Hydrocephalus*

Hypoxia and brain trauma can lead to bleeding inside brain ventricles, which can cause ventricular swelling due to excessive cerebrospinal fluid (CSF). This damages white matter in the brain and impairs the growth of the cerebral cortex, significantly impacting memory, attention, perception, thought, language and consciousness. Hydrocephalus occurs as a secondary result of other brain injuries in a cascade where damage occurs in a feedback loop to cause more damage. This is usually treated using a shunt system to relieve pressure on the brain from CSF.

Permanent damage to a baby’s brain can occur when a baby experiences a lack of oxygen (hypoxia), head trauma or complications around the time of birth.  Hypoxia and trauma can cause bleeding inside the ventricles of the brain, called an intraventricular hemorrhage (IVH).  This can progress to significant swelling of the ventricles, called hydrocephalus.  The swelling is caused by too much cerebral spinal fluid (CSF).  Hydrocephalus can lead to damaged blood vessels and destruction of white matter in the brain, and the cerebral cortex may not develop properly.  White matter is important because it regulates the electrical signals between cells called neurons.  It is responsible for transmitting information throughout the brain, to the spinal cord, and outside the brain to the rest of the body.  These signals control our bodily functions; nerve cells that transmit signals to the brain, or neurons that regulate breathing or heart rate, would be unable to perform their tasks without white matter.  The cerebral cortex plays a key role in memory, attention, perceptual awareness, thought, language and consciousness.  Hydrocephalus can cause a child to have brain / developmental impairment with many lifelong conditions, such as seizures, cerebral palsy, and developmental delays that affect reasoning, memory, speech or other learning and communication abilities.

Medical Explanations

Causes of Hydrocephalus

Although other conditions can cause hydrocephalus, it many times begins with IVH.  Then there is a disturbance in CSF dynamics, which leads to progressive dilation (swelling) of the brain ventricles, which then results in hydrocephalus.

The progression of hydrocephalus is as follows:

  1. IVH (or other brain insult)
  2. Disturbance in CSF dynamics
  3. Swelling of the ventricles
  4. Hydrocephalus

IVH AND OTHER BRAIN INSULTS

Hydrocephalus often begins with an injury to the brain that causes IVH.  Premature babies are more susceptible to IVH because blood vessels and other parts of their brains are fragile.  Conditions that can cause IVH in a term or preterm baby include the following:

  • Hypoxic ischemic encephalopathy (lack of oxygen or blood flow to the brain)
  • Abnormal changes in blood pressure
  • Trauma from prolonged labor
  • Trauma from a difficult birth, which can occur when a baby is large for its gestational age (macrosomic), the mother has a small pelvis or the baby’s head or body cannot easily fit through the birth canal (CPD), or forceps or vacuum extractors are used to facilitate delivery
  • Oxygen deprivation or head trauma from use of Pitocin or Cytotec
  • Abnormal presentation that causes head trauma, such as a breech or face presentation

*The most common cause of acquired (after birth) hydrocephalus in a baby is a germinal matrix hemorrhage (GMH), also known as a periventricular-intraventricular hemorrhage.   GMH occurs primarily in premature infants.  This is because activity in the germinal matrix peaks at 8 – 23 weeks of gestation.  The germinal matrix helps create the fully-formed brain; cells develop and migrate out of this structure during brain development.  Damage to the germinal matrix at weeks 8-23 can cause severe brain damage and death.  A GMH occurs when there is bleeding into the part of the germinal matrix that is connected to the lateral ventricles.  When this occurs, there may be subsequent rupture into the lateral ventricles.  A GMH causes the ventricles to be filled with blood and degradation products, and this, among other things, causes dilation of the ventricles.  GMH leads to a lot of stress on the areas around it, as well as congestion of the blood vessels.  All of these factors can lead to white matter injury and permanent brain damage.

Other conditions that can lead to hydrocephalus include the following:

  • Intrauterine infections, such as syphilis, cytomegalovirus (CMV), rubella and toxoplasmosis
  • Brain infections, such as meningitis or viral infections.
  • Hemorrhage (severe and sudden bleeding) into the subarachnoid space, which is the space in between the layers that cover the brain.
  • Hemorrhage into the ventricles of the brain, which can be caused by ruptured vessels and trauma.

DISTURBANCE IN CSF DYNAMICS, SWELLING OF THE VENTRICLES AND BRAIN DAMAGE

CSF is a liquid that completely surrounds the brain and spinal cord tissue.  The liquid circulates throughout the brain before being reabsorbed into the blood through the brain ventricles.  In normal conditions, the absorption of CSF generally matches the volume of CSF being formed each day.  CSF protects the brain and spinal cord against any jarring forces, acting as a shock absorber.  CSF is comprised of a mixture of proteins that supports brain cell development.

Hydrocephalus results from an imbalance between the intracranial CSF inflow and outflow, and is most often caused by inadequate absorption of CSF or obstruction of CSF circulation.  When there is severe insult to the brain due to conditions such as oxygen deprivation (hypoxia), trauma or infection, the brain swells.  Although the initial swelling may subside, the injury may result in damage to one or more ventricles, causing a blockage or damage to tissue responsible for absorption of CSF.  Since the body is constantly making CSF, any delayed absorption or obstruction results in buildup of the fluid, causing the ventricles to swell.  This, in turn, leads to pressure on surrounding brain matter, pushing aside the soft tissue of the brain and leading to further brain damage.

More specifically, hydrocephalus and brain damage can occur due to the following events:

  • Dilation of the ventricles usually is uneven.  The front and back horns of the ventricles typically enlarge first, and to the greatest extent.  This enlargement disrupts the lining of the ventricles, which allows CSF to move directly into the brain tissue.  This reduces a lot of CSF pressure, but also leads to fluid going into the spaces within the membranes that cover the brain, with progressive involvement and damage of white matter.
  • As the hydrocephalus progresses, fluid build up and ischemia (restricted blood flow) develop in the white matter that surrounds the ventricles (the periventricular brain tissue).  This leads to destruction of the white matter.
  • With further progression of this process, the membrane that covers the brain can become obliterated over the brain hemispheres.  (The gray brain matter at the cortex may be reduced, but gray matter is better preserved than white matter.) This eventually leads to the vascular system becoming compressed, and pressure in the veins increasing in certain areas.
  • The vessels in the brain become weak, and there is a loss of vessels in the white matter, which leads to white matter destruction.  Damage to the white matter also  is caused by changes in cells that are part of the central nervous system.

Signs of Hydrocephalus

When birth trauma results in hydrocephalus, evidence of fluid buildup may be present within days.  There may be an appearance of puffiness or swelling at the temples, at the bridge of the nose, or possibly around the eyes.  Diagnosing the condition early and treating it quickly can help limit any long-term problems.  Long-term effects mainly depend on the cause of the fluid buildup, how severe it is, and how the baby responds to treatment.

A very visible sign of hydrocephalus is an enlarged head.  Rapid head growth is a sign of increased intracranial pressure (ICP).  The growth occurs because the bones of the skull are not yet fused, so the head can stretch and grow in an attempt to decrease the pressure in the brain.  Symptoms of hydrocephalus also may include vomiting, sleepiness, irritability, an inability to look upward, and seizures.

If left completely untreated, hydrocephalus can cause the brain stem to become so compressed that the heart can stop or breathing may cease.  The compression of the cerebellum, while not as serious as a compressed brain stem, may lead to problems with swallowing, speaking, and breathing, or it can cause cerebral palsy to develop in fetuses and infants

Diagnosing Hydrocephalus

Many diagnostic tools are available to aid physicians and neurologists in the diagnosis of hydrocephalus, including:

  • Cerebral angiography, which is a test used to detect blockages of the arteries or veins.  Images are taken after a dye is injected into the area.
  • Ultrasonography uses sound waves to take pictures of soft tissue in the brain.
  • Computed tomography, also known as a CT scan, takes two-dimensional images of organs, bones, and tissue.  Neurological CT scans are used to view the brain and spine.
  • Magnetic Resonance Imaging (MRI) uses computer-generated radio waves and a magnetic field to produce detailed images of body structures including tissue, organs, bones, and nerves.
  • CSF analysis involves the removal of a small amount of fluid for testing.
  • Electroencephalography (EEG) monitors brain activity through the skull.  EEG is used to help diagnose conditions such as certain seizure disorders, brain damage from head injuries, and inflammation of the brain and / or spinal cord.
  • An external pressure transducer can be used to measure the ICP.

Treating Hydrocephalus

Hydrocephalus is manageable.  The most common treatment involves surgical placement of a shunt system.

Using tubes, this system redirects the flow of fluid from an area of buildup to the abdominal cavity where it can be absorbed as part of the circulatory process.  A valve within the shunt system allows physicians to adjust the flow to normalize pressure.  Physicians insert a tube long enough to accommodate the child’s growth, thereby potentially eliminating the need for another surgery.

Hydrocephalus, Birth Injuries and Cerebral Palsy

Prevention of HIE, head trauma, infection and other conditions that can cause hydrocephalus is of utmost importance.  If hydrocephalus occurs, prompt diagnosis and treatment are essential.  The longer hydrocephalus is allowed to occur without proper treatment, the more severe the brain damage, which can lead to cerebral palsy.  When risk factors for hydrocephalus or signs and symptoms of the condition occur, close monitoring of the baby is essential.  Due to the severity of the condition, diagnostic tests must be performed when there is any suspicion of brain hemorrhage or hydrocephalus.

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