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Empty Sella Syndrome

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to Rodney J. Schlosser, MD, Professor and Director of Rhinology/Sinus Surgery, Department of Otolaryngology - Head and Neck Surgery, Medical University of South Carolina, for assistance in the preparation of this report.

Synonyms of Empty Sella Syndrome

  • 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.

Empty Sella Syndrome Resources

Organizations:

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