Hydrocephalus

Ever came across the word “Hydrocephalus”? Well, even if you have not, you can guess it has the literal meaning of water and brain. Getting to the point?

In simple scientific terms, it is explained as a medical condition where the baby has either acquired or been born with the state of abnormal accumulation of excessive fluid particularly cerebrospinal fluid (CSF) inside the deep ventricles or cavities of the brain. Consequently, leading to the augmented skull pressure inside the skull, ventricles tend to broaden erratically.

Too much fluid pressure damages the brain’s functions and can result in even death if left untreated. Regrettably, it is an incurable disease but science has advanced enough to offer beneficial treatments. After treatment, the patient can experience a wide array of physical incapacities.
Now, you must be thinking HOW and WHY!
As far as WHY is concerned, generically it happens when an imbalance is caused between the CSF production and the amount of CSF fluid being absorbed into the blood.

What are its types?

Communicating OR Nonobstructive Hydrocephalus – Abnormal cerebrospinal fluid reabsorption without any visible ventricular or subarachnoid space obstruction.
Non-Communicating OR Obstructive Hydrocephalus – Abnormally low cerebrospinal fluid absorption due to flow obstruction in ventricles or subarachnoid space.
Ex Vacuo – One of the rarest types that shows the signs of cerebral ventricular enlargement along with subarachnoid spaces. The usual causes include brain atrophy, post-traumatic brain injuries, and a few psychological disorders.
And, remember!! It is not caused due to higher CSF pressure.
Normal Pressure OR Chronic Communicating Hydrocephalus – Visible expanded cerebral ventricles accompanied with the occasionally raised CSF pressure.

What could be the possible reasons?

Now, you must have gotten an idea that it all happens due to the unnecessarily and fatal increased CSF. But, WHY it increases or HOW it is not properly produced and absorbed?The reasons for the excessive CSF in the brain’s ventricles can be:

• Partial obstruction
• PoorCSF absorption
• CSF overproduction

 Congenital Hydrocephalus

• Aqueductal Stenosis (most common)
• Neural-tube defects
• Arachnoid cysts
• Dandy-Walker syndrome
• Arnold–Chiari malformation

Apart from the above-mentioned diseases, the reasons could be:

• Hereditary abnormalities blocking the cerebrospinal fluid flow
• Developmental disorders related to the brain, spine, or spinal cord
• Premature birth
• Bleeding within the ventricles
• Infections during pregnancy particularly rubella

 Acquired Hydrocephalus

• CNS infections
• Meningitis
• Brain tumors
• Head trauma
• Toxoplasmosis
• Painful intracranial hemorrhages

 Clinical manifestations

Typically, if we talk about its signs and symptoms, they are totally dependent on the patient’s age, type, or cause of hydrocephalus and severity. Depending on the age, if the baby is born with hydrocephalus, he or she will show unnecessary rapid head growth/expansion. Apart from the growth, the patient will experience vomiting or nausea, drowsiness, seizures, and will have eyes pointing downwards. In older children, young adults, and middle-aged adults, the characteristic manifestations are headache, vision problems, body balance and gait issues with poor coordination, and the slowest developmental growth. In certain severe cases, the loss of bladder control, urinary incontinence, anger issues, irritability, bizarre personality changes, and low cognition including dementia can be observed.

Well, the “Normal pressure hydrocephalus” is featured with a distinctive symptom triad including:

1. Dementia
2. Apraxic gait
3. Urinary incontinence

 Points to Ponder

• Learning disabilities and short-term memory loss are common.
• Children with hydrocephalus since birth will have the tendency to exhibit long-term complications regarding speech and language.
• At peak pressure, the person will bear a significant hearing loss.

 Medical Diagnosis

 Neurological examination
As the problem is inside brain cavities and related to the flow of the fluid, the best way to diagnose this medical condition is through a detailed clinical neurological examination performed by a skillful neurologist with the application of advanced and accurate brain imaging techniques. The major areas that are studied are:

• Muscular strength along with reflexes
• Body and movement coordination
• Body and gait balance
• Extensive eye check-up to observe their movements and vision accuracy
• Auditory evaluation
• Mental functioning affecting personality and mood

 Brain imaging 

• Performed from 15-35 weeks of gestation, ultrasound can help diagnose the condition before the baby is born.
• Perform a painless fetal MRI as it will provide you thorough brain imageries to confirm the diagnosis. It will show if the ventricles have significantly enlarged, the CSF has obstructed flow or brain tissue has inflammation.
• Computed tomography
• Spinal tap OR lumbar puncture is performed by injecting a needle in the lower back. It is specified to estimate CSF pressure level while analyzing the fluid flow.
• Intracranial pressure monitoring is a necessity for measuring the pressure. Another benefit is its ability to detect the severity of brain swelling.
• The fundoscopic examination is comparatively expensive, critical, and difficult to perform requiring a special devicefor examining the optic nerve. If you see a swelling it means ICP is elevated.

 Treatment options

• Medication – Antibiotics if it involves infection as it will clear the infection and if not, then the end treatment must be the surgery.
• Surgery – If the blockage needs to be surgically removed or fluid should be drained (temporary hydrocephalus). Majorly recommended in case of resolving the underlying syndrome.
• Wait & see approach – Only an option if hydrocephalus is not showing any fatal symptoms. Without feeling the need for a specific treatment, only careful monitoring is recommended.

Short-term and long-term treatments

If you are looking for a short-term escape, EVD (external ventricular drain) is found enough to provide satisfactory relief.

For long-term treatment, the use of a cerebral shunt is advised. It is performed by placing a ventricular catheter to normalize the flow by removing the possible obstruction inside the cerebral ventricles. It works for the malfunctioning arachnoid granulations, as well. The main purpose of the shunt is to drain the accumulated and excessive fluid to support the resorption of the CSF.

The total sessions of 2 to 5 are required for the shunt treatment over the life span as it demands constant maintenance.

Complications 

But it can result in certain complications. The most prominent ones are over drainage or under drainage of CSF, mechanical failure, obstruction, or catheter infection. 

Other supportive treatments 

• Rehabilitation therapies
• Educational interventions
• Occupational and developmental therapies to develop life skills
• Mental health providers and social workers for counseling and emotional support to improve social behaviors
• Special education to tackle learning incapacities