Version May 2024
INTRODUCTION — Hydrocephalus is a disorder in which an excessive amount of cerebrospinal fluid accumulates within the cerebral ventricles and/or subarachnoid spaces, resulting in ventricular dilation and often with increased intracranial pressure (ICP) [1,2].
The management and prognosis of hydrocephalus in children will be reviewed here. The pathophysiology, etiology, clinical features, and diagnosis of hydrocephalus are discussed separately. (See "Hydrocephalus in children: Physiology, pathogenesis, and etiology" and "Hydrocephalus in children: Clinical features and diagnosis".)
This topic will focus on the management and prognosis of obstructive and communicating hydrocephalus, which are almost always associated with increased ICP. Normal pressure hydrocephalus, a condition seen predominantly in adults in which the cerebral ventricles are pathologically enlarged, but the ICP is not elevated, is discussed separately. (See "Normal pressure hydrocephalus".)
The prevention and initial management of hydrocephalus associated with intraventricular hemorrhage in preterm neonates is also discussed in greater detail separately. (See "Germinal matrix and intraventricular hemorrhage (GMH-IVH) in the newborn: Management and outcome".)
TERMINOLOGY — The following terms are used this topic:
●Obstructive hydrocephalus – Obstructive hydrocephalus (also called noncommunicating hydrocephalus) refers to excess accumulation of cerebrospinal fluid (CSF) due to structural blockage of CSF flow within the ventricular system. This is the most common form of hydrocephalus in children and is almost always associated with increased intracranial pressure (ICP). (See "Hydrocephalus in children: Physiology, pathogenesis, and etiology", section on 'Obstruction'.)
●Communicating hydrocephalus – Communicating hydrocephalus refers to CSF accumulation due to impaired absorption in the subarachnoid spaces. Rarely, it is caused by excessive CSF production. Communicating hydrocephalus is also typically associated with increased ICP. (See "Hydrocephalus in children: Physiology, pathogenesis, and etiology", section on 'Impaired absorption' and "Hydrocephalus in children: Physiology, pathogenesis, and etiology", section on 'Excessive production'.)
●Arrested hydrocephalus - arrested hydrocephalus refers to hydrocephalus that is nonprogressive because alternate pathways of CSF absorption develop or because normal mechanisms for CSF handling become re-established.
There is some overlap in these categories. Many causes of hydrocephalus have both obstructive and absorptive components (table 1), and the absorptive component of the hydrocephalus may change over time.
●Normal pressure hydrocephalus – In normal pressure hydrocephalus (NPH), the cerebral ventricles are pathologically enlarged, but the ICP is not elevated. NPH is most often seen in adults over the age of 60 years. NPH is discussed separately. (See "Normal pressure hydrocephalus".)
●Ventriculomegaly – Ventriculomegaly is a general term used to describe enlargement of the ventricles as seen on neuroimaging. Ventriculomegaly is a common finding in all forms of hydrocephalus. It is also seen in other conditions that are not associated with hydrocephalus (eg, brain atrophy).
The forms of hydrocephalus described above are distinct from two radiographic findings that include the same word:
●"Hydrocephalus ex-vacuo" – This term refers to enlargement of the CSF spaces caused by reduced volume of brain tissue due to atrophy (image 1) or malformation. It is not accompanied by increased ICP.
●"Benign external hydrocephalus" – Benign external hydrocephalus (image 2) (also called "benign enlargement of the subarachnoid space" or "benign extra-axial fluid of infancy") is a relatively common cause of macrocephaly in infancy and frequently occurs in other family members [3,4]. As the name implies, the condition is self-limited and affected infants usually do not require any intervention. (See "Macrocephaly in infants and children: Etiology and evaluation", section on 'Benign enlargement of the subarachnoid space'.)
MANAGEMENT
Overview — Timely referral to a pediatric neurosurgeon is important to ensure appropriate management of children with hydrocephalus. In addition, referral to a pediatric neurologist is often warranted, particularly if there are associated conditions such as seizures and/or developmental delays.
Most cases of hydrocephalus are progressive, meaning that neurologic deterioration will occur if the hydrocephalus is not effectively and continuously treated. For most patients, the most effective treatment is surgical drainage, using a shunt or third ventriculostomy. Rare exceptions include cases of hydrocephalus caused by a vein of Galen malformation, in which embolization of the malformation may be more appropriate than surgical drainage [5,6].
Rarely, hydrocephalus is not progressive because alternate pathways of cerebrospinal fluid (CSF) absorption develop or because normal mechanisms for CSF handling become re-established. This is known as "arrested hydrocephalus." In this case, shunting is unnecessary.
Management approach — The need for and timing of surgical intervention in patients with hydrocephalus is determined by the severity of symptoms and the neuroimaging findings:
Acute rapidly progressive hydrocephalus — Patients with acute rapidly progressive hydrocephalus require urgent surgical intervention, typically with a CSF shunt or endoscopic third ventriculostomy (ETV). If the hydrocephalus is due to a structural cause such as a resectable brain tumor, the appropriate intervention consists of removal of the tumor, often with intraoperative external ventricular drain (EVD) placement. (See 'CSF diversion procedures' below.)
Temporizing measures may be needed for patients with a life-threatening presentation (eg, signs of herniation) and those who are too unstable to undergo surgery, and may include:
●Ventriculostomy – In cases of rapidly progressive hydrocephalus, a temporary EVD may be needed until a permanent shunt can be placed or until the hydrocephalus resolves spontaneously [7,8]. EVD placement can be lifesaving in this setting. An EVD is a small catheter inserted through the skull usually into the lateral ventricle, which is typically connected to a closed collecting device to allow for drainage of CSF (figure 1). The EVD can also be connected to a transducer to measure ICP. The major complications associated with EVD are catheter occlusion and infection.
●Diuretics – The diuretics furosemide and acetazolamide decrease CSF production. They have been used for short periods in slowly progressive hydrocephalus in patients too unstable for surgery, but they are generally less effective than ventriculostomy.
In newborn infants with posthemorrhagic hydrocephalus, treatment with diuretics is generally not effective and is associated with complications. This issue is discussed in a separate topic review. (See "Germinal matrix and intraventricular hemorrhage (GMH-IVH) in the newborn: Management and outcome".)
●Serial lumbar punctures – Serial lumbar punctures are not recommended as a temporizing measure for most patients with acute rapidly progressive hydrocephalus. An exception is for preterm infants with posthemorrhagic hydrocephalus, for whom repeated lumbar punctures are sometimes used as a temporizing measure prior to placement of a more definitive CSF diverting device. However, the routine use of serial LPs as a preventive measure in neonates with intraventricular hemorrhage does not appear to be effective and is not recommended [9]. This issue is discussed in greater detail separately. (See "Germinal matrix and intraventricular hemorrhage (GMH-IVH) in the newborn: Risk factors, clinical features, screening, and diagnosis".)
Symptomatic and high-risk patients — Surgical intervention (with CSF shunt or ETV) is generally indicated in patients with hydrocephalus if any of the following are present:
●Symptoms (eg, headaches, vomiting, irritability, developmental delays, focal neurologic deficits, papilledema)
●Progression of ventriculomegaly on neuroimaging
●Clear obstruction of the CSF pathway evident on neuroimaging
The choice of surgical procedure is based largely on the age of the patient and the underlying cause. (See 'CSF diversion procedures' below.)
Asymptomatic and low-risk patients — Asymptomatic patients who lack findings suggestive of elevated ICP (eg, papilledema, bulging fontanel), are achieving expected developmental milestones, and do not have severe ventriculomegaly or obvious obstruction of the CSF pathway on neuroimaging can be managed with watchful waiting. Young infants are followed with serial head measurements, monthly or bi-monthly head ultrasounds, and assessment of gross motor skills. Ultrasound is generally preferred for this monitoring because it is time-efficient, inexpensive, and doesn't require sedation. If there is ongoing clinical concern after the anterior fontanelle closes, magnetic resonance imaging (MRI) is the preferred modality for follow-up neuroimaging. Limited imaging with ultrafast MRI can also be used as an alternative to ultrasonography in young infants. If signs or symptoms of increased ICP develop and/or there is progression of ventriculomegaly on imaging, surgical intervention is generally warranted. (See 'CSF diversion procedures' below.)
CSF DIVERSION PROCEDURES
Choice of procedure — Cerebrospinal fluid (CSF) shunts have long been the standard treatment for hydrocephalus in children (see 'CSF shunt' below). Endoscopic third ventriculostomy (ETV) is an alternative approach that has several advantages over CSF shunting in that it is relatively low-cost, durable, and potentially avoids the long-term complications that frequently occur with ventriculoperitoneal (VP) shunts (ie, infection and/or malfunction). However, ETV is not effective in all patients. As discussed below, the success of ETV depends upon the age of the patient, the cause of hydrocephalus, and upon previous complications [10-14]. (See 'Endoscopic third ventriculostomy' below.)
Criteria for selection of patients for ETV versus shunting are not standardized and practice varies considerably. The 2014 evidence-based guidelines of the American Association of Neurological Surgeons (AANS) and the Congress of Neurological Surgeons (CNS) concluded that outcomes of the two procedures are generally equivalent and they did not advocate for one approach over the other [15].
The ETV success score can help identify patients who are likely to benefit from ETV (table 2) [13]. Higher values of the success score appear to predict the probability of ETV success. Ideally, the success score should be 80 or greater; however, some experts may recommend ETV if the score is greater than 50.
In our practice, we use the following approach:
●We generally perform ETV for patients with third or fourth ventricular outlet obstruction or with clear aqueductal stenosis and for those with pineal region tumors and tectal tumors, because these respond well to ETV.
●We generally perform a CSF shunt procedure rather than ETV in patients with a history of intraventricular hemorrhage (IVH), meningitis, or previous shunting, because the likelihood of success with ETV is low in this setting. However, if patients with these disorders also have acquired aqueductal stenosis, we generally attempt ETV prior to pursuing shunting, because we have had moderate success with this approach.
●We generally do not perform ETV for treatment of obstructive hydrocephalus in infants <3 months old, because the likelihood of success is low (around 25 percent) in this age group [13,16]. These infants may need a CSF shunt. Alternatively, ETV with choroid plexus cauterization may be an option. (See 'ETV with choroid plexus cauterization' below.)
●For children in whom ETV is unsuccessful (ie, hydrocephalus progresses following ETV), we generally perform a shunting procedure, because repeating the ETV acutely is not likely to be successful [17].
CSF shunt
Shunt procedure — A mechanical shunt system is placed to prevent the excessive accumulation of CSF. The shunt allows CSF to flow from the ventricles into the systemic circulation or to the peritoneum where it is absorbed, bypassing the site of mechanical or functional obstruction to absorption. Shunts consist of the following components (figure 2):
●Ventricular catheter – A catheter is placed into one of the lateral ventricles (usually the right). In placing the ventricular catheter, entry from the skull is directed to access the ventricle without penetrating eloquent cortex. Frontal or occipital-parietal entry points are most commonly used, with the aim of positioning the tip of the catheter in the frontal horn, away from the choroid plexus. The optimal entry point is unclear. A 2014 systematic review of five studies that evaluated the impact of entry point on shunt survival concluded that the available evidence is insufficient to recommend one entry point (frontal or occipital) over the other. Technical adjuvants such as ventricular endoscopic placement, computer-assisted electromagnetic guidance, or ultrasound guidance are sometimes used to guide placement or the ventricular catheter; however, the available data are insufficient to determine if use of these technologies is associated with reduced complication rates and/or improved shunt function [18].
●Valve – The catheter is connected to a one-way valve system (usually placed beneath the scalp of the postauricular area) that opens when the pressure in the ventricle exceeds a certain value. The ventricular pressure decreases as fluid drains, resulting in closure of the valve until the pressure increases again. Many different shunt system components are available and they function with a variety of pressure, flow, and siphon control characteristics [19]. The design of these systems has evolved with the aim of reducing failure rates and other complications. Antisiphon devices (either intrinsic in the valve mechanism or as a separate device) have been developed to provide progressive resistance to flow to counteract the siphoning that occurs when negative pressure is exerted with vertical positioning. Programmable valves allow alterations in valve function to be made without a surgical procedure. A 2014 systematic review of 22 studies comparing different shunt components concluded that the available evidence did not demonstrate a clear advantage for any specific shunt component, mechanism, or valve design over another [19].
●Distal catheter – The distal end of the system is connected to a catheter that is most commonly placed in the peritoneal cavity (VP shunt). Less commonly (eg, if peritoneal placement is not feasible), the distal catheter is inserted in the right atrium of the heart (ventriculoatrial [VA] shunt) or pleural space (ventriculopleural shunt).
Shunt complications — In general, complications of shunted hydrocephalus are due to malfunction of the shunt. If the shunt malfunctions and if the mechanism causing the hydrocephalus is still active, symptoms of hydrocephalus recur, and a shunt revision or other drainage procedure is required. (See 'Shunt malfunction' below.)
Shunt malfunction — Malfunction may be caused by infection or mechanical failure. Approximately 40 percent of standard shunts malfunction within the first year after placement, and 5 percent per year malfunction in subsequent years [20-22]. Prompt evaluation for possible shunt malfunction should be performed in patients with shunts who develop new or worsening signs or symptoms of elevated intracranial pressure (ICP) (eg, headache, vomiting, lethargy, papilledema, irritability). Consultation with a neurosurgeon should occur early in the evaluation.
●Infection – Patients with CSF shunts (eg, ventriculoperitoneal, ventriculoatrial, or ventriculopleural shunts) are at risk for shunt infections. Shunt infection is a common complication, occurring in approximately 5 to 15 percent of procedures [21,23,24]. This may lead to ventriculitis [25], may promote the development of loculated compartments of CSF, and may contribute to impaired cognitive outcome and death [24]. The risk of shunt infections appears to be higher in newborns compared with older infants and children [26].
Infections can occur at any time, but most occur in the first six months after shunt placement. This is an important consideration in deciding when to tap shunts to evaluate a fever, especially when there is no clinical or radiographic evidence of mechanical shunt failure. Increasing abdominal pain associated with peritoneal signs and/or fever is a common presentation of shunt infection in patients with VP shunts. Abdominal ultrasound may demonstrate pseudocyst. Shunt infection must be considered in a child with a shunt who develops persistent fever. Antibiotics should be started, but this treatment alone is often not effective. In most cases, an infected shunt must be removed, and an external ventricular drain must temporarily be placed. (See "Infections of cerebrospinal fluid shunts", section on 'Device removal' and "Infections of cerebrospinal fluid shunts", section on 'Antibiotic therapy'.)
Diagnosis, treatment, and prevention of shunt infections are discussed in greater detail separately. (See "Infections of cerebrospinal fluid shunts".)
●Mechanical failure – Mechanical shunt failure is another important cause of shunt failure. Like shunt infection, it is most common during the first year after shunt placement [24]. The majority of shunt failures result from obstruction at the ventricular catheter [24]. Fractured tubing is the cause of shunt failure in approximately 15 percent of cases. Other causes include shunt migration (partial or complete) and excessive CSF drainage (overdrainage). Mechanical failure requires prompt recognition and surgical intervention.
●Evaluation and diagnosis – Evaluation for shunt malfunction typically includes a detailed neurologic examination, neuroimaging (usually with computed tomography [CT] or rapid brain magnetic resonance imaging [MRI]), and plain radiographs of the shunt tubing pathway (shunt series). In some cases, a shunt tap by the neurosurgical team may provide useful information (eg, elevated pressure suggestive of shunt malfunction, abnormal CSF indices suggestive of infection).
Shunt malfunction can be diagnosed on the basis of any of the following:
•Interval increase in ventricular size on neuroimaging study; however, as many as 30 percent of patients may lack this finding [27].
•Highly concerning neurologic findings (eg, new focal deficits, papilledema, severe lethargy), even in the absence of increased ventricular size.
•Fractured, displaced, or kinked shunt tubing seen on imaging (in the setting of suggestive signs or symptoms).
•Elevated CSF pressure and/or poor CSF flow as assessed by tapping the shunt.
•Persistent symptoms (eg, headaches, vomiting, lethargy) despite appropriate nonsurgical management.
If the findings of the evaluation are not consistent with shunt malfunction, other possible causes of the symptoms should be investigated (eg, infection, seizures, gastrointestinal pathology).
●Management – Shunt malfunction is managed surgically. Most shunt failures result from obstruction of the ventricular catheter. Shunt infections are managed with both medical and surgical interventions (ie, externalization of the shunt and intravenous antibiotics) as discussed in detail separately. (See "Infections of cerebrospinal fluid shunts", section on 'Treatment'.)
Overdrainage — Overdrainage can cause functional shunt failure, which causes subnormal ICP (particularly in the upright position) and which is associated with characteristic neurologic symptoms such as postural headache and nausea [20]. Overdrainage greatly reduces the size of the ventricles causing the catheter to lie against the ependyma and choroid plexus, and these tissues block the holes in the catheter. Overdrainage can lead to slit-ventricle syndrome, which is characterized by small or slit-like ventricles, coupled with transient episodes of symptoms of raised ICP [28]. Changes in shunt design to address the problem of overdrainage include valves designed to open at different pressures and selected based upon the patient's characteristics; anti-siphoning devices to minimize the siphon effect caused by changes in posture; and valves that regulate by flow rather than by pressure differences.
Other complications — Other less common complications are related to the end site of CSF drainage. Potential complications in patients with VP shunts include perforation of viscus and intestinal obstruction. Patients with VA shunts may develop thrombosis associated with the atrial catheter, cor pulmonale, or very rarely may develop glomerulonephritis ("shunt nephritis"), which is related to chronic infection. Patients with ventriculopleural shunts may develop pleural effusions which occasionally produce symptoms.
Endoscopic third ventriculostomy
ETV procedure — ETV involves creating an opening in the floor of the third ventricle to allow CSF to flow into the prepontine cistern and the subarachnoid space. ETV may be used in the initial treatment of selected cases of obstructive hydrocephalus and as an alternative to shunt revision; it is generally not effective for patients with communicating hydrocephalus (ie, hydrocephalus resulting from impaired CSF absorption). Some experts consider ETV the treatment of choice for aqueductal stenosis, although approximately 20 percent of patients still require shunting [29]. The success of the procedure depends upon the age of the patient, the cause of hydrocephalus, and upon previous complications [10-14].
If ETV is performed, it is important to monitor the patient postoperatively with serial clinical examinations and imaging to determine if the procedure was successful. If the hydrocephalus progresses, a shunting procedure generally is performed, because repeating the ETV acutely is not likely to be successful [17]. (See 'Follow-up' below.)
ETV success rate — The ETV success score can be used to estimate the likelihood of early success (table 2). The score was developed and validated using a dataset of 618 consecutive ETV procedures performed at 12 international institutions [13]. Older age at the time of the procedure (ie, >1 year old) is the strongest predictor of success; other important predictors include noninfectious hydrocephalus etiology (eg, aqueductal stenosis, tectal tumor, myelomeningocele, IVH), and lack of previous shunt.
In an analysis of 618 ETV procedures performed at 12 international institutions, the overall success of ETV assessed six months after the procedure was 66 percent [13]. In a follow-up study, the same investigators compared outcomes with ETV and shunting in a cohort of children with newly diagnosed hydrocephalus treated with either ETV (489 patients) or shunt insertion (720 patients) [30]. Among patients with high predicted ETV success (ie, ETV success score ≥80), cumulative reoperation-free survival at 36 months was greater with ETV compared with shunting (72 percent versus 54 percent). However, among patients with moderate and low ETV success scores, outcomes were similar with the two procedures. For patients with moderate ETV success scores (ie, 50 to 70), reoperation-free survival at 36 months was approximately 50 percent in both groups; and for those with low ETV success scores (ie, ≤40), reoperation-free survival at 36 months was approximately 38 percent in both groups.
In another multicenter, prospective study involving 336 children who underwent initial ETV and were followed for at least 18 months, 42 percent of patients had documented failure of their ETV requiring repeat hydrocephalus surgery during follow-up [14]. Two-year reoperation-free survival was 58 percent. The majority of failures (83 percent) occurred within six months of surgery and the ETV success score was a strong predictor of success. The cohort represented a selected group of patients; most patients (85 percent) were older than 12 months and most (81 percent) had not undergone prior shunt placement. The most common etiologies were aqueductal stenosis (25 percent) and midbrain or tectal lesions (21 percent).
Ongoing clinical trials are evaluating outcomes with ETV compared with shunting in children with communicating [31] and noncommunicating hydrocephalus [32].
ETV complications — Complications of ETV were described in a 2012 systematic review of 24 case series reporting outcomes of >2500 ETV procedures in children and adults with hydrocephalus due to a variety of etiologies, most commonly tumor (37 percent) and aqueductal stenosis (26 percent) [33]. The overall complication rate was 8.8 percent, including intraoperative hemorrhage (3.9 percent), infection (1.8 percent), CSF leak (1.7 percent), and other surgical complications (eg, thalamic infarct and subdural, intracerebral, and epidural hematoma: [1.1 percent]). Permanent morbidity, including hemiparesis, gaze palsy, memory disorders, altered consciousness, and/or hypothalamic dysfunction, occurred in 2.1 percent. Postoperative mortality was 0.2 percent, and two patients experienced delayed sudden death (ie, >2 years following ETV) due to acute hydrocephalus from stoma occlusion.
In a subsequent multicenter prospective study involving 336 ETV procedures in pediatric patients, postoperative complications included CSF leak (4.4 percent), hyponatremia (3.9 percent), pseudomeningocele (3.9 percent), severe bleeding (1.8 percent), thalamic contusion (1.8 percent), venous injury (1.5 percent), hypothalamic contusion (1.5 percent), and major arterial injury (0.3 percent) [14]. Visible forniceal injury was seen more commonly in this cohort than previously reported (17 percent of cases). New neurologic deficits occurred in 1.5 percent of cases, with 0.5 percent being permanent.
ETV with choroid plexus cauterization — Because of the high rate of failure of ETV, particularly in young infants, researchers have suggested adding choroid plexus cauterization (CPC) to ETV in attempt to improve the efficacy of the procedure. Based on results from studies performed predominantly in sub-Saharan Africa [34,35], the combined ETV and CPC procedure was introduced in the United States and Canada beginning in the late 2000s to early 2010s [36-38]. However, a 2020 systematic review, with five studies (two prospective) and 963 patients found no significant difference in success rates comparing ETV with ETV plus CPC, with the exception of a subgroup analysis suggesting benefit in sub-Saharan African populations [39]. Further data are needed, particularly regarding the long-term effects of CPC, before ETV with CPC can be recommended as a routine practice.
CPC is technically challenging and requires skill with the flexible endoscope. In order to perform the procedure successfully, the surgeon must access the entire lateral ventricle choroid plexus bilaterally. Inadequate lighting in the ventricle may limit the amount of choroid plexus cauterization, and possibly complicate the procedure. In addition, the choroid plexus is not the only site for CSF production, so even if cauterization is successful, the procedure may not adequately address the hydrocephalus in some cases.
FOLLOW-UP
●Office visits and examination – Patients who undergo surgical treatment for hydrocephalus require long-term follow-up with a neurosurgeon. Following the initial surgical intervention, patients should be seen within two to four weeks, or sooner if concerning symptoms develop. Visits can then be spaced out if the child is stable.
●Neuroimaging – Neuroimaging studies are typically obtained postoperatively. Postoperative imaging for children who have undergone endoscopic third ventriculostomy (ETV) should include a magnetic resonance cerebrospinal fluid (CSF) flow study to demonstrate flow through the ventriculostomy. (See "Hydrocephalus in children: Clinical features and diagnosis", section on 'Neuroimaging'.)
Once ventricular size is stabilized on neuroimaging, and the parents or guardians are comfortable and familiar with the signs to watch for, follow-up imaging is done once every few years or if the patient presents with symptoms of shunt malfunction. (See 'Shunt malfunction' above.)
For children with hydrocephalus, the goals of treatment are to return the CSF flow and intracranial pressure (ICP) to levels that are as near to normal as possible and to promote normal neurologic development. The effectiveness of surgical intervention is assessed using both clinical and radiologic findings. Neuroimaging indicators of effective treatment include [40]:
•Reduction in ventricle size
•Presence of a flow void in the third ventriculostomy site (for patients who underwent third ventriculostomy)
•Resolution of periventricular edema
However, imaging findings do not always correlate with important clinical outcomes such as neurocognitive sequelae. In particular, ventricular size alone appears to be a poor indicator of success or failure of surgical intervention [40].
●Neurodevelopmental monitoring – Close neurodevelopmental monitoring is an important aspect of the long-term management of children with treated hydrocephalus and should be included in all routine health maintenance visits. If concerns arise, appropriate referrals should be made. (See "Developmental-behavioral surveillance and screening in primary care", section on 'Approach to surveillance'.)
●Echocardiogram – For patients with ventriculoatrial (VA) shunts, we typically perform echocardiograms annually. Unlike ventriculoperitoneal (VP) shunts, VA shunts can manifest with cardiac vegetations and/or cor pulmonale decades after shunt insertion. It is important to understand these long-term complications and to follow patients appropriately into adulthood.
OUTCOME — The outcome of hydrocephalus depends upon the etiology, the associated abnormalities, and the complications, such as infection.
Survival — Survival in untreated hydrocephalus is poor. Approximately 50 percent of affected patients die before three years of age, and approximately 80 percent die before reaching adulthood [20]. Treatment markedly improves the outcome for hydrocephalus not associated with tumor, with 89 and 95 percent survival in two reports [26,41].
Epilepsy — Seizures occur frequently in children with shunted hydrocephalus [41-43]. In one report from of 802 children treated with ventriculoperitoneal (VP) shunt and followed for a mean of eight years, 32 percent had epilepsy [43]. Seizures may be present at the time the diagnosis of hydrocephalus is made. However, shunt placement and complications also predisposed to epilepsy.
The incidence of seizures varies according to the etiology of hydrocephalus. In the study described above, the risks in patients with infection, cerebral malformations or intraventricular hemorrhage (IVH), and spina bifida were approximately 50, 30, and 7 percent, respectively [43].
Seizures are associated with poor cognitive outcome. In the study described above, fewer children with seizures had normal cognition (intelligence quotient [IQ] >90) compared with those without seizures (24 versus 66 percent) [43].
Seizures in this setting can be subclinical or can occur exclusively at night [44]. Electroencephalogram (EEG) monitoring should be considered in patients with neurologic deterioration who do not appear to have shunt failure or infection.
Functional outcome — Functional outcome is dependent on many factors, including degree of prematurity, other central nervous system (CNS) malformations, other congenital abnormalities, and epilepsy, as well as sensory and motor impairments [20]. The Hydrocephalus Outcome Questionnaire is a useful tool to measure the physical, emotional, cognitive, and social function of hydrocephalic children, aspects of health that are often overlooked [45,46].
In a study that reported outcomes of 129 children who underwent shunt placement before the age of 2 years between 1979 and 1982 and who were followed up for at least 10 years, motor deficits, visual or auditory deficits, and epilepsy occurred in 60, 25, and 30 percent of patients, respectively [41]. IQ was >90 in 32 percent and was <50 in 21 percent. Attendance at a normal school was possible for 60 percent, although one-half were one to two years behind for their age or were having difficulties. Of the remainder, 31 percent were in special classes or were institutionalized, and 9 percent were not considered educable.
In another series of 155 children who underwent first-time VP shunt insertion between 1978 and 1983 and were followed for 10 years, 11 percent died during the follow-up period [26]. Among survivors, approximately 60 percent attended a normal school. Children with hydrocephalus caused by infection or by IVH were more likely to need special education services than were those with congenital hydrocephalus (52 and 60 percent versus 29 percent).
Another study assessed cognitive outcome at 5 to 10 years of age in 73 children with hydrocephalus born between 1989 and 1993 [47]. IQ was ≥85 in 33 percent, 70 to 84 in 30 percent, 50 to 69 in 21 percent, and <50 in 16 percent. Median IQ was decreased among those who were born preterm compared with term (median IQ score 68 versus 76); among those with isolated hydrocephalus at birth compared with those with hydrocephalus and myelomeningocele or with acquired hydrocephalus (median IQ score 60 versus 77); and among those with cerebral palsy and/or epilepsy compared with those without (median IQ score 66 versus 78). There was a discrepancy between median verbal and performance IQ (90 and 76, respectively), which has been noted in other studies [48].
In extremely low birth weight infants, hydrocephalus associated with IVH and a shunt correlates with adverse neurodevelopmental outcomes at 18 to 22 months follow-up, compared with children with and without severe IVH and with no shunt [49]. Nonhemorrhagic ventriculomegaly in extremely preterm infants is also associated with increased likelihood of neurodevelopmental impairment [50]. Neurodevelopmental outcomes in preterm infants with IVH is discussed in greater detail separately. (See "Germinal matrix and intraventricular hemorrhage (GMH-IVH) in the newborn: Management and outcome", section on 'Outcome'.)
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Pediatric hydrocephalus".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword[s] of interest.)
●Basics topics (see "Patient education: Hydrocephalus in babies and children (The Basics)")
SUMMARY AND RECOMMENDATIONS
●Timely referral to a pediatric neurosurgeon is important to ensure appropriate management of children with hydrocephalus. In addition, referral to a pediatric neurologist is often warranted, particularly if there are associated conditions such as seizures and/or developmental delays. Most cases of hydrocephalus are progressive, meaning that neurologic deterioration will occur if the hydrocephalus is not effectively and continuously treated. (See 'Management' above.)
●The need for and timing of surgical intervention in patients with hydrocephalus is determined by the severity of symptoms and the neuroimaging findings (see 'Management approach' above):
•Patients with acute rapidly progressive hydrocephalus require urgent surgical intervention, typically with a cerebrospinal fluid (CSF) shunt (figure 2) or endoscopic third ventriculostomy (ETV). Temporizing measures may be needed for patients with a life-threatening presentation (eg, signs of herniation) and those who are too unstable to undergo surgery. In these circumstances, management often consists of placement of a temporary external ventricular drain (figure 1). (See 'Acute rapidly progressive hydrocephalus' above.)
•Surgical intervention (with CSF shunt or ETV) is also generally indicated in patients with hydrocephalus who are symptomatic (eg, headaches, vomiting, irritability, developmental delays, focal neurologic deficits, papilledema), have progression of ventriculomegaly on neuroimaging, and/or have clear obstruction of the CSF pathway evident on neuroimaging. The choice of surgical procedure is based largely on the age of the patient and the underlying cause. (See 'CSF diversion procedures' above and 'Choice of procedure' above.)
•Asymptomatic patients who lack findings suggestive of elevated intracranial pressure (ICP) (eg, papilledema, bulging fontanel), are achieving expected developmental milestones, and do not have severe ventriculomegaly or obvious obstruction of the CSF pathway on neuroimaging can be managed with watchful waiting. (See 'Asymptomatic and low-risk patients' above.)
●Surgical treatment of hydrocephalus consists of CSF shunt insertion or ETV (see 'CSF diversion procedures' above):
•A CSF shunt is a mechanical system that is placed to prevent the excessive accumulation of CSF (figure 2). It consists of a ventricular catheter, a one-way valve system that opens when the pressure in the ventricle exceeds a certain value, and a distal catheter that is most commonly placed in the peritoneal cavity (ventriculoperitoneal [VP] shunt). Less commonly (eg, if peritoneal placement is not feasible), the distal catheter is inserted in the right atrium of the heart (ventriculoatrial [VA] shunt) or pleural space (ventriculopleural shunt). Complications of CSF shunts are most commonly due to infection or mechanical failure. (See 'CSF shunt' above.)
•ETV involves creating an opening in the floor of the third ventricle to allow CSF to flow into the prepontine cistern and the subarachnoid space. ETV may be used in the initial treatment of selected cases of obstructive hydrocephalus and as an alternative to shunt revision; it is generally not effective for patients with communicating hydrocephalus (ie, hydrocephalus resulting from impaired CSF absorption). The success of ETV depends upon the age of the patient, the cause of hydrocephalus, and upon previous complications. The ETV success score can help identify patients who are likely to benefit from ETV (table 2). (See 'Endoscopic third ventriculostomy' above.)
●Patients who undergo surgical treatment for hydrocephalus require long-term follow-up with a neurosurgeon. Once the ventricle size become stabilized on follow-up imaging, and the parents or guardians are comfortable and familiar with the signs to watch for, follow-up imaging is done once every few years or if the patient presents with symptoms of shunt malfunction. For children with hydrocephalus, the goals of treatment are to return the CSF flow and ICP to levels that are as near to normal as possible and to promote normal neurologic development. (See 'Follow-up' above.)
●Prompt evaluation for possible shunt malfunction should be performed in patients with shunts who develop new or worsening signs or symptoms of elevated ICP (eg, headache, vomiting, lethargy, papilledema, irritability). Consultation with a neurosurgeon should occur early on in the evaluation. (See 'Shunt malfunction' above.)
●The outcome of hydrocephalus depends upon the etiology, the associated abnormalities, and the complications such as infection. (See 'Outcome' above.)
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