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Hydrocephalus

Hydrocephalus is a condition in which excess fluid accumulates in the brain. The name is derived from the Greek “hydro,” meaning water, and “cephalus,” meaning or pertaining to the head. In the case of hydrocephalus, the fluid that gathers on the brain is cerebrospinal fluid. The effects of excess fluid on the brain include a widening of the ventricles and excessive pressure on the tissues of the brain.

In a healthy infant, cerebrospinal fluid flows through the ventricles, exits into cisterns (closed spaces that serve as reservoirs) at the base of the brain, bathes the surfaces of the brain and spinal cord, and then is reabsorbed into the blood stream. The fluid serves the important function of maintaining a specific buoyancy level in the intracranial space to absorb shock, providing nutrients to the brain tissues and helping the brain adapt to changes in blood volume in the intracranial compartments.

Hydrocephalus results when inflow and drainage of cerebrospinal fluid are disrupted in the cranial spaces. Congenital hydrocephalus is present at birth and may be caused by prenatal occurrences during fetal development or genetic abnormalities. Acquired hydrocephalus develops at the time of birth or at some point afterward.

Disruption of cerebrospinal fluid flow can occur as a result of cerebral sinus-thrombic or arterial-ischemic stroke, intracranial hemorrhage, intraventricular hemorrhage, subarachnoid hemorrhage, direct head trauma, infection, tumors, or complications from surgery. Other risk factors include prematurity, traumatic brain injury and instrumental deliveries.   

The symptoms of hydrocephalus in the neonatal period are different from those seen in children or adults. Hydrocephalus that occurs prenatally can be detected by an ultrasound, showing enlarged ventricles in the fetal brain. Usually, hydrocephalus will be noticed at birth if a child has an unusually large head or if there is increase in the child’s head circumference during the neonatal period. Babies with hydrocephalus will often be overly sleepy or inactive, irritable, and let out shrill cries. Other symptoms include vomiting, a “sun-setting” or downwardly-facing gaze, and seizures.

Treatment for hydrocephalus is usually dependent on the severity of the condition. Typically, surgical shunts are placed in the brain to help drain cerebrospinal fluid from the ventricles, normalizing intracranial pressure. Far less frequently, hydrocephalus is transient and resolves on its own. Medications such as acetazolamide and furosemide, which decrease cerebrospinal fluid secretion, and isosorbide, which increases cerebrospinal fluid reabsorption, are used in cases where acute management is necessary.

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