National Organization for Rare Disorders, Inc.

Empty Sella Syndrome

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

Disorder Subdivisions

  • None

General Discussion

The primary form of empty sella syndrome is a rare inherited disorder of the brain that is transmitted as an autosomal dominant trait. The disorder is characterized by an empty space filled with cerebrospinal fluid in the sella turcica area of the brain. The area fills with fluid as a result of a defect in the sella diaphragm. Symptoms and findings may include unusual facial features, a highly-arched palate, moderate short stature, increased bone density (osteosclerosis), and normal pituitary function.

The secondary form of this disorder is caused by another underlying disorder or defect (e.g., surgery, radiation therapy, etc.). In the idiopathic form of the disease, the exact cause in not known. This form of the disorder affects mostly obese, middle-aged women.

Resources

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com

National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda, MD 20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Email: braininfo@ninds.nih.gov
Internet: http://www.ninds.nih.gov/

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). 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

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  8/8/2007
Copyright  1989, 1998, 1999, 2007 National Organization for Rare Disorders, Inc.


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Topic Contents
 Synonyms
 Disorder Subdivisions
 General Discussion
 Resources
 For a Complete Report