Empty sella syndrome

If there are symptoms, people with empty sella syndrome can have headaches and vision loss. Additional symptoms would be associated with hypopituitarism.{{Cite book |url=https://books.google.com/books?id=Qd-vvNh0ee0C&q=headaches+as+symptoms+of+elevated+spinal+fluid+pressure++empty+sella+syndrome&pg=PA1256 |title=Goldman's Cecil Medicine |vauthors=Goldman L, Schafer AI |date=2012 |publisher=Elsevier Health Sciences |isbn=978-1-4377-1604-7 |edition=24th |page=1256 |language=en |access-date=8 March 2017}}

Additional symptoms are as follows:{{citation needed|date=October 2019}}

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

File:Pituitary gland small.gif ]]

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 or surgery damages the gland, this is an acquired manner of the condition.
• patients with idiopathic intracranial hypertension will have empty sella on MRI{{Cite journal |vauthors=Ranganathan S, Lee SH, Checkver A, Sklar E, Lam BL, Danton GH, Alperin N |date=August 2013 |title=Magnetic resonance imaging finding of empty sella in obesity related idiopathic intracranial hypertension is associated with enlarged sella turcica |journal=Neuroradiology |volume=55 |issue=8 |pages=955–961 |doi=10.1007/s00234-013-1207-0 |pmc=3753687 |pmid=23708942}}
• The cause of primary empty sella syndrome is a congenital defect (diaphragma sellae)

The normal mechanism of the pituitary gland sees that it controls the hormonal system, which therefore has an effect on growth, sexual development, and adrenocortical function. The gland is divided into anterior and posterior.{{Cite book |url=https://www.ncbi.nlm.nih.gov/books/NBK279389/ |title=How does the pituitary gland work? |date=19 April 2018 |work=InformedHealth.org |publisher=Institute for Quality and Efficiency in Health Care}}

Its pathophysiology is such that individuals affected with the condition can have cerebrospinal fluid build-up, which in turn causes intracranial pressure leading to headaches for the individual.{{Cite book |url=https://books.google.com/books?id=Q30_QeaGvLYC&q=empty+sella+syndrome+mechanism+pituitary+gland+nih&pg=PA281 |title=The Encyclopedia of Neuropsychological Disorders |vauthors=Noggle CA, Dean RS, Horton AM |date=2012-01-01 |publisher=Springer Publishing Company |isbn=978-0-8261-9854-9 |page=282 |language=en}}

File:Empty Sella MRT T2 sag 002.jpg]]

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 {{Cite book |last=National Organization for Rare Disorders |url=https://books.google.com/books?id=99YPDvFWBB0C&q=Empty+sella+syndrome++diagnosis+NORD&pg=PA530 |title=NORD Guide to Rare Disorders |date=2003 |publisher=Lippincott Williams & Wilkins |isbn=978-0-7817-3063-1 |page=530 |language=en |access-date=11 March 2017}}

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.{{Cite web |title=Empty sella syndrome |url=https://rarediseases.info.nih.gov/diseases/6331/empty-sella-syndrome |website=Genetic and Rare Diseases Information Center}} Primary empty sella syndrome is associated with obesity and increase in intracranial pressure in women.{{Cite journal |vauthors=Fouad W |date=1 June 2011 |title=Review of empty sella syndrome and its surgical management |journal=Alexandria Journal of Medicine |volume=47 |issue=2 |pages=139–147 |doi=10.1016/j.ajme.2011.06.005 |doi-access=free}} 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, surgery, or radiation therapy. 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.{{medical citation needed|date=March 2017}}

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.{{Cite journal |vauthors=González-Tortosa J |date=April 2009 |title=[Primary empty sella: symptoms, physiopathology, diagnosis and treatment] |trans-title=Primary empty sella: symptoms, physiopathology, diagnosis and treatment |journal=Neurocirugia |language=es |volume=20 |issue=2 |pages=132–151 |doi=10.1016/s1130-1473(09)70180-0 |pmid=19448958}}

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.

{{Reflist|32em}}

{{refbegin}}

• {{Cite book |last=National Organization for Rare Disorders |url=https://books.google.com/books?id=99YPDvFWBB0C&q=empty+sella+syndrome&pg=PA530 |title=NORD Guide to Rare Disorders |date=2003 |publisher=Lippincott Williams & Wilkins |isbn=978-0-7817-3063-1 |language=en |access-date=8 March 2017}}
• {{Cite book |url=https://books.google.com/books?id=FVfzRvaucq8C&q=empty+sella+syndrome&pg=PA107 |title=Principles and Practice of Endocrinology and Metabolism |vauthors=Becker KL |date=January 2001 |publisher=Lippincott Williams & Wilkins |isbn=978-0-7817-1750-2 |language=en}}

{{refend}}

{{Medical resources

| DiseasesDB = 31523

| ICD10 = {{ICD10|E23.6}}

| ICD9 = {{ICD9|253.8}}

| ICDO =

| OMIM =

| MedlinePlus = 000349

| eMedicineSubj =

| eMedicineTopic =

| MeshID = D004652

| Orphanet = 91354

| SNOMED CT = 237722004

}}

{{Commons}}

{{Scholia|topic}}

{{Pituitary disease}}

{{Authority control}}

Category:Syndromes with obesity

Category:Syndromes affecting the endocrine system

Category:Rare syndromes