HYDROCEPHALOUS

Hydrocephalous is a condition in which accumulation of cerebrospinal fluid occurs within the brain. This typically causes increase pressure inside the skull.

• Associated birth defects includes :

              neural tube defects

              aqueductal stenosis

• Other causes include:

             meningitis

             brain tumors

            Traumatic brain injuries

            Intraventricular haemorrhage

           Subarchnoid haemorrhage

Clinical presentation of hydrocephalous varies with chronicity.

• Acute dilatation of ventricular system is more likely to manifest with nonspecific sign and symptoms of increased intracranial pressure (ICP).
• Chronic dilatation ( especially in older population) may have a more insidious onset presenting.
• Symptoms of increased ICP may includes headach, vomiting, nausea, papilledema,sleepiness or coma with increase levels of CSF.
• Hearing loss due to CSF creating pressure on auditory pathways or disrupting the communication of inner ear fluid.
• Elevated ICP of different etiologies have been linked to sensorineural hearing loss (SNHL). Transient SNHL has been reported after the loss of CSF with shunt surgeries
• Hearing loss is rear but well-known sequel of procedures resulting in CSF loss.
• Elevated ICP may result uncal or tonsillar herniation, with resulting life-threatening brain stem compression.
• Hakim’s triad of gait instability, urinary incontinence, and dementia is a relatively typical manifestation of the distinct entity normal-pressure hydrocephalus.

Focal nurological deficits may also occur, such as abducences nerve palsy and vertical gaze palsy (parinaud syndrome due to compression of the quadrigeminal plate,where the neural centers coordinating the conjugated vertical eye movement are located

• The symptoms depend on the cause of the blockage,the person’s age, and how much brain tissue has been damaged by the swelling.
• In infants with hydrocephalus, CSF builds up in the central nervous system, causing the fontanelle (soft spot) to bulge and the head to be larger than expected. Early symptoms may also include:
• Eyes that appear to gaze downward
• Irritatability
• Seizures
• Separated sutures
• Sleepiness
• Vomiting
• Symptoms that may occur in older childrens can includes:
• Brief, shrill, high-pitched cry
• Changes in personality, memory, or the ability to reason to
• think
• Changes in facial appearance and eye spacing(craniofacial disproportion)
• Crossed eyes or uncontrolled eye movements.
• Difficulty sleepiness
• Excessive sleepiness
• Headches
• Irritability, poor temper control
• Loss of bladder control
• Loss of coordination and trouble walking
• Muscle spasticity
• Slow growth (child 0-5 years)
• Delayed milestones
• Failure to thrive
• Slow or restricted movements
• Vomiting

1. Congenital – foetus develop hydrocephalous in utero during fetal development. The most common cause of hydrocephalus is aqueductal stenosis. Which occurs when the narrow passage between the third and fourth ventricles in the brain is blocked or too narrow to allow sufficient cerebral spinal fluid to drain .fluid accumulates in the upper ventricles,causing hydrocephalus. Other causes:

           Neural tube defect
           arachnoid cysts

Dandy-walker syndrome

Arnold-chiari malformation

2. Acquired- the condition is acquired as a consequences of CNS infections meningitis, brain tumors, head trauma, toxoplasmosis, or intracranial hemorrhage is usually painful.

The cause of hydrocephalus is not known with certainty and is probably multifactorial. It may be caused by impaired CSF flow, reabsorption, or excessive CSF production.

• Obstruction to CSF flow hinders its free passage through the ventricular system and subarachnoid space(e.g., stenosis of the cerebral aqueduct or obstruction of the interventricular foramina secondary to tumors, hemorrhages, infections or congenital malformations) and can cause increases in ICP.
• Hydrocephalus can also be caused by overproduction of CSF (relative obstruction) (e.g., choroid plexus papilloma, villous hypertrophy).
• Bilateral uretericobstruction is a rare, but reported, cause of hydrocephalus.

Hydrocephalus can be classified into communicating and noncommunicating (obstructive). Both forms can be either congenital or acquired.

Communicating

communicating hydrocephalus, also known as nonobstructive hydrocephalus, is caused by impaired CSF reabsorption in the absence of any obstructive to CSF flow between the ventricles and subarchnoid space.

This may be due to functional impairement of the arachnoidal granulations), which are located along the superior sagittal sinus, and is the site of CSF reabsorption back into the venous system.

Non- communicating

Non communicating hydrocephalus, or obstructive hydrocephalus is caused by a CSF- flow obstruction.

• Foramen of monro obstruction may lead to dilation of one , or if large enough( e.g., in colloid cyst), both lateral ventricls.
• The aqueduct of sylvius, normally narrow,may be obstructed by a number of genetic or acquired lesions ( e.g. atresia, ependymitis, hemorrage, or tumor) and lead to dilation of both lateral ventricles, as well as the third ventricle.
• Fourth ventricle obstruction leads to dilatation of the aqueduct, as well as the lateral and third ventricles ( e.g. chairi malformation)
• The foramina of Luschkaand foramen of Magendie may be obstructed due to congenital malformation (e.g., Dandy-Walker malformation).

Normal pressure hydrocephalus
When hydrocephalus occurs in adults, CSF levels rise but the amount of pressure is usually normal. It still causes the brain to swell and can lead to impaired functioning. In adults, this condition usually results from conditions that prevent CSF from flowing. However, in some cases, there is no known cause.

You might be at higher risk if you’ve experienced any of the following:

• brain-related infections such as meningitis
• head injuries
• bleeding from a blood vessel in your brain
• brain surgery

Hydrocephalus can cause permanent brain damage, so it’s important that you recognize symptoms of this condition and seek medical attention. The condition is more common in children, but it can affect people of any age.

Early signs of hydrocephalus in infants include:

• bulging fontanel, which is the soft spot on the surface of the skull
• a rapid increase in head circumference
• eyes that are fixed downward
• seizures
• extreme fussiness
• vomiting
• excessive sleepiness
• poor feeding
• low muscle tone and strength

Symptoms or signs that affect toddlers and older children include:

• short, high-pitched cries
• personality changes
• changes in facial structure
• crossed eyes
• headaches
• muscle spasms
• delayed growth
• trouble eating
• extreme sleepiness
• irritability
• loss of coordination
• loss of bladder control
• larger than normal head
• trouble staying awake or waking up
• vomiting or nausea
• seizures

Symptoms in young and middle-aged adults include:

• chronic headaches
• loss of coordination
• difficulty walking
• bladder problems
• vision problems
• poor memory
• difficulty concentrating

If you suspect that you or your child has hydrocephalus, your doctor will perform a physical exam to look for signs and symptoms. In children, doctors check for eyes that are sunken in, slow reflexes, a bulging fontanel, and a head circumference that is larger than normal for their age.

Your doctor may also use an ultrasound to get a closer look at the brain. These tests use high-frequency sound waves to create images of the brain. This type of ultrasound can only be done in babies whose fontanel (soft spot) is still open.

Magnetic resonance imaging (MRI) scans can be used to look for signs of excess CSF. MRIs use a magnetic field and radio waves to make a cross-sectional image of the brain.

Computerized tomography (CT) scans can also help diagnose hydrocephalus in children and adults. CT scans use several different X-rays to form a cross-sectional image of the brain. These scans can show enlarged brain ventricles that result from too much CSF.

Surgical treatments
Hydrocephalus is treated with one of two surgical options:

• A shunt (tube) is surgically inserted into the brain and connected to a flexible tube placed under the skin to drain the excess fluid into either the chest cavity or the abdomen so it can be absorbed by the body. 
• Endoscopic Third Ventriculostomy (ETV) improves the flow of CSF out of the brain.   A tiny hole is made at the bottom of the third ventricle and the CSF is diverted there to relieve pressure.  Sometimes this is done in conjunction with choroid plexus cauterization to try and decrease the production of CSF.  Choroid plexus cauterization uses electric current to burn the CSF-producing tissue (i.e., the choroid plexus) in the lateral ventricles in the brain, so it produces less CSF. 

Shunt systems generally function well but they can fail to properly drain the CSF due to mechanical failure or infection.  When this happens the CSF once again begins to build up in the brain and earlier symptoms may recur.   To reduce the buildup of CSF, the clogged shunt system is replaced to restore drainage of CSF.  
Shunts require monitoring and regular medical checkups.  Multiple surgeries may be needed to repair or replace a shunt throughout a person’s lifetime.  Seek medical help immediately if symptoms develop that suggest the shunt system is not working properly.

Signs and symptoms of shunt malfunction may include:

• headache
• double vision or sensitivity to light
• nausea or vomiting
• soreness of the neck or shoulder muscles
• seizures
• redness or tenderness along the shunt tract
• low-grade fever
• sleepiness or exhaustion
• reoccurrence of hydrocephalus symptoms

Other treatment

Many people diagnosed with hydrocephalus benefit from rehabilitation therapies and educational interventions.  Treatment by an interdisciplinary team of medical professionals, rehabilitation specialists, and educational experts is critical to a positive outcome.

Supportive therapies for children may include:

• occupational and developmental therapists who can help children learn life skills and develop social behaviors
• special education teachers who can help tackle learning disabilities
• mental health providers or social workers who can provide emotional support and help families find services.

Adults may also require similar support, including social workers, occupational therapists, and specialists in dementia care.

If left untreated, hydrocephalus can be fatal.  Early diagnosis and successful treatment improve the chance for a good recovery.

With the benefits of surgery, rehabilitative therapies, and educational interventions, many people with hydrocephalus live relatively normal lives.

The symptoms of NPH 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.

Regardless of the different surgical management, children with hydrocephalus still have some disabilities. Therefore, early involvement with physiotherapists via different methods of rehabilitation is essential, whether surgical or non-surgical management is required. Additionally, successful shunting is usually related to more obvious and rapid improvements in rehabilitation efforts. Specific treatment procedures are numerous, functional training for activities of daily living; therapeutic exercise; manual techniques such as mobilization and stretching; and therapeutic modalities.

Physiotherapy goals are aimed at:

• Improving functional skills and reducing secondary impairment, such as obesity, contractures, and fractures, which could delay developmental skills. Furthermore, physiotherapists can work with children in their home and in the hospital or clinic, depending on their medical conditions and age.
• Motor control (co-ordination of muscles and limbs), learning theories, and development are factors that contribute to occurrence of motor behaviour (how the muscles and limbs react to movement, control, development, and learning). These factors include not only the central nervous system (brain and spinal cord) as the driving force, but also biomechanical (human movement principles), psychological, social, and environmental components.
• Teaching and practicing skills under these theories is task-oriented (specific to everyday movements e.g. sitting to standing), which should be intermittent and repetitive. A high level of learning occurs via a child’s problem solving instead of by the therapist's hands-on facilitation. It is also important that emphasis is placed on family centred care and treatment in natural environments.
• The mutual goal is usually to increase functional activity, which in turn, decreases disability.

These goals should be achieved by:

• Promoting physical milestones of achievement such as sitting, crawling, standing
• Optimising mobility independence
• Improving balance and coordination via exercise
• Stretching tight muscles via exercise
• Strengthening weak muscles via exercise
• Increasing quality of life and confidence
• Improving endurance and exercise tolerance