Hydrocephalus
Roya Sohaey, MD
DIFFERENTIAL DIAGNOSIS
Common
Chiari II Malformation
Aqueductal Stenosis, Late
Dandy-Walker Continuum: Classic
Less Common
Intracranial Hemorrhage
Encephalomalacia
Rare but Important
Obstructing Mass
Choroid Plexus Papilloma
ESSENTIAL INFORMATION
Key Differential Diagnosis Issues
Routine lateral ventricle measurement
Measure atria of lateral ventricle
At level of choroid plexus glomus
Measure inner to inner wall
Normal atrial measurements
< 10 mm between 14-40 wks
Ventriculomegaly
Mild: 10-12 mm
Moderate: 12-15 mm
Severe: > 15 mm
Use transvaginal ultrasound
Cephalic presentation
Frontal fontanelle as acoustic window
Best for corpus callosum visualization
Color Doppler to show pericallosal artery
Helpful Clues for Common Diagnoses
Chiari II Malformation
Hindbrain herniation
Causes obstructive hydrocephalus
Variable ventriculomegaly
55% at time of diagnosis
33% progress during pregnancy
Variable calvarial measurements
Normal or small in 2nd trimester
Macrocephaly if ↑ ventriculomegaly
Posterior fossa compression
Small or obliterated cisterna magna
Variable cerebellar compression
“Banana sign” if cerebellum wraps around midbrain
“Lemon-shaped” calvarium
Frontal bone narrowing and “tenting”
Almost always with spina bifida
90% lumbosacral
80% with overlying sac
Aqueductal Stenosis, Late
Aqueduct of Sylvius narrowing/obstruction
↑ 3rd and lateral ventricles
Normal 4th ventricle/posterior fossa
Progressive hydrocephalus
Initial normal or mild ventriculomegaly
Macrocephaly common near term
X-linked form
Bickers-Adams syndrome
< 5% of all cases
50% recurrence risk in male fetuses
Adducted thumbs is additional finding
90% with mental retardation
More severe retardation if X-linked
Dandy-Walker Continuum: Classic
Most severe cystic posterior fossa malformation
Absent cerebellar vermis
Cystic dilatation of 4th ventricle
Variable ventriculomegaly
Ultrasound findings
Splayed cerebellar hemispheres
Small/absent cerebellum if severe
Posterior fossa cyst
Associated findings
Dysgenesis of corpus callosum
Encephalocele, neural tube defect
Cleft lip/palate
Cardiac defects
Polycystic kidneys
Helpful Clues for Less Common Diagnoses
Intracranial Hemorrhage
Most common locations include: Subependymal, germinal matrix, intraventricular, intraparenchymal, subdural
Ventriculomegaly from 2 causes
Ventricle findings
Hyperechoic intraventricular clot
Echogenic cerebrospinal fluid
Bulky irregular choroid plexus
Echogenic irregular ependyma
Causes
Encephalomalacia
Brain parenchyma destruction
Ultrasound findings
Early: Periventricular heterogeneity
Late: Cystic change
Causes
Hypoperfusion from any cause
Infection
Teratogen exposure
Helpful Clues for Rare Diagnoses
Obstructing Mass
Mass or mass-like lesions obstruct cerebrospinal flow
Cystic mass
Arachnoid cyst
Glioependymal cyst
Cystic teratoma
Solid mass
Solid teratoma
Astrocytoma or other rare tumor
Choroid plexus papilloma
Mass-like lesion
Vein of Galen malformation
Arteriovenous fistula
Intracranial hemorrhage
Choroid Plexus Papilloma
Hydrocephalus from over production of cerebral spinal fluid (CSF)
Often rapid onset
Well-defined, lobular, hyperechoic mass
Occurs anywhere in ventricular system
Lateral ventricle most common
Mass may also obstruct ventricle causing asymmetric enlargement