Hydrocephalus

On this page

Definition & Types - Brainy Waterworks

  • Definition: Excess CSF → ventricular dilation ± ↑ICP.
  • Types:
    • Communicating (Non-obstructive): Impaired CSF reabsorption (arachnoid villi) or rare CSF overproduction (choroid plexus papilloma).
      • Causes: Post-meningitis, SAH.
    • Non-communicating (Obstructive): CSF flow blocked within ventricles/outlets.
      • Causes: Aqueductal stenosis, tumors, Chiari malformations.
    • Normal Pressure Hydrocephalus (NPH): Communicating type. Triad: Gait disturbance, Dementia, Urinary incontinence (📌 Wet, Wobbly, Wacky).
    • Hydrocephalus ex-vacuo: Ventricular dilation from brain atrophy; normal ICP. Not true hydrocephalus. CSF circulation & hydrocephalus obstruction sites

⭐ Aqueductal stenosis is the most common cause of congenital hydrocephalus.

Pathophysiology - CSF Traffic Jam

Hydrocephalus: Imbalance in CSF production, flow, or absorption.

  • Mechanisms causing CSF build-up:
    • Obstruction (Non-communicating): Blockage within ventricular pathways.
      • 📌 Aqueductal stenosis: Most common site.
      • Others: Tumors, IVH, congenital malformations.
    • Impaired Absorption (Communicating): Dysfunction at arachnoid granulations.
      • Post-infectious (meningitis), post-hemorrhagic (SAH).
    • Overproduction (Rare): e.g., Choroid plexus papilloma.
  • Consequences: Ventricular dilation → ↑ICP, neurological deficits.

CSF circulation, hydrocephalus etiology, and treatments

⭐ Aqueductal stenosis: most common cause of congenital hydrocephalus; key site for non-communicating type.

Clinical Features - Spotting the Swell

  • Infants (Open Fontanelles/Sutures):
    • Head: Rapidly ↑ head circumference (HC), crossing centiles.
    • Fontanelle: Bulging, tense anterior.
    • Sutures: Split, widened.
    • Scalp veins: Dilated, prominent.
    • Macewen sign ("cracked pot" sound). 📌 "Mac's Pot"
    • Eyes: "Setting sun" sign (downward gaze).
    • General: Irritability, poor feeding, vomiting.
    • Infant Hydrocephalus Clinical Signs
  • Older Children (Fused Sutures):
    • ↑ ICP signs:
      • Headache (AM, improves with vomiting).
      • Nausea, vomiting (AM, projectile).
      • Papilledema.
      • CN VI palsy (diplopia).
    • Other: Gait ataxia, spasticity, cognitive decline.
    • Late: Cushing's triad (hypertension, bradycardia, irregular respirations).

⭐ "Setting sun" sign: Paresis of upward gaze in infants, due to pressure on tectal plate (Parinaud's syndrome).

Diagnosis - Brain Scan Clues

  • Imaging:
    • USG (infants): Initial.
    • CT: Acute.
    • MRI: Gold standard (etiology).
  • Ventriculomegaly Signs:
    • Evans' Index > 0.3.
    • Temporal horn dilatation (early).
    • "Mickey Mouse" sign (frontal horns, coronal). 📌
  • Other Clues:
    • Periventricular lucency/hyperintensity (CSF seepage).
    • Effaced sulci.
    • Corpus callosum: Stretched, upward bowed.
  • Type (MRI):
    • Non-communicating: Obstruction (e.g., aqueductal stenosis → triventricular hydrocephalus).
    • Communicating: Diffuse dilatation, patent pathways.

⭐ Triventricular hydrocephalus (dilated lateral/3rd ventricles, normal 4th) on MRI strongly suggests aqueductal stenosis.

Management & Complications - Drain & Outlook

  • Surgical (Mainstay):
    • VP Shunt: Most common; programmable/fixed pressure valves.
    • ETV (Endoscopic Third Ventriculostomy): For non-communicating hydrocephalus; ETV Success Score (ETVSS) guides.
  • Medical (Adjunctive/Temporary): Acetazolamide, Furosemide (↓CSF production).
  • Shunt Complications:
    • Infection (5-15%): S. epidermidis/aureus.
    • Malfunction/Obstruction: Signs of ↑ICP.
    • Overdrainage: Subdural collections, slit ventricle syndrome.
  • Outlook: Variable; depends on cause, timely management. Lifelong follow-up.

    ⭐ Shunt infection, often by Staphylococcus epidermidis, is the most frequent serious complication, necessitating shunt removal, EVD, and antibiotics.

VP shunt system in a child with hydrocephalus

High-Yield Points - ⚡ Biggest Takeaways

  • Hydrocephalus is an abnormal CSF accumulation causing ventricular dilatation and ↑ICP.
  • Key types: Communicating (impaired absorption) and Non-communicating/Obstructive (CSF flow blockage).
  • Infants present with rapid head growth, bulging fontanelle, and sunset sign.
  • Older children show headache, vomiting, papilledema, and gait ataxia.
  • Common causes include aqueductal stenosis, meningitis, IVH, and posterior fossa tumors.
  • Diagnosis via ultrasound in infants; CT/MRI are definitive imaging.
  • Mainstay of treatment is ventriculoperitoneal (VP) shunt; ETV for obstructive types.

Practice Questions: Hydrocephalus

Test your understanding with these related questions

All of the following are causes of vasogenic-type cerebral edema except which of the following?

1 of 5

Flashcards: Hydrocephalus

1/10

Congenital hydrocephalus often presents initially with macrocephaly, muscle _____tonicity and hyperreflexia, and a bulging fontanelle

TAP TO REVEAL ANSWER

Congenital hydrocephalus often presents initially with macrocephaly, muscle _____tonicity and hyperreflexia, and a bulging fontanelle

hyper

browseSpaceflip

Enjoying this lesson?

Get full access to all lessons, practice questions, and more.

Start Your Free Trial