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