What is hydrocephalus and how is it managed?

Hydrocephalus is a condition in which cerebrospinal fluid (CSF) accumulates in the brain's ventricles, causing them to enlarge and creating pressure on the surrounding brain tissue. CSF is normally produced in the ventricles, circulates around the brain and spinal cord, and is reabsorbed. In hydrocephalus, this balance is disrupted: either too much CSF is produced, the normal pathways are blocked (obstructive hydrocephalus), or reabsorption is impaired. Untreated, the pressure from excess CSF damages brain tissue. The primary treatment for hydrocephalus in children and adults is a ventriculoperitoneal (VP) shunt: a surgically implanted device that drains excess CSF from the ventricle through a valve and tube to the abdominal cavity where it is reabsorbed. Endoscopic third ventriculostomy (ETV) is an alternative to shunting for some types of hydrocephalus, creating an opening in the floor of the third ventricle to allow CSF flow. Both treatments require ongoing monitoring.

What are the signs of shunt malfunction and why is recognising them critical?

Shunt malfunction (when the shunt stops working correctly, either through blockage, breakage, disconnection, or infection) is a medical emergency. When the shunt fails, CSF pressure builds up again, threatening brain function and life if not treated promptly. The signs of shunt malfunction vary by age. In infants: bulging fontanelle (soft spot), rapidly increasing head circumference, vomiting, irritability, downward deviation of the eyes (sunsetting sign), poor feeding, lethargy. In older children and adults: headache (often severe, worse in the morning or lying down), nausea and vomiting, blurred or double vision, drowsiness, irritability, decreased alertness, change in behaviour or school performance, seizures. In people with baseline cognitive or communication impairment: behavioural changes, regression to earlier skill levels, increased irritability, or altered responsiveness may be the only signs. All families and support workers must know the individual's specific shunt malfunction signs and the action to take: if shunt malfunction is suspected, go to the emergency department immediately. Do not wait to see if symptoms improve.

Does hydrocephalus qualify for NDIS?

Hydrocephalus can qualify for NDIS when it causes significant permanent functional impairment. Hydrocephalus that was present from birth (congenital) or acquired in childhood often causes lasting effects on cognitive function, motor skills, or both, which may qualify for NDIS. Hydrocephalus that occurs in adults from a treatable cause (such as normal pressure hydrocephalus, which is most common in older adults) and is treated successfully may not leave permanent disability. For children with congenital hydrocephalus or hydrocephalus secondary to conditions like spina bifida, the functional impairment is typically significant and NDIS access is straightforward. A detailed assessment by an OT, neuropsychologist, or paediatrician documenting the functional impacts is needed for the NDIS access request. For adults with hydrocephalus acquired before 65, NDIS is the pathway; for older adults, My Aged Care applies.

What cognitive effects does hydrocephalus commonly cause?

The cognitive effects of hydrocephalus depend on the cause, the timing of diagnosis and treatment, the duration and severity of raised intracranial pressure, and whether there are other brain conditions present (as in hydrocephalus associated with spina bifida or prematurity). Common cognitive effects include: processing speed difficulties, with tasks taking longer to complete than for peers; working memory difficulties, affecting the ability to hold and manipulate information; attention and concentration difficulties; difficulties with planning and sequencing tasks (executive function); visual-spatial processing difficulties (common in hydrocephalus, particularly when the posterior brain regions are affected); and learning difficulties in reading, writing, and mathematics. The profile varies significantly between individuals; neuropsychological assessment provides the most detailed and accurate picture of an individual's cognitive strengths and difficulties. Some people with treated hydrocephalus have no lasting cognitive effects; others have significant and lifelong cognitive support needs.

What school adjustments help children with hydrocephalus?

School adjustments for children with hydrocephalus should be based on a neuropsychological assessment documenting specific cognitive strengths and difficulties. Common adjustments include: extended time for assessments and tasks (processing speed difficulties mean tasks take longer, not that the child cannot do them); reduced quantity of work required (rather than extended time in some cases, doing fewer items of the same task); access to a scribe or keyboard for written work if written output is disproportionately difficult; preferential seating and reduced distractions for attention difficulties; breakdown of multi-step instructions into single steps with visual supports; regular check-ins from the teacher to ensure the child has understood; and teacher education about the specific cognitive profile. For children with physical effects of hydrocephalus (from associated conditions such as spina bifida), additional physical and mobility adjustments are also needed. A Student Support Plan or Individual Education Plan documents these adjustments formally.

What is the relationship between hydrocephalus and spina bifida?

Hydrocephalus is strongly associated with spina bifida myelomeningocele (the most severe form of spina bifida): approximately 80 to 90% of children with myelomeningocele develop hydrocephalus, typically requiring shunt treatment. The two conditions often co-exist as part of a complex involving Chiari II malformation (a structural brain abnormality also associated with spina bifida). The combined effects of spina bifida, hydrocephalus, and Chiari II create a complex neurological and orthopaedic profile including: mobility impairment from the spina bifida-related paralysis; continence difficulties (bladder and bowel); the cognitive effects of hydrocephalus; and the potential for shunt-related complications across a lifetime. Families managing this combination need a coordinated team spanning neurosurgery, urology, orthopaedics, physiotherapy, OT, speech pathology (if relevant), and neuropsychology, coordinated by a support coordinator and informed by their GP.

What is normal pressure hydrocephalus and how is it different from childhood hydrocephalus?

Normal pressure hydrocephalus (NPH) is a form of hydrocephalus that occurs primarily in adults over 60. Unlike the raised intracranial pressure seen in acute hydrocephalus, NPH is characterised by enlarged ventricles with CSF pressure at the upper limit of normal. The classic triad of symptoms is gait disturbance (slow, shuffling, unsteady walking), urinary incontinence, and cognitive impairment (particularly slowed thinking and memory difficulties). NPH is potentially reversible with treatment (CSF drainage via shunt or serial lumbar punctures); improvement in gait and cognition after treatment confirms the diagnosis. NPH is sometimes confused with Parkinson's disease or dementia because the gait and cognitive features overlap. The distinction matters because NPH can be treated. A geriatrician or neurologist investigates NPH through brain imaging (enlarged ventricles on MRI or CT), clinical assessment, and often a lumbar puncture trial. Support needs for NPH are similar to those for other causes of gait impairment and cognitive change in older adults.

How do support workers assist people with hydrocephalus safely?

Support workers working with people with hydrocephalus have two primary responsibilities beyond their general support role. First, shunt malfunction recognition: all support workers must be trained in the specific signs of shunt malfunction for the individual they support and must understand that if any of those signs appear, they take the person to the emergency department immediately without waiting. This is a non-negotiable emergency protocol. Second, cognitive support: many people with hydrocephalus have processing speed, memory, or executive function difficulties that affect how they receive and act on information. Support workers should: give instructions one step at a time; allow additional processing time before expecting a response or action; use visual reminders and schedules; not interpret slow response as unwillingness; and maintain routine and predictability which reduces the cognitive load of daily life.

Hydrocephalus Family Guide: Monitoring Function Changes and Coordinating Community Supports

Key Points

Shunt malfunction is a medical emergency; all family members and support workers must know the individual’s specific warning signs and the immediate action is to go to the emergency department, not wait
Hydrocephalus causes a variable profile of cognitive effects; neuropsychological assessment is the most accurate way to identify the specific profile and inform school adjustments and daily support strategies
NDIS access is typically straightforward for children with congenital hydrocephalus or hydrocephalus associated with spina bifida; supporting documentation from an OT, neuropsychologist, or paediatrician is needed
Approximately 80 to 90% of children with spina bifida myelomeningocele develop hydrocephalus; the combined management is complex and requires coordinated care across multiple specialists
Normal pressure hydrocephalus in adults over 60 is potentially reversible with treatment; the classic triad is gait disturbance, urinary incontinence, and cognitive impairment; it should not be assumed to be Parkinson’s disease or dementia without investigation
Support workers must prioritise two things: emergency shunt malfunction recognition, and cognitive support strategies that accommodate processing speed and memory difficulties

Hydrocephalus: Community Life and Coordinated Support

Hydrocephalus is a condition that affects people across the entire lifespan, from premature infants who develop post-haemorrhagic hydrocephalus to adults over 60 with normal pressure hydrocephalus. The functional impact ranges from none (successfully treated with no lasting effects) to significant and lifelong cognitive, motor, and independence effects depending on the cause, timing, and individual factors.

For families living with hydrocephalus, the ongoing monitoring and emergency vigilance required by shunt management is a constant backdrop to daily life. On top of this, many people with hydrocephalus have functional impairments requiring support in school, work, and community life. Coordinating these supports across the right providers requires understanding the condition, knowing the funding pathways, and building a team that communicates.

Living with a Shunt

How the Shunt Works

A ventriculoperitoneal (VP) shunt consists of:

A ventricular catheter: placed in the brain’s lateral ventricle
A valve: regulates the pressure at which CSF drains
A peritoneal catheter: carries the CSF to the abdominal cavity

Modern shunts use programmable valves that can be adjusted non-invasively using an external magnet to change the pressure setting. This allows the neurosurgeon to adjust the shunt without surgery in response to changing needs.

Shunt Monitoring: Routine and Emergency

Routine monitoring:

Regular neurosurgery review (at least annually for children; variable for adults)
Imaging (MRI or CT) to monitor ventricle size and shunt position
GP awareness and management of general health (fever, which can be related to shunt infection, should always be taken seriously)
Annual review of cognitive and functional status, ideally with neuropsychological input

Emergency monitoring: The most important skill for any person living with hydrocephalus and their family is recognising the signs of shunt malfunction. Every family should have:

A written list of the individual’s specific shunt malfunction signs (these vary between individuals)
Clear instructions: emergency department immediately, not a GP appointment, not waiting overnight
A contact number for the neurosurgery team for clinical queries when symptoms are ambiguous

Shunt Infection

Shunt infection (typically from Staphylococcus epidermidis or other skin organisms) is a serious complication requiring neurosurgical management. Signs include fever, redness or swelling along the shunt tract, meningism (stiff neck, sensitivity to light), or any of the shunt malfunction signs. Shunt infection requires removal of the infected shunt and systemic antibiotics followed by reinsertion of a new shunt.

Cognitive Effects and Daily Support

The cognitive effects of hydrocephalus are highly variable and individual-specific. Common areas of difficulty:

Processing Speed

Slowed processing speed is one of the most consistent cognitive effects. The person understands; they simply take longer to process information and formulate responses. Implications:

Conversations feel slower; the person may need more time to respond than is socially expected
Written work takes longer; tasks in school or work that have time limits are disproportionately difficult
Support workers and family should allow additional time without rushing or completing sentences

Working Memory

Working memory affects the ability to hold information in mind while doing something with it. Implications:

Following multi-step instructions without writing them down is difficult
Remembering what was just said while doing another task is difficult
Single-step instructions with written or visual backup work better

Executive Function

Planning, initiating, organising, and shifting between tasks can all be affected. Implications:

Transitioning between activities requires preparation and support
Self-directed multi-step tasks (getting ready in the morning, planning a study schedule) may need external scaffolding
Visual schedules and checklists support independent task completion

Visual-Spatial Processing

Many people with hydrocephalus have visual-spatial processing difficulties (not related to vision acuity but to how the brain processes visual-spatial information). This affects: reading maps; understanding spatial relationships; some mathematics; handwriting and page organisation.

School: Getting the Adjustments Right

Neuropsychological Assessment First

Before developing school adjustments, a neuropsychological assessment is the most valuable investment. The neuropsychologist provides:

Detailed cognitive profile across all relevant domains
Identification of cognitive strengths (which are often considerable) alongside areas of difficulty
Specific, evidence-based recommendations for school
A formal report that supports NDIS access and school adjustment requests

The Student Support Plan

Request a formal Student Support Plan (IEP) based on the neuropsychological recommendations. Key adjustments to include:

Extended time: For assessments and written work; the amount of extension (25%, 50%, or unlimited) should be based on the neuropsychological assessment
Keyboard access: If written output is disproportionately difficult relative to verbal ability
Scribe provision: For examinations if written output is severely affected
Instruction modifications: Multi-step instructions broken into single steps; written as well as verbal instructions
Reduced distraction environment: For children with significant attention difficulties
Regular check-ins: Teacher or aide checking that the child has understood and is on track

NAPLAN and Senior Examination Adjustments

Australia’s standardised testing systems (NAPLAN, state senior examinations) have processes for applying for adjustments based on documented disability. These require the neuropsychological assessment report and supporting documentation. Apply well in advance of the examination period.

Hydrocephalus Associated with Spina Bifida

For children with myelomeningocele spina bifida, hydrocephalus is part of a much larger clinical picture. The combined management team for spina bifida with hydrocephalus includes:

Neurosurgeon (shunt management)
Spina bifida coordinator (team coordination)
Urologist (bladder management: clean intermittent catheterisation is standard for bladder paralysis)
Orthopaedic surgeon (scoliosis, hip, and foot management)
Physiotherapist (mobility, strength, orthotics)
OT (daily living, equipment, school)
Neuropsychologist (cognitive assessment)
Dietitian (bowel management and nutrition)

The spina bifida clinic (available at children’s hospitals in most states) coordinates this team for children. Adults with spina bifida and hydrocephalus often find the transition to adult services fragmented; a support coordinator helps maintain continuity.

The Provider Team

Provider	Role	Funding
Neurosurgeon	Shunt management, monitoring, revisions	Medicare
Neurologist / paediatrician	Neurological monitoring, referrals	Medicare
Neuropsychologist	Cognitive assessment, school recommendations	NDIS, private
OT	Daily living, equipment, home assessment, school consultation	NDIS, Medicare CDM
Physiotherapist	Mobility, motor skills (if motor impairment present)	NDIS, Medicare CDM
Speech pathologist	Communication, if affected	NDIS, Medicare CDM
Support coordinator	NDIS plan management, team coordination	NDIS Capacity Building
Support workers	Personal care, community access, cognitive support	NDIS Core

Key Resources

Hydrocephalus Australia - peer support, information, and advocacy
Spina Bifida and Hydrocephalus Association of Queensland - state-based support for people with both conditions
NDIS - funding access and plan information
Australian Spinal Muscular Atrophy Association - where relevant for differential diagnosis discussions

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Andre Smith All articles

Co-founder & CEO

Andre is the co-founder and CEO of Carevo. He holds a Bachelor of Commerce, majoring in Marketing, and a Bachelor of Arts from UNSW Sydney, where his majors were International Relations, Politics, Information Systems, and Media and Communications, graduating in 2014, and went through the UNSW 10x Founders accelerator in 2023.

Hydrocephalus Family Guide: Monitoring Function Changes and Coordinating Community Supports

Key Points

Hydrocephalus: Community Life and Coordinated Support