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Empty Sella Syndrome
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Empty Sella Syndrome (ESS) is a disorder that
involves the sella turica, a bony structure at the base of
the brain that surrounds and protects the pituitary gland. ESS is a
condition that is often discovered during tests for pituitary
disorders, when radiological imaging of the pituitary gland reveals
a sella turica that appears to be empty. There are two types of ESS:
primary and secondary. Primary ESS happens when a
small anatomical defect above the pituitary gland increases pressure
in the sella turica and causes the gland to flatten out along the
interior walls of the sella turica cavity. Primary ESS is associated
with obesity and high blood pressure in women. The disorder
sometimes results in a build-up of fluid pressure inside the skull
and the pituitary gland may be smaller than usual. Secondary
ESS is the result of the pituitary gland regressing within
the cavity after an injury, surgery, or radiation therapy.
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. In children, ESS may be
associated with early onset of puberty, growth hormone deficiency,
pituitary tumors, or pituitary gland dysfunction. MRI scans are
useful in evaluating ESS and differentiating it from other disorders
that produce an enlarged sella.
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Unless the syndrome results in other medical
problems, treatment for endocrine dysfunction associated with
pituitary malfunction is symptomatic and supportive. In some cases,
surgery may be needed.
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ESS is not a life-threatening illness.
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The NINDS supports and conducts studies aimed at
understanding neurological conditions such as ESS. The goal of this
research is to understand what causes these disorders and to
discover new ways to diagnose, treat, and prevent them.
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National Organization for Rare Disorders
(NORD)
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