is placed in a saddle-like compartment in the skull, the sella turcica. Primary empty sella syndrome occurs whenever one of the layers (arachnoid) covering the outside of the brain comes out into the sella and puts pressure on the pituitary. 

Secondary empty sella syndrome is caused by:

  • Injury or trauma
  • Pituitary gland tumor
  • Infection
  • Surgery
  • Radiation therapy

The pituitary gland controls other glands by the pituitary hormone, these are listed below:

  • Thyroid
  • Adrenal glands
  • Testicles
  • Ovaries

Any abnormality within the pituitary gland can result into problems with those glands and abnormal hormone secretion from the glands.

Symptoms

In most of the cases, empty sella syndrome does not exhibit any symptoms or loss of functionality in the pituitary gland. Possible symptoms are:

Diagnosis

Often, empty sella syndrome is discovered by radiological diagnosis of the head and the brain by MRI or CT scan. In most of the cases, the pituitary gland shows normal functionality, though the clinician may still examine the pituitary gland tracing any loss of functionality. Sometimes high pressure in the brain is detected by tests like:

  • Lumbar puncture (Spinal tap)
  • Examination of retina done by an ophthalmologist

Treatment

For primary empty sella syndrome, no treatment is required if the pituitary gland shows normal functionality. However, medication may be prescribed attending unnatural hormone profile. For secondary empty sella syndrome, treatments are related to replacement of the lacking hormones. Sometimes, surgery is required repairing the sella turcica.

Prognosis

Primary empty sella syndrome neither causes any health problems nor affects life expectancy. A possible complication related to primary empty sella syndrome is hyperprolactinemia, a condition in which a person has higher-than-normal levels of the hormone prolactin in the blood. Secondary empty sella syndrome may lead to pituitary gland disease or reduction of the amount of pituitary hormone.

If affected individuals develop any symptoms regarding abnormal pituitary function (e.g. menstrual cycle problems, impotence) medical attention is needed.

References

Article

  1. Guitelman M, Garcia Basavilbaso N, Vitale M, et al. Primary empty sella (PES): a review of 175 cases. Pituitary. 2013; 16(2):270-4.
  2. Lenz AM, Root AW. Empty sella syndrome. Pediatr Endocrinol Rev. 2012; 9(4):710-5.
  3. Aruna P, Sowjanya B, Reddy PA, et al. Partial empty sella syndrome: a case report and review. Indian J Clin Biochem. 2014; 29(2):253-6.
  4. Paroder V, Miller T, Cohen MM Jr, et al. Absent sella turcica: a case report and a review of the literature Fetal Pediatr Pathol. 2013; 32(5):375-83.
  5. Durodoye OM, Mendlovic DB, Brenner RS, et al. Endocrine disturbances in empty sella syndrome: case reports and review of literature. Endocr Pract. 2005; 11(2):120-4.
  6. Dange N, Redhu R, Kawale J, et al. Primary amenorrhea due to empty sella: an underestimated entity. Turk Neurosurg. 2012; 22(4):499-501.
  7. Giustina A, Aimaretti G, Bondanelli M, et al. Primary empty sella: Why and when to investigate hypothalamic-pituitary function. J Endocrinol Invest. 2010; 33(5):343-6.
  8. Kim JH, Ko JH, Kim HW, et al. Analysis of empty sella secondary to the brain tumors. J Korean Neurosurg Soc. 2009; 46(4):355-9.
  9. González-Tortosa J. Primary empty sella: symptoms, physiopathology, diagnosis and treatement. Neurocirugia (Astur). 2009;20(2):132-51.
  10. Naing S, Frohman LA. The empty sella. Pediatr Endocrinol Rev. 2007;4(4):335-42.

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Last updated: 2018-06-22 03:38