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What is Hydrocephalus?
Hydrocephalus, also known as "water in the brain," is a medical condition in which there is an 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, tunnel vision, and mental disability. Hydrocephalus can also cause death. The name derives from the Greek words ὑδρο- (hydro-) "water", and κέφαλος (kephalos) "head".
The data concerning the incidence and prevalence of this condition are hard to establish since there is no national registry or data base with regards of the patients carrying Hydrocephalus and the disorders tightly associated with the disease, however, it is believed that this condition affects 1 in 500 children. To date, most of these cases are diagnosed in the prenatal stage or in its early years of childhood.
The causes of hydrocephalus are still not well understood. Hydrocephalus may result from inherited genetic abnormalities (such as the genetic defect that causes aqueductal stenosis) or developmental disorders (such as those associated with neural tube defects including spina bifida and encephalocele). Other possible causes include complications of premature birth such as intraventricular hemorrhage, diseases such as meningitis, tumors, traumatic head injury, or subarachnoid hemorrhage, which block the exit of CSF from the ventricles to the cisterns or eliminate the passageway for CSF into the cisterns.
Symptoms of hydrocephalus vary with age, disease progression, and individual differences in tolerance to the condition. For example, an infant's ability to compensate for increased CSF pressure and enlargement of the ventricles differs from an adult's. The infant skull can expand to accommodate the buildup of CSF because the sutures (the fibrous joints that connect the bones of the skull) have not yet closed. In infancy, the most obvious indication of hydrocephalus is often a rapid increase in head circumference or an unusually large head size. Other symptoms may include vomiting, sleepiness, irritability, downward deviation of the eyes (also called "sunsetting"), and seizures. Older children and adults may experience different symptoms because their skulls cannot expand to accommodate the buildup of CSF. Symptoms may include headache followed by vomiting, nausea, papilledema (swelling of the optic disk which is part of the optic nerve), blurred or double vision, sunsetting of the eyes, problems with balance, poor coordination, gait disturbance, urinary incontinence, slowing or loss of developmental progress, lethargy, drowsiness, irritability, or other changes in personality or cognition including memory loss. Symptoms of normal pressure hydrocephalus include, problems with walking, impaired bladder control leading to urinary frequency and/or incontinence, and progressive mental impairment and dementia. An individual with this type of hydrocephalus may have a general slowing of movements or may complain that his or her feet feel "stuck." Because some of these symptoms may also be experienced in other disorders such as Alzheimer's disease, Parkinson's disease, and Creutzfeldt-Jakob disease, normal pressure hydrocephalus is often incorrectly diagnosed and never properly treated. Doctors may use a variety of tests, including brain scans (CT and/or MRI), a spinal tap or lumbar catheter, intracranial pressure monitoring, and neuropsychological tests, to help them accurately diagnose normal pressure hydrocephalus and rule out any other conditions. The symptoms described in this section account for the most typical ways in which progressive hydrocephalus manifests itself, but it is important to remember that symptoms vary significantly from one person to the next.
Hydrocephalus is diagnosed through clinical neurological evaluation and by using cranial imaging techniques such as ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), or pressure-monitoring techniques. A physician selects the appropriate diagnostic tool based on an individual's age, clinical presentation, and the presence of known or suspected abnormalities of the brain or spinal cord.
Hydrocephalus is most often treated by surgically inserting a shunt system. This system diverts the flow of CSF from the CNS to another area of the body where it can be absorbed as part of the normal circulatory process. A shunt is a flexible but sturdy plastic tube. A shunt system consists of the shunt, a catheter, and a valve. One end of the catheter is placed within a ventricle inside the brain or in the CSF outside the spinal cord. The other end of the catheter is commonly placed within the abdominal cavity, but may also be placed at other sites in the body such as a chamber of the heart or areas around the lung where the CSF can drain and be absorbed. A valve located along the catheter maintains one-way flow and regulates the rate of CSF flow. A limited number of individuals can be treated with an alternative procedure called third ventriculostomy. In this procedure, a neuroendoscope — a small camera that uses fiber optic technology to visualize small and difficult to reach surgical areas — allows a doctor to view the ventricular surface. Once the scope is guided into position, a small tool makes a tiny hole in the floor of the third ventricle, which allows the CSF to bypass the obstruction and flow toward the site of resorption around the surface of the brain.
La Hidrocefalia se diagnostica mediante una evaluación neurológica clínica y mediante el uso de técnicas de imágenes craneales tales como la ultrasonografía, la tomografía computarizada (CT), las imágenes de resonancia magnética (MRI) o técnicas de vigilancia de la presión. El médico selecciona la herramienta diagnóstica apropiada tomando como base la edad, la presentación clínica del paciente, y la presencia de otras anormalidades conocidas o sospechadas del cerebro o la médula espinal.
Shunt systems are not perfect devices. Complications may include mechanical failure, infections, obstructions, and the need to lengthen or replace the catheter. Generally, shunt systems require monitoring and regular medical follow up. When complications occur, the shunt system usually requires some type of revision. Some complications can lead to other problems such as overdraining or underdraining. Overdraining occurs when the shunt allows CSF to drain from the ventricles more quickly than it is produced. Overdraining can cause the ventricles to collapse, tearing blood vessels and causing headache, hemorrhage (subdural hematoma), or slit-like ventricles (slit ventricle syndrome). Underdraining occurs when CSF is not removed quickly enough and the symptoms of hydrocephalus recur. In addition to the common symptoms of hydrocephalus, infections from a shunt may also produce symptoms such as a low-grade fever, soreness of the neck or shoulder muscles, and redness or tenderness along the shunt tract. When there is reason to suspect that a shunt system is not functioning properly (for example, if the symptoms of hydrocephalus return), medical attention should be sought immediately.
The signs and symptoms of shunt malfunction are the same as for hydrocephalus itself: headaches, nausea, vomiting, irritability, change in behavior or intellectual performance, etc.
Malfunction in infants:
• Enlargement of the baby's head
• Fontanel is full and tense when the infant is upright and quiet
• Prominent scalp veins
• Swelling along the shunt tract
• Vomiting
• Irritability
• Sleepiness
• Downward deviation of the eyes
• Seizures
The prognosis for individuals diagnosed with hydrocephalus is difficult to predict, although there is some correlation between the specific cause of the hydrocephalus and the outcome. Prognosis is further complicated by the presence of associated disorders, the timeliness of diagnosis, and the success of treatment. The degree to which relief of CSF pressure following shunt surgery can minimize or reverse damage to the brain is not well understood. Affected individuals and their families should be aware that hydrocephalus poses risks to both cognitive and physical development. However, many children diagnosed with the disorder benefit from rehabilitation therapies and educational interventions and go on to lead normal lives with few limitations. Treatment by an interdisciplinary team of medical professionals, rehabilitation specialists, and educational experts is critical to a positive outcome. Left untreated, progressive hydrocephalus may be fatal. The symptoms of normal pressure hydrocephalus usually get worse over time if the condition is not treated, although some people may experience temporary improvements. While the success of treatment with shunts varies from person to person, some people recover almost completely after treatment and have a good quality of life. Early diagnosis and treatment improves the chance of a good recovery.
• Do not touch the shunt, unless you are under physician's instructions.
• Do not lay the child on the side the shunt is placed, otherwise you may cause skin abrasion.
• Once the child moves freely, he will be able to react to pain, so he will find a position that is comfortable.
• Monitor the appearance of redness or irritation of the skin around or on top of the shunt.
• Monitor for a leveled or sunken fontanel (top part of the head).
• Be aware of the appearance of any syntoms of a shunt malfunction.
Remember, a shunt is a foreign object in our body, which is a sort of a synthetic piping that may malfunction due to: The obstruction of cerebrospinal fluids. It may disconnect from any of its parts. It may break (usually from the distal catheter) The shunt may drain more or less cerebrospinal fluids from the expected. Infection
While the shunt is working properly, there is no need to change it or replace it. In some cases, due to the child's growth the catheter may break or get obstructed or displaced from the valve, in which case the patient will present valve disorder and parent should get aware by this. Around 6 out of 10 children, at some point in their life, will need their shunt checked out or replaced. Other cases in which a shunt may be replaced is caused by infection.
At the The National Institute of Pediatrics, General Hospital of Mexico & Hospital Infantil Federico Gómez, which are government institutions, as well as the ABC Medical Center, renowned private institution, all of them located in Mexico, DF. As for the rest of the country, every state has its general hospital o pediatric hospital where you will find help or a proper channel to reach Mexico City and find treatment for your child.
. www.hydroassoc.org
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