Hydrocephalus

BabyHydrocephalus is a very serious type of infant brain injury in which the ventricle spaces inside the brain become flooded by a backup of cerebrospinal fluid (CSF). Hydrocephalus can occur in adults but it is most common in newborn infants who experience some type of external trauma to the head during childbirth.

What is Hydrocephalus?

Hydrocephalus literally means "water in the brain" ("hydro" is a Greek word for water and "cephalus" refers to the head). Hydrocephalus is a medical condition that occurs when cerebrospinal fluid (CSF) becomes blocked and starts to build up within the brain eventually flooding into the ventricle cavities. CSF is a vital fluid that is constantly produced inside the ventricles of the brain. As the CSF is produced it gets released from the ventricles and it is supposed to circulate down to the spinal cord before getting absorbed into the blood. Hydrocephalus occurs when this normal outflow of CSF from the brain down to the spine gets blocked. The blockage prevents the release of CSF but the brain continues to produce it so it begins to back up, like water in a clogged plumbing system. As the CSF accumulates it eventually floods into the brain ventricles. The flooding within the ventricles creates internal outward pressure inside the brain which can cause devastating internal damage.

Different Types of Hydrocephalus

Although hydrocephalus is usually a birth injury resulting from external head trauma during delivery, it can also be caused by a congenital defect. Congenital hydrocephalus is triggered by a developmental abnormality that occurs during fetal growth and may be genetically passed down. The most common abnormalities that cause congenital hydrocephalus is aqueductal stenosis Abnormalities that can trigger congenital hydrocephalus can sometimes be diagnosed during pregnancy. When properly diagnosed these conditions can be managed so good prenatal care is an important preventative measure. When hydrocephalus is the result of head trauma (or another perinatal event) during labor and delivery, it often described as "acquired" rather than congenital.

Whether acquired or congenital, hydrocephalus is classified into 1 of 2 different types based on exactly where the cerebrospinal fluid is being blocked within its circulatory path:

  • Communicating Hydrocephalus: communicating hydrocephalus (also called "non-obstructive") occurs when the CSF circulation is being blocked somewhere outside of the brain.
  • Non-Communicating Hydrocephalus: when the outflow of CSF is actually being blocked somewhere inside the brain and ventricles, the condition is classified as non-communication or "obstructive" hydrocephalus.
Causes of Congenital Hydrocephalus

The most common developmental abnormaliy that causes congenital hydrocephalus is aqueductal stenosis. It occurs when the narrow pathway connecting the 4th and 3rd ventricles (called the aqueduct of Sylvius) becomes compressed and blocked. The compression is usually the result of either an infection or a tumor. A neural tube defect is another common cause of congenital hydrocephalus. This occurs when the spinal cord is exposed during fetal development and CSF leaks out.

Causes of Acquired Hydrocephalus

Acquired hydrocephalus is caused by some external trauma or another event that occurs during labor and delivery which triggers a blockage of normal CSF flow and absorption. Injury from external trauma the head during a difficult vaginal delivery is the leading cause of acquired hydrocephalus. When the baby's delicate head is subjected to excessive pressure during childbirth it can rupture blood vessels and cause swelling within the. The internal inflammation in the brain can obstruct pathways and block or restrict the outflow of CSF. The blockage causes a buildup of CSF that eventually floods into the ventricles. Hydrocephalus can also be caused by intraventricular hemorrhage which occurs when blood vessels around the brain ventricles rupture and block CSF flow. This frequently occurs in premature babies with underdeveloped blood vessels.

Hydrocephalus Treatment & Prognosis

Sadly there is no effective cure or treatment for hydrocephalus and it is often difficult to prevent. Complex neurosurgery is currently the only option for treatment of hydrocephalus. The surgery attempts to clear the blockage of CSF and restore normal circulation of the fluid out of the brain. This is typically accomplished by surgically implanting a ventriculoperitoneal shunt inside the head. Any type of major brain surgery like this is always a difficult and very high risk, but this is particularly true for newborn infants. There are a host of potential post-surgical complications that can arise and the shunts have to be replaced after just a few years.

Hydrocephalus is an extremely dangerous medical condition that can be potentially fatal. The internal pressure caused by a buildup of CSF within the brain can do extensive internal damage as well as disrupt normal brain development. Hydrocephalus is also a progressive condition which means it can and often does get worse over time. If left undiagnosed and untreated, the pressure within the brain from hydrocephalus do enough damage to cause death.