Hydrocephalus

Notes

## Basic introduction

  • This is defined as the disturbance of CSF formation, flow, or absorption, leading to an increase in volume occupied by this fluid in the CNS.
  • In infants, hydrocephalus is characterized by rapid increase in head circumference but in older children and adults the skulls cannot expand to accommodate the buildup of CSF.

## Statistics

  • Globally, congenital hydrocephalus affects about 0.5 - 2 in every 1000 births.
  • In East Africa, there are > 6,000 new cases of hydrocephalus per year the majority of which are caused by neonatal infections.

## Types of Hydrocephalus

  • The two main classes of hydrocephalus are congenital and acquired hydrocephalus
  • Hydrocephalus is further categorized into communicating and noncommunicating hydrocephalus
  • Communicating hydrocephalus involves obstruction in the subarachnoid space while noncommunicating hydrocephalus involves the obstruction within the ventricles
  • Normal pressure hydrocephalus (NPH) is more common in patients > 60 yrs. This type of hydrocephalus develops gradually, enlarging the ventricles and increasing pressure in the brain
Symptoms
  • Enlargement of the head pre or post-partum
  • Bulging and/or tense fontanelles in infants
  • Infants feeding poorly and they may be vomiting
  • Dilated scalp veins
  • McEwen’s sign (a “cracked pot” sound when the head is percussed)
  • Setting-sun sign (a margin of sclera beneath the upper eyelid)
  • Brow overhanging the roof of orbit
  • Nystagmus
  • Transillumination
  • Obstructed labor
  • Blurred and/or double vision
  • Reduced mental activity
  • Headaches
  • Gait disturbance
  • Drowsiness
  • Difficulty in walking
  • Dementia
  • Seizures
  • Urinary incontinence
  • Parkinsonism
  • Babinski response in one or both feet / increased reflexes
Diagnosis
  • Cranial ultrasound
  • Skull X-ray
  • CT scan
  • Screening for infections
  • EEG in patient with seizures
  • MRI
Differential
  • Acute Subdural Hematoma in the ED
  • Brainstem Gliomas
  • Epidural Hematoma
  • Abscess Intracranial Hemorrhage
  • Pituitary Tumors
  • Subdural Empyema
Prevention
  • Some types of hydrocephalus can be prevented by prompt treatment of infections, especially in infants.
Management

## Shunt placements (the most common method of treatment)

  • Ventriculoperitoneal (VP) shunt (most common)
  • Ventriculoatrial (VA) shunt
  • Lumboperitoneal shunt
  • Torkildsen shunt
  • Ventriculopleural shunt

## Rapid-onset hydrocephalus with elevated intracranial pressure

  • Ventricular tap in infants
  • Open ventricular drainage in children and adults
  • Lumbar puncture (in posthemorrhagic and postmeningitic hydrocephalus)
  • VP or VA shunt

## Other treatment

  • Endoscopic third ventriculostomy combined with choroid plexus cauterization (popular in East Africa)

## Complications of Shunt placements

  • Mechanical failure
  • Infections
  • Obstructions
  • Need to lengthen or replace the catheter
  • Overdraining (that can lead to the collapse of ventricles and tearing of blood vessels, which in turn can cause headache and subdural hematoma)
  • Underdraining (that can cause the symptoms of hydrocephalus to recur)
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