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:

• Benign intracranial pressure
• Liquorrhea 
• Isolated growth hormone deficiency
• Optic disc swelling
• Visual abnormalities 

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

• 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]

  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]

• Pseudotumor

  An extensive work-up disclosed the presence of pseudotumor cerebri and an empty sella turcica. [ncbi.nlm.nih.gov]

  […] cerebri Commonly Associated Conditions Benign intracranial hypertension (BIH) Obesity Pseudotumor cerebri Hypertension Diabetes mellitus Autoimmune hypophysitis: There have been case reports where it was noted that patients would eventually develop ESS [unboundmedicine.com]

  POSNER First published June 1, 1975, DOI: https://doi.org/10.1212/WNL.25.6.565 Abstract Eight patients had both the “primary empty sella syndrome,” diagnosed by the finding of an air-filled sella turcica at pneumoencephalography, and pseudotumor cerebri [neurology.org]

  Primary empty sella is seen during pregnancy, obesity, and pseudotumor cerebri (a condition of increased spinal fluid pressure seen in obesity and associated with vision loss). [pituitary.org]

• Infertility

  […] enlarged Pituitary hemorrhage Pituitary infarction Posterior pituitary disorder Primary hypothalamic insufficiency Rathkes pouch cyst Rathke's pouch cyst Renon-Delille syndrome Secondary hypothalamic insufficiency Secondary pituitary - hypothalamic infertility [icd9data.com]

  Individuals with secondary ESS can sometimes have symptoms that reflect the loss of pituitary functions, such as the ceasing of menstrual periods, infertility, fatigue, and intolerance to stress and infection. [ninds.nih.gov]

  Individuals with secondary ESS due to destruction of the pituitary gland have symptoms that reflect the loss of pituitary functions, such as the ceasing of menstrual periods, infertility, fatigue, and intolerance to stress and infection. [mediconotebook.com]

  Damage to the pituitary may lead to symptoms such as infertility, cessation of menstrual periods (amenorrhea), fatigue and intolerance to infections and stress. [news-medical.net]

• Galactorrhea

  Abstract The presence of galactorrhea and/or hyperprolactinemia in patients with the primary empty sella syndrome (PESS) has been proposed to be of hypothalamic etiology. [ncbi.nlm.nih.gov]

  Jones JR, DeHempel PAC, Kemmann E, et al: Galactorrhea and amenorrhea in a patient with an empty sella. Obstet Gynecol 49:S9, 1977. Google Scholar 54. [link.springer.com]

  Diagnosis and Management of Galactorrhea. American Family Physician [On-line journal]. [labtestsonline.it]

  Observation It consists in a 47 years old female patient, who has been followed up since the age of 31 years for bilateral galactorrhea and a spaniomenorrhea. There has been no associated drug intake. [omicsonline.org]

• Weight Gain

  Weight gain in some populations (e.g. the Pima Indians of Arizona) is predicted by a lower physical activity but in most instances it seems unlikely that reduced physical activity accounts for more than 40% of the weight gain. [ncbi.nlm.nih.gov]

• Rhinorrhea

  Following a shunt procedure, ICP reverted to normal and the CSF rhinorrhea ceased for 10 weeks. The sella turcica was packed with muscle after the CSF rhinorrhea recurred. [ncbi.nlm.nih.gov]

  Finally, CSF rhinorrhea is a complication of empty sella syndrome in approx 10% of patients (147,148). [78stepshealth.us]

• Sneezing

  Its insidious onset may occur with a sneezing or coughing episode which may lead to an incorrect diagnosis of allergic rhinitis or vasomotor rhinorrhoea. [ncbi.nlm.nih.gov]

  Also, sneezing and coughing should be avoided, as these increase the pressure within the skull and favor a continuity of CSF leak. [symptoma.com]

• Hypertension

  (BIH) Obesity Pseudotumor cerebri Hypertension Diabetes mellitus Autoimmune hypophysitis: There have been case reports where it was noted that patients would eventually develop ESS after autoimmune hypophysitis ( 3 ). [unboundmedicine.com]

  In primary empty sella syndrome, this is usually the result of benign intracranial hypertension. Accordingly, appropriate therapy for the latter should be initiated. [78stepshealth.us]

  Otolaryngologists should be familiar with this disease entity and the implications intracranial hypertension has on patient management. [ncbi.nlm.nih.gov]

• Visual Impairment

  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]

  New technique for Chiasmapexy using iliac crest bone graft: 2 cases of visual impairment caused by empty sella syndrome. [revistas.unilibre.edu.co]

• Hunger

  This part of the nervous system manages the balance of energy, heat and water in the body, which includes things like body temperature, heartbeat, urination, sleep, hunger and thirst. [ncbi.nlm.nih.gov]

• Amenorrhea

  The case of a 37-year-old woman with secondary amenorrhea and clear signs of hyperandrogenism is reported. [ncbi.nlm.nih.gov]

  Damage to the pituitary may lead to symptoms such as infertility, cessation of menstrual periods (amenorrhea), fatigue and intolerance to infections and stress. [news-medical.net]

  Jones JR, DeHempel PAC, Kemmann E, et al: Galactorrhea and amenorrhea in a patient with an empty sella. Obstet Gynecol 49:S9, 1977. Google Scholar 54. [link.springer.com]

  Low-grade hyperprolactinemia (of stalk compression origin), because of its long-term adverse effects, should be treated if associated with symptoms of hypogonadism, such as amenorrhea, and is often exquisitely responsive to low-dose dopamine agonist therapy [78stepshealth.us]

• Secondary Amenorrhea

  The case of a 37-year-old woman with secondary amenorrhea and clear signs of hyperandrogenism is reported. [ncbi.nlm.nih.gov]

  Endocrine Studies Clinical endocrine dysfunction is rare in adult patients with the empty sella syndrome; significant abnormalities are largely restricted to secondary amenorrhea, decreased libido, and loss of pituitary reserve. [pituitaryadenomas.com]

• Headache

  Headache Happiness Headache Happiness! Stop Your Headache BEFORE IT STARTS. How To Get Rid Of Your Headache BEFORE It Starts! The pain can be AGONIZING Headaches can stop you from doing all the things you love. [78stepshealth.us]

  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]

• Papilledema

  We describe a patient with CSF rhinorrhea who developed headache, papilledema, and visual disturbance after surgical repair of the CSF leak. The leak was repaired by the placement of a septal cartilage plug with a free mucosal suture graft. [ncbi.nlm.nih.gov]

  Three had visual symptoms and four had papilledema at the time of examination. [neurology.org]

  Do you have symptoms of high intracranial pressure (papilledema, headaches?). [msworld.org]

  Complaints of blurred vision or ophthalmologic findings such as papilledema, decreased acuity, enlarged blind spot, and optic atrophy are likely to be the result of coexisting intracranial hypertension. [78stepshealth.us]

• 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]

• Stroke

  (Updated October 26, 2010).NINDS Pituitary Tumors Information Page.National Institute of Neurological Disorders and Stroke [On-line information].Available online at http://www.ninds.nih.gov/disorders/pituitary_tumors/pituitary_tumors.htm.Accessed on October [labtestsonline.it]

  The most common manifestation is some degree of paralysis, but small strokes may occur without symptoms. Usually caused by arteriosclerosis, it often results in brain damage. Iron An essential mineral. [digitalnaturopath.com]

  The National Institute of Neurological Disorders and Stroke. (September 10, 2003). http://www.ninds.nih.gov/health_and_medical/disorders/emptysella.htm. Scott J. Polzin, MS, CGC [encyclopedia.com]

• Dizziness

  Other neurological complaints include memory loss, balance impairment, dizziness, seizures, and rhinorrhea. Papilledema, decreased visual acuity, and visual field loss are somewhat less common (10 percent). [phoenixrising.me]

  Stroke A sudden loss of brain function caused by a blockage or rupture of a blood vessel that supplies the brain, characterized by loss of muscular control, complete or partial loss of sensation or consciousness, dizziness, slurred speech, or other symptoms [digitalnaturopath.com]

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

  Empty sella is a radiological diagnosis based on CT or MR investigation. Either a normal sized (empty sella) or enlarge sella (empty enlarged sella) presents partly or totally filled with cerebrospinal fluid. [thamburaj.com]

  The empty sella syndrome (ESS) is well documented in adults, and although the same phenomenon of herniation of the arachnoid space into the enlarged sella turcica has been noted in children, it is not widely known that children suffer from this syndrome [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]

  What is empty sella syndrome? Empty sella syndrome (ESS) may occur if you have an enlarged sella turcica. This is a bony structure where the pituitary gland sits at the base of the brain. [hopkinsmedicine.org]

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:

• CSF rhinorrhea
• Visual disturbance
• Severely increased intracranial pressure

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.

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

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:

• Head injury or trauma
• Tumor of the pituitary gland
• Radiation therapy
• Infection
• Surgery
• Rare disorders (e.g. Sheehan syndrome) 

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. 

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.

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.

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].

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:

• Impotence (Erectile dysfunction)
• Low libido (Lack of sexual desire)
• Absent or irregular menstruation
• Nipple discharge
• Headaches
• Fatigue (Low energy)

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.

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