Hydrocephalus is a Greek word that means excessive accumulation of fluid in the brain. This excess fluid causes an abnormal dilation of the spaces (ventricles) in the brain causing great damaging pressure on the brain tissues.
Acquired hydrocephalus can affect people of all ages and can be caused by an injury or illness developed at birth. It can also be congenital, when it is present at birth and can be caused by environmental influences during the development of the fetus during pregnancy, or by genetic predisposition.
Hydrocephalus occurs when there is an imbalance between the amount of fluid the brain produces and how the body is able to process it. It is not known exactly why hydrocephalus occurs.
The causes of hydrocephalus are not very well defined. Both can be due to genetic or congenital inheritance when the fetus has bled before birth, due to infections such as toxoplasmosis or syphilis from the mother, due to fetal development disorders such as those associated with neural tube defects, including spina bifida , or due to a genetic abnormality.
Other possible causes are complications of premature birth, such as intraventricular hemorrhage, diseases such as meningitis, tumors, traumatic head injury or hemorrhage that blocks the exit of the ventricles to the cisterns and eliminates the cisterns themselves.
Symptoms vary with age, disease progression, and individual differences in disease tolerance. The most obvious symptom of hydrocephalus is a larger than normal head. The baby's skull can expand a lot in the first year to accommodate the increased cerebrospinal fluid because the sutures (the fibrous joints that connect the skull bones) have not yet closed.
You may notice that the fontanelle (white part of the baby's head) is bulging and firm. By increasing pressure on the baby's brain, he may have:
- Much irritation
- Too much sleep
- Lack of appetite
- Diversion of the eyes
But the most obvious indication of hydrocephalus is typically the rapid increase in head circumference or an unusually large head size.
In older children and adults, the symptoms are different because their skull cannot expand to accommodate the increased cerebrospinal fluid. Symptoms may include headaches followed by vomiting, nausea, blurred or double vision, downward drift of the eyes, problems with balance, poor coordination, gait disturbances, urinary incontinence, reduction or loss of developmental progress, lethargy, drowsiness, irritability, or other changes in personality or consciousness, including memory loss.
The symptoms described in this section are related to the most typical forms in which progressive hydrocephalus occurs; however, it is important to remember that symptoms vary greatly from one person to another.
Only a physician can diagnose hydrocephalus, through a clinical neurological evaluation and through cranial imaging techniques such as ultrasonography or sonography, computed tomography (CT), magnetic resonance imaging (MRI), or pressure monitoring techniques. Sometimes the problem can be detected before delivery with a fetal ultrasound, although in most cases it is detected when the baby is born.
The physician will select the appropriate diagnostic tool based on the patient's age, clinical presentation, and the presence of other known or suspected brain or spinal cord abnormalities.
Regarding the treatment of hydrocephalus, it is recommended to do it immediately, before 4 months of age to help limit or avoid brain damage. Treatment is most often done through the surgical placement of a drainage system known as a 'bypass'.
This system consists of a long, flexible tube with a valve that keeps brain fluid flowing in the right direction and at the right speed. Fluid is drained and its flow diverted from one place within the central nervous system to another area of the body (such as the abdomen or heart) where it can be absorbed as part of the circulatory process. This referral system requires regular medical monitoring and follow-up.
This entire process can suffer complications such as excessive or insufficient drainage, which can be caused by mechanical failure, infections, obstructions or the need to prolong or replace the catheter. When that happens, subsequent surgery may be necessary to replace the faulty part or the entire shunt system. When a shunt system is found to be not working properly (for example, if symptoms of hydrocephalus reappear), medical attention should be sought immediately.
A child born with hydrocephalus, even if he has received adequate treatment for the problem, will always have to be observed by the doctor. In the check-ups, the pressure in the brain will be evaluated, possible signs that the fluid has accumulated again, the symptoms that the child presents such as headaches, vision problems, problems when walking or speaking, etc. will be observed. As the child grows, it is necessary to observe problems with brain development such as delays in learning, problems in motor skills and speech difficulties.
It is necessary to be aware that hydrocephalus presents risks for both the cognitive and physical development of the child. However, many children who have been diagnosed with the disorder benefit from rehabilitative therapy and educational interventions that help them lead normal lives with few limitations. Treatment by an interdisciplinary team of medical professionals, rehabilitation specialists, and educational experts is vital to a positive outcome.
Prognosis for patients diagnosed with hydrocephalus is difficult to predictAlthough there is some correlation between the specific cause of hydrocephalus and the outcome of the condition. Treatment of patients with hydrocephalus saves and sustains the life of the patient. If left untreated, progressive hydrocephalus, with rare exceptions, is fatal.
NINDS - National Institute of Neurological Disorders and Stroke
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