Empty sella syndrome is the condition when the

pituitary gland The pituitary gland or hypophysis is an endocrine gland in vertebrates. In humans, the pituitary gland is located at the base of the human brain, brain, protruding off the bottom of the hypothalamus. The pituitary gland and the hypothalamus contr ...

shrinks or becomes flattened, filling the sella turcica with cerebrospinal fluid instead of the normal pituitary. It can be discovered as part of the diagnostic workup of pituitary disorders, or as an incidental finding when imaging the brain.

Signs and symptoms

If there are symptoms, people with empty sella syndrome can have headaches and vision loss. Additional symptoms would be associated with hypopituitarism. Additional symptoms are as follows: * Abnormality of the middle ear ossicles * Cryptorchidism * Dolichocephaly * Arnold-Chiari type I malformation * Meningocele * Patent ductus arteriosus * Muscular

hypotonia Hypotonia is a state of low muscle tone (the amount of tension or resistance to stretch in a muscle), often involving reduced muscle strength. Hypotonia is not a specific medical disorder, but it is a potential manifestation of many different dis ...

* Platybasia

Cause

The cause of this condition is divided into primary and secondary, as follows: * The cause of this condition in terms of ''secondary empty sella syndrome'' happens when a

tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

or surgery damages the gland, this is an acquired manner of the condition. * patients with idiopathic intracranial hypertension will have empty sella on MRI * The cause of ''primary empty sella syndrome'' is a

congenital A birth defect is an abnormal condition that is present at childbirth, birth, regardless of its cause. Birth defects may result in disability, disabilities that may be physical disability, physical, intellectual disability, intellectual, or dev ...

defect ( diaphragma sellae)

Mechanism

The normal mechanism of the pituitary gland sees that it controls the

hormonal A hormone (from the Greek participle , "setting in motion") is a class of signaling molecules in multicellular organisms that are sent to distant organs or tissues by complex biological processes to regulate physiology and behavior. Hormones a ...

system, which therefore has an effect on growth, sexual development, and adrenocortical function. The gland is divided into ''anterior'' and ''posterior''. Its pathophysiology is such that individuals affected with the condition can have cerebrospinal fluid build-up, which in turn causes

intracranial The cranial cavity, also known as intracranial space, is the space within the skull that accommodates the brain. The skull is also known as the cranium. The cranial cavity is formed by eight cranial bones known as the neurocranium that in human ...

pressure leading to ''headaches'' for the individual.

Diagnosis

The diagnosis of empty sella syndrome, done via examination (and test), may be linked to early onset of puberty, growth hormone deficiency, or pituitary gland dysfunction (at an early age). Additionally there is: ::::::::*CT scan ::::::::* MRI scans

Classification

There are two types of empty sella syndrome: ''primary'' and ''secondary''. * ''Primary'' empty sella syndrome occurs 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 empty sella syndrome is associated with

obesity Obesity is a medical condition, considered by multiple organizations to be a disease, in which excess Adipose tissue, body fat has accumulated to such an extent that it can potentially have negative effects on health. People are classifi ...

and increase in intracranial pressure in women. In most cases, especially in people with primary empty sella syndrome, there are no symptoms and it does not affect life expectancy or health. Some researchers have estimated that less than 1% of affected people ever develop symptoms of the condition. * ''Secondary'' empty sella syndrome is the result of the pituitary gland regressing within the cavity after an

injury Injury is physiological damage to the living tissue of any organism, whether in humans, in other animals, or in plants. Injuries can be caused in many ways, including mechanically with penetration by sharp objects such as teeth or with ...

surgery Surgery is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery s ...

, or

radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...

. Individuals with secondary empty sella syndrome due to destruction of the pituitary gland have symptoms that reflect the loss of pituitary functions, such as intolerance to stress and infection.

Differential diagnosis

The major differential to consider in empty sella syndrome is intracranial hypertension, of both

unknown Unknown or The Unknown may refer to: Film and television Film * The Unknown (1915 comedy film), ''The Unknown'' (1915 comedy film), Australian silent film * The Unknown (1915 drama film), ''The Unknown'' (1915 drama film), American silent drama ...

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, surgery may be needed in some cases.

References

External links

{{Authority control Syndromes with obesity Syndromes affecting the endocrine system Rare syndromes