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Flashcards in dit deck (38)
  • Hydrocephalus, from the Greek words hydro (water) and kefala (head), is one of the most common pediatric and infant conditions.

  • Annual specialized health expenditures for hydrocephalus cost approximately \(2 billion in the United States and beyond \)1 billion in sub-Saharan Africa.

  • Hydrocephalus occurs due to an abnormal buildup of cerebrospinal fluid (CSF) in the ventricular spaces of the brain.

  • The buildup of CSF in hydrocephalus leads to increased intracranial pressures (ICPs), splaying of cranial suture lines, and various neurologic symptoms and deficits.

  • The morbidity burden of hydrocephalus can be very high, especially in infants, with possible long-term neurologic sequelae including cerebral palsy, epilepsy, and cognitive and behavioral delays.

  • Hippocrates was one of the first to describe accumulation of water in the brain as hydrocephalus in 400 BC.

  • By the 1800s, it was accepted that the 'water' in hydrocephalus was CSF, and symptoms resulted from increased ICP.

  • Direct drainage of CSF, either as a ventriculostomy from the brain or a lumbar puncture from the thecal sac, provided symptom relief for hydrocephalus.

  • By the early 1900s, surgical treatments for hydrocephalus, such as third ventriculostomies and ventricular shunting to the peritoneum, proved more effective and safe.

  • Hydrocephalus refers to the clinical manifestations of increased ICPs, including increasing head circumference and irritability in infants.

  • Ventriculomegaly refers to the radiographic increase in ventricle system size.

  • Macrocephaly refers to a physical head circumference that is above the 98th percentile age-standardized measurement.

  • While hydrocephalus can present with ventriculomegaly and macrocephaly, these conditions can also occur due to other causes and are not exclusive to a hydrocephalus diagnosis.

  • Almost 400,000 new cases of infant hydrocephalus are diagnosed worldwide each year, approximating a rate of 20 to 110 per 100,000 live births.

  • Up to 75% of the global burden of hydrocephalus falls in countries of Africa, Latin America, and Southeast Asia.

  • The incidence of hydrocephalus is higher in low- and middle-income countries (123 per 100,000 births) than in high-income countries (79 per 100,000 births).

  • Disparities in hydrocephalus incidence are driven by quality and access to prenatal care and education, endemic infections, poorer diets, and limited access to diagnostic imaging.

  • In low- and middle-income countries, materials such as imaging equipment, shunt valves, and endoscopy equipment are unavailable, limiting the efficacy of hydrocephalus treatment.

  • There are likely many more patients in low- and middle-income countries who do not receive timely treatment as in high-income countries.

  • While in utero, fetal ventriculomegaly can be detected on surveillance ultrasonography.

  • Fetal ventriculomegaly is defined as a greater-than-normal diameter of the lateral ventricle atria, typically greater than 10 mm.

  • Specialist interpretation of prenatal imaging is required because ventriculomegaly can also result from abnormal brain development and not necessarily reflect hydrocephalus caused by increased ICPs.

  • In infants, signs of hydrocephalus are more subtle than the classic symptoms seen in older children and adults.

  • Neonates are born with open fontanelles and skull sutures, which can accommodate increases in ICP more so than a more mature skull.

  • Early signs of hydrocephalus in infants are mostly physical rather than symptomatic.

  • Early signs of hydrocephalus in infants include: - growing head circumference crossing multiple percentiles over time - bulging fontanelles - separated (splayed) cranial sutures.

  • As untreated hydrocephalus progresses, patients may present with: - Initially: vomiting, irritability, lethargy - Later: seizures, downward deviated gaze, and episodes of apnea and bradycardia.

  • An infant with evolving hydrocephalus commonly presents with irritability and vomiting, which can be confused with infant colic or viral infection.

  • Parents of an infant with hydrocephalus may note increasing head size and a 'full head', which refers to the fontanelles being pushed outward due to increasing ICPs.

  • Head imaging is the most useful procedure to diagnose or rule out hydrocephalus.

  • A large head circumference or macrocephaly alone can also arise due to benign anatomic macrocephaly or benign subdural collections like hygromas.

  • Distinguishing benign macrocephaly from hydrocephalus is important because benign conditions can be misconstrued as traumatic in nature.

  • On imaging of hydrocephalus in infants, enlarged ventricular systems can often be appreciated.

  • Head ultrasonography is typically the preferred initial imaging modality for neonates due to lack of radiation exposure and ease at bedside.

  • Magnetic resonance imaging without contrast, particularly using the cine flow sequence, can identify subtle flow-related abnormalities to diagnose hydrocephalus types.

  • CSF is a clear, acellular liquid derived from plasma.

  • CSF circulates in the subarachnoid space of the central nervous system, covering the brain and spine.

  • CSF plays a crucial role in: - protecting - nourishing - removing waste from the central nervous system - Providing mechanical shock absorbance.