Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Hydrocephalus (pronunciation IPA: /?ha??d?o?’s?f?l?s/) is a term derived from the Greek words “hydro” meaning water, and “cephalus” meaning head, and this condition is sometimes known as “water on the brain”. People with hydrocephalus have abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles, or cavities, of the brain. This may cause increased intracranial pressure inside the skull and progressive enlargement of the head, convulsion, and mental disability.
Hydrocephalus is usually due to blockage of CSF outflow in the ventricles or in the subarachnoid space over the brain. In a person without hydrocephalus, CSF continuously circulates through the brain, its ventricles and the spinal cord and is continuously drained away into the circulatory system. Alternatively, the condition may result from an overproduction of the CSF fluid, from a congenital malformation blocking normal drainage of the fluid, or from complications of head injuries or infections.[1]
Regardless of cause, the fluid accumulates in the ventricles. Compression of the brain by the accumulating fluid eventually may cause convulsions and mental retardation. These signs occur sooner in adults, whose skulls no longer are able to expand to accommodate the increasing fluid volume within. Fetuses, infants, and young children with hydrocephalus typically have an abnormally large head, excluding the face, because the pressure of the fluid causes the individual skull bones — which have yet to fuse — to bulge outward at their juncture points. Another medical sign, in infants, is a characteristic fixed downward gaze with whites of the eyes showing above the iris, as though the infant were trying to examine its own lower eyelids.[2] Hydrocephalus occurs in about one out of every 500 live births[3] and was routinely fatal until surgical techniques for shunting the excess fluid out of the central nervous system and into the blood or abdomen were developed. Hydrocephalus is detectable during prenatal ultrasound examinations.
Usually, hydrocephalus does not cause any intellectual disability if recognized and properly treated. A massive degree of hydrocephalus rarely exists in typically functioning people, though such a rarity may occur if onset is gradual rather than sudden.[4]
Hydrocephalus was first described by the ancient Greek physician Hippocrates, but it remained an intractable condition until the 20th century, when shunts and other neurosurgical treatment modalities were developed. Although 1 Million Americans suffer from hydrocephalus, it remains a lesser-known medical condition. Relatively small amounts of research is conducted to improve treatments for hydrocephalus, and to this day there remains no cure for the condition.
Hydrocephalus affects one in every 500 live births, making it one of the most common developmental disabilities, more common than Down syndrome or deafness.[3] According to the NIH website, there are an estimated 700,000 children and adults living with hydrocephalus, and it is the leading cause of brain surgery for children in the United States. There are over 180 different causes of the condition, one of the most common being brain hemorrhage associated with premature birth.
One of the most performed treatments for hydrocephalus, the cerebral shunt, has not changed much since it was developed in 1960. The shunt must be implanted through neurosurgery into the patient’s brain, a procedure which itself may cause brain damage. An estimated 50% of all shunts fail within two years, requiring further surgery to replace the shunts. In the past 25 years, death rates associated with hydrocephalus have decreased from 54% to 5% and the occurrence of intellectual disability has decreased from 62% to 30%.
In the United States, the healthcare cost for hydrocephalus has exceeded $1 billion per year, but is still much less funded than research on other diseases including juvenile diabetes.[5]
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