Hydrocephalus

Disease/Disorder

Definition

Hydrocephalus is an increase in total cerebrospinal fluid (CSF) volume or a nonphysiologic distribution of CSF within the brain. Hydrocephalus can be classified as communicating (non-obstructive), noncommunicating (obstructive), hypersecretory, or normal pressure.^1-4

Etiology

Hydrocephalus can occur when there is

• Increased production of CSF. This is rare and occurs typically in the setting of choroid plexus tumor and is more common in children.⁵
• Obstruction of the ventricular system secondary to aqueduct stenosis or mass lesion such as tumor. This is the most common type in congenital hydrocephalus.³
• Inadequate reabsorption of CSF. Reabsorption of CSF by the arachnoid granulations is blocked by blood products (e.g., subarachnoid hemorrhage [SAH]) or infectious material (e.g., meningitis). This is the most common type of post-traumatic hydrocephalus.^1,3

Ex vacuo hydrocephalus occurs when the ventricular space is enlarged due to loss of brain parenchyma, typically after trauma or stroke. This is a descriptive term and does not imply the patient has clinical findings of hydrocephalus. Ventricular enlargement alone does not establish a diagnosis of hydrocephalus and must be interpreted in the context of CSF dynamics and the patient’s clinical presentation.^3,13 Table 1 summarizes the major types of ventricular enlargement, highlighting their underlying pathophysiology, clinical presentation, and distinguishing features.

Table 1. Comparison of the Major Types of Hydrocephalus and Ventricular Enlargement

Adapted from Refs. 3, 6, and 13.

Epidemiology including risk factors and primary prevention

The overall global prevalence of hydrocephalus across all age groups is about 85 per 100,000 individuals.⁶

Hydrocephalus is most common in infancy due to congenital malformation and intraventricular hemorrhage in premature infants. The estimated incidence of congenital hydrocephalus varies by region of the world. It was previously reported as lowest in North America at 68 per 100,00 births and highest in Latin America at 316 per 100,000 births. About 15% of children with myelomeningocele are born with significant hydrocephalus, and up to 80-90% eventually develop it due to deformities associated with the associated Chiari II malformation that obstruct CSF flow.⁷

Among adults aged 19-64 years, the prevalence of hydrocephalus is estimated to be 11 per 100,000 individuals, but this increases substantially with age. Prevalence rises to approximately 175 per 100,000 individuals among those aged 65–80 years, and up to 400 per 100,000 in individuals older than 80 years.⁶

In adults older than 60 years, NPH represents the most common subtype. The reported incidence of NPH ranges from 1.1 to 5.5 new cases per 100,000 individuals per year.⁷

Ventricular dilation has been noted in up to 72% of patients with severe brain injury. Clinically significant hydrocephalus following TBI greatly varies in the literature. It is important to note that some patients have ex vacuo changes without clinical findings.³

Subarachnoid hemorrhage is a risk factor for hydrocephalus and is highest in those with hemorrhage due to an aneurysm.

Hydrocephalus is relatively common and reversible cause of disorders of consciousness (DOC). An incidence rate of 38.4% has been reported in patients presenting with DOC.⁸

Patho-anatomy/physiology

CSF is normally produced at a rate of 25 mL/hr by the choroid plexus in the lateral ventricles and reabsorbed by the arachnoid granulations in the sagittal sinus at the same rate. The volume of CSF is between 125-150 mL, of which ~20% is contained in the ventricles.⁹

CSF flows through the ventricular system beginning in the lateral ventricles, passing into the third ventricle via the foramina of Monro. It then travels through the cerebral aqueduct (aqueduct of Sylvius) into the fourth ventricle. From there, it exits through the paired lateral foramina of Luschka and the median foramen of Magendie to enter the subarachnoid space, where it circulates around the brain and spinal cord before ultimately being reabsorbed through the arachnoid granulations into the dural venous sinuses.

Acutely after injury, blood products within the ventricular system can block the foramen of Sylvius or the fourth ventricle and cause an outflow obstruction, which results in hydrocephalus. Aqueduct stenosis or Chiari malformation may be decompensated by TBI resulting in a relative CSF outflow blockage or obstructive hydrocephalus.

Post-traumatic hydrocephalus may also be of the communicating type due to blood products and proteins accumulating in the arachnoid granulations, preventing reabsorption of CSF.

Normal pressure hydrocephalus may develop after trauma but may also be idiopathic. Decreased turgor in the aging brain may allow normal pressures to produce hydrocephalus.

Hydrocephalus ex vacuo occurs due to loss of brain parenchyma after injury, which allows the CSF space to enlarge.

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

Historically, patients with congenital or pediatric hydrocephalus would develop unremitting head enlargement followed by several physical and neurobehavioral complications. Introduction of shunting or CSF diversion has improved outcomes in this population.

Disease progression in TBI patients is variable, depending on whether it is an acute or chronic presentation. Acute hydrocephalus may present with headache, nausea, vomiting, lethargy, and/or change in mental status and may progress to coma and death if untreated. In the subacute or chronic state, failure to progress with rehabilitation or a decline in cognition or behavior may indicate hydrocephalus. Earlier shunting has been associated with improved outcomes in TBI patients.¹⁰

The natural course of NPH is progression of symptoms over time. The deterioration is only partially reversible leading to recommendations for earlier surgical management.¹¹

As previously mentioned, hydrocephalus is a potential reversible cause of disorders of consciousness.^8,12

Essentials of Assessment

History

History of trauma, central nervous system disease, or central congenital malformations should be elicited.

Acute hydrocephalus can present with headache, visual changes, nausea, vomiting, a decline in mental status, or lethargy, indicating increased intracranial pressure.

In patients with TBI, there may be the additional symptoms of failure to progress with rehabilitation therapies or a decline in performance. Other subtle signs may also be indicative of hydrocephalus including: abulia, perseveration, emotional lability, mutism, apraxia, decreased initiation or changes in bowel and bladder in the absence of infection.

Normal pressure hydrocephalus presents in the elderly with dementia, including memory loss and forgetfulness, gait ataxia and urinary incontinence. Gait is typically bradykinetic, wide-based with short steps and has been described as “magnetic.”¹³

Physical examination

Physical examination should include vital signs, mental status exam, behavioral observation, cranial nerve exam including fundoscopic evaluation, speech and language, motor and sensory testing, reflexes, and gait assessment. Observation of craniectomy site should be included as appropriate.

Cushing’s triad of hypertension, bradycardia and hypoventilation may be present.³

Enlarged head circumference and firm fontanelles can occur in infants with hydrocephalus.¹

Papilledema or a bulging of a craniectomy site may occur.³

Frontal and subcortical deficits including forgetfulness and decreased attention are present in NPH. The presence of aphasia or agnosia are not typically seen with NPH. The typical gait pattern seen in patients with NPH is bradykinetic, broad-based, magnetic, and shuffling. Unlike Parkinson’s, tremor and rigidity are not characteristic of NPH.¹³

For a patient with a shunt in place, abdominal pain or palpable pseudocyst may be present when the distal shunt is occluded.³

Functional assessment

Gait and balance should be assessed, including base of support and step length. Mental status examination should include comparison with previous evaluations, when available.

Serial functional outcome measures provide an objective means of monitoring functional status over time and detecting worsening mobility or cognition. Measures of gait, balance, and consciousness (e.g., gait speed and the Coma Recovery Scale-Revised [CRS-R]) may be useful depending on the clinical population.^8,13

Laboratory studies

Laboratory studies to rule out other etiologies contributing or causing a decline in cognition and gait should include evaluation for infection and electrolyte abnormalities.

Lumbar puncture or shunt tap (if present) with CSF analysis should be performed if meningitis is suspected.

Imaging

Computerized tomography (CT) scan will show ventricular dilation and is helpful in identifying the existence and nature of the CSF obstruction.

Magnetic resonance imaging (MRI) offers more structural information of potential pathologic causes of hydrocephalus. MRI also provides imaging of the Sylvian aqueduct and foramen of Monro. Decreased signal in the Sylvian aqueduct indicates hydrocephalus.

Ex vacuo hydrocephalus due to injury-related atrophy results in sulcal prominence. As previously mentioned, this is a descriptive term and does not imply the patient has clinical findings of hydrocephalus.

There are several morphological changes which can be observed on CT or MRI. This includes an increased Evans index (>0.3). The Evans index is the ratio of the maximum width of the frontal horns of the lateral ventricles and the maximal internal diameter of the skull at the same level. This index is particularly useful in patients with suspected NPH.¹³ However, the Evans index should be interpreted cautiously in patients with significant cerebral atrophy or prior decompressive craniectomy, as ventricular enlargement or altered cranial anatomy may reduce its diagnostic accuracy.^13,14

Shunt series is a set of radiographic images to assess for mechanical causes of shunt failure.

Nuclear medicine shunt patency studies determine if CSF is flowing through the shunt system by injection a small volume of radiotracer in the shunt reservoir. This allows for measurement of flow through the catheters and valve.

Supplemental assessment tools

CSF tap test can be performed to evaluate for normal pressure hydrocephalus. Prior to removal of >30 ml of CSF, neurologic status is evaluated. After CSF removal, the patient’s neurological and functional status is reassessed. If improvement is seen after CSF removal, shunting may be helpful.¹³

The tap test can be extended to prolonged drainage by catheter for 3-5 days. This has been shown to be more sensitive and predictive of shunt success.¹³

Early predictions of outcomes

Patients with longer duration of coma, increased age, decompressive craniectomy and subarachnoid hemorrhage (SAH) are reported to be at increased risk of developing post-traumatic hydrocephalus. Following craniectomy, there is disruption of intracranial pressure dynamics resulting in decreased CSF outflow.¹⁴

Flattening of the sulci with ventriculomegaly and periventricular lucencies on CT indicate that the response to shunting is typically favorable.³

Untreated, acute hydrocephalus can progress to coma and death. External ventriculostomy is used to prevent this progression. Delayed treatment can slow or disrupt neuroplasticity, repair and recovery.

When shunting is performed for normal pressure hydrocephalus (NPH), slowness of gait rather than disequilibrium is more likely to improve. Significant improvement in cognitive function may occur after shunting for NPH as well.¹³

Social role and social support system

Hydrocephalus can significantly affect mobility, cognition, and independence, thereby altering a patient’s social roles and daily functioning. As a result, patients often become more reliant on support systems, including family members, caregivers, and community resources. Family members are often the first to notice subtle changes in cognition or behavior, which may signal the development of hydrocephalus or dysfunction of an existing shunt and should prompt further evaluation.  It is important for medical providers to educate patients and their families on symptom recognition (e.g., shunt malfunction) and ensuring access to necessary services such as rehabilitation, home health support, and transportation.

Professional issues

For the low-level brain-injured patient with hydrocephalus, the decision to place a shunt should be considered in the context of other life-sustaining, quality of life measures. Expectations of outcomes following shunting should be discussed with family members.

Rehabilitation Management and Treatments

Available or current treatment guidelines

Medical management targets decreasing the rate of CSF formation or increasing CSF reabsorption.

Carbonic anhydrase inhibitors, such as acetazolamide, decrease CSF production. It has been used in combination with furosemide, a loop diuretic, however, its efficacy and safety have been questioned.¹⁵ Isosorbide, a hypertonic osmotic agent, increases CSF reabsorption. Medical treatments are used as a temporary measure prior to more definitive management.

External ventriculostomy is recommended for the treatment of acute hydrocephalus. If the obstruction persists, ventriculoperitoneal shunt (VP shunt) may be needed for long-term management.

A typical VP shunt consists of a proximal catheter, which is usually placed in the frontal horn of the lateral ventricle, a valve that regulates the pressure and prevents retrograde flow of the shunted CSF, and distal tubing that is usually placed in the peritoneal space.

Endoscopic third ventriculostomy, or membrane fenestration, involves creating a hole in the floor of the third ventricle, allowing communication with the underlying cisterns and subarachnoid space. This approach is particularly effective in cases of obstructive hydrocephalus, such as aqueductal stenosis.³

At different disease stages

• If shunt failure is clinically suspected, radiographic evaluation is warranted. Plain radiography is recommended to check for disconnection and catheter kinking, breakage or migration. CT of the head is recommended to evaluate ventricular size. Nuclear medicine shunt patency studies evaluate the patency with radiotracer. Sonography can be used to evaluate the patency of the peritoneal end of the shunt. MRI evaluates for infection or hemorrhage.¹⁶
• Replacement or revision of the shunt may be necessary if shunt occlusion or disconnection is present.
• Shunt infection occurs in 6-15% of cases and most commonly is related to Staphylococcus. Low-grade fever, malaise, irritability and nausea are presenting features. Diagnosis is made by shunt tap and is based on culture results. Shunt removal or externalization in combination with antibiotics is recommended.³
• Shunt overdrainage may result in orthostatic hypotension, dizziness, nausea, vomiting and diplopia. Overdrainage can also lead to hygromas or hematomas. Antisiphon devices and valves have been developed to counteract negative standing intracranial pressure. Programmable valves can be adjusted to maximize optimal CSF pressure balance.^17,18,19 Of note, shunts with programmable valves require checking the settings before and after MRI to ensure there was no inadvertent shunt readjustment.

Rehabilitation following shunt placement

Following VP shunt placement, rehabilitation should resume as soon as the patient is medically stable. Early mobilization should include monitoring for orthostatic symptoms, such as dizziness, headache, nausea, or hypotension, which may suggest shunt overdrainage. Clinicians should also monitor for changes in cognition, arousal, gait patterns, balance, continence and functional participation. These may reflect both successful CSF diversion and potential shunt complications.^3,10,17-19

Serial functional outcome measures can help track recovery and guide rehabilitation. The same useful previously discussed (e.g., gait speed, TUG test, Berg Balance Scale, CRS-R) can be used to assess response to treatment.^8,13

Patient & family education

Family education should be provided so that any change in mental status, behavior, mobility, or bladder function prompts them to seek medical evaluation for possible hydrocephalus or shunt malfunction.

Emerging/unique interventions

In patients where definitive placement of a shunt is not recommended, routine large volume lumbar punctures may also be effective in managing symptoms.

Recent studies have evaluated a novel shunt approach for NPH, in which patients undergo a minimally invasive procedure using a percutaneous femoral access technique to place a device at the within the cerebral venous system, specifically creating a connection between the CSF and the venous circulation. This approach, referred to as the “eShunt,” offers a less invasive alternative to traditional shunt placement.²⁰

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

It is important to note that hydrocephalus is a clinical diagnosis and should not be diagnosed or excluded by imaging alone.

A high index of suspicion should be maintained by clinicians treating patients at risk for hydrocephalus. In the context of neurotrauma or neurologic disease, subtle changes in cognition and behavior should prompt consideration of hydrocephalus.

In patients with posttraumatic hydrocephalus, earlier shunting predicted improved recovery.¹⁰

In people presenting with a disorder of consciousness, hydrocephalus should be excluded before a diagnosis of permanent vegetative state.^8,12

Routine imaging of patients with shunts is not recommended and should be based on a change in mental status, physical exam, or performance in rehabilitation therapies.

Cutting Edge/Emerging and Unique Concepts and Practice

Research shows that traumatic brain injury may cause ependymal ciliary loss which decreased CSF flow and increase the likelihood of hydrocephalus.²¹

Alterations in genes that regulate ciliary structure and motility have emerged as potential biomarkers for specific subtypes of hydrocephalus, with the potential to enhance diagnostic precision and support the development of personalized treatment strategies.²²

Recent data is increasing our knowledge of the molecular mechanisms by which inflammation contributes to the pathogenesis of hydrocephalus. This includes TLR4-regulated CSF cytokines, immune cells, and signaling pathways that can lead to choroid plexus hypersecretion, ependymal denudation, and scarring of CSF pathways. These may be potential targets for therapeutic intervention.²³

The glymphatic system plays a role in CSF regulation, however, the extent remains unclear. An imaging study in patients with idiopathic normal pressure hydrocephalus revealed significant suppression of glymphatic clearance.^22,24

Deferoxamine has been shown to decrease acute hydrocephalus after traumatic brain injury in animal studies.²⁵

Furthermore, in patients with NPH and coexisting type 2 diabetes mellitus taking sodium/glucose cotransporter 2 (SGLT2) inhibitors, recent studies have observed a reduction in the brain ventricle size.²⁶

As artificial intelligence and neuroimaging advances continue, the development of more accurate models will likely emerge to achieve better outcomes.

Gaps in the Evidence-Based Knowledge

There is a lack of understanding of exact pathophysiology underlying hydrocephalus.  This has led to gaps in evidence-based knowledge involving primary and secondary prevention of hydrocephalus.

References

1. Braddom RL. Physical Medicine and Rehabilitation. 3rd ed. New Brunswick, NJ: Saunders; 2007:1139,1178,1273,1418.
2. Delisa JA. Physical Medicine and Rehabilitation Principles and Practice. 4th ed. Hagerstown, MD: Lippincott; 2005:1689.
3. Zasler ND. Brain Injury Medicine. New York: Demos; 2007:583-592.
4. Cardenas DD, Hooton TM. Medical Complications in Physical Medicine and Rehabilitation. Demos; 2015:119-121.
5. Trevisi G, Frassanito P, Di Rocco C. Idiopathic cerebrospinal fluid overproduction: case-based review of the pathophysiological mechanism implied in the cerebrospinal fluid production. Croat Med J. 2014 Aug 28;55(4):377-87.
6. Schulz LN, Redwan A, Edwards S, Hamilton MG, Isaacs AM. Hydrocephalus pathophysiology and epidemiology. Neurosurg Clin N Am 2025;36:113–126.
7. Dewan MC, Rattani A, Mekary R, Glancz LJ, Yunusa I, Baticulon RE, Fieggen G, Wellons JC III, Park KB, & Warf BC. Global hydrocephalus epidemiology and incidence: systematic review and meta-analysis. J Neurosurg. 2019;130(4):1065-1079.
8. Zhang B, Huang K, Karri J, O’Brien K, DiTommaso C, Li S. Many Faces of the Hidden Souls: Medical and Neurological Complications and Comorbidities in Disorders of Consciousness. Brain Sciences. 2021;11(5):608.
9. Wright BLC, Lai JTF, Sinclair AJ. Cerebrospinal fluid and lumbar puncture: a practical review. J Neurol. 2012;259:1530-1545.
10. Kowalski RG, Weintraub AH, Rubin BA, Gerber DJ, Olsen AJ. Impact of timing of ventriculoperitoneal shunt placement on outcome in posttraumatic hydrocephalus. J Neurosurg. 2019;130(2):406-417.
11. Andrén K, Wikkelso C, Tisell M, et al. Natural course of idiopathic normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry. 2014;85:806-810.
12. Pickard JD, Coleman MR, Czosnyka M. Hydrocephalus, ventriculomegaly and the vegetative state: a review. Neuropsychol Rehabil. 2005;15:224-236.
13. Shprecher D, Schwalb J, Kurlan R. Normal pressure hydrocephalus: diagnosis and treatment. Curr Neurol Neurosci Rep. 2008;8(5):371-376.
14. Choi I, Park HK, Chang JC, Cho SJ, Choi SK, Byun BJ. Clinical factors for the development of posttraumatic hydrocephalus after decompressive craniectomy. J Korean Neurosurg Soc. 2008;43(5):227-231. doi:10.3340/jkns.2008.43.5.227
15. Whitelaw A, Kennedy CR, Brion LP. Diuretic therapy for newborn infants with posthemorrhagic ventricular dilatation. Cochrane Database Syst Rev. 2001(2):CD002270.
16. Goeser CD, McLeary MS, Young LW. Diagnostic imaging of ventriculoperitoneal shunt malfunctions and complications. Radiographics. 1998;18:635-651.
17. Friemann FB, Sprung C. Shunting with gravitational valves: can adjustments end the era of revisions for overdrainage-related events? Neurosurg. 2012;117(6):1197-1204. doi:10.3171/2012.8.JNS1233.
18. Trinh VT, Duckworth EA. Revision to an adjustable non-siphon control valve in low pressure hydrocephalus: therapeutic siphoning and a new perspective on NPH: series of 3 cases and review of the literature. Clin Neurol Neurosurg. 2012 (Jul 4). [Epub ahead of print]
19. Thomale UW, Gebert AF, Haberl H, Schulz M. Shunt survival rates by using the adjustable differential pressure valve combined with a gravitational unit (proGAV) in pediatric neurosurgery. Childs Nerv Syst. 2012 (Nov 8). [Epub ahead of print]
20. Malek A.M., Beneduce B.M., Heilman C.B. Endovascular shunting for communicating hydrocephalus using a biologically inspired transdural cerebrospinal fluid valved eShunt® implant. Neurosurg Clin. 2024;35:379–387. doi: 10.1016/j.nec.2024.03.004.
21. Xiong G, et al. Traumatic brain injury-induced ependymal ciliary loss decreases cerebral spinal fluid flow. J Neurotrauma. 2014 Aug 15;31(16):1396-404.
22. Roşu A-I, Andrei D, Ghenciu LA, Bolintineanu SL. Hydrocephalus: Molecular and Neuroimaging Biomarkers in Diagnosis and Management. Biomedicines. 2025; 13(7):1511. https://doi.org/10.3390/biomedicines13071511
23. Karimy JK, Reeves BC, Damisah E, et al. Inflammation in acquired hydrocephalus: pathogenic mechanisms and therapeutic targets. Nat Rev Neurol. 2020 May;16(5):285-296.
24. Ringstad G, Vatnehol SAS, Eide PK. Glymphatic MRI in idiopathic normal pressure hydrocephalus. Brain. 2017;140:2691-2705.
25. Zhao J, et al. Deferoxamine attenuates acute hydrocephalus after traumatic brain injury in rats. Transl Stroke Res. 2014 Oct;5(5):586-594.
26. Sadagopan NS, Khazanchi R, Jain R, Heimberger AB, Magill ST. Impact of SGLT2 inhibitors on cerebrospinal fluid dynamics and implications for hydrocephalus management. J Clin Invest. 2025;135(15):e188584. Published 2025 Jun 10. doi:10.1172/JCI188584

Original Version of the Topic

Carolyn Geis, MD. Hydrocephalus. 6/07/2013

Previous Revision(s) of the Topic

Mary Russell, MD. Hydrocephalus. 3/29/2017

Jacob Jeffers, MD, Mary Russell, MD. Hydrocephalus. 5/18/2023

Author Disclosure

Michelle Andary, DO
Nothing to Disclose

Jacob Jeffers, MD
Nothing to Disclose

Mary Russell, MD
Nothing to Disclose