Irregular Lateral Ventricles
Bronwyn E. Hamilton, MD
DIFFERENTIAL DIAGNOSIS
Common
CSF Shunts and Complications
Surgical Defects
Periventricular Leukomalacia
Cerebral Infarction, Chronic
Porencephalic Cyst
Chiari 2
Less Common
Heterotopic Gray Matter
Tuberous Sclerosis Complex
Metastases, Intracranial, Other
Intraventricular Webs or Adhesions
CMV, Congenital
Schizencephaly
Rare but Important
Hemimegalencephaly
Holoprosencephaly
Holoprosencephaly Variants
ESSENTIAL INFORMATION
Key Differential Diagnosis Issues
Irregular ventricles may be the result of obstruction, chronic volume loss/or congenital deformities
Obstruction: Mass effect, “ballooned” appearing ventricles, & transependymal CSF migration
Volume loss: Ventricle irregularity with brain parenchymal loss
Congenital: Look for associated findings (colpocephaly, subependymal nodules)
Ventricular deformities may become permanent despite relief of obstruction, due to parenchymal atrophy or acquired ventricular non-compliance
Enhancement may help differentiate etiologies
Helpful Clues for Common Diagnoses
CSF Shunts and Complications
Common complications include shunt obstruction/breakage, infection, overdrainage
Acquired ventricular non-compliance may result in ventricle deformity
Surgical Defects
Often evident from prior shunt tract or burr hole
Deformity is chronic
Overlying skull or scalp also shows defect
Periventricular Leukomalacia
“Wavy” margins or undulating lateral ventricular contours typical
Cysts or ill-defined T2 hyperintensity in periventricular white matter (WM)
Colpocephaly common & reflects predominant posterior WM loss
Cerebral Infarction, Chronic
Vascular territory wedge-shaped area of encephalomalacia
Results in compensatory or “ex vacuo” dilation of the regional ventricle, due to volume loss
Porencephalic Cyst
Cystic space in brain parenchyma with enlarged adjacent ventricle, may communicate with ventricle
Cyst may cause mild mass effect (from CSF pulsations)
Chiari 2
Pointed anterior horns, colpocephaly
Small posterior fossa, tectal “beaking”, downward herniation of cerebellar tissue through foramen magnum
Associated with a lumbar myelomeningocele
Helpful Clues for Less Common Diagnoses
Heterotopic Gray Matter
Subependymal nodules follow gray matter signal & do not enhance
May be seen with epilepsy or incidental
Tuberous Sclerosis Complex
Subependymal nodules lining the ventricles characteristic
Mostly along striothalamic groove
Calcify with increasing age
Cortical & subcortical tubers are usually multifocal ± mild mass effect
Tubers are most easily seen on FLAIR
Rarely tubers may calcify or enhance
Enhancing mass at foramen of Monro = subependymal giant cell astrocytoma
Metastases, Intracranial, Other
CSF seeding of primary CNS tumors, lymphoma or systemic malignancy may cause irregular ventricles
May result in ventricular nodules which can deform the ventricles
Intraventricular Webs or Adhesions
May be congenital or acquired (prior hemorrhage, infection or tumor)
Contours of ventricles may be rounded or balloon-like due to obstructive symptoms
Contrast ventriculography or cine CSF can be helpful to assess for evidence of physiological flow obstruction
CMV, Congenital
White matter volume loss
Periventricular calcifications are common
Polymicrogyria & cortical malformations may be seen
Schizencephaly
Outward “dimpling” of lateral ventricle suggests schizencephaly
Look for gray matter lining the CSF cleft
Helpful Clues for Rare Diagnoses
Hemimegalencephaly
Hamartomatous overgrowth of part/all of a hemisphere
Lateral ventricle ipsilateral to enlarged hemisphere is usually bizarre-shaped & typically enlarged
Holoprosencephaly
Congenital structural forebrain anomalies defined by degree of frontal lobe fusion
All types have absent septum pellucidum & frontal lobe fusion anomaly
Alobar: Monoventricle, often incompletely covered posteriorly by brain (“dorsal cyst”)
Semilobar: Anterior horns absent, partial occipital & temporal horns
Lobar: Anterior lateral ventricle may be deficient
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