Arachnoid Cysts A h id C t

Dean D. Lin Department of Neurosurgery University of Fl id U i it f Florida November 24, 2004

Outline
Epidemiology Presentation Locations Imaging Characteristics Pathology Treatment

Definition

Benign, congenital, intra-arachnoidal intraspacespace-occupying lesions filled with clear CSF-like fluid CSF-

Epidemiology
Incidence: 1% of intracranial mass lesions Age: 75% present during childhood
Spinal: S i l 5th d decade, M F d M=F

Gender: M:F = 3:1 Left side involved twice as frequently q y Genetics: typically sporadic, non-syndromic non-

Etiology
Poorly Understood Older hypothesis: litti or Old h th i splitting diverticulum of developing arachnoid Newer hypothesis: failure of frontal & yp temporal embryonic meninges to merge at sylvian fissue y

Etiology
Potential mechanisms:
Active Acti e secretion of CSF-like fl id CSFfluid by cyst wall Distention by CSF pulsations Entrapment by one-way/ball-valve one-way/ballflow Osmotic gradient Os ot c g ad e t

Spine: defect of septum posticum (thin membranous partition of ti

the dorsal thoracic spinal cord)

Associated Abnormalities
Temporal lobe hypoplasia Hematoma subdural and intra-cystic intraTearing of bridging veins Associated with mild head injury j y

Macrocephaly Spinal arachnoid cysts: kyphoscoliosis

Outline
Epidemiology Presentation Locations Imaging Characteristics Pathology Treatment

Presentation
Natural history unclear y
Most cranial cysts do not enlarge Spinal cysts frequently enlarge

6060-80% symptomatic Most common symptoms:

Headache Seizure Focal deficits

Presentation
Other signs/symptoms: g y p
Protrusion of skull, widen spinal canal

Suprasellar
Visual impairment Endocrinopathies (up to 60% suprasellar cysts) BobbleBobble-head doll syndrome
2-3/second AP bobbing

Spine pain/cord compression

Intermittent claudication, spasticity Worse with Valsalva

Outline
Epidemiology Presentation Locations Imaging Characteristics Pathology Treatment

Locations

Locations
Sylvian fissue/middle fossa y
Cerebellopontine angle Quadrigeminal cistern Vermian Sellar/suprasellar Interhemispheric

49%
11% 10% 9% 9% 9%

Spine - most commonly in Thoracic region Typically dorsal

Extra- or intra-dural

Outline
Epidemiology Presentation Locations Imaging Characteristics Pathology Treatment

Radiographic Appearance
CT: well-demarcated cystic mass wellExtraExtra-axial exerts mass effect CSFCSF-like density No enhancement Expands/remodels bone E pands/remodels Intracystic hemorrhage hyperdense (rare)

CTA: MCA vessels posteriorly displaced

Radiographic Appearance
MRI: well-demarcated cystic well-demarcated,
T1WI and T2WI: isointense to CSF No enhancement

Flair: Suppressed Fl i S d DiffusionDiffusion-weighted: No restriction

Radiographic Appearance
CTCT-Myelogram
May or may not communicate with subarachnoid space Largely replaced by MRI More important for spinal arachnoid cysts

Galassi Classification
Middle fossa arachnoid cysts
Type I: small, lenticular; yp , ; located at temporal pole;
Communicates with subarachnoid space

Type II: involves anterior and intermediate segments of Sylvian fissue; quadrangular
Partially communicates with subarachnoid space

Type III: entire Sylvian fissue, bony expansion of middle fossa; mass effect
Minimal Mi i l communication with i ti ith subarachnoid space Marked shift

Arachnoid Cysts
Imaging Middle Fossa

Elevation of lesser sphenoid wing, thinning of squamous bone

Frontal displacement of greater wing

Arachnoid Cysts
Imaging Middle Fossa

Type I
Rarely treated

Arachnoid Cysts
Imaging Middle Fossa

Type I

Arachnoid Cysts
Imaging Middle Fossa

Type II

Treat if symptomatic

Arachnoid Cysts
Imaging Middle Fossa

Type III
Complete re-expansion frequently not achieved

Arachnoid Cysts
Imaging - CT

Arachnoid Cysts
Imaging Sellar/suprasellar

Frequently present with obstructive hydrocephalus, visual impairment, and endocrinopathies

Arachnoid Cysts
Imaging Cerebellopontine angle

Arachnoid Cysts
Imaging Quadrigeminal and Clival

Obstructive hydrocephalus

Arachnoid Cysts
Imaging Vermian

Arachnoid Cysts
Imaging Posterior fossa

Arachnoid Cysts
Imaging hemorrhagic

Intracystic hemorrhage y g and subdural hematoma

Acute intracystic hemorrhage y g with fluid-fluid level

Spinal Arachnoid Cysts S i l A h id C t

Arachnoid/Meningeal Cysts
Spine Classification Nabors et al., 1988 Type I: Extradural: No nerve roots
Type IA: extradural arachnoid cyst Type IB: sacral meningocele Fibrous li i Fib lining

Type II: Extradural: Roots involved

Tarlov cysts Fibrous lining

Type III: Intradural arachnoid cysts

+ arachnoid lining g Different T2 signal sometimes (no pulsations)

Arachnoid Cysts
Spine
Intradural versus Extradural: Etiologies
Intradural: arachnoid diverticulum or adhesion or trabecular proliferation, either congenital or secondary to trauma/infection Extradural: associated with a dural defect; ball-valve balleffect causes enlargement

Workup includes MRI followed by CT-myelogram p y CT- y g

Arachnoid Cysts
Imaging S i I i Spine

Arachnoid Cysts
Imaging Spine

Arachnoid Cysts
Imaging Spine

Arachnoid Cysts
Imaging Spine

Differential Diagnosis Diff ti l Di i

Arachnoid Cysts
Imaging Differential Diagnosis Differential includes any cystic tumors
JPAs Craniopharyngiomas Hemangioblastoma

Also any non-neoplastic cyst nonPorencephalic cyst Neurenteric cyst

Arachnoid Cysts
Imaging Differential Diagnosis Primary differential: epidermoid cysts

Epidermoid Cysts
Imaging Differential Diagnosis

Arachnoid vs Epidermoid Cysts

Differentiate with FLAIR and DWI

Epidermoid p

Arachnoid cyst y

Arachnoid Cysts
Imaging Differential Diagnosis

Arachnoid cyst versus mega cisterna magna

ACs have mass effect

AC

Mega CM

Outline
Epidemiology Presentation Locations Imaging Characteristics Pathology Treatment

Pathology

Gross: thin, translucent cyst wall filled with CSF

Pathology
Microscopic: cyst lined by flattened arachnoid cells
Sometimes with proliferated trabeculae

Outline
Epidemiology Presentation Locations Imaging Characteristics Pathology Treatment

Treatment
Controversial:
Shunting versus excision/fenestration

Treatment
Shunting
Pros:
easy to perform immediate cyst decompression

Cons:
Frequently need to shunt both ventricle and cyst Infection Recurrence Visualization bridging veins SlitSlit-cyst syndrome (symptoms of elevated ICP but decompressed cyst)

Treatment
Excision/fenestration
Goal: decompression with shunt-independence p shuntp Pros:
Relatively R l ti l easy No foreign material implanted Excellent visualization

Cons:
Some increased recurrence rate depending on techniques (scarring, adhesions) May still require shunting

Treatment
Excision/fenestration Techniques:
Open craniotomy Endoscopic fenestration
Most ff ti f M t effective for suprasellar cysts, esp. with ll t ith opening of lamina terminalis

Keyhole craniotomy
Recently shown to be very effective: 80-95% 80success rate with middle fossa cysts

Treatment
Excision/fenestration

Pre-op

Post-op

Treatment
Spinal arachnoid cysts Laminectomy and excision Closure of dural defect with extradural cysts May also require shunting (intradural)

Happy Th k i i H Thanksgiving