After a diagnosis of hydrocephalus, the first thing to do is to get a clear picture of what it means so that you can take the next steps with your child in a calm and realistic manner.
The good news is that in most cases your baby will develop normally, even with treated hydrocephalus.
And even if not, or only to a limited extent, together with good medical and therapeutic care, you and your child will certainly make the best of it.
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Definition: This Is What Is Meant By Hydrocephalus
One in about 2000 babies is born with hydrocephalus. This rare disorder of the fluid balance in the brain is usually not quite as threatening as it sounds. Provided it is treated early.
In order for you to be knowledgeable and prepared and to be able to take action yourself in time, you will learn everything important here about understanding the disease, about the various triggers and manifestations as well as about the symptoms.
You will also find helpful information about treatment options, risks, and possible late effects in this overview, which will give you initial information about the “pressure in the head”. The term hydrocephalus is the exact translation of the scientific name hydrocephalus (also hydrocephalus) from the Greek.
Other terms for it are cerebrospinal fluid, cerebrospinal fluid congestion or cerebrospinal fluid outflow disorder. They all describe a malfunction in the fluid-filled cavities in the brain: these four ventricles are abnormally enlarged.
Sometimes the outer slit spaces are also affected.
Pressure In The Head: What Exactly Happens
The causes of hydrocephalus can vary widely, but there is always an imbalance between the formation and reabsorption of cerebrospinal fluid.
You can imagine it as follows: To supply the brain cells, there are no blood vessels in the brain, but a special fluid, the cerebrospinal fluid or CSF for short. Incidentally, it also protects against damage caused by concussions.
It is extracted from the blood as a clear ultrafiltrate in a special plexus (choroid plexus) at the blood-brain barrier. It looks like water. About 500 to 700 milliliters of cerebrospinal fluid are thus produced daily in adults.
The fluid surrounds the external and all internal brain surfaces, fills the ventricles, and circulates freely between various passageways and channels. It flows because, at several points in the brain and spinal cord, cerebrospinal fluid is continuously removed from the brain space and returns to the blood, for disposal, so to speak.
In total, 150 milliliters of fluid flow around and flush the brain in constant circulation. Even small imbalances between production and recirculation, however, can cause pressure fluctuations.
Excessive overpressure or underpressure in the long term has an unfavorable effect on brain function. In hydrocephalus, positive pressure usually predominates: congestion has occurred, either due to an outflow disorder or, more rarely, due to too much cerebrospinal fluid.
The cerebral ventricles, or ventricles, are oversized, bulging, and exerting pressure on brain tissue. And even when there is no pressure in the head, the CSF takes up space that a healthy brain needs.
When Does Hydrocephalus Develop?
Anyone can develop hydrocephalus at any age or stage of development. This is because pressure in the head has many different possible triggers and types.
Certain forms form in the womb, some right after birth, or somewhat later due to acquired causes as an infant or toddler. Others are typical for people aged 60 and older.
In short, no one is immune. At this point, we will focus primarily on hydrocephalus in children.
What Types Of Hydrocephalus Exist And What Causes It
Very different causes also cause different types of hydrocephalus. First, we distinguish between congenital (primary) and acquired (secondary) hydrocephalus.
Maldevelopments already in the fetus can also be genetically caused; current research is investigating the mechanisms.
In addition, a purely descriptive distinction can be made as to whether only the internal ventricles are affected, only the external CSF spaces, or both. Finally, physicians classify the disease according to underlying cause.
An Overview And Explanation Of The Technical Terms:
Primary (Congenital) Triggers
Null gene mutations, various malformations during brain development, very rare bony malformations, or hereditary autosomal recessive hydrocephalus.
Secondary (Acquired) Triggers
Surgery, trauma, tumors, cerebral hemorrhage, infections such as toxoplasmosis, meningitis, encephalitis, cerebrospinal fluid overproduction due to plexus papilloma (benign tumor of the choroid plexus), thrombosis (blood clots), rhesus incompatibility, and cysts.
Internal cerebrospinal fluid congestion in the ventricles.
External CSF congestion in the outer CSF space between the meninges.
A combination of H. internus and externus.
Internal channels or drains are blocked, i.e., obstructed or narrowed, caused by tumors, inflammation or hemorrhage. With 60 percent the most frequent form.
CSF reabsorption is disturbed, usually caused by adhesions following infection or bleeding in the outer CSF space. Second most common form (30 percent).
Increased CSF production due to brain inflammation or poisoning with carbon monoxide or mercury (10 percent).
Hydrocephalus (EX) Vacuo
Underlying the ventricular dilations is decreased brain mass. Either by embryonic underdevelopment or in old age by shrinkage of the brain mass.
Normal Pressure Hydrocephalus
There are two subtypes here:
- Idiopathic, also called old-age hydrocephalus, has as yet unknown causes for the ventricular dilatations and can apparently be a late consequence of infantile hydrocephalus.
- Secondary is the result of craniocerebral trauma, meningitis, or hemorrhage. Common to both is that only short-term elevated intracranial pressure is measurable and typically these three symptoms occur: Gait disturbance, dementia, urinary incontinence (uncontrollable urge to urinate).
Symptoms: How Hydrocephalus Manifests In Children
Fetuses, newborns, and infants show different signs of the disease than young children, schoolchildren or even adults. This is mainly due to the fact that the skull is not yet fully developed and is highly deformable.
On the one hand, this is fortunate for the still growing and maturing brain, because the skull simply yields to the pressure in the head and initially relieves the sensitive structures.
Typical Symptoms In Infants
- disproportionately rapid head growth, severe “cranial inflation”.
- Fontanelle may bulge.
- Sunset phenomenon. This refers to a peculiar eye position: the gaze is always directed downward and the pupil and iris look like a setting sun on the horizon of the lower eyelid against the upper white of the eye.
- Drinking weakness.
- Developmental delays.
After ossification and cranial closure, other symptoms come to the fore from about one year of age, especially the so-called.
Cranial Pressure Signs:
- Nausea with fasting vomiting.
Other Signs Of The Disease:
- Visual disturbances (double vision).
- Respiratory failure.
- Hair growth disorders.
- Gait disorders.
- Fatigue, lethargy and dozing off.
- Disturbances of consciousness up to coma.
- Brainstem entrapment: life-threatening displacement of brain matter toward the occipital hole, with disruption of circulatory and respiratory control.
How Hydrocephalus Is Diagnosed
Imaging techniques are key to detecting hydrocephalus. The most common, because it is the least stressful, is sonography, or ultrasound.
Early hydrocephalus can often be diagnosed during a screening examination during pregnancy (prenatal diagnosis). In newborns or infants, ultrasound is also the best method for diagnosing hydrocephalus, because the fontanelle allows a good “view” of the brain structures.
Later or in addition, an MRI (magnetic resonance imaging) or a CT (computer tomography) may be necessary for reliable clarification and identification of the cause. In addition, a pressure measurement may also provide important information.
Or a puncture of the spinal cord (lumbar puncture): if the leakage of cerebrospinal fluid leads to immediate relief, hydrocephalus is most likely present.
Why The Pressure In The Head Must Be Treated
If you observe or even suspect any of the above noticeable signs, have your pediatrician take a look! The probability that hydrocephalus will spontaneously regress is zero.
On the contrary, it will continue to progress and the pressure in the head will increase more and more. The earlier hydrocephalus is detected, the better the treatment success and the chances of normal development.
It is important that the right therapy is given at the right time. The stretching of the skull is reversible for a certain time and your child may be able to compensate for the consequences of the intracranial pressure.
If not acted upon, the risk of disability and even fatality is high. Life-threatening brainstem entrapment with cessation of circulation and breathing may occur.
This Is How Hydrocephalus Is Treated
The main goal is to equalize the pressure in the head as quickly as possible. Short-term medication (diuretics) can reduce fluid retention, but only surgical repair of the imbalances in the brain offers a permanent solution.
The silver bullet is when an anatomical cause can be found and removed. A tumor, for example, a cyst, or a canal obstruction.
If only the outflow tract of a cerebral ventricle is blocked (aqueductal stenosis of the third ventricle), the neurosurgeon will drill a small opening in the floor of the ventricle, called an endoscopic ventriculostomy (ETV).
In this way, he creates a drain into the lower cisterns. Surgery on the fetus is also possible, although rare. However, the most common procedure is to implant a cerebrospinal fluid drain, or shunt.
This is a thin tube system usually made of plastic and silicone with a regulating pressure valve. Through a small drilled hole in the skull, the neurosurgeon inserts one of the tube ends (ventricular catheter) into a brain chamber.
From there, he passes a silicone tube under the skin behind the ear (where the valve is located) and along the neck to the abdomen. Here, a catheter of the shunt ends in the peritoneum (ventriculo-peritoneal shunt, VP shunt).
In this way, the CSF can drain into the abdomen, where it is easily reabsorbed. It is also possible to drain it into the ventricle (VA shunt). You can hardly see the tube under the skin and it does not disturb your baby.
Is Hydrocephalus Curable?
In cases with neurosurgically correctable cause yes, but in many cases not. To date, there are types of hydrocephalus in children that cannot be definitively cured.
Specialty clinics such as the one in Leipzig are able to restore normal CSF circulation in certain childhood hydrocephalus forms through minimally invasive endoscopic neurosurgery (MIEN).
Many other children with hydrocephalus require therapy throughout their lives to prevent pressure in the head. However, initiated in time, normal life is possible for very many affected individuals with shunt.
In the case of hydrocephalus in the newborn and infant, timely action is most important. Although the pressure in the head is usually not dangerous at first, once ossification of the fontanel and closure of the cerebral sutures begins, there is a threat of a permanent oversized skull.
This is the other side of its initial compliance. With increasing intracranial pressure in the now more rigid skull capsule, the risk of permanent maldevelopment and damage to brain structures increases.
The latter then also applies to hydrocephalus in the older child and adult.
Signs of brain damage become noticeable through disturbances or delayed further development of:
- Motor skills: turning, crawling, sitting, walking.
Much of this can be recovered through early intervention. The vast majority of children continue to develop normally.
Nevertheless, a doctor can never predict with certainty whether permanent brain damage has already occurred. The clinical picture is too varied and complex for that. Especially with congenital forms a prognosis is difficult, often mental disabilities remain.
What Life With Hydrocephalus Looks Like
If the hydrocephalus could not be surgically repaired, your child will henceforth live with a shunt that keeps the pressure in the head under control. The shunt is monitored closely and replaced with new ones as the child grows.
When the child is fully grown, the shunt can remain implanted for many years until a replacement is due. But infections and other side effects cannot be ruled out.
If there are neurological consequences and developmental disorders, you and your child will be accompanied therapeutically: In a rehab facility and also afterward, it will be specifically promoted and supported to catch up or limit.
And very many adults would never notice the disease due to “pressure in the head”.
Are There Risk Factors?
Yes, some conditions favor hydrocephalus in children. One of these is premature birth. Premature babies are at higher risk for brain hemorrhages due to their particular vulnerability, immaturity and susceptibility to stress. These can trigger hydrocephalus.
Babies with Spina bifida (open back), i.e. a malformation of the spinal cord and spinal column, also develop hydrocephalus with a frequency of 80 percent due to disturbed cerebrospinal fluid resorption.
Frequently associated with hydrocephalus are also some congenital anomalies, such as the hereditary disease monosomy 4p (Wolf-Hirschhorn syndrome) or certain brain malformations: the Arnold-Chiari malformation and the Dandy-Walker syndrome.