Intracranial Cysts: Lateral
Anne Kennedy, MD
DIFFERENTIAL DIAGNOSIS
Common
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Choroid Plexus Cyst
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Arachnoid Cyst
Less Common
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Schizencephaly
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Arteriovenous Fistula
Rare but Important
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Porencephaly
ESSENTIAL INFORMATION
Key Differential Diagnosis Issues
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Assessment of fluid-filled intracranial structure
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Could it be a prominent normal structure such as lateral ventricle?
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Mild ventriculomegaly
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Choroid displaced from medial wall
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Atria of lateral ventricle measures 10-12 mm
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Look for signs of trisomy 21
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Look for additional brain abnormalities
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Colpocephaly: Tear drop shape to dilated occipital horns seen in agenesis of corpus callosum
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Ventricles parallel
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Absent cavum septi pellucidi
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Loss of normal branch pattern of anterior cerebral artery
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Stenogyria
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Once abnormal lateral ventricle excluded, determine location
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Is it in the choroid plexus?
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Is it in the brain substance (i.e., intra-axial)?
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Is it outside the brain substance (i.e., extra-axial)?
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Is it replacing normal brain tissue?
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Porencephaly
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Schizencephaly
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Is it a space-occupying lesion displacing brain tissue?
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Arachnoid cyst
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Arteriovenous fistula
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Is it vascular?
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Could it be a thrombosed vascular structure?
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Hypoechoic structure with increased through transmission
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Often contains faint low level echoes
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Usually tubular or round
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Helpful Clues for Common Diagnoses
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Choroid Plexus Cyst
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Cyst > 2 mm with defined wall, within choroid plexus
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Variable size
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Variable number
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May be unilateral or bilateral
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May be single or multiple
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Look for associated signs of trisomy 18
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Abnormal posturing
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Facial cleft
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Congenital heart disease
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Neural tube defects
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Omphalocele
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Arachnoid Cyst
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Extra-axial cyst displaces brain parenchyma
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Unilocular
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Avascular
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Most common over convexities
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1/3 fetal cases are in posterior fossa
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May enlarge and cause hydrocephalus due to mass effect at foramen of Monro or aqueduct of Sylvius
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Helpful Clues for Less Common Diagnoses
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Schizencephaly
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Wedge-shaped defect in brain parenchyma
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Extends from lateral ventricle to inner table of skull
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Edges of cleft are lined by gray matter
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Unilateral or bilateral
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Open lip or closed lip
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Open lip: Wedge-shaped defect, edges separated by cerebrospinal fluid-filled cleft
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Closed lip: Edges of defect are in contact; unlikely to be detected on prenatal ultrasound
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Size varies from small to “giant”
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Consider MR to look for associated heterotopia, polymicrogyria, pachygyria, septo-optic dysplasia
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Arteriovenous Fistula
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Hypoechoic structure on grayscale images
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Blood flow on Doppler evaluation when patent
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Thrombosed AVF is tubular/round structure with low level internal echoes but no flow on Doppler evaluation
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MR will show blood product signal even when flow is absent
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May be intra-axial, but dural (i.e., extra-axial) more common in fetus
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May be associated with high output cardiac failure
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May be associated with polyhydramnios
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Look for associated intraventricular or parenchymal hemorrhage
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Look for associated ischemic damage secondary to vascular “steal”
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Cortical thinning, ventriculomegaly, microcephaly
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MR more sensitive than ultrasound for detection of encephalomalacia
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Helpful Clues for Rare Diagnoses
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Porencephaly
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Porencephalic cyst replaces damaged brain
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Intracerebral cavitation due to injury
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Usually connected with lateral ventricle
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Look for associated intracranial hemorrhage
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Destructive process may present with mild ventriculomegaly and progress to encephalomalacia/porencephaly over time
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Other Essential Information
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Prognosis is variable depending on underlying condition
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Choroid plexus cysts
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Seen in 1% of normal second trimester exams: No clinical significance if isolated finding
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Seen in 50% of fetuses with trisomy 18: Usually associated with multiple other findings
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Arachnoid cyst
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Prognosis good if isolated
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May require shunt or excision if large or causing obstructive hydrocephalus
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Schizencephaly
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Prognosis highly variable depending on size and bilaterality
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Septo-optic dysplasia is common association: Visual impairment and hypothalamic/pituitary disturbance
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Bilateral giant open lip schizencephaly → severe functional deficit ± epilepsy
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Arteriovenous fistula
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If brain is normal at birth, treatment recommended to prevent complications of hydrocephalus, progressive neurological deterioration, and acute bleed
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