Empty Sella Syndrome

Empty Sella Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Empty Sella Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • empty sella turcica
  • ESS

Disorder Subdivisions

  • primary empty sella syndrome
  • secondary empty sella syndrome

General Discussion

Empty sella syndrome is a rare disorder characterized by enlargement or malformation of a structure in the head known as the sella turcica. The sella turcica is a saddle-shaped depression located in the bone at the base of skull (sphenoid bone), in which resides the pituitary gland. In empty sella syndrome, the malformed sella turcica is often either partially or completely filled with cerebrospinal fluid. As a result, the pituitary gland is often compressed and flattened so that the sella turcica appears empty. Most individuals with empty sella syndrome do not have any associated symptoms. Occasionally, headaches or pituitary dysfunction may occur. Empty sella syndrome may occur as a primary disorder, for which the cause is unknown (idiopathic), or as a secondary disorder, in which it occurs due to an underlying condition or disorder such as a pituitary tumor or trauma in the pituitary region.

Symptoms

The symptoms of empty sella syndrome may vary from one person to another. In most cases, especially in individuals with primary empty sella syndrome, there are no associated symptoms (asymptomatic). Often, empty sella syndrome is discovered incidentally on x-ray examination when individuals are being evaluated for other reasons.



The most common symptom potentially associated with empty sella syndrome is chronic headaches. However, it is unknown whether headaches develop because of empty sella syndrome or are a coincidental finding. Many individuals with empty sella syndrome have high blood pressure (hypertension).



In rare cases, individuals with empty sella syndrome have developed increased pressure within the skull (benign intracranial pressure), leakage of cerebrospinal fluid from the nose (cerebrospinal rhinnorhea), swelling of the optic disc due to increased cranial pressure (papilledema), and abnormalities affecting vision such as loss of clarity of vision (visual acuity).



The pituitary gland is usually not affected. The pituitary gland is a small gland located near the base of the skull that stores several hormones and releases them into the bloodstream as needed by the body. These hormones regulate many different bodily functions. Although a rare occurrence, some abnormal or decreased pituitary function can occur (hypopituitarism). A specific finding in some affected individuals, including children, has been isolated growth hormone deficiency.



Individuals with secondary empty sella syndrome are more likely to develop abnormalities affecting vision and decreased function of the pituitary.

Causes

The exact, underlying cause of primary empty sella syndrome is unknown (idiopathic).



Researchers believe that a defect in the diaphragma sellae that is present at birth (congenital defect) plays a role in the development of primary empty sella syndrome. The diaphragma sellae is a fold of dura mater (the outermost layer of the membranes that line the brain and spinal cord). The diaphragma sellae covers the sphenoid bone where the sella turcica and the pituitary are located. In some affected individuals a tear in the diaphragma sellae allows the underlying membranes to push through (herniate), which allows cerebrospinal fluid to leak out and accumulate in the sella turcica. The pressure exerted by the fluid can flatten or enlarge the sella turcica. Consequently, the pituitary becomes compressed and flattened as well. In some individuals with primary empty sella syndrome the diaphragma sellae is absent at birth. The exact role that defects in the diaphragma sella play is the development of primary empty sella syndrome is unknown. Whether it causes primary empty sella syndrome directly, occurs as part of a larger disease process or is only a predisposing factor to the development of the disorder is unresolved.



Secondary empty sella syndrome is caused by a variety of different conditions including injury or trauma to the head, pituitary tumors, infection, radiation therapy, surgery on the pituitary region, or rare disorders such as Sheehan syndrome.

Affected Populations

Primary empty sella syndrome affects women more often than men. Most cases occur in middle-aged women who are obese and have high blood pressure (hypertension). Because most people with empty sella syndrome do not have symptoms and may go undiagnosed, determining the disorder's true frequency in the general population is difficult. Some researchers have estimated that less than 1 percent of individuals with empty sella syndrome ultimately develop symptoms associated with the disorder.

Standard Therapies

Diagnosis

A diagnosis of empty sella syndrome is made based upon identification of characteristic symptoms, a detailed patient history, a thorough clinical evaluation and specialized tests such as x-ray imaging techniques. Such imaging techniques may include computerized tomography (CT) scanning and magnetic resonance imaging (MRI). During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of certain tissue structures. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs, tissues and structures such as the sella turcica.



Treatment

Many individuals with empty sella syndrome do not have any symptoms and do not require treatment. When symptoms do occur, treatment is directed toward the specific symptoms that are apparent in each individual. If the pituitary is affected, then replacement therapy for specific hormones should be administered as needed. Surgery may be necessary when cerebrospinal fluid leaks from the nose (CSF rhinnorhea).

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



For information about clinical trials conducted in Europe, contact:

https://www.clinicaltrialsregister.eu/

References

TEXTBOOKS

Greenberg MS, Ed. Handbook of Neurosurgery. 6th ed. Thieme. New York, NY; 2006:499.



Colleran K. Primary Empty Sella Syndrome. NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:530-531.



Becker KL, Ed. Principles and Practice of Endocrinology and Metabolism. 3rd ed. Lippincott, Williams & Wilkins. Philadelphia, PA; 2001:107.



JOURNAL ARTICLES

Komada H, Yamamoto M, Okubo S, et al. A case of hypothalamic panhypopituitarism with empty sella syndrome: case report and review of the literature. Endocr J. 2009;56:585-589.



Woodworth BA, Prince A, Chiu AG, et al. Spontaneous CSF leaks: a paradigm for definitive repair and management of intracranial hypertension. Otolaryngol Head Neck Surg. 2008;138:715-720.



Naing S, Frohman LA. The empty sella. Pediatr Endocrinol Rev. 2007;4:335-342.



Del Monte P, Foppiani L, Cafferata C, Marugo A, Bernasconi D. Primary "empty sella" in adults: endocrine findings. Endocr J. 2006;53:803-809.



Valensi P, Combes M, Perret G, et al. TSH and prolactin responses to thyrotropin releasing hormone (TRH) and domperidone in patients with empty sella syndrome. J Endocrinol Invest. 1996;19:293-297.



Braatvedt CD, Corrall RM. The empty sella syndrome: much ado about nothing. Br J Hosp Med. 1992;47:523-525.



INTERNET

National Institute of Neurological Disorders and Stoke. Empty Sella Syndrome Information Page. October 26, 2010. Available at: http://www.ninds.nih.gov/disorders/emptysella/emptysella.htm Accessed:February 4, 2013.

Resources

Pituitary Network Association

P.O. Box 1958

Thousand Oaks, CA 91358

USA

Tel: (805)499-9973

Fax: (805)480-0633

Email: info@pituitary.org

Internet: http://www.pituitary.org



NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801

Bethesda, MD 20824

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

TDD: (301)468-5981

Internet: http://www.ninds.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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