434 lines
26 KiB
Markdown
434 lines
26 KiB
Markdown
---
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title: "Empty Sella"
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docid: "39a0d2d1-1439-4558-8f5d-86a2a6d93e3a"
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authors:
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- key: "5cff4116-3654-4b3a-bb75-5ebe0b8c9850"
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value: "Anne G. Osborn, MD, FACR"
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breadcrumbs:
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-
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name: "Brain"
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slug: "brain"
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treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8"
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-
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name: "Anatomy-Based Diagnoses"
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slug: "anatomy-based-diagnoses"
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treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629"
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-
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name: "Sella and Pituitary"
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slug: "sella-and-pituitary"
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treeNodeId: "9afaeeb6-661c-49be-b55f-5bdc1c98a53e"
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-
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name: "Miscellaneous"
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slug: "miscellaneous"
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treeNodeId: "7941c33d-0063-41a2-b035-39440c09b829"
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-
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name: "Empty Sella"
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slug: "empty-sella"
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treeNodeId: null
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category: "Brain"
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documentVersionId: "91612570-e8d2-417b-8345-8226e2028fbf"
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imageCount: 18
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lastUpdated: "08/10/20"
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pageDescription: "Empty Sella"
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pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Sella and Pituitary, Miscellaneous, Empty Sella"
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pageTitle: "Empty Sella | STATdx"
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enhancedTitle: "Empty Sella"
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type: "DX"
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references: true
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breadcrumbs:
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- "Brain"
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- "Diagnosis"
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- "Anatomy-Based Diagnoses"
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- "Sella and Pituitary"
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- "Miscellaneous"
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- "Empty Sella"
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---
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# KEY FACTS
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- ## Terminology
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- Sella partially filled with arachnoid-lined CSF collection
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- Primary empty sella
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- Common normal variant (15% of brain MRs), incidental finding
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- Normal or increased CSF pressure
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- Near-normal volume of compressed pituitary tissue
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- Secondary empty sella
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- Prior pituitary surgery, radiation, or injury
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- ## Imaging
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- Intrasellar CSF, pituitary flattened against sellar floor
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- Bony sella may be normal or moderately enlarged (secondary to pulsatile CSF)
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- Bony margins intact, not eroded/demineralized
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- Infundibular stalk, pituitary gland enhance normally
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- Fluid exactly like CSF
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- Suppresses completely on FLAIR
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- Does not restrict on DWI
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- ## Top Differential Diagnoses
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- Idiopathic intracranial hypertension
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- Secondary intracranial hypertension
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- Arachnoid cyst
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- Pituitary apoplexy
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- Pituitary anomalies
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- ## Pathology
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- "Deficient" diaphragma sellae
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- Dural covering of sella is incomplete (widened)
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- Leaves large opening for infundibular stalk
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- Allows intrasellar herniation of arachnoid with CSF from suprasellar subarachnoid cistern above
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- ## Clinical Issues
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- Mostly incidental, asymptomatic (adults)
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- F:M = 5:1
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- Headache, visual disturbances if related to intracranial hypertension
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- Frequent endocrine abnormalities in children
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# TERMINOLOGY
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- ## Abbreviations
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- Empty sella (ES)
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- ## Definitions
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- Herniation of suprasellar arachnoid and cerebrospinal fluid (CSF) through wide diaphragma sellae into bony sella turcica
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- Sella turcica is partially filled with CSF
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- Rarely completely empty
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- Pituitary gland
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- Almost never completely absent
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- Thin, flattened rim of residual pituitary tissue
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- Generally at posteroinferior sellar floor
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- Primary or secondary
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- Primary empty sella
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- Common normal variant (15% of brain MRs), incidental finding
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- Normal or increased CSF pressure
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- Near-normal volume of compressed pituitary tissue
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- No history of trauma, surgery, radiation
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- Patients typically endocrinologically normal
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- Secondary empty sella
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- Many etiologies
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- Surgery
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- Radiation
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- Bromocriptine therapy
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- Trauma
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- Sheehan syndrome (postpartum pituitary necrosis)
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- Pituitary apoplexy
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- Pituitary abscess
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Intrasellar CSF with pituitary gland flattened against sellar floor
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- Bony sella may be normal or large
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- ### Location
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- Intrasellar CSF
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- ### Size
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- Variable
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- ## Imaging Recommendations
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- ### Best imaging tool
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- Sagittal T1WI
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- Coronal T2WI
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- ## CT Findings
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- ### NECT
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- CSF-like herniation of CSF into bony sella
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- Bony sella typically appears normal
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- May also be moderately enlarged (secondary to pulsatile CSF)
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- Bony margins intact, not eroded/demineralized
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- ### CECT
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- Infundibular stalk and pituitary gland enhance normally
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- Occasionally intrasellar CSF collection may be asymmetric
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- Stalk may appear tilted to one side
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- ## MR Findings
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- ### T1WI
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- Primary empty sella
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- Fluid looks exactly like CSF
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- Stalk usually midline
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- Gland + stalk = anchor sign on coronal imaging
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- Stalk may be tilted to one side if intrasellar CSF herniation is asymmetric
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- 3rd ventricle, hypothalamus usually normal
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- Rare: Herniation of optic chiasm, anterior 3rd ventricle into sella
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- Secondary empty sella
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- Look for changes of transsphenoidal hypophysectomy
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- Defect in sellar floor
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- Fat packing
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- May cause distortion of stalk, chiasm
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- Stalk and pituitary remnant(s) may be scarred/adhesed to side or bottom of sella turcica
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- ### T2WI
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- Fluid exactly like CSF
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- ### FLAIR
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- Intrasellar fluid suppresses completely on FLAIR
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- ### DWI
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- No restriction
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- ### T1WI C+
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- Primary empty sella
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- Stalk, gland enhance normally
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- No other abnormalities
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- Secondary empty sella
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- Gland and stalk may be adhesed/distorted
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# DIFFERENTIAL DIAGNOSIS
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- [Idiopathic Intracranial Hypertension](/document/idiopathic-intracranial-hypertensi-/d7a0a1b6-1d94-473c-9fe9-021443969f9f)
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- Often not truly "idiopathic" (e.g., dural venous sinus stenosis)
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- Usually obese female, 20-40 years
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- Headache, papilledema
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- Intraoptic protrusion of optic nerve head
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- Enlarged optic nerve sheaths ± empty sella
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- Ventricles may appear slit-like
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- Subarachnoid spaces (cisterns, surface sulci) may be small
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- ## Secondary Intracranial Hypertension
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- Increased intracranial pressure caused by
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- Obstructive hydrocephalus (intra-/extraventricular)
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- Mass (neoplasm, etc.)
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- Dilated anterior recesses of 3rd ventricle herniate into sella
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- Look for mass, evidence for transependymal CSF migration
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- [Arachnoid Cyst](/document/arachnoid-cyst/d25aaeb3-5b3c-4483-99dc-2757468eedb9)
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- Suprasellar arachnoid cyst may herniate into bony sella
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- Bony sella often enlarged, eroded/expanded
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- Look for 3rd ventricle or optic chiasm displaced by CSF-containing mass
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- Cyst walls may be visible on thin-section imaging
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- [Pituitary Apoplexy](/document/pituitary-apoplexy/43efc995-d33c-4ac1-be70-e3237eec9fc9)
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- Acute: Pituitary gland usually enlarged, not small
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- Usually hemorrhagic
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- Look for rim enhancement around periphery of enlarged, nonenhancing gland
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- Chronic: May cause empty sella
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- [Pituitary Anomalies](/document/pituitary-anomalies/09ca9b54-a3d9-43fd-a9cc-4c0212b578a1)
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- Ectopic posterior pituitary "bright spot"
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- May cause small pituitary gland
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- Infundibular stalk short, "stubby"
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- Bony sella often small, shallow appearing
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- Sella can appear partially empty
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- Persisting embryonal infundibular recess of 3rd ventricle
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- Can mimic empty sella (rare)
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- Pituitary stalk duplication
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- Rare
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- Look for 2 thin stalks
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- Sella may appear partially empty
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- [Sheehan Syndrome](/document/pituitary-apoplexy/43efc995-d33c-4ac1-be70-e3237eec9fc9)
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- Original clinical description
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- Postpartum hemorrhage
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- Pituitary necrosis
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- Lactation failure
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- Hypopituitarism
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- Anterior pituitary necrosis
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- Leaves small residual pituitary gland
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- Result = empty sella
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- May occur years after pregnancy
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- Slow clinical progression over years suggests factors other than ischemia may be involved
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- Necrosis may be caused by antihypothalamus, antipituitary antibodies
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- Pituitary autoimmunity may perpetuate hypopituitarism
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- [Epidermoid Cyst](/document/epidermoid-cyst/704c5ddf-e1f7-4a5d-a1b8-5b0e603170d9)
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- True intrasellar epidermoid cyst very rare
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- Off midline > midline
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- Usually extension from cerebellopontine angle epidermoid
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Primary empty sella
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- Deficient diaphragma sellae
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- Dural covering of sella is incomplete (widened)
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- Leaves widened dural opening for infundibular stalk
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- Allows intrasellar herniation of arachnoid with CSF from suprasellar subarachnoid cistern above
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- Compresses pituitary gland against sellar floor
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- Traction on infundibular stalk may cause alteration in visual system
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- Pulsatile CSF may gradually enlarge sella
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- Secondary empty sella
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- Common: Surgery, bromocriptine therapy, radiation
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- Less common: Pituitary apoplexy, pituitary abscess
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- Rare: Pituitary necrosis in viral hemorrhagic fever (e.g., hanta)
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- ## Gross Pathologic & Surgical Features
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- Diaphragma sellae appears widened, gaping
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- Intrasellar herniation of arachnoid-containing CSF
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Incidental, usually asymptomatic
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- Headache
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- Visual disturbances 1-15%
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- Idiopathic intracranial hypertension (IIH)
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- Optic chiasm herniation into ES may cause visual symptoms
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- Endocrine disturbances
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- 20% of adults have subtle laboratory abnormalities
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- Majority (70%) of children with ES have endocrine abnormalities
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- ## Demographics
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- ### Age
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- Peak incidence between 50-60 years
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- Increased CSF pressure presents earlier (30-40 years)
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- ### Sex
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- F:M = 5:1
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- ### Epidemiology
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- 10-15% found incidentally on imaging
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- ## Natural History & Prognosis
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- Both primary and secondary empty sella usually benign, do not require treatment
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- If related to IIH, can result in vision loss or CSF leak
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- Hormonal replacement therapy may be required in some cases
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- Surgery (rare)
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- "Chiasmapexy" to elevate optic chiasm if severe visual disturbances caused by inferior displacement of optic chiasm into empty sella
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- CSF rhinorrhea may require surgical intervention
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# DIAGNOSTIC CHECKLIST
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- ## Consider
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- Incidental, normal variant in older adults
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- Additional findings of IIH in younger females (e.g., dilated optic nerve sheaths, papilledema, dural venous sinus narrowing)
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- Look for endocrine abnormalities in children
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- ## Image Interpretation Pearls
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- Intrasellar fluid follows CSF **exactly**on all sequences
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8bb93cd6-d836-4878-89c3-865ebc070aea
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## References
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# Selected References
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1. [Byrne N et al: Symptomatic primary tethered optic chiasm: Technical case report. Oper Neurosurg (Hagerstown). ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32386310%5Bpmid%5D)
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1. [Chen H et al: A case report of empty Sella syndrome secondary to Hantaan virus infection and review of the literature. Medicine (Baltimore). 99(14):e19734, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32243412%5Bpmid%5D)
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1. [Guinto G et al: Osseous remodeling technique of the sella turcica: a new surgical option for primary empty sella syndrome. World Neurosurg. 126:e953-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30877013%5Bpmid%5D)
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1. [Kirigin Biloš LS et al: Empty sella in the making. World Neurosurg. 128:366-70, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31128314%5Bpmid%5D)
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1. [Rehder D: Idiopathic intracranial hypertension: Review of clinical syndrome, imaging findings, and treatment. Curr Probl Diagn Radiol. ePub, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31056359%5Bpmid%5D)
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1. [Seo YS et al: Bitemporal hemianopsia associated with empty sella syndrome. J Craniofac Surg. 30(8):2660-1, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31449212%5Bpmid%5D)
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1. [Atci IB et al: Prognosis of hormonal deficits in empty sella syndrome using neuroimaging. Asian J Neurosurg. 13(3):737-41, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30283536%5Bpmid%5D)
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1. [Auer MK et al: Primary empty sella syndrome and the prevalence of hormonal dysregulation. Dtsch Arztebl Int. 115(7):99-105, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29510819%5Bpmid%5D)
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1. [Barzaghi LR et al: Treatment of empty sella associated with visual impairment: a systematic review of chiasmapexy techniques. Pituitary. 21(1):98-106, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29027644%5Bpmid%5D)
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1. [Chiloiro S et al: Diagnosis of endocrine disease: Primary empty sella: A comprehensive review. Eur J Endocrinol. ePub, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28780516%5Bpmid%5D)
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1. [Kyung SE et al: Enlargement of the sella turcica in pseudotumor cerebri. J Neurosurg. 120(2):538-42, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24313606%5Bpmid%5D)
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1. [Saindane AM et al: Factors determining the clinical significance of an "empty" sella turcica. AJR Am J Roentgenol. 200(5):1125-31, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23617499%5Bpmid%5D)
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## Images
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### Selected Images
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*Sagittal graphic shows an empty sella (ES). The extension of arachnoid with CSF through the diaphragma sellae <img src='img/arrows/WS.png'/> flattens and displaces the pituitary gland <img src='img/arrows/BO.png'/> posteroinferiorly against the sellar floor.*
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*Sagittal graphic shows an empty sella (ES). The extension of arachnoid with CSF through the diaphragma sellae <img src='img/arrows/WS.png'/> flattens and displaces the pituitary gland <img src='img/arrows/BO.png'/> posteroinferiorly against the sellar floor.*
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*Sagittal T1 MR in a 59-year-old woman with blurry vision and a primary hypopituitarism shows an enlarged, mostly CSF-filled sella turcica <img src='img/arrows/WC.png'/>. The pituitary gland appears flattened along the sellar floor and a posterior pituitary "bright spot" is absent.*
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*Sagittal T1 C+ FS MR in the same patient shows a thin rim of enhancing pituitary gland <img src='img/arrows/WO.png'/> compressed against the expanded sellar floor. The infundibulum is kinked over the dorsum sellae <img src='img/arrows/WC.png'/>.*
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*Coronal T2 MR in the same patient shows the expanded sella has a thin rim of compressed pituitary gland lining the sellar floor <img src='img/arrows/WO.png'/>. The sella is filled with CSF exactly the same signal intensity as the fluid in Meckel caves and lateral ventricles. The diagnosis was primary ES.*
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*Sagittal T1 MR shows an incidental finding of a partially ES <img src='img/arrows/WC.png'/>.*
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*Coronal T1 C+ MR shows the enhancing infundibular stalk and pituitary gland <img src='img/arrows/WS.png'/>. This configuration with the stalk in the midline and a curvilinear pituitary gland has been called the anchor sign because of its resemblance to a ship's anchor <img src='img/arrows/WC.png'/>.*
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*Sagittal T1 MR shows a 40-year-old man with an incidentally found partially ES. No endocrine laboratory abnormalities were reported. Notice the enlarged, bony sella with thin rim of pituitary tissue <img src='img/arrows/CO.png'/>.*
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*Axial T2 MR in the same patient shows the bony sella is expanded and filled with CSF <img src='img/arrows/WO.png'/>. A normal pituitary infundibulum <img src='img/arrows/BC.png'/> is present in the midline.*
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*Sagittal T1 C+ FS MR shows an ES secondary to surgery for pituitary macroadenoma. There is little pituitary tissue apparent along the enlarged sellar floor <img src='img/arrows/CO.png'/>.*
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*Coronal T2 MR of a secondary ES in the same patient demonstrates that the sella is filled with CSF <img src='img/arrows/CO.png'/>. Note the thinned optic chiasm <img src='img/arrows/CS.png'/> retracted downward toward the sella.*
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### Additional Images
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*Axial gross pathology shows a primary empty sella found incidentally at autopsy. Note the wide opening of the diaphragma sellae <img src='img/arrows/BO.png'/> and CSF <img src='img/arrows/BC.png'/> largely filling the bony sella. (Courtesy M. Sage, MD.)*
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*Low-power micropathology shows primary empty sella with downward herniation of CSF-filled subarachnoid space <img src='img/arrows/BS.png'/> into the sella. The pituitary gland <img src='img/arrows/BO.png'/> is flattened against the sellar floor. (Courtesy W. Kucharczyk, MD.)*
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*Coronal T2 MR at 3T shows CSF within the sella turcica <img src='img/arrows/CS.png'/> surrounding the infundibular stalk <img src='img/arrows/CC.png'/>. The pituitary gland <img src='img/arrows/CO.png'/> is flattened against the sellar floor in this patient with a primary empty sella.*
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*Sagittal T1 MR demonstrates a thinned pituitary gland <img src='img/arrows/CS.png'/> along the floor of the mostly empty sella in a 34-year-old woman who had postpartum anterior pituitary gland necrosis (Sheehan syndrome) 10 years prior to imaging.*
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*Coronal T2 MR in the same patient shows a thin, nearly inapparent pituitary gland remnant along the sellar floor <img src='img/arrows/CS.png'/>. The history distinguishes Sheehan syndrome from an incidental finding of partial empty sella.*
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*Sagittal T1 C+ MR shows a 71-year-old woman with a sellar and suprasellar arachnoid cyst mimicking an empty sella. Note the normally enhancing pituitary infundibulum <img src='img/arrows/CS.png'/> and pituitary tissue <img src='img/arrows/CO.png'/> displaced anteriorly by the CSF intensity arachnoid cyst.*
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*Sagittal T2 MR in the same patient shows the lack of CSF flow artifact within the cyst <img src='img/arrows/CS.png'/> compared to the flow within the suprasellar and interpeduncular cisterns <img src='img/arrows/CO.png'/>.*
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*Coronal T2 MR shows primary empty sella seen as incidental finding on screening IAC MR. The sella is filled with CSF and the pituitary gland <img src='img/arrows/WS.png'/> is flattened against the sellar floor.*
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