Empty Sella Syndrome
| Classification according to ICD-10 | |
|---|---|
| E23.6 | Other pituitary disorders |
| ICD-10 online (WHO version 2019) | |
The empty sella syndrome (ESS; syndrome of the empty sella turcica ) is a relatively common neuroradiological or pathological finding, which often corresponds to an anatomical standard variant , but can also represent a hormonal disease .
The sella turcica (German: "Turk's saddle") is a cranial fossa in the middle of the middle cranial fossa ( fossa cranii media ), in which the pituitary gland normally lies, which produces numerous hormones and thus regulates processes such as growth , reproduction and metabolism .
In the empty sella syndrome, however, there is no typical form of the pituitary gland in this cranial fossa, but rather a hernia-like expansion of the subarachnoid space filled with cerebrospinal fluid ( arachnoidocele ) into the sella turcica. As a result, the pituitary gland is pressed to the bottom of the sella turcica and appears to be "rolled out". As a result of compression of the pituitary gland can cause endocrine come disorders.
The empty sella syndrome was first described in 1951.
Epidemiology
Empty Sella Syndrome is found in unselected autopsy material and in neuroradiological findings in 6-20% of cases.
etiology
The primary form of the empty sella syndrome is based on an insufficiently developed diaphragm sellae (part of the hard meninges that spans the sella turcica and separates the pituitary gland from the base of the brain), with the subarachnoid space yielding to the CSF pressure into the sella expands into it.
Primary empty sella syndrome is more common in women, and more often when they have high blood pressure , are overweight , have headaches or have visual disturbances .
The secondary form of the disease is often the result of increased intracranial pressure , such as idiopathic intracranial hypertension or increased intracranial pressure due to brain tumors or hydrocephalus. A secondary empty sella syndrome can also occur after an operation on a pituitary tumor , as postpartum necrosis ( Sheehan's syndrome ), as a result of a heart attack or as a result of a traumatic brain injury or radiation .
pathology
Morphologically, a shell-like reshaped, pressure-atrophic pituitary gland can be seen within an often enlarged sella .
In general, there is a "partial" empty sella syndrome when the sella turcica is at most 50% full with CSF, and a complete syndrome with more than 50% CSF filling.
clinic
The Empty Sella Syndrome is often asymptomatic in its primary form and is therefore sometimes also regarded as an anatomical variant of the norm. Often, however , there are overweight , high blood pressure and headaches .
How often endocrine dysfunctions such as hyperprolactinemia , central diabetes insipidus , hypopituitarism , hypothalamic hypothyroidism or hypogonadism occur is controversial. In a meta-analysis of the published studies, hormonal disorders were found with a prevalence of 52%. According to the authors, with a prevalence of empty sella syndrome of 2–20%, this would mean that 1–10% of the population would have pituitary disorders. The prevalence is only 50 per 100,000, so that the authors of the meta-analysis assume a considerable publication and selection bias . Nevertheless, it is recommended that if an empty sella syndrome is discovered by chance, a basic hormone diagnosis is carried out and further diagnosis, including stimulation tests, is only initiated if there are further abnormalities.
The authors of the meta-analysis recommend the determination of morning cortisol , free thyroxine , estradiol or testosterone , IGF-1 and prolactin as basic hormone diagnostics .
Individual evidence
- ↑ a b U. Riede, M. Werner, H. Schäfer: General and special pathology. 5th edition. Thieme, 2004, ISBN 3-13-683305-8 .
- ^ W. Busch: Morphology of sella turcica and its relation to the pituary gland Virchows Archiv 1951, Volume 320, Issue 5 of September 1951, pages 437-58
- ↑ a b c d e Matthias K. Auer, Mareike R. Stieg, Alexander Crispin, Caroline Sievers, Günter K. Stalla, Anna Kopczak: Primary Empty Sella Syndrome and Prevalence of Hormonal Disorders Deutsches Ärzteblatt 2018, Volume 115, Issue 7 from February 16, 2018, pages 99-105, DOI: 10.3238 / aerztebl.2018.0099
