hydrocephalus

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hydrocephalus brain MRI CT scan enlarged ventricles

This composite figure includes radiological imaging of the brain in the axial plane, illustrating the evolution of hydrocephalus associated with ventriculoperitoneal shunt (VPS) malfunction. Panels A and B are T2-weighted MRI scans. Image A shows a significantly dilated, entrapped right lateral ventricle exhibiting a bright, hyperintense signal characteristic of cerebrospinal fluid accumulation due to right-sided shunt failure. Image B shows the same patient shortly after, displaying a reduction in ventricular volume and normalized morphology, indicating a temporary improvement in hydrocephalus. Panel C is a non-contrast axial CT scan showing a recurrence of hydrocephalus. A high-density (bright) linear structure is visible traversing the right cerebral parenchyma and terminating in the enlarged right lateral ventricle, representing the radio-opaque proximal shunt catheter. A separate circular hyperdensity within the calvarium denotes a shunt component or connector. This series highlights the diagnostic utility of MRI and CT in monitoring shunt-dependent hydrocephalus and identifying complications like entrapped ventricles.

This composite figure includes radiological imaging of the brain in the axial plane, illustrating the evolution of hydrocephalus associated with ventriculoperitoneal shunt (VPS) malfunction. Panels A and B are T2-weighted MRI scans. Image A shows a significantly dilated, entrapped right lateral ventricle exhibiting a bright, hyperintense signal characteristic of cerebrospinal fluid accumulation due to right-sided shunt failure. Image B shows the same patient shortly after, displaying a reduction in ventricular volume and normalized morphology, indicating a temporary improvement in hydrocephalus. Panel C is a non-contrast axial CT scan showing a recurrence of hydrocephalus. A high-density (bright) linear structure is visible traversing the right cerebral parenchyma and terminating in the enlarged right lateral ventricle, representing the radio-opaque proximal shunt catheter. A separate circular hyperdensity within the calvarium denotes a shunt component or connector. This series highlights the diagnostic utility of MRI and CT in monitoring shunt-dependent hydrocephalus and identifying complications like entrapped ventricles.

A series of five longitudinal neuroimaging studies (axial sections) demonstrating dynamic changes in ventricular size over a two-and-a-half-month period. The series primarily consists of brain MRI (T1-weighted and FLAIR sequences) and one CT scan (April 19). The images illustrate a progression from baseline ventricular morphology on February 15 to progressive ventriculomegaly by March 27, where the lateral ventricles are markedly enlarged and rounded, suggestive of hydrocephalus. Subsequent scans from April 19 and April 30 show a resolution of this enlargement, with the ventricles returning to near-baseline dimensions. This clinical progression demonstrates the fluctuation of ventricular volume, often seen in cases of intermittent hydrocephalus or following neurosurgical intervention such as shunt placement. The images provide high-contrast visualization of the cerebrospinal fluid (CSF) spaces relative to the brain parenchyma, cortical sulci, and periventricular structures, serving as a teaching tool for longitudinal monitoring of intracranial pressure-related changes.

A series of five longitudinal neuroimaging studies (axial sections) demonstrating dynamic changes in ventricular size over a two-and-a-half-month period. The series primarily consists of brain MRI (T1-weighted and FLAIR sequences) and one CT scan (April 19). The images illustrate a progression from baseline ventricular morphology on February 15 to progressive ventriculomegaly by March 27, where the lateral ventricles are markedly enlarged and rounded, suggestive of hydrocephalus. Subsequent scans from April 19 and April 30 show a resolution of this enlargement, with the ventricles returning to near-baseline dimensions. This clinical progression demonstrates the fluctuation of ventricular volume, often seen in cases of intermittent hydrocephalus or following neurosurgical intervention such as shunt placement. The images provide high-contrast visualization of the cerebrospinal fluid (CSF) spaces relative to the brain parenchyma, cortical sulci, and periventricular structures, serving as a teaching tool for longitudinal monitoring of intracranial pressure-related changes.

This figure presents two axial cross-sections of the brain highlighting neuroanatomical changes. Image A is a non-contrast Computed Tomography (CT) scan of the head demonstrating significant ventriculomegaly. A red arrow points to the markedly enlarged lateral and third ventricles, which occupy a disproportionate volume relative to the brain parenchyma. Image B is a T2-weighted Magnetic Resonance Image (MRI) with contrast from the same patient. This image clarifies the etiology of the ventricular enlargement, with a red arrow indicating pronounced cerebral atrophy. Key visual findings in image B include the prominent narrowing of cortical gyri and the compensatory widening of the sulci (hydrocephalus ex vacuo). The comparison between these two modalities is used to distinguish between communicating hydrocephalus and secondary ventricular enlargement due to global tissue loss. These diagnostic images are essential for evaluating geriatric patients presenting with the clinical triad of gait instability, urinary incontinence, and cognitive decline, often seen in normal pressure hydrocephalus (NPH) or neurodegenerative processes.

This figure presents two axial cross-sections of the brain highlighting neuroanatomical changes. Image A is a non-contrast Computed Tomography (CT) scan of the head demonstrating significant ventriculomegaly. A red arrow points to the markedly enlarged lateral and third ventricles, which occupy a disproportionate volume relative to the brain parenchyma. Image B is a T2-weighted Magnetic Resonance Image (MRI) with contrast from the same patient. This image clarifies the etiology of the ventricular enlargement, with a red arrow indicating pronounced cerebral atrophy. Key visual findings in image B include the prominent narrowing of cortical gyri and the compensatory widening of the sulci (hydrocephalus ex vacuo). The comparison between these two modalities is used to distinguish between communicating hydrocephalus and secondary ventricular enlargement due to global tissue loss. These diagnostic images are essential for evaluating geriatric patients presenting with the clinical triad of gait instability, urinary incontinence, and cognitive decline, often seen in normal pressure hydrocephalus (NPH) or neurodegenerative processes.

This composite diagnostic image presents a comparative study between Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) of the brain in the context of Idiopathic Normal Pressure Hydrocephalus (iNPH). (a, b) Axial CT and T1-weighted MRI at the vertex level demonstrate 'disproportionately enlarged subarachnoid space hydrocephalus' (DESH), characterized by narrow sulci at the high convexity and midline, contrasting with focally enlarged sulci elsewhere. (c, d) Coronal CT and T1-weighted MRI sections highlight significant ventriculomegaly and prominently dilated Sylvian fissures, a classic diagnostic feature of iNPH. (e, f) Axial CT and T2-FLAIR MRI sequences at the level of the lateral ventricles illustrate extensive periventricular white matter changes. These appear as hypodense regions on CT and hyperintense signals on T2-FLAIR, surrounding the dilated lateral ventricles. The images serve to illustrate how both CT and MRI can identify key morphological markers of hydrocephalus, including sulcal effacement, sylvian fissural widening, and white matter signal alterations for clinical evaluation.

This composite diagnostic image presents a comparative study between Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) of the brain in the context of Idiopathic Normal Pressure Hydrocephalus (iNPH). (a, b) Axial CT and T1-weighted MRI at the vertex level demonstrate 'disproportionately enlarged subarachnoid space hydrocephalus' (DESH), characterized by narrow sulci at the high convexity and midline, contrasting with focally enlarged sulci elsewhere. (c, d) Coronal CT and T1-weighted MRI sections highlight significant ventriculomegaly and prominently dilated Sylvian fissures, a classic diagnostic feature of iNPH. (e, f) Axial CT and T2-FLAIR MRI sequences at the level of the lateral ventricles illustrate extensive periventricular white matter changes. These appear as hypodense regions on CT and hyperintense signals on T2-FLAIR, surrounding the dilated lateral ventricles. The images serve to illustrate how both CT and MRI can identify key morphological markers of hydrocephalus, including sulcal effacement, sylvian fissural widening, and white matter signal alterations for clinical evaluation.

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Hydrocephalus

Definition

Hydrocephalus is an increase in the volume of CSF within the ventricular system, typically resulting in ventricular enlargement. The literal meaning is "water on the brain," but this is a non-specific term - it must be distinguished from ventriculomegaly due to brain atrophy (hydrocephalus ex vacuo), which does not benefit from intervention. - Robbins & Kumar Basic Pathology, p. 821; Grainger & Allison's Diagnostic Radiology, p. 2028

CSF Physiology

CSF is produced by the choroid plexus of the lateral ventricles (approximately 20 mL/hour; total volume ~150 mL). It flows:
Lateral ventricles → Foramen of Monro → Third ventricle → Cerebral aqueduct (of Sylvius) → Fourth ventricle → Foramina of Magendie (midline) & Luschka (lateral) → Subarachnoid space → Absorbed by arachnoid granulations along the superior sagittal sinus
The balance between production and resorption regulates CSF volume. Cerebrovascular pulsations also influence net flow.
CSF Pathways diagram showing ventricular system
CSF pathways - Bailey and Love's Short Practice of Surgery, 28th Ed.

Classification

1. Noncommunicating (Obstructive / Intraventricular) Hydrocephalus

Obstruction occurs within the ventricular system. CSF cannot flow freely between ventricles and subarachnoid space.
  • The ventricles proximal to the obstruction enlarge; distal ventricles remain normal
  • Most commonly blocked at the cerebral aqueduct (aqueductal stenosis)
  • Can cause very sudden deterioration, coma, and death
  • Lumbar puncture carries risk of brainstem herniation

2. Communicating (Extraventricular Obstructive) Hydrocephalus

Obstruction occurs outside the ventricular system - impaired resorption at the arachnoid granulations. The entire ventricular system is enlarged.
  • Causes: post-hemorrhagic, post-infectious, raised CSF protein, meningeal carcinomatosis

3. Hydrocephalus ex Vacuo

Compensatory enlargement of CSF spaces secondary to loss of brain parenchyma (atrophy, infarction, neurodegeneration). Not a true hydrocephalus - no raised ICP; does not benefit from shunting.

4. Overproduction (Rare)

Choroid plexus papilloma/carcinoma - may also cause obstruction by mass effect or hemorrhage.

Etiology by Age

Age GroupCommon Causes
Neonate/InfantPosthemorrhagic (germinal matrix hemorrhage in preterms <1500g), postinfective (in utero infection → aqueductal scarring), Chiari II malformation (with myelomeningocele), Dandy-Walker malformation, aqueductal stenosis/gliosis
Older childPosterior fossa neoplasms (cerebellum/brainstem), aqueductal stenosis, meningitis sequelae
AdultSubarachnoid hemorrhage, colloid cyst of 3rd ventricle (ball-valve effect), ependymoma of 4th ventricle, cerebellar hemorrhage/infarction (compresses aqueduct), meningeal infections/tumors, NPH
Premature infants <1500g have a ~25% risk of progressive ventricular enlargement after intraventricular hemorrhage; approximately 5% ultimately require shunting.

Clinical Features

Infants (sutures open, before age ~2 years)

  • Progressive macrocephaly - head circumference crossing centile lines (the most reliable sign)
  • Frontal bossing
  • Tense, bulging anterior fontanelle
  • Sutural diastasis, scalp vein engorgement, calvarial thinning
  • "Sunsetting" eyes - failure of upward gaze (Parinaud sign due to dorsal midbrain compression)
  • Lateral rectus palsy (CN VI false localizing sign)
  • Leg spasticity (corticospinal tracts stretched around enlarged ventricles)
  • "Cracked-pot" sound on skull percussion (McEwen sign)

Older Children (sutures fused)

  • Early morning headache, nausea, vomiting (raised ICP)
  • Papilledema
  • Diplopia (CN VI palsy)
  • Ataxia and gait disturbance
  • With long-standing disease: endocrine dysfunction (short stature, menstrual irregularity, diabetes insipidus)

Adults - Acute Hydrocephalus

  • Headaches, papilledema, diplopia
  • Mental status changes
  • Sudden death from acute pressure surge

Adults - Chronic Hydrocephalus / Normal Pressure Hydrocephalus (NPH)

Hakim's Triad:
  1. Gait disturbance - apraxic ("magnetic") gait - inability to lift feet as if stuck to the floor; wide-based; resembles Parkinsonism (most responsive to treatment)
  2. Cognitive impairment - subcortical dementia: psychomotor slowing, apathy, preserved language/spatial skills
  3. Urinary incontinence
NPH may be idiopathic (~1/3) or secondary to trauma, SAH, or infection. LP reveals normal or minimally elevated opening pressure - but long-term monitoring shows intermittent nocturnal pressure spikes.

Gross Pathology

Hydrocephalus - coronal section showing dilated lateral ventricles
Coronal brain section showing grossly dilated lateral ventricles (red boxes) and posterior horns (yellow boxes) - Robbins & Kumar Basic Pathology
Long-standing hydrocephalus causes periventricular white matter atrophy (thinning of white matter from pressure), flattening of gyri, and interstitial edema from transependymal fluid seepage.

Imaging

CT

  • Dilated temporal horns (early and sensitive sign of noncommunicating hydrocephalus)
  • Periventricular interstitial (transependymal) edema: hypodense "halo" around ventricles on CT, hyperintense on T2/FLAIR MRI
  • Small sulci and obliterated basal cisterns (distinguish from atrophic ventriculomegaly where sulci are prominent)

MRI (preferred)

  • Shows all of the above plus cause of obstruction
  • Aqueductal stenosis: focal narrowing on sagittal MRI, usually at level of superior colliculi; lateral and 3rd ventricles dilated, 4th ventricle normal
  • NPH: DESH pattern (Disproportionately Enlarged Subarachnoid Space Hydrocephalus) - tight sulci at high convexity with enlarged Sylvian fissures
CT and MRI demonstrating iNPH with DESH pattern and periventricular changes
Idiopathic NPH: CT and MRI showing ventriculomegaly, DESH, and periventricular white matter changes
Longitudinal MRI series showing progressive ventriculomegaly and resolution
Serial MRI demonstrating dynamic ventricular changes in hydrocephalus
  • Grainger & Allison's Diagnostic Radiology, p. 2028-2033

Pathophysiology of Ventricular Enlargement

Acute noncommunicating hydrocephalus progresses rapidly - 80% of maximal ventricular enlargement occurs within 6 hours of obstruction, driven by continued CSF production despite rising pressure. This is followed by a slower phase where periventricular interstitial edema develops. Once it stabilizes, CSF pressure may paradoxically normalize (the basis for NPH). - Bradley and Daroff's Neurology in Clinical Practice, p. 1854

Treatment

Surgical CSF Diversion

ProcedureDetails
External Ventricular Drain (EVD)Acute/emergency; also allows ICP monitoring
Ventriculoperitoneal (VP) shuntMost common permanent option; one-way pressure-responsive valve; drains to peritoneal cavity
Ventriculoatrial (VA) shuntUsed when peritoneal cavity unavailable
Endoscopic Third Ventriculostomy (ETV)Creates opening in floor of 3rd ventricle → CSF drains to suprasellar cistern; preferred for aqueductal stenosis; avoids shunt hardware
Removal of obstructing lesionWhen a resectable mass is the cause

Shunt Complications

  • Malfunction/obstruction - choroid plexus or glial tissue blocking catheter tip; presents with recurrent hydrocephalus symptoms; revision required
  • Infection - requires externalization and antibiotic treatment
  • Over-drainage - subdural hematomas, slit ventricle syndrome
  • In children, revisions are frequently needed as the child grows
  • Shunt tubing integrity is assessed by plain X-ray ("shunt series"); obstruction and fluid tracking along tubing suggest malfunction

NPH - Diagnostic Work-up Before Shunting

  • Lumbar puncture (large-volume tap test) - remove 30-50 mL CSF; observe for gait improvement in the next 24-48 hours (positive tap test predicts shunt response)
  • Continuous ICP monitoring or external lumbar drainage over several days
  • A 2024 Cochrane review (PMID 39105473) evaluated shunting for iNPH; a 2025 systematic review (PMID 40045804) focused on standardizing the tap test protocol

Special Topics

Hydrocephalus ex Vacuo

Compensatory CSF expansion due to parenchymal loss - no intervention indicated. Distinguished from true hydrocephalus by prominent sulci (not compressed), no periventricular edema, and clinical features of the underlying neurodegenerative or vascular disease.

Fetal Hydrocephalus

A 2025 systematic review (PMID 40825578) evaluated surgical interventions for fetal hydrocephalus - in utero management remains largely investigational.

Post-Subarachnoid Hemorrhage

Acute hydrocephalus (obstructive or communicating) develops after SAH. Initial management: EVD insertion for ICP control and CSF drainage. Many patients progress to chronic hydrocephalus requiring VP shunt. - Goldman-Cecil Medicine, p. 3954

Sources: Bradley and Daroff's Neurology in Clinical Practice | Grainger & Allison's Diagnostic Radiology | Robbins & Kumar Basic Pathology | Goldman-Cecil Medicine | Bailey and Love's Short Practice of Surgery, 28th Ed.
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