The World of Pregnancy

Congenital Hydrocephalus: Causes, Symptoms & Treatment!

Congenital hydrocephalus represents the excess accumulation of cephalosporidic fluid which can be found at birth in our brain.

Congenital Hydrocephalus

The excess of fluid leads to an increased intracranial pressure in the child, which in turn leads to brain damage and loss of physical and mental abilities.

The prompt diagnosis and treatment may prevent a lot of these issues, allowing the child to develop normally. However, there may be minor issues, such as learning difficulties.

Causes

Congenital hydrocephalus is caused by an imbalance in the cerebrospinal fluid production (CSF) as well as the ability of the organism to distribute and absorb this fluid. Typically, CSF circulates at the level of brain compartments known as ventricles, then surrounds the brain and spinal cord offering nutrition and protection.

Signs & Symptoms

The symptoms of this birth defect occur either at birth or during the first 9 months of life. The first obvious symptom is macrocephaly, meaning that the child`s head is larger than normal. Children who experience this congenital abnormality may have other signs, like:

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  • Sleepiness;
  • Blurred/double vision;
  • Feeling ill;
  • Changes in the mental state ()confusion);
  • Difficulty walking;
  • Not being able to control the bladder;
  • Neck pain;

Investigations

This congenital malformation can sometimes be found before birth by fetal ultrasound. Still, most cases of congenital hydrocephalus are found after birth. This birth defect is suspected in babies who have large heads, but there are also other mental or physical changes which might occur.

Treatment

Treatment for this congenital abnormality is intended to decrease the amount of CSF in the brain that results in decreased intracranial pressure. The earlier is the treatment started, the bigger are the chances of preventing any possible brain injuries, since most children who experience this birth defect are more likely to the risk of developing various disabilities, like memory or visual disorders. Still, if this medical condition is found and treated rapidly, the children will be able to live a normal life. The effects of this abnormality on long term depend on the cause, the treatment`s response and the severity of the condition.

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Initial Treatment

Immediately after a newborn gets diagnosed with this birth defect, in the first 48 hours he`ll be surgically placed in the brain a shunt which will facilitate the drainage of excess CSF. In general, it starts at the cerebral ventricle level, it passes under the scalp skin after the ear, then descends along the throat and generally ends in the abdomen where the excess CSF is absorbed. Removing excess cerebrospinal fluid may reduce intracranial pressure, which results in the prevention of brain damage.

On occasion, urgent action may be needed to decrease intracerebral tension before implanting the shunt.

Maintenance Treatment

Maintenance treatment of hydrocephalus is generally represented by a requirement for strict supervision by experts such as neurosurgeons, pediatricians, neurologists or family doctors. During period investigations, the doctor will measure the cranial perimeter of the child, assess the medical condition and neurological development. It may also be possible for the physician to recommend a computer tomography (CT) or period nuclear magnetic resonance to be sure that CSF drainage is done properly. Parents need to be careful to symptoms of obstruction or infection of the shunt, as excessive CSF may build up and lead to serious brain damage.

Treatment if the Illness Worsens

Children who were treated early for hydrocephalus generally don`t develop long-term disability or brain damage. However, there`s no permanent treatment for this medical condition. Cerebrospinal fluid may accumulate in the brain that leads to signs like poor functioning of the shunt or infection.

Outpatient Treatment

The home treatment of the child with this medical condition consists of monitoring the illness by the entourage for the early observation of the signs that indicate the intracranial hypertension.

Advice for Parents

Being the parent of a child with this condition is pretty demanding. Although most kids are treated quite early before any cerebral lesions may become permanent, uncertainty about the possibility of recurrence of signs of intracranial hypertension is rather stressful. Sometimes this birth defect produces brain lesions that are permanent and which in turn lead to mental and physical disabilities. That is why the parents of such a kid are recommended to take care of their own person so that they can be able to take care of the child.

  • Parents should measure the cranial perimeter from time to time and be careful to any signs of intracranial hypertension like drowsiness, frequent vomiting, irritability, visual disturbance, cerebral palsy or decreased appetite. The occurrence of these signs indicates its poor functioning.
  • Parents need to pay attention to any signs which happen after the first childhood like neck pain, urinary incontinence, confusional or behavioral disorders, cramps or motor issues.
  • Infection of the shunt produces fever, among the signs described above.

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The doctor should be contacted as soon as the above signs are noticed.

  • Around 40% from shunts will fail in the first 2 years. The poor functioning of these causes leads to excessive accumulation of CSF and signs like decreased appetite, cerebral palsy, drowsiness, irritability and visual disturbance. After first childhood, there`re other signs that may occur like motor issues, urinary incontinence, neck pain or cramps.
  • There same signs occur if the shunt gets infected, along with fever. The chances for the shunt to get infected are of 3% to 15%, and this happens in the first 3 months from implantation.
  • The cause of infection or poor functioning of the shunt needs to be diagnosed in order to be treated. For instance, a lumbar puncture may show an infection and spot the bacterium that is involved, which allow the doctor to prescribe the most efficient medication.

Also, CSF might accumulate in an excessive manner in the brain after an unsuccessful vetriculostomy as well.  If this may occur, it will be required the implantation of a shunt or another ventriculostomy.

If the accumulation of CSF consist an emergency, then it will be drained by using a procedure that involves penetrating the scalp until the patient gets stabilized enough to withstand a surgical intervention. – Read this!

Permanent brain lesions because of this congenital abnormality need specific treatment for this issue. For instance, the treatment will concentrate on a few problems, like motor skills or speech development.

For non-communicable (congenital) congenital hydrocephalus, it`s generally performed a procedure known as endoscopic ventriculostomy. In infants, this specific medical procedure isn`t recommended, but might be performed at a later age. For ventriculostomy, the doctor will insert an endoscope through a very small orifice in the attempt of removing ventricular obstruction by simply letting the CSF flow freely and then absorb without implanting a shunt. It was seen as a definitive solution for this birth defect, but it has been noticed in time that this solution can actually fail. Thus, it`s crucial to monitor the patients for an early discovery of intracranial hypertension.

Depending on the treatment type, the family of the child must be alert to any symptom of brain damage, like loss of mental or physical abilities or learning difficulties. Learning difficulties or problems will be treated depending on their own specificity (for instance, the speaking difficulty will be treated through speech therapy).

The doctor will advise the parents if the child has to receive any special care or some restrictions involving his lifestyle. If issues may arise from his brain injury, then the parents of the child will be referred to a specialized staff.

Image courtesy of myhealth.alberta.ca
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