Kalpana Kalpana (Editor)

Empty sella syndrome

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ICD-9-CM
  
253.8

MedlinePlus
  
000349

DiseasesDB
  
31523

MeSH
  
D004652

Empty sella syndrome

Empty sella syndrome (abbreviated ESS) is where the pituitary gland shrinks or becomes flattened, filling the sella turcica, with cerebrospinal fluid on imaging instead of the normal pituitary. ESS can be found in the workup of pituitary disorders, or as an incidental finding when imaging the brain.

Contents

Classification

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 turcica and causes the gland to flatten out along the interior walls of the sella turcica cavity. Primary ESS is associated with obesity and increase in intracranial pressure in women.
  • 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 intolerance to stress and infection.
  • Signs/symptoms

    If there are symptoms, people with empty sella syndrome can have headaches as symptoms which subsides when lying down. Additional symptoms are as follows:

  • Abnormality (middle ear ossicles)
  • Cryptorchidism
  • Dolichocephaly
  • Arnold-Chiari type I malformation
  • Meningocele
  • Patent ductus arteriosus
  • Muscular hypotonia
  • Platybasia
  • Diagnosis

    The diagnosis of ESS, done via examination and test, may be associated with early onset of puberty, growth hormone deficiency, pituitary tumors, or pituitary gland dysfunction.Additionally there is:

  • Low testosterone (or hypogonadism)
  • MRI scans
  • Differential diagnosis

    The major differential to consider in empty sella syndrome is intracranial hypertension, of both unknown and secondary causes, and an epidermoid cyst, which can mimic cerebrospinal fluid due to its low density on CT scans, although MRI can usually distinguish the latter diagnosis.

    Treatment

    In terms of management, unless the syndrome results in other medical problems, treatment for endocrine dysfunction associated with pituitary malfunction is symptomatic and thus supportive;however, in some cases, surgery may be needed.

    References

    Empty sella syndrome Wikipedia