Acta Neurologica Belgica (2021) 121:823–836

https://doi.org/10.1007/s13760-021-01626-0

REVIEW ARTICLE

Non‑traumatic pediatric intracranial hypertension: key points

for different etiologies, diagnosis, and treatment
Nir Shimony1,2,3,4 · Meleine Martinez‑Sosa5 · Brooks Osburn5 · George I. Jallo1,2,5

Received: 3 December 2020 / Accepted: 2 February 2021 / Published online: 7 April 2021
© Belgian Neurological Society 2021

Abstract
Intracranial hypertension can be an acute life-threatening event or slowly deteriorating condition, leading to a gradual loss
of neurological function. The diagnosis should be taken in a timely fashioned process, which mandates expedite measures
to save brain function and sometimes life. An optimal management strategy is selected according to the causative etiology
with a core treatment paradigm that can be utilized in various etiologies. Distinct etiologies are intracranial bleeds caused
by traumatic brain injury, spontaneous intracranial hemorrhage (e.g., neonatal intraventricular hemorrhage), or the rare
pediatric hemorrhagic stroke. The other primary pediatric etiologies for elevated intracranial pressure are intracranial mass
(e.g., brain tumor) and hydrocephalus related. Other unique etiologies in the pediatric population are related to congenital
diseases, infectious diseases, metabolic or endocrine crisis, and idiopathic intracranial pressure. One of the main goals of
treatment is to alleviate the growing pressure and prevent the secondary injury to brain parenchyma due to inadequate blood
perfusion and eventually inadequate parenchymal oxygenation and metabolic state. Previous literature discussed essential
characteristics of the treatment paradigm derived mainly from pediatric brain traumatic injuries’ treatment methodology.
Yet, many of these etiologies are not related to trauma; thus, the general treatment methodology must be tailored carefully for
each patient. This review focuses on the different possible non-traumatic etiologies that can lead to intracranial hypertension
with the relevant modification of each etiology’s treatment paradigm based on the current literature.

Keywords Intracranial pressure · Conservative · Etiology · Surgery

Introduction two primary groups based on the etiology of the derange-

ment—primary intracranial hypertension (also known as
Intracranial hypertension (IH) is a general term for excessive idiopathic intracranial hypertension or pseudotumor cer-
pressure within the cranial vault. It usually presents with ebri) and secondary intracranial hypertension (variable eti-
headaches, alterations in the level of consciousness, visual ologies—trauma, hydrocephalus, infection, tumors, acute
disturbances, and kids’ developmental or cognitive delays. subarachnoid hemorrhage, Chiari malformation, medication-
The etiologies for IH vary and are usually separated into induced, etc.). Another way to categorize increased intrac-
ranial pressure is by severity and acuity. Sustained chronic
* Nir Shimony intracranial hypertension usually is better tolerated and
nshimony@geisinger.edu presented with chronic headaches, visual disturbances, and
even developmental and cognitive delays. On the other hand,
1
Department of Neurosurgery, Johns Hopkins Medicine, acute IH (e.g., traumatic brain injury) is not well tolerated
Institute for Brain Protection Sciences, Johns Hopkins All
Children’s Hospital, St. Petersburg, FL, USA and can lead to acute brain herniation—this was referred
2 to by some as “brain codes”, which in analogy to cardiac
Department of Neurosurgery, Johns Hopkins University
School of Medicine, Baltimore, MD, USA codes, signify extreme cases of devastating neurological epi-
3 sodes that mandate immediate action to save brain tissue and
Geisinger Medical Center, Neuroscience Institute, Danville,
PA, USA prevent further injury and even death [1]. Besides specific
4 etiologies that have a component of the mass lesion (brain
Geisinger Commonwealth School of Medicine, Scranton, PA,
USA tumor, intracranial bleeding, etc.) with or without secondary
5 brain edema, most of the other etiologies pathophysiological
University of South Florida, Tampa, FL, USA

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824 Acta Neurologica Belgica (2021) 121:823–836

mechanism leads to elevated pressure as a result of the sec- agree that when the suspicion for elevated ICP is present, a
ondary brain edema. Brain edema is traditionally classified stepwise tier directed paradigm should be initiated, with an
as vasogenic or cytotoxic [1, 2]. Further understanding of elevation of the head of the bed, control blood pressure and
the pathophysiology leads to the current paradigm stating glycemic status, and initiation of hyperosmolar treatment
this is a continuum process, with different stages and types as needed. In the pediatric population, Yildizdas et al. [13]
of edema (usually cytotoxic edema, then ionic edema, and conducted a retrospective study of 67 children with cerebral
last vasogenic edema) [3]. Cytotoxic edema is created by edema caused by different etiologies. In their research, the
progressive cell swelling mainly because of the accumu- endpoint was the duration of the comatose state and mortal-
lation of fluids and sodium. Vasogenic edema is primarily ity for patients that were treated aggressively for elevated
created by the disruption of the blood–brain barrier (BBB). ICP by either mannitol, hypertonic saline, or a combination
This leads to the extracellular accumulation of fluid in the of the two. They found that those treated by mannitol did
brain parenchyma. worse and deduced that hypertonic saline has some advan-
The management paradigm of elevated intracranial pres- tages over mannitol. Yet, other publications failed to achieve
sure is based on pediatric traumatic brain injury research the same results and even found mannitol benefits like treat-
and management. Early detection of increased intracranial ment choice [14, 15]. In 2016, Burgess et al. published a
pressure is an essential first step to implement the right ther- systematic review of RCTs comparing the two modalities
apeutic measures [4]. In many cases, imaging and clinical in TBI treatment [16]. They stated that if taking all consid-
evaluation is enough to diagnose elevation in intracranial erations into account and not just reducing ICP, there is no
pressure. In some cases, as part of diagnosis and monitor- superiority of any agent over the other. They concluded that
ing of treatment’s effectiveness, direct ICP monitoring via choice of therapy could be decided based on safety and that
intraparenchymal or intraventricular catheter is considered. in different scenarios, different agents can be used when tak-
Direct ICP measurement is considered by many the gold ing the safety profile into account [16].
standard and the most reliable technique. However, although The paradigm for non-traumatic elevated ICP is different
relatively low risk, it still bears the risk for intraparenchy- in several aspects, and one of them is the possible use of
mal or ventricular hemorrhage as well as an intracranial high dose corticosteroids, which can be very beneficial in
infection [5]. This leads to the development of a variety of cases of intracranial mass effect like a new brain tumor but
non-invasive diagnostic tools to measure ICP [6]. The use can be even harmful to some degree when given in the case
of direct ICP measurement for non-traumatic etiologies is of IH as a result of brain trauma [10, 12]. If the condition
highly controversial, and its use is judged based on the case continues to evolve, most agree there is a need to control ICP
and the possible benefit of invasive monitoring [7–9]. and cerebral perfusion pressure (CPP) by more aggressive
This review thoroughly describes the different etiologies, measures like intubation, ventilation, and possible invasive
suggested diagnosis, monitoring, and treatment for non-trau- ICP monitoring. The use of induced hypothermia is still
matic etiologies that lead to IH in the pediatric population. highly controversial, even for the pediatric age group. Advo-
cates mention the good response that was observed among
Methodology neonates treated with hypothermia for hypoxic-ischemic
insults [17, 18]. In a multicenter phase III randomized con-
A thorough literature review was made using PubMed. trolled trial for severe pediatric TBI comparing induced
We used general keywords for elevated intracranial pres- hypothermia versus maintaining normothermia, it was found
sure concerning the pediatric population (e.g., pediatric that induced hypothermia did not lead to greater mortality
elevated intracranial hypertension, pediatric elevated intrac- but also did not achieve a better neurological outcome for the
ranial pressure, elevated intracranial pressure in children). patients (much more patients needed intervention in the nor-
After reviewing the non-traumatic etiologies, we chose the mothermia group, but it did not reach statistical significance)
most commonly discussed etiologies in the literature and [19]. The latest update for the pediatric age group from the
ran another literature review for each subcategory. These brain trauma foundation published in 2019, still describes
etiologies are described in this review based on the different the controversy with a Level II evidence stating there is no
papers that were reviewed for each subcategory. benefit over normothermia for improving outcome. Yet, for
overall ICP reduction, there is a level III recommendation
to use moderate hypothermia [12]. For the non-traumatic
Core treatment paradigm etiologies of pediatric IH, there is no evidence to support
either way, and we advocate using extreme caution when
The treatment paradigm derived mainly from trauma studies choosing this tool. In the case where ICP is refractory, the
and the development of Brain Trauma Foundation Guide- use of paralytic agents can be discussed (although contro-
lines for traumatic brain injuries [10–12]. Most clinicians versial) and the consideration for barbiturate-induced coma

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or decompressive craniectomy. Hemispheric decompressive to brain tumors will be treated with debulking or resection
craniectomy is known to increase the cranial cavity volume of the tumor. In other cases, when secondary hydrocephalus
and thus reduce intracranial pressure, increase perfusion cannot be relieved by tumor resection, CSF diversion will
pressure, and preserve cerebral blood flow [20]. The use of be needed.
decompressive craniectomy in non-traumatic IH settings is
again highly controversial, with no evidence to support its Infections
use besides some small series and case reports.
Meningitis is an infection of the brain covering mainly the
Different etiologies for non‑traumatic pediatric dura, arachnoid, and subarachnoid space. Encephalitis and
intracranial pressure brain abscesses are infections of the brain itself, either the
grey or white matter fibers. The symptoms can vary widely
Pediatric hypoxic brain injury usually includes injuries with from mild neurological changes with or without fever to
devastating results, such as near-drowning or prolonged severe neurological deterioration, septic shock, and even
resuscitation after cardiac arrest. The management of this death. The imaging modality is usually MRI, focusing on
kind of injury usually will consist of supportive systemic T1 with gadolinium, T2, and FLAIR sequences.
treatment with some measurement for controlling brain Acute severe bacterial meningitis can develop into a
swelling. There is a big controversy regarding intracranial severe infection across the central nervous system with
pressure monitoring, mainly because it appears not to alter devastating consequences [24–27]. The high mortality
the outcome [21]. Another group of pathological conditions and morbidity rates are mainly due to raised intracranial
that is atraumatic and known to be related to significant brain pressure and herniation. Although better understanding
edema or swelling is related to metabolic disorders, such as has been achieved in recent years, the mortality rate is
Reye’s syndrome. Past literature showed the benefit of ICP still very high with traditional therapies for increased ICP
monitoring for Reye’s syndrome, but like near-drowning, [28]. The treatment paradigm is derived mainly from the
invasive monitoring is quite controversial regarding other trauma setting or acute malignant stroke syndrome. One
anoxic encephalopathies [8]. For fulminant hepatic failure, of the possible explanations is collateral damage, such as
there is controversy as well, but a growing body of litera- vascular injury (that can potentially lead to areas of cer-
ture suggests the benefit of controlling the ICP even as a ebral ischemia and stroke), cerebritis, and the evolution of
bridge for transplantation. The elevated ICP in high-grade brain abscess. In some cases, vascular injury leads to small
liver encephalopathy is a leading cause of mortality, and punctate hemorrhagic spots that eventually can evolve into
aggressive measures to control it have shown benefit [22]. In a severe infection and even significant brain infarct [29, 30].
a recent review regarding different pathologies that can lead Encephalitis is an inflammation of the brain parenchyma
to elevated intracranial pressure, the authors describe panel that can be presented as a significant neurological deficit.
recommendations for various pathologies stating the insuf- It primarily involves the brain yet can involve the meninges
ficient literature and evidence regarding pathologies that are in some cases (meningoencephalitis). The impairment of
not TBI related [23]. The literature focuses mainly on the viral encephalitis can result from focal or diffuse inflamma-
major pathologies, such as TBI, stroke, sinus vein throm- tion and vascular arthropathies, including pediatric stroke
bosis, IIH, and infections. There is even less published data [26]. Among the pediatric population, the most common
regarding the pediatric age group, and hence the treatment form of encephalitis is infection from the Herpes simplex
paradigm is deduced from the adult age group literature. The virus. The infectious manifestations are devastating, with
treatment paradigm in many cases will be similar, as men- more than 70% mortality rate is reported in untreated cases,
tioned above. Still, there is a lack of evidence in many issues and almost all the patients will not return to their neurologi-
regarding the different treatment aspects (e.g., invasive ICP cal baseline [31, 32]. Treatment success depends mainly on
monitoring). In this review, we will focus on the significant early anti-viral treatment. A significant reduction in mortal-
etiologies discussed in the literature regarding pediatric non- ity (in some publications to 20%) and significantly favorable
traumatic elevated ICP. results (around half of the patients will go back to baseline)
can be achieved with the administration of Acyclovir [32].
Brain tumors The disease-associated inflammatory process is usually focal
and tends to classically affect the temporal lobe. However, in
One of the significant non-traumatic etiologies of elevated some cases, parenchymal hemorrhage and areas of necrosis
ICP among the pediatric age group is mass-like lesions, such can be developed within the involved area, leading to mass
as brain tumors. The discussion of pediatric brain tumors effect and intracranial hypertension. In severe cases, even
and their treatment paradigm is lengthy and beyond this to brain herniation [33]. The peak rise in ICP varies for the
review’s scope. Most cases of elevated ICP that are related individual patient but has been described in adults around

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the 12th day of illness, whereas in children, the data are still under steroidal treatment [45]. Most authors advocate for
lacking [34]. Barnett et al. demonstrated in their publication their use in a delayed fashion after initiation of the anti-viral/
that within two weeks, half of the patients died due to severe bacterial treatment [45–47]. Hyperosmolar treatment is very
mass effects causing a devastating elevation in intracranial widely practiced in the setting of elevated ICP in trauma as
pressure despite treatment to alleviate the pressure [34]. well as for elevated ICP due to infectious derived etiologies.
Hence, a better understanding of the patient’s ICP is needed Barbiturate coma is a medical therapy that is used for low-
in the setting of acute and severe encephalitis [35]. Another ering cerebral metabolic demand, which leads to less blood
important entity of encephalomyelitis is acute disseminated influx to the brain and hence to a reduction in the intracra-
encephalomyelitis (ADEM). This is an inflammatory demy- nial pressure [35]. This therapy should be initiated after the
elinating immune-mediated disorder that usually will erupt failure of the previous tiers or the initial case was extremely
in childhood [36]. There is a strong association between severe demanding use of early radical measures. Barbiturate
ADEM and infectious process or after vaccine administra- coma requires continuous EEG monitoring (to achieve burst
tion. It is believed that the reactive immune response of the suppression) as well as hemodynamic monitoring due to sig-
body leads to pathologic transient autoimmune response nificant hypotensive crisis risk. In infectious diseases, sev-
directed at myelin or other self-antigens in the CNS [36–39]. eral descriptions are supporting the early use of barbiturates
The medical treatment of ADEM is mainly high dose corti- coma in severe cases [48, 49]. The use of hypothermia has
costeroids with a good prognosis as more than 50% achieve been debated for the last decade, especially in the pediatric
full recovery. Still, mortality is up to 12%, and refractory population. Yet, as for now although the mechanism has
elevated ICP is the cause of most cases. been postulated in few publications it cannot be advocated.
In cases of encephalitis or encephalomyelitis, when Surgical treatment in elevated ICP derived from infec-
elevated ICP symptoms are suspected, there is a need to tious disease etiology is scarce. In the pediatric population,
consider ICP monitoring and CPP management. Yet, clear it is mainly anecdotal and focused on previously published
management guidelines are missing, and the benefit of ICP case reports. Yet, it has been described in the adult popula-
measurement and surgical intervention is controversial when tion [50]. Several publications advocate the use of decom-
managing severe herpes encephalitis [40]. Furthermore, the pressive craniectomy leading to high GOS of 4–5. Decom-
management paradigm for encephalitis is related to ICP pressive craniectomy was shown to be a significant and
monitoring and ICP management from the traumatic brain effective treatment methodology in patients with significant
injury (TBI) literature, which is a different etiology. As with mass effect secondary to encephalitis that failed other treat-
the other entities with elevated ICP, the treatment should ments [20, 35]. Yet, cases need to be selected carefully since
progress gradually with accurate monitoring of ICP in cases although functional outcome results after decompressive
where there is no reliable neurological assessment. Several craniectomy look promising, there is not enough evidence
groups tried to describe a decision-making mechanism to to recommend the routine use of this tool in the setting of
identify the children with potential elevated intracranial encephalitis [35]. In case the diagnosis is of a brain abscess
pressure before devastating result happen. In 2020, Kosten- the treatment paradigm usually involves tissue diagnosis
niemi et al. published their scale of MeningiSSS (Meningitis with or without mass reduction or complete removal of the
Swedish Survival Score) trying to predict those that will abscess followed with broad-spectrum antibiotics that will
benefit from strict monitoring of their ICP [41–44]. They be narrowed according to microbiology answers.
compared the results of their scale to different known scales
and were focused at the presence of altered mental status Stroke—malignant MCA, others
and leukopenia and circulatory distress, and found it use-
ful in identifying children later having to undergo invasive Pediatric ischemic stroke is a devastating disease with
procedures to monitor or manage their ICP as a result of significant mortality and morbidity. According to differ-
bacterial meningitis [41]. ent publications, the mortality rate 1 month after severe
Medical management of increased ICP includes differ- pediatric stroke is around 12%, and most of the survivors
ent methods for relieving intracranial pressure and preserve will have significant neurological deficits[51–53]. The
tissue oxygenation and blood supply [35]. Treatments, such annual incidence of pediatric stroke differentiates accord-
as hyperventilation, osmotherapy, corticosteroids, barbi- ing to etiology, with acute ischemic and hemorrhagic stroke
turate coma, and induced hypothermia, were described in being the most common (1–5 for hemorrhagic stroke and
previous reviews, and the overall management is similar to 2–13/100.000 children for ischemic stroke), while cerebral
TBI management. Corticosteroids are being widely used in venous thrombosis (CVT) is the least common as possible
different neurological and neurosurgical entities to reduce etiology (0.5–1.0 for every 100,000 children) [54–56]. Pedi-
cerebral edema. Their use in infectious disease is question- atric stroke is usually ischemic rather than hemorrhagic. The
able because of the possible increased viral or bacterial load treatment in pediatric stroke derived mainly from the adult

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literature but had come to the establishment of the treatment use of barbiturate come is as relevant as DC for intractable
paradigm that suggests the identification of the etiology, pos- elevated ICP in face of the outcome of both treatments, we
sible acute intervention, and mainly the prevention of further can summarize that only when maximal medical manage-
progression by diminution brain metabolic demands (pre- ment of ICP has failed, the use of DC should be considered
vention of drop in oxygen saturation, hyperthermia, hypo- or in children suffering from a pediatric stroke [67].
hyperglycemia, hypo- or hypertension, seizures, infections,
etc.) [57]. Large ischemic stroke can lead to a significant Sinus venous thrombus—spontaneous, trauma‑related
increase in the ICP, which might lead to brain herniation,
and significant morbidity, and even mortality [51]. Decom- Cerebral sinus vein thrombosis (CSVT) is a known phe-
pressive hemicraniectomy (DC) was shown already in sev- nomenon that can happen in a variety of clinical statuses.
eral publications to be an important and significant treatment The brain vasculature system allows the blood to be drained
modality to achieve immediate reduction for the rapid rise through the different venous sinuses. In case one of the
in ICP and is known to reduce mortality and improving out- major sinuses is occluded, there is a steep rise in ICP due
comes among adult patients with malignant stroke [58]. Yet, to lack of blood drainage from the brain. CSVT and arterial
the use of this radical tool is preserved for cases in which stroke differ for both the clinical evolution as well as the
more conservative methods, including osmolar treatment, natural history [68]. CSVT accounts for 0.5% of all stroke
prevention of hyperglycemia and fever, and even barbiturate cases [69]. The respective incidence in children and neo-
coma, failed to relieve the elevated ICP. The devastating nates is higher than in adults [70–72]. Most of the cases will
numbers of mortality and morbidity were the driving force involve either the superior sagittal sinus (SSS) or transverse
behind the randomized controlled trials (RCTs) DECIMAL, sinus (TS). Etiologies may vary widely. The most common
DESTINY, and HAMLET. These trials showed that the use causes are prothrombotic states, either genetically imposed
of DC can significantly reduce mortality (case fatality reduc- or acquired. Other pathologies include malignancy, hema-
tion closely estimated as 50–75%) without increasing the tological pathologies, inflammatory systemic disorders,
risk for severe functional outcomes. The results were for postoperative related, infections (i.e. mastoiditis), trauma,
patients under the age of 60 years and that was operated on jugular vein catheterization, and more [73–76]. Recent pub-
for decompressive craniectomy within 48 h from the stroke lication focused on the risk factors for CSVT among pediat-
[58, 59]. Most stroke patients will not develop the devas- ric patients found that in a multivariant analysis head/neck
tating elevation of ICP mandate treatment. In the special infection, head/neck trauma, and mechanical ventilation are
setting of large MCA (Middle cerebral artery) stroke, there independent risk factors for the development of pediatric
is the potential of developing malignant MCA syndrome, CSVT [72].
which is a life-threatening event because of acute elevation The management paradigm includes diagnosis by imag-
of intracranial pressure and brain herniation secondary to ing, usually either MRI with a specific focus on venous sys-
the development of severe brain swelling. The incidence of tem (MRV), CTV, or even venography (Fig. 1). The treat-
malignant MCA stroke in the pediatric population is much ment goal is to stop the progression of the thrombotic event
smaller in comparison to adults (2% vs. 10%) [60]. The use as well as understanding the causative etiology. In cases
of ICP monitoring is less common among these patients and where there is altered mental status and suspected elevated
the treatment decision is usually derived from clinical status ICP, urgent steps are taken to control ICP, measure it, and
and imaging [61]. Yet, in cases of more aggressive treat- actively lowering it [77]. In 2008 and later in 2011, sev-
ment like barbiturate come, ICP measurement might become eral important manuscripts were published with the recom-
significant for decision-making to anticipate the need for mended use of anticoagulation for children [78–81].
DC. The American Heart Association guideline for the early ICP management in the setting of sinus vein thrombosis
management of adults with ischemic stroke recommends DC includes several treatment options, such as acetazolamide
as a lifesaving measurement in cases of severe malignant administration, external ventricular drainage (that allows
stroke. As mentioned above, DC in the right cases within ICP monitoring as well as an effective treatment to lower
48 h will decrease mortality and can assist in preventing excessive pressure), CSF shunt insertion, or even DC [68].
further neurological deterioration [58, 62–65]. The use of Decompressive craniectomy was found to be effective for
DC in pediatric ischemic stroke has become more common CSVT when there is large parenchymal hemorrhage, or an
in recent years with acknowledgment of its benefits for the acute mass effect secondary to cerebral swelling and failure
adult population. There is no clear evidence regarding when of conservative treatment for decreasing intracranial pres-
DC should be done in case malignant stroke develops. Delay sure [82]. The use of thrombectomy either endovascular or
in utilizing DC can lead to a worse prognosis and in some surgical has been an issue for debate in recent years. Some
cases to hemorrhagic conversion [66]. When considering centers advocate the use of endovascular treatment first
all recent data, including the recent data suggesting that the when the patient starts to have neurological deterioration or

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Fig. 1  Transverse sinus throm-

bosis. Sinus vein thrombosis
can be seen in regular CT scan
as hyper density (a) around
the sinus (as a result of blood
stasis), and as a filling defect in
CT venogram (b)

have significant findings in imaging and conservative treat- > 70% of patients [91]. Yet, bilateral papilledema or optic
ment failed [83]. Yet, when taking the published data that disc swelling is the most common finding on an exam with
we have today, in cases where the pressure is building up a frequency of 87% in children [94]. In pre-pubertal chil-
within the brain in a more diffuse pattern, a better treat- dren, incidentally found bilateral papilledema is common
ment paradigm will be measuring neurological status and and it has been reported in as high as 33% of the cases
ICP when needed, use of mannitol or hypertonic saline, and in the literature. However, papilledema is not identified
even barbiturate coma. DC in these cases should be the last in every patient and although it is often found bilater-
result [84, 85]. ally, it can also be seen unilaterally. If papilledema is left
untreated, it can lead to sudden or gradual blindness as the
Idiopathic primary increased ICP increased pressure causes circumferential compression of
the retinal ganglion cells in the optic nerve [86]. Visual
Idiopathic intracranial hypertension (IIH) is diagnosed in field cuts are seen in 74–85% of patients on presentation
patients with elevated intracranial pressure with normal and the most common defect is an enlarged blind spot [92,
brain parenchyma and small ventricles that lack an identifi- 95, 96]. Transient visual obscurations (TVO) described as
able cause for elevated pressure. IIH is seen predominantly blacking out of the vision for approximately one second
in young women of childbearing age and among obese girls, are also widely reported and tend to be exacerbated by
however, it can be seen across all ages, genders, a wide range either straining, Valsalva, bending, etc. Blurry vision is a
of body mass indices (BMI), ethnicities, and socioeconomic very common, however, nonspecific complaint. Diplopia
statuses. The pathophysiology of IIH remains uncertain. has been reported in 16–42.3% of patients [90, 93, 97].
Incidence of IIH in pediatrics has been difficult to esti- Unilateral abducens palsy can present as esotropia and
mate given the paucity of appropriate studies in this pop- diplopia when looking towards the side of the affected
ulation. It has been reported that 37% of all cases of IIH eye. In the literature, cranial nerve VI palsies have been
are children, and 90% of these are between the ages of reported as low as 12% of patients and as high as 60% of
5–15 years old [86]. Previous studies found the incidence of cases [91, 93, 98, 99]. Tinnitus is also a common symptom
primary intracranial hypertension (PIH) to be 0.47–1.2 per seen in 40–50% of patients and is often pulsatile and uni-
100,000 children, higher than that of secondary intracranial lateral [90, 91]. Other symptoms of increased intracranial
hypertension (SIH), which was 0.32 per 100,000 children pressure are also seen, and in rare cases, even spontaneous
[87–89]. CSF leak can be the presenting symptom [92, 96, 98]. It
The presentation of patients with PIH varies with age. It is important to note that the symptoms can be similar to
is harder to diagnose in younger children due to more sub- those caused by posterior fossa lesions including but not
tle symptoms and the ability to communicate their symp- limited to cranial nerve VII palsy, ataxia, nuchal rigidity,
toms [90]. The most common presenting symptom and the Babinski sign [100].
most consistent symptom is headaches, which is present in The latest criteria include (1) signs and symptoms of ele-
30–96.5% of patients [91–93]. Headaches are described as vated intracranial pressure as discussed above, (2) lack of
worse in the morning and can be exacerbated by straining, focal neurologic deficits (excluding cranial nerve IV and VI
coughing, Valsalva, and bending down. Visual changes palsies), (3) normal CSF studies, (4) CSF opening pressure
are the second most common symptom and are present in of > 18 cm H20 in children < 8 years old or > 25 cm H20 in

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children > 8 years old or < 8 years old without optic nerve Treatment for IIH should be designed by a multidiscipli-
edema, and (5) normal or small ventricles [89]. nary team involving a primary care doctor, a neurologist,
Diagnostic studies consist of MRI to rule out a mass a dietitian, an ophthalmologist, and if there is an ultimate
lesion and a lumbar puncture for opening pressure (OP) and need for surgical intervention, neurosurgery. The main rea-
CSF studies. This is important to note in infants that have son to treat IIH is to prevent blindness and to provide pain
open sutures, as the increased ICP may lead to an increas- relief. The first line of treatment is medical management.
ing head circumference and a bulging fontanelle, but not In patients who are overweight, this consists of first and
papilledema [100]. Magnetic resonance venography (MRV) foremost a weight loss plan. It has been shown that weight
is the test of choice to rule out venous sinus thrombosis and loss of as little as 6% body weight can result in resolution of
is especially necessary for atypical patients, atypical pres- papilledema [106]. Alternatives for weight loss include diet,
entations, patients with recent infections in sinuses or ears, exercise, and in adults, it may include surgical interventions
patients refractory to routine medical treatment, and patients like laparoscopic banding and gastric bypass.
with a more sudden presentation. MRV has also been recom- Acetazolamide is the first-line medication for IIH in both
mended in children as these are considered atypical patients adults and children. Acetazolamide is a carbonic anhydrase
and data shows 11% of cases are positive for venous sinus inhibitor that leads to decreased CSF production [107]. If
thrombosis [100, 101]. acetazolamide is not effective, other possible medications
Imaging studies should reveal normal brain parenchyma are furosemide and methazolamide. Furosemide can be used
and normal to small ventricles. Findings that are common alone or with acetazolamide as a synergistic effect has been
in patients with intracranial hypertension include slit-like reported. It is important to note the possible side effects
ventricles, empty sella, protrusion of the optic nerve head of these medications. Acetazolamide can lead to a metal-
anteriorly, flattening of the posterior globe, dilatation of the lic taste and furosemide to hypokalemia. Given the need
optic nerve sheath, abnormal appearance of the optic nerve to monitor potassium levels and replace potassium accord-
through its orbital portion, enhancement of the posterior ingly, furosemide is considered a second-line medication
sclera (Fig. 2) [95, 102, 103]. Presence of these findings when treating children [108]. Topiramate and methazola-
assists in diagnosis, but their absence does not rule out IIH. mide are both weak carbonic anhydrase inhibitors used as
A lumbar puncture (LP) serves as a diagnostic test, may well. Octreotide can be used in adults, however, in children,
provide patients with temporary relief of symptoms caused it is not recommended given that it inhibits growth hormone
by elevated ICP, and it also assists in treatment decisions as and IGF-1 [109]. Lastly, corticosteroids can be used in com-
by removing some CSF it demonstrates whether CSF diver- bination with acetazolamide in cases of sudden rapid visual
sion would be effective. However, it is important to note that decline [90].
the patient’s response to the LP is not diagnostic of IIH. The Surgical interventions are indicated if medical manage-
pressure would return to what it was prior to LP within 1–2 h ment fails if a patient does not tolerate medical manage-
[104, 105]. In equivocal cases, the intracranial pressure can ment, if papilledema is severe, or if vision loss is rapidly
be monitored for an extended period via lumbar drain or progressing as weight loss and medications take some time
intracranial pressure monitor. to work [90]. Options for surgical interventions include (1)

Fig. 2  Idiopathic intracranial

pressure. Previously known as
pseudotumor cerebri, this entity
by definition should be without
any intracranial etiology that
can result in mass effect. One of
the signs being used in recent
years is the T2 changes in the
optic nerve sheath (a—axial,
b—coronal), as a sign for
intracranial pressure and builds
up of papillary edema

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830 Acta Neurologica Belgica (2021) 121:823–836

CSF diversion via ventriculoperitoneal shunt (VPS) or lum- has been studied in patients with craniosynostosis and
bar-peritoneal shunt (LPS) placement and (2) optic nerve it can be used to diagnose elevated ICP and make treat-
sheath fenestration (ONSF). Serial lumbar punctures are ment decisions regarding surgical intervention and tim-
advocate by some as spontaneous remissions are common ing of surgery if determined necessary. The threshold for
within months to a year of onset. Surgeries like subtemporal elevated ICP differs across the literature and across age
or suboccipital decompressions are not in the frontline of the groups, which limits the generalization of study results for
surgical armamentarium and are used rarely. guidelines and the application of these results in general
CSF diversion is ideal for patients that complain of practice. The most common parameters in the literature
headaches as their primary symptom. Shunting decreases are as follows: ICP is normal if it is < 10 mmHg, ICP is
intracranial pressure and helps relieve both headaches and borderline if it measures 10–15 mmHg, and ICP is con-
papilledema. Debate still exists regarding what type of shunt sidered elevated if > 15 mmHg [115, 117–119]. Yet others
is better for patients with IIH. Abubaker et al. compared VPS suggested that a more sensitive way of diagnosing patients
and LPS. The study determined that VPS has a failure rate with elevated ICP is to calculate the frequency of ICP
of 14% and a revision rate of 30% whereas LPS has a failure elevations > 20 mmHg, rather than making the diagnosis
rate of 11% and a revision rate of 60% [110]. based on the calculated mean ICP [120].
Patients should be followed for at least 2 years with serial The expected findings of increased ICP and imaging
imaging to rule out any obscure, developing, or difficult to reflective of increased ICP are not common in patients
diagnose the secondary cause of intracranial hypertension with craniosynostosis [114, 118]. Possible symptoms of
[86]. Outcome data are scarce for the pediatric population. increased ICP in these patients are persistent headaches,
43–68% of adult patients describe a persistent, yet differ- sleep disturbance, irritability, nausea, deteriorating atten-
ent headache after treatment [100]. Papilledema does not tion span or academic performance, mental delay [120];
resolve in about 15% of patients [86]. Permanent visual loss however, these cognitive declines and mental delay find-
is reported in 2–24% of patients in the literature [86]. Esti- ings are wildly debated in the literature as to their associa-
mates for recurrence are around 18–20%, but a relationship tion with increased ICP and the long-term effects of ICP
between BMI and risk of recurrence has not been identified elevations on function and development in the presence
[111]. of other factors that can lead to these same developmental
issues [116]. Papilledema is not commonly identified in
Craniosynostosis young children with non-syndromic craniosynostosis and
raised ICP [118, 121]. On imaging, one can see decreased
Craniosynostosis is a condition that is diagnosed when- subarachnoid spaces, sulcal effacement, beaten copper
ever there is premature closure of one or more of the skull appearance of the calvarium with a sensitivity of 82% and
sutures. Incidence has been reported as 1 in 2000–2500 live specificity of 27% [118]. Tuite et al. studied 123 patients
births [112]. Craniosynostosis is classified by the specific with craniosynostosis focusing on imaging findings and
suture or sutures involved, and it can be syndromic or non- ICP [119]. Certain imaging patterns were found to be
syndromic, with 80–90% of hospitalized children having the normal in different age groups and not others. They iden-
nonsyndromic subtype [112, 113]. Craniosynostosis can lead tified that in children < 18 months old diffuse beaten cop-
to increased ICP, especially in syndromic craniosynostosis per patterns, effacement of the basal cisterns or the sulci
cases. In this subgroup, increased ICP has been reported were strong predicting factors for the presence of elevated
in 30–50% of cases [114]. Elevated ICP is more common ICP [122]. It is crucial to note that those findings did not
in patients with multiple sutures that are prematurely fused predict elevated ICP in older children, as they are seen in
than in patients with single fused sutures [115]. However, these patients even in the presence of normal ICP. Hydro-
elevated ICP has been reported in 11–14% of cases with cephalus was the most sensitive indicator of elevated ICP
a single fused suture [114]. Craniofacial dysmorphic syn- when accounting for all patients; however, they found that
dromes are typically associated with the fusion of multiple hydrocephalus only detected 40% of patients with elevated
sutures and special care has to be taken when evaluating ICP. They noted that suture diastasis and dorsum sella
these patients for interventions as their risk for increased erosion had a specificity of at least 90% for elevated ICP
ICP is higher than single suture craniosynostosis (i.e. Crou- [119]. When analyzing imaging findings for patients with
zon, Apert, Pfeiffer, Kleeblattschädel syndromes, amongst craniosynostosis, age is a very important factor that assists
others). in determining the relevance of any findings and whether
Increases in ICP can lead to abnormal development of it will change the treatment plan.
the brain with significant consequences for these children The gold standard to diagnose elevated ICP is invasive
[116]. An older study identified that for the first 6 years of monitor placement. ICP monitoring is indicated for chil-
life, ICP increased with age [115, 116]. ICP monitoring dren who present with persistent symptoms of increased

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Acta Neurologica Belgica (2021) 121:823–836 831

ICP in the absence of any objective symptoms on imaging Hydrocephalus

or ophthalmologic evaluation [117].
Surgery for correction of craniosynostosis is only nec- Hydrocephalus is a well-known pathology in the pediat-
essary in about 20% of cases [86]. Most children with mild ric population. Many etiologies can lead to hydrocephalus
craniosynostosis do not show significant symptoms, even among the pediatric population. A full discussion of this
in the presence of elevated ICP. In the presence of elevated topic is beyond the scope of this review.
ICP, early surgery is indicated for cranial vault expansion
(for kids with fused sutures). Cranial vault expansion has
been shown to relieve symptoms of increased ICP [123].
Conclusion

Chiari malformation Increased intracranial pressure is a potentially devastating

process, especially in the young population. Different etiolo-
A Chiari malformation is characterized typically by a gies can lead to increased pressure due to brain edema, mass
hindbrain abnormality and CSF flow disturbance. There effect, or obstructive hydrocephalus. When clinical signs and
are several types, from Chiari 0–Chiari IV; however, they symptoms suggesting the rise of intracranial pressure are
do not relate to each other embryologically [124]. Treat- present, urgent measures need to be taken to preserve cere-
ment via posterior fossa decompression relieves symptoms bral tissue perfusion, oxygenation, and hence brain function.
and reduction in the size of the syrinx [125]. A Chiari I
malformation, or simply a Chiari malformation, is the most
common type and it is defined by elongated “pegged” cer-
ebellar tonsils with > 5 mm of tonsillar herniation through Summary
the foramen magnum in a way that creates compaction of
the foramen magnum. It presents later in life than the other • The gold standard to diagnose elevated ICP is invasive
types, typically in adulthood during the second or third monitor placement
decade of life [124, 126]. The most common presenting • The treatment is a tier-based treatment similar to the trau-
symptoms are pain, most commonly suboccipital pressure- matic brain injury guidelines
like headaches (81% of cases) exacerbated by straining, • For Herpes simplex encephalitis, acyclovir therapy
physical activity, Valsalva, and bending down [126]. Chi- dramatically reduces mortality, and around half of the
ari, I malformation is associated with syringomyelia in patients return to normal function.
20–85% of cases [124, 127, 128]. The alterations of CSF • Some studies are supporting the utility of decompressive
flow in these patients are believed to cause the syrinx. craniotomy in severe encephalitis, but true evidence is
The surgical intervention in Chiari malformation is lacking.
intended to decompress the posterior fossa and brainstem • For adults with malignant stroke, decompressive hemi-
and reestablish CSF flow. Surgery includes a suboccipi- craniectomy is an effective and recommended treatment
tal decompression and depending on the case, a cervical for elevated ICP that failed conservative treatment, which
laminectomy, duraplasty, and tonsillar shrinking. Asymp- can reduce mortality and improve functional outcome.
tomatic patients do not require treatment. It is recommended to consider the same treatment meth-
The need for CSF diversion in those patients that under- odology in the pediatric population, although the lack of
went decompression surgery is not clear. The previous age-specific data.
publication found a significant abnormal pulsatile pres- • For patients with CSVT, decompressive craniectomy
sure gradient between the intracranial and lumbar com- should be considered in the presence of large mass effect
partments, which is even worse in patients with a syrinx (e.g., intracerebral hemorrhage or malignant cerebral
[129]. Yet, the second intervention for CSF diversion after edema) with signs for decompensation (pending hernia-
decompression surgery is rare [130]. tion).
Acquired Chiari, I malformations can occur when there • For idiopathic intracranial hypertension, surgical inter-
is a significant gradient between the intracranial and cer- ventions are only indicated if medical management fails,
vical compartments. This can occur in the setting of over if a patient does not tolerate medical management, if
drainage from a lumboperitoneal shunt or in patients with papilledema is severe, or if vision loss is rapidly pro-
CSF leaks. Patients with hydrocephalus or idiopathic gressing as weight loss and medications take some time
intracranial hypertension can also develop downward her- to work.
niation of tonsils and an acquired Chiari I malformation
[128].

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832 Acta Neurologica Belgica (2021) 121:823–836

Author contributions NS—Text drafting, text revision, conception 12. Kochanek PM, Tasker RC, Carney N, Totten AM, Adelson PD,
and design of the work, literature review, supervising, image editing. Selden NR et al (2019) Guidelines for the management of pediat-
MM-S—Text drafting and editing, literature review. BO—Text draft- ric severe traumatic brain injury, third edition: update of the brain
ing and editing, literature review. GIJ—Text revision, conception and trauma foundation guidelines, executive summary. Neurosurgery.
design of the work, supervising and final approval for publication, https://​doi.​org/​10.​1093/​neuros/​nyz051
image editing. 13. Yildizdas D, Altunbasak S, Celik U, Herguner O (2006) Hyper-
tonic saline treatment in children with cerebral edema. Indian
Funding None. Pediatr 43(9):771–779
14. Marcin JP, Glaser N, Barnett P, McCaslin I, Nelson D, Trainor
J et al (2002) Factors associated with adverse outcomes in chil-
Compliance with ethical standards dren with diabetic ketoacidosis-related cerebral edema. J Pediatr
141(6):793–797
Conflict of interest Dr. George Jallo has a consulting agreement with 15. Sherry NA, Levitsky LL (2008) Management of diabetic ketoaci-
Integra. Drs. Shimony, Martinez-Sosa, Osburn have no financial rela- dosis in children and adolescents. Paediatr Drugs 10(4):209–215
tionships relevant to this article. 16. Burgess S, Abu-Laban RB, Slavik RS, Vu EN, Zed PJ (2016)
A systematic review of randomized controlled trials comparing
Ethical approval Not applicable or needed for this article. hypertonic sodium solutions and mannitol for traumatic brain
injury: implications for emergency department management. Ann
Informed consent Not applicable or needed for this article Pharmacother 50(4):291–300. https://​doi.​org/​10.​1177/​10600​
28016​628893
17. Gunn AJ (2000) Cerebral hypothermia for prevention of
brain injury following perinatal asphyxia. Curr Opin Pediatr
12(2):111–115
References 18. Gunn AJ, Gluckman PD, Gunn TR (1998) Selective head cool-
ing in newborn infants after perinatal asphyxia: a safety study.
1. Cadena R, Shoykhet M, Ratcliff JJ (2017) Emergency neurologi- Pediatrics 102(4 Pt 1):885–892
cal life support: intracranial hypertension and herniation. Neuro- 19. Adelson PD, Wisniewski SR, Beca J, Brown SD, Bell M, Mui-
crit Care. https://​doi.​org/​10.​1007/​s12028-​017-​0454-z zelaar JP et al (2013) Comparison of hypothermia and normo-
2. Klatzo I (1967) Presidental address. Neuropathological aspects thermia after severe traumatic brain injury in children (Cool
of brain edema. J Neuropathol Exp Neurol. 26(1):1–14 Kids): a phase 3, randomised controlled trial. Lancet Neurol
3. Simard JM, Kent TA, Chen M, Tarasov KV, Gerzanich V (2007) 12(6):546–553. https://d​ oi.o​ rg/1​ 0.1​ 016/S
​ 1474-4​ 422(13)7​ 0077-2
Brain oedema in focal ischaemia: molecular pathophysiology and 20. Pérez-Bovet J, Garcia-Armengol R, Buxó-Pujolràs M, Lorite-
theoretical implications. Lancet Neurol 6(3):258–268. https://d​ oi.​ Díaz N, Narváez-Martínez Y, Caro-Cardera JL et al (2012)
org/​10.​1016/​S1474-​4422(07)​70055-8 Decompressive craniectomy for encephalitis with brain her-
4. Steinborn M, Friedmann M, Makowski C, Hahn H, Hapfelmeier niation: case report and review of the literature. Acta Neu-
A, Juenger H (2016) High resolution transbulbar sonography rochir (Wien) 154(9):1717–1724. https:// ​ d oi. ​ o rg/ ​ 1 0. ​ 1 007/​
in children with suspicion of increased intracranial pressure. s00701-​012-​1323-3
Childs Nerv Syst 32(4):655–660. https:// ​ d oi. ​ o rg/ ​ 1 0. ​ 1 007/​ 21. Bohn DJ, Biggar WD, Smith CR, Conn AW, Barker GA (1986)
s00381-​015-​3001-2 Influence of hypothermia, barbiturate therapy, and intracranial
5. Luerssen TG (1997) Intracranial pressure: current status in moni- pressure monitoring on morbidity and mortality after near-
toring and management. Semin Pediatr Neurol 4(3):146–155 drowning. Crit Care Med 14(6):529–534. https://​doi.​org/​10.​
6. Wiegand C, Richards P (2007) Measurement of intracranial pres- 1097/​00003​246-​19860​6000-​00002
sure in children: a critical review of current methods. Dev Med 22. Paschoal FM, Nogueira RC, Ronconi KA, de Lima OM, Teixeira
Child Neurol 49(12):935–941. https://​doi.​org/​10.​1111/j.​1469-​ MJ, Bor-Seng-Shu E (2016) Multimodal brain monitoring in ful-
8749.​2007.​00935.x minant hepatic failure. World J Hepatol. 8(22):915–923. https://​
7. Sala F, Abbruzzese C, Galli D, Grimaldi M, Abate MG, Sganz- doi.​org/​10.​4254/​wjh.​v8.​i22.​915
erla EP et al (2009) Intracranial pressure monitoring in pediatric 23. Cook AM, Morgan Jones G, Hawryluk GWJ, Mailloux P,
bacterial meningitis: a fancy or useful tool? A case report. Min- McLaughlin D, Papangelou A et al (2020) Guidelines for the
erva Anestesiol 75(12):746–749 acute treatment of cerebral edema in neurocritical care patients.
8. Le Roux PD, Jardine DS, Kanev PM, Loeser JD (1991) Pediat- Neurocrit Care 32(3):647–666. https:// ​ d oi. ​ o rg/ ​ 1 0. ​ 1 007/​
ric intracranial pressure monitoring in hypoxic and nonhypoxic s12028-​020-​00959-7
brain injury. Childs Nerv Syst 7(1):34–39. https://​doi.​org/​10.​ 24. Flores-Cordero JM, Amaya-Villar R, Rincón-Ferrari MD,
1007/​BF002​63831 Leal-Noval SR, Garnacho-Montero J, Llanos-Rodríguez AC
9. Odetola FO, Bratton SL (2005) Characteristics and immediate et al (2003) Acute community-acquired bacterial meningitis in
outcome of childhood meningitis treated in the pediatric inten- adults admitted to the intensive care unit: clinical manifesta-
sive care unit. Intensive Care Med 31(1):92–97. https://​doi.​org/​ tions, management and prognostic factors. Intensive Care Med
10.​1007/​s00134-​004-​2501-4 29(11):1967–1973. https://​doi.​org/​10.​1007/​s00134-​003-​1935-4
10. Carney N, Totten AM, O’Reilly C, Ullman JS, Hawryluk GW, 25. Hsu CL, Chang CH, Wong KN, Chen KY, Yu CJ, Yang PC
Bell MJ et al (2017) Guidelines for the management of severe (2009) Management of severe community-acquired septic men-
traumatic brain injury, fourth edition. Neurosurgery 80(1):6–15. ingitis in adults: from emergency department to intensive care
https://​doi.​org/​10.​1227/​NEU.​00000​00000​001432 unit. J Formos Med Assoc 108(2):112–118. https://​doi.​org/​10.​
11. Kochanek PM, Carney N, Adelson PD, Ashwal S, Bell MJ, Brat- 1016/​S0929-​6646(09)​60041-3
ton S et al (2012) Guidelines for the acute medical management 26. Abulhasan YB, Al-Jehani H, Valiquette MA, McManus A,
of severe traumatic brain injury in infants, children, and adoles- Dolan-Cake M, Ayoub O et al (2013) Lumbar drainage for
cents—second edition. Pediatr Crit Care Med 13(Suppl 1):S1-82. the treatment of severe bacterial meningitis. Neurocrit Care
https://​doi.​org/​10.​1097/​PCC.​0b013​e3182​3f435c 19(2):199–205. https://​doi.​org/​10.​1007/​s12028-​013-​9853-y

13
Acta Neurologica Belgica (2021) 121:823–836 833

27. Franco-Paredes C, Lammoglia L, Hernández I, Santos-Preciado morbidity in guatemalan children with acute bacterial menin-
JI (2008) Epidemiology and outcomes of bacterial meningitis in gitis. Pediatr Infect Dis J 34(7):724–8. https://​doi.​org/​10.​1097/​
Mexican children: 10-year experience (1993–2003). Int J Infect INF.​00000​00000​000720
Dis 12(4):380–386. https://​doi.​org/​10.​1016/j.​ijid.​2007.​09.​012 44. Pelkonen T, Roine I, Monteiro L, Cruzeiro ML, Pitkäranta
28. Lindvall P, Ahlm C, Ericsson M, Gothefors L, Naredi S, Koski- A, Kataja M et al (2012) Prognostic accuracy of five simple
nen LO (2004) Reducing intracranial pressure may increase sur- scales in childhood bacterial meningitis. Scand J Infect Dis
vival among patients with bacterial meningitis. Clin Infect Dis 44(8):557–65. https://​doi.​org/​10.​3109/​00365​548.​2011.​652666
38(3):384–390. https://​doi.​org/​10.​1086/​380970 45. Ramos-Estebanez C, Lizarraga KJ, Merenda A (2014) A sys-
29. Kim YS, Sheldon RA, Elliott BR, Liu Q, Ferriero DM, Täu- tematic review on the role of adjunctive corticosteroids in her-
ber MG (1995) Brain injury in experimental neonatal menin- pes simplex virus encephalitis: is timing critical for safety and
gitis due to group B streptococci. J Neuropathol Exp Neurol efficacy? Antivir Ther 19(2):133–9. https://​doi.​org/​10.​3851/​
54(4):531–539 IMP26​83
30. Breeze RE, McComb JG, Hyman S, Gilles FH (1989) CSF pro- 46. Kamei S, Sekizawa T, Shiota H, Mizutani T, Itoyama Y,
duction in acute ventriculitis. J Neurosurg 70(4):619–622. https://​ Takasu T et al (2005) Evaluation of combination therapy
doi.​org/​10.​3171/​jns.​1989.​70.4.​0619 using aciclovir and corticosteroid in adult patients with her-
31. Whitley RJ, Kimberlin DW (2005) Herpes simplex encephalitis: pes simplex virus encephalitis. J Neurol Neurosurg Psychiatry
children and adolescents. Semin Pediatr Infect Dis 16(1):17–23. 76(11):1544–9. https://​doi.​org/​10.​1136/​jnnp.​2004.​049676
https://​doi.​org/​10.​1053/j.​spid.​2004.​09.​007 47. Fitch MT, van de Beek D (2008) Drug Insight: steroids in CNS
32. Steiner I, Budka H, Chaudhuri A, Koskiniemi M, Sainio K, infectious diseases—new indications for an old therapy. Nat
Salonen O et al (2010) Viral meningoencephalitis: a review of Clin Pract Neurol 4(2):97–104. https://​doi.​org/​10.​1038/​ncpne​
diagnostic methods and guidelines for management. Eur J Neu- uro07​13
rol 17(8):999-e57. https://​doi.​org/​10.​1111/j.​1468-​1331.​2010.​ 48. Tiroumourougane SV, Raghava P, Srinivasana S, Badrinath
02970.x (2003) Management parameters affecting the outcome of Japa-
33. Singhi P, Saini AG, Sahu JK, Kumar N, Vyas S, Vasishta RK nese encephalitis. J Trop Pediatr 49(3):153–6
et al (2015) Unusual clinical presentation and role of decompres- 49. Solomon T, Dung NM, Kneen R, Thao T, Gainsborough M, Nis-
sive craniectomy in herpes simplex encephalitis. J Child Neurol alak A et al (2002) Seizures and raised intracranial pressure in
30(9):1204–7. https://​doi.​org/​10.​1177/​08830​73814​546688 Vietnamese patients with Japanese encephalitis. Brain 125(Pt
34. Barnett GH, Ropper AH, Romeo J (1988) Intracranial pressure 5):1084–93
and outcome in adult encephalitis. J Neurosurg 68(4):585–8. 50. Schwab S, Jünger E, Spranger M, Dörfler A, Albert F, Steiner
https://​doi.​org/​10.​3171/​jns.​1988.​68.4.​0585 HH et al (1997) Craniectomy: an aggressive treatment approach
35. Safain MG, Roguski M, Kryzanski JT, Weller SJ (2015) A in severe encephalitis. Neurology 48(2):412–7
review of the combined medical and surgical management in 51. Shah S, Murthy SB, Whitehead WE, Jea A, Nassif LM (2013)
patients with herpes simplex encephalitis. Clin Neurol Neurosurg Decompressive hemicraniectomy in pediatric patients with
128:10–6. https://​doi.​org/​10.​1016/j.​cline​uro.​2014.​10.​015 malignant middle cerebral artery infarction: case series and
36. Granget E, Milh M, Pech-Gourg G, Paut O, Girard N, Lena review of the literature. World Neurosurg 80(1–2):126–33.
G et al (2012) Life-saving decompressive craniectomy for https://​doi.​org/​10.​1016/j.​wneu.​2013.​06.​001
acute disseminated encephalomyelitis in a child: a case report. 52. Farooq MU, Abbed KM, Fletcher JJ (2009) Decompressive hemi-
Childs Nerv Syst 28(7):1121–4. https:// ​ d oi. ​ o rg/ ​ 1 0. ​ 1 007/​ craniectomy in a 19-month-old female after malignant cerebral
s00381-​012-​1733-9 infarction. Pediatr Neurosurg 45(2):146–50. https://​doi.​org/​10.​
37. Alexander M, Murthy JM (2011) Acute disseminated encephalo- 1159/​00020​9654
myelitis: treatment guidelines. Ann Indian Acad Neurol 14(Suppl 53. Gandhi SK, McKinney JS, Sedjro JE, Cosgrove NM, Cabrera J,
1):S60-4. https://​doi.​org/​10.​4103/​0972-​2327.​83095 Kostis JB et al (2012) Temporal trends in incidence and long-
38. Borras-Novell C, García Rey E, Perez Baena LF, Jordan Garcia I, term case fatality of stroke among children from 1994 to 2007.
Catella Cahiz D, Cambra F (2015) Therapeutic plasma exchange Neurology 78(24):1923–9. https://​doi.​org/​10.​1212/​WNL.​0b013​
in acute disseminated encephalomyelitis in children. J Clin Apher e3182​59e25c
30(6):335–9. https://​doi.​org/​10.​1002/​jca.​21388 54. Simma B, Höliner I, Luetschg J (2013) Therapy in pediatric
39. Koelman DL, Chahin S, Mar SS, Venkatesan A, Hoganson GM, stroke. Eur J Pediatr 172(7):867–75. https://​doi.​org/​10.​1007/​
Yeshokumar AK et al (2016) Acute disseminated encephalo- s00431-​012-​1863-9
myelitis in 228 patients: a retrospective, multicenter US study. 55. Agrawal N, Johnston SC, Wu YW, Sidney S, Fullerton HJ (2009)
Neurology 86(22):2085–93. https://d​ oi.o​ rg/1​ 0.1​ 212/W ​ NL.0​ 0000​ Imaging data reveal a higher pediatric stroke incidence than prior
00000​002723 US estimates. Stroke 40(11):3415–21. https://​doi.​org/​10.​1161/​
40. Kumar G, Kalita J, Misra UK (2009) Raised intracranial pressure STROK​EAHA.​109.​564633
in acute viral encephalitis. Clin Neurol Neurosurg 111(5):399– 56. Steinlin M, Pfister I, Pavlovic J, Everts R, Boltshauser E, Capone
406. https://​doi.​org/​10.​1016/j.​cline​uro.​2009.​03.​004 Mori A et al (2005) The first three years of the Swiss Neuropae-
41. Johansson Kostenniemi U, Karlsson L, Silfverdal SA, Mehle C diatric Stroke Registry (SNPSR): a population-based study of
(2020) MeningiSSS: a new predictive score to support decision incidence, symptoms and risk factors. Neuropediatrics 36(2):90–
on invasive procedures to monitor or manage the intracerebral 7. https://​doi.​org/​10.​1055/s-​2005-​837658
pressure in children with bacterial meningitis. Neurocrit Care 57. Lanthier S, Armstrong D, Domi T, deVeber G (2005) Post-var-
32(2):586–95. https://​doi.​org/​10.​1007/​s12028-​019-​00792-7 icella arteriopathy of childhood: natural history of vascular ste-
42. Bijlsma MW, Brouwer MC, Bossuyt PM, Heymans MW, van nosis. Neurology 64(4):660–3. https://​doi.​org/​10.​1212/​01.​WNL.​
der Ende A, Tanck MW et al (2016) Risk scores for outcome in 00001​51851.​66154.​27
bacterial meningitis: systematic review and external validation 58. Vahedi K, Hofmeijer J, Juettler E, Vicaut E, George B, Algra A
study. J Infect 73(5):393–401. https://d​ oi.o​ rg/1​ 0.1​ 016/j.j​ inf.2​ 016.​ et al (2007) Early decompressive surgery in malignant infarc-
08.​003 tion of the middle cerebral artery: a pooled analysis of three
43. Olson D, Lamb MM, Gaensbauer JT, Todd JK, Halsey NA, randomised controlled trials. Lancet Neurol 6(3):215–22. https://​
Asturias EJ et al (2015) Risk factors for death and major doi.​org/​10.​1016/​S1474-​4422(07)​70036-4

13
834 Acta Neurologica Belgica (2021) 121:823–836

59. Marklund N (2017) Decompressive craniectomy, ICP monitor- dural sinus thrombosis: results of the International Study on
ing and secondary necrosectomy as treatment options in patients Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke
presenting with malignant ischemic infarctions extending beyond 35(3):664–70. https://​doi.​org/​10.​1161/​01.​STR.​00001​17571.​
the middle cerebral artery territory. Acta Neurochir (Wien). 76197.​26
https://​doi.​org/​10.​1007/​s00701-​017-​3331-9 75. Ferro JM (2006) Causes, predictors of death, and antithrombotic
60. Smith SE, Kirkham FJ, Deveber G, Millman G, Dirks PB, Wirrell treatment in cerebral venous thrombosis. Clin Adv Hematol
E et al (2011) Outcome following decompressive craniectomy for Oncol 4(10):732–3
malignant middle cerebral artery infarction in children. Dev Med 76. Stam J (2005) Thrombosis of the cerebral veins and sinuses.
Child Neurol 53(1):29–33. https://​doi.​org/​10.​1111/j.​1469-​8749.​ N Engl J Med 352(17):1791–8. https://​doi.​org/​10.​1056/​NEJMr​
2010.​03775.x a0423​54
61. Schwab S, Aschoff A, Spranger M, Albert F, Hacke W (1996) 77. Lebas A, Chabrier S, Fluss J, Gordon K, Kossorotoff M, Nowak-
The value of intracranial pressure monitoring in acute hemi- Göttl U et al (2012) EPNS/SFNP guideline on the anticoagulant
spheric stroke. Neurology 47(2):393–8 treatment of cerebral sinovenous thrombosis in children and neo-
62. Vahedi K, Vicaut E, Mateo J, Kurtz A, Orabi M, Guichard JP nates. Eur J Paediatr Neurol 16(3):219–28. https://​doi.​org/​10.​
et al (2007) Sequential-design, multicenter, randomized, con- 1016/j.​ejpn.​2012.​02.​005
trolled trial of early decompressive craniectomy in malignant 78. Monagle P, Chalmers E, Chan A, deVeber G, Kirkham F, Mas-
middle cerebral artery infarction (DECIMAL Trial). Stroke sicotte P et al (2008) Antithrombotic therapy in neonates and
38(9):2506–17. https://​doi.​org/​10.​1161/​STROK​EAHA.​107.​ children: American college of chest physicians evidence-based
485235 clinical practice guidelines. Chest 133(6 Suppl):887S-968S.
63. Hofmeijer J, Kappelle LJ, Algra A, Amelink GJ, van Gijn J, van https://​doi.​org/​10.​1378/​chest.​08-​0762
der Worp HB et al (2009) Surgical decompression for space- 79. Roach ES, Golomb MR, Adams R, Biller J, Daniels S, Deveber
occupying cerebral infarction (the Hemicraniectomy After Mid- G et al (2008) Management of stroke in infants and children: a
dle Cerebral Artery infarction with Life-threatening Edema scientific statement from a Special Writing Group of the Ameri-
Trial [HAMLET]): a multicentre, open, randomised trial. Lan- can Heart Association Stroke Council and the Council on Car-
cet Neurol 8(4):326–33. https://d​ oi.o​ rg/1​ 0.1​ 016/S
​ 1474-4​ 422(09)​ diovascular Disease in the Young. Stroke 39(9):2644–91. https://​
70047-X doi.​org/​10.​1161/​STROK​EAHA.​108.​189696
64. Jüttler E, Bösel J, Amiri H, Schiller P, Limprecht R, Hacke W 80. Saposnik G, Barinagarrementeria F, Brown RD, Bushnell CD,
et al (2011) DESTINY II: DEcompressive Surgery for the Treat- Cucchiara B, Cushman M et al (2011) Diagnosis and manage-
ment of malignant INfarction of the middle cerebral arterY II. Int ment of cerebral venous thrombosis: a statement for healthcare
J Stroke 6(1):79–86. https://​doi.​org/​10.​1111/j.​1747-​4949.​2010.​ professionals from the American Heart Association/American
00544.x Stroke Association. Stroke 42(4):1158–92. https://​doi.​org/​10.​
65. Jüttler E, Unterberg A, Woitzik J, Bösel J, Amiri H, Sakowitz 1161/​STR.​0b013​e3182​0a8364
OW et al (2014) Hemicraniectomy in older patients with exten- 81. Chalmers E, Ganesen V, Liesner R, Maroo S, Nokes T, Saun-
sive middle-cerebral-artery stroke. N Engl J Med 370(12):1091– ders D et al (2011) Guideline on the investigation, management
100. https://​doi.​org/​10.​1056/​NEJMo​a1311​367 and prevention of venous thrombosis in children. Br J Haema-
66. Grant GA (2015) Is there a role for decompressive craniectomy tol 154(2):196–207. https://​doi.​org/​10.​1111/j.​1365-​2141.​2010.​
in children after stroke? World Neurosurg 83(1):44–5. https://​ 08543.x
doi.​org/​10.​1016/j.​wneu.​2013.​08.​049 82. Einhäupl K, Bousser MG, de Bruijn SF, Ferro JM, Martinelli I,
67. Hutchinson PJ, Kolias AG, Timofeev IS, Corteen EA, Czos- Masuhr F et al (2006) EFNS guideline on the treatment of cer-
nyka M, Timothy J et al (2016) Trial of decompressive craniec- ebral venous and sinus thrombosis. Eur J Neurol 13(6):553–9.
tomy for traumatic intracranial hypertension. N Engl J Med https://​doi.​org/​10.​1111/j.​1468-​1331.​2006.​01398.x
375(12):1119–30. https://​doi.​org/​10.​1056/​NEJMo​a1605​215 83. Nyberg EM, Case D, Nagae LM, Honce JM, Reyenga W, Seinfeld
68. Filippidis A, Kapsalaki E, Patramani G, Fountas KN (2009) J et al (2017) The addition of endovascular intervention for dural
Cerebral venous sinus thrombosis: review of the demographics, venous sinus thrombosis: single-center experience and review of
pathophysiology, current diagnosis, and treatment. Neurosurg literature. J Stroke Cerebrovasc Dis 26(10):2240–7. https://​doi.​
Focus 27(5):E3. https://​doi.​org/​10.​3171/​2009.8.​FOCUS​09167 org/​10.​1016/j.​jstro​kecer​ebrov​asdis.​2017.​05.​006
69. Bousser MG, Ferro JM (2007) Cerebral venous thrombosis: an 84. Ferro JM, Crassard I, Coutinho JM, Canhão P, Barinagarremen-
update. Lancet Neurol 6(2):162–70. https://​doi.​org/​10.​1016/​ teria F, Cucchiara B et al (2011) Decompressive surgery in cer-
S1474-​4422(07)​70029-7 ebrovenous thrombosis: a multicenter registry and a systematic
70. Agnelli G, Verso M (2008) Epidemiology of cerebral vein and review of individual patient data. Stroke 42(10):2825–31. https://​
sinus thrombosis. Front Neurol Neurosci 23:16–22. https://​doi.​ doi.​org/​10.​1161/​STROK​EAHA.​111.​615393
org/​10.​1159/​00011​1258 85. Ferro JM, Bousser MG, Canhão P, Coutinho JM, Crassard I,
71. deVeber G, Andrew M, Adams C, Bjornson B, Booth F, Buck- Dentali F et al (2017) European Stroke Organization guideline
ley DJ et al (2001) Cerebral sinovenous thrombosis in children. for the diagnosis and treatment of cerebral venous thrombosis—
N Engl J Med 345(6):417–23. https://​doi.​org/​10.​1056/​NEJM2​ endorsed by the European Academy of Neurology. Eur J Neurol
00108​09345​0604 24(10):1203–13. https://​doi.​org/​10.​1111/​ene.​13381
72. Sellers A, Meoded A, Quintana J, Jallo G, Amankwah E, Nguyen 86. Greenberg MS (2016) Handbook of neurosurgery.
ATH et al (2020) Risk factors for pediatric cerebral sinovenous 87. Gordon K (1997) Pediatric pseudotumor cerebri: descriptive
thrombosis: A case–control study with case validation. Thromb epidemiology. Can J Neurol Sci 24(3):219–21
Res 194:8–15. https://​doi.​org/​10.​1016/j.​throm​res.​2020.​06.​013 88. Bursztyn LL, Sharan S, Walsh L, LaRoche GR, Robitaille J, De
73. Canhão P, Ferro JM, Lindgren AG, Bousser MG, Stam J, Bari- Becker I (2014) Has rising pediatric obesity increased the inci-
nagarrementeria F et al (2005) Causes and predictors of death dence of idiopathic intracranial hypertension in children? Can J
in cerebral venous thrombosis. Stroke 36(8):1720–5. https://​doi.​ Ophthalmol 49(1):87–91. https://​doi.​org/​10.​1016/j.​jcjo.​2013.​09.​
org/​10.​1161/​01.​STR.​00001​73152.​84438.​1c 015
74. Ferro JM, Canhão P, Stam J, Bousser MG, Barinagarremen- 89. Gillson N, Jones C, Reem RE, Rogers DL, Zumberge N, Aylward
teria F, Investigators I (2004) Prognosis of cerebral vein and SC (2017) Incidence and demographics of pediatric intracranial

13
Acta Neurologica Belgica (2021) 121:823–836 835

hypertension. Pediatr Neurol 73:42–7. https://​doi.​org/​10.​1016/j.​ intracranial hypertension: is there a place for octreotide? Cepha-
pedia​trneu​rol.​2017.​04.​021 lalgia 31(16):1679–80. https://​doi.​org/​10.​1177/​03331​02411​
90. Aylward SC, Reem RE (2017) Pediatric intracranial hyperten- 424211
sion. Pediatr Neurol 66:32–43. https://​doi.​org/​10.​1016/j.​pedia​ 110. Abubaker K, Ali Z, Raza K, Bolger C, Rawluk D, O’Brien D
trneu​rol.​2016.​08.​010 (2011) Idiopathic intracranial hypertension: lumboperitoneal
91. Sivaswamy L (2016) Pediatric intracranial hypertension . Pedi- shunts versus ventriculoperitoneal shunts–case series and litera-
atr Neurol Briefs 30(10):39. https://​doi.​org/​10.​15844/​pedne​urbri​ ture review. Br J Neurosurg 25(1):94–9. https://​doi.​org/​10.​3109/​
efs-​30-​10-2 02688​697.​2010.​544781
92. Dessardo NS, Dessardo S, Sasso A, Sarunić AV, Dezulović MS 111. Ravid S, Shahar E, Schif A, Yehudian S (2015) Visual outcome
(2010) Pediatric idiopathic intracranial hypertension: clinical and and recurrence rate in children with idiopathic intracranial hyper-
demographic features. Coll Antropol 34(Suppl 2):217–21 tension. J Child Neurol 30(11):1448–52. https://d​ oi.o​ rg/1​ 0.1​ 177/​
93. Aylward SC, Aronowitz C, Roach ES (2016) Intracranial 08830​73815​569306
hypertension without papilledema in children. J Child Neurol 112. Di Rocco F, Arnaud E, Renier D (2009) Evolution in the fre-
31(2):177–83. https://​doi.​org/​10.​1177/​08830​73815​587029 quency of nonsyndromic craniosynostosis. J Neurosurg Pediatr
94. Mallery RM, Friedman DI, Liu GT (2014) Headache and the 4(1):21–5. https://​doi.​org/​10.​3171/​2009.3.​PEDS0​8355
pseudotumor cerebri syndrome. Curr Pain Headache Rep 113. LoPresti M, Buchanan EP, Shah V, Hadley CM, Monson LA,
18(9):446. https://​doi.​org/​10.​1007/​s11916-​014-​0446-z Lam S (2017) Complete resolution of papilledema in syndromic
95. Friedman DI, Liu GT, Digre KB (2013) Revised diagnostic crite- craniosynostosis with posterior cranial vault distraction. J Pediatr
ria for the pseudotumor cerebri syndrome in adults and children. Neurosci 12(2):199–202. https://d​ oi.o​ rg/1​ 0.4​ 103/j​ pn.J​ PN_1​ 5_1​ 7
Neurology 81(13):1159–65. https://d​ oi.o​ rg/1​ 0.1​ 212/W
​ NL.0​ b013​ 114. Gault DT, Renier D, Marchac D, Jones BM (1992) Intracranial
e3182​a55f17 pressure and intracranial volume in children with craniosynos-
96. Babikian P, Corbett J, Bell W (1994) Idiopathic intracranial tosis. Plast Reconstr Surg 90(3):377–81
hypertension in children: the Iowa experience. J Child Neurol 115. Renier D, Sainte-Rose C, Marchac D, Hirsch JF (1982) Intracra-
9(2):144–9. https://​doi.​org/​10.​1177/​08830​73894​00900​208 nial pressure in craniostenosis. J Neurosurg 57(3):370–7. https://​
97. Aylward SC, Waslo CS, Au JN, Tanne E (2016) Manifesta- doi.​org/​10.​3171/​jns.​1982.​57.3.​0370
tions of pediatric intracranial hypertension from the intracranial 116. Takahashi Y, Hori M, Shimoji K, Miyajima M, Akiyama O,
hypertension registry. Pediatr Neurol 61:76–82. https://​doi.​org/​ Arai H et al (2017) Changes in delta ADC reflect intracra-
10.​1016/j.​pedia​trneu​rol.​2016.​04.​007 nial pressure changes in craniosynostosis. Acta Radiol Open
98. Cinciripini GS, Donahue S, Borchert MS (1999) Idiopathic 6(9):2058460117728535. https://​doi.​org/​10.​1177/​20584​60117​
intracranial hypertension in prepubertal pediatric patients: 728535
characteristics, treatment, and outcome. Am J Ophthalmol 117. Baird LC, Gonda D, Cohen SR, Evers LH, LeFloch N, Levy
127(2):178–82 ML et al (2012) Craniofacial reconstruction as a treatment for
99. Kesler A, Fattal-Valevski A (2002) Idiopathic intracranial hyper- elevated intracranial pressure. Childs Nerv Syst 28(3):411–8.
tension in the pediatric population. J Child Neurol 17(10):745–8. https://​doi.​org/​10.​1007/​s00381-​011-​1615-6
https://​doi.​org/​10.​1177/​08830​73802​01701​01401 118. Wall SA, Thomas GP, Johnson D, Byren JC, Jayamohan J,
100. Friedman DI, Jacobson DM (2002) Diagnostic criteria for idi- Magdum SA et al (2014) The preoperative incidence of raised
opathic intracranial hypertension. Neurology 59(10):1492–5 intracranial pressure in nonsyndromic sagittal craniosynostosis is
101. Standridge SM, O’Brien SH (2008) Idiopathic intracranial underestimated in the literature. J Neurosurg Pediatr 14(6):674–
hypertension in a pediatric population: a retrospective analysis 81. https://​doi.​org/​10.​3171/​2014.8.​PEDS1​425
of the initial imaging evaluation. J Child Neurol 23(11):1308–11. 119. Tuite GF, Evanson J, Chong WK, Thompson DN, Harkness WF,
https://​doi.​org/​10.​1177/​08830​73808​318056 Jones BM et al (1996) The beaten copper cranium: a correlation
102. Maralani PJ, Hassanlou M, Torres C, Chakraborty S, King- between intracranial pressure, cranial radiographs, and computed
stone M, Patel V et al (2012) Accuracy of brain imaging in the tomographic scans in children with craniosynostosis. Neurosur-
diagnosis of idiopathic intracranial hypertension. Clin Radiol gery 39(4):691–9
67(7):656–63. https://​doi.​org/​10.​1016/j.​crad.​2011.​12.​002 120. Eide PK, Helseth E, Due-Tønnessen B, Lundar T (2002) Assess-
103. Fraser JA, Leung AE (2014) Reversibility of MRI features of ment of continuous intracranial pressure recordings in childhood
pseudotumor cerebri syndrome. Can J Neurol Sci 41(4):530–2 craniosynostosis. Pediatr Neurosurg 37(6):310–20. https://​doi.​
104. Dandy WE (1937) Intracranial pressure without brain tumor: org/​10.​1159/​00006​6311
diagnosis and treatment. Ann Surg 106(4):492–513 121. Bannink N, Joosten KF, van Veelen ML, Bartels MC, Tasker
105. Johnston I, Paterson A (1974) Benign intracranial hypertension. RC, van Adrichem LN et al (2008) Papilledema in patients with
I. Diagnosis and prognosis. Brain 97(2):289–300 Apert, Crouzon, and Pfeiffer syndrome: prevalence, efficacy of
106. Johnson LN, Krohel GB, Madsen RW, March GA (1998) The role treatment, and risk factors. J Craniofac Surg 19(1):121–7. https://​
of weight loss and acetazolamide in the treatment of idiopathic doi.​org/​10.​1097/​SCS.​0b013​e3181​5f4015
intracranial hypertension (pseudotumor cerebri). Ophthalmol- 122. Rosenberg JB, Shiloh AL, Savel RH, Eisen LA (2011) Non-
ogy 105(12):2313–7. https://​doi.​org/​10.​1016/​S0161-​6420(98)​ invasive methods of estimating intracranial pressure. Neurocrit
91234-9 Care 15(3):599–608. https://d​ oi.o​ rg/1​ 0.1​ 007/s​ 12028-0​ 11-9​ 545-4
107. McCarthy KD, Reed DJ (1974) The effect of acetazolamide 123. Cohen SR, Dauser RC, Newman MH, Muraszko K (1993) Surgi-
and furosemide on cerebrospinal fluid production and choroid cal techniques of cranial vault expansion for increases in intrac-
plexus carbonic anhydrase activity. J Pharmacol Exp Ther ranial pressure in older children. J Craniofac Surg. 4(3):167–76
189(1):194–201 (discussion 74–6)
108. Schoeman JF (1994) Childhood pseudotumor cerebri: clinical 124. Strayer A (2001) Chiari I malformation: clinical presentation and
and intracranial pressure response to acetazolamide and furosem- management. J Neurosci Nurs 33(2):90–6
ide treatment in a case series. J Child Neurol 9(2):130–4. https://​ 125. Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ (1998) The reso-
doi.​org/​10.​1177/​08830​73894​00900​205 lution of syringohydromyelia without hindbrain herniation after
109. Deftereos SN, Panagopoulos G, Georgonikou D, Karageorgiou posterior fossa decompression. J Neurosurg 89(2):212–6. https://​
CE, Karageorgiou E, Piadites G (2011) Treatment of idiopathic doi.​org/​10.​3171/​jns.​1998.​89.2.​0212

13
836 Acta Neurologica Belgica (2021) 121:823–836

126. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, with Chiari malformation type 1: a prospective observational
Wolpert C et al (1999) Chiari I malformation redefined: clinical study. Acta Neurochir (Wien). 157(8):1411–23. https://​doi.​org/​
and radiographic findings for 364 symptomatic patients. Neuro- 10.​1007/​s00701-​015-​2465-x (discussion 23)
surgery 44(5):1005–17 130. Frič R, Eide PK (2016) Perioperative monitoring of pulsatile
127. Attenello FJ, McGirt MJ, Gathinji M, Datoo G, Atiba A, Wein- and static intracranial pressure in patients with Chiari malforma-
gart J et al (2008) Outcome of Chiari-associated syringomyelia tion type 1 undergoing foramen magnum decompression. Acta
after hindbrain decompression in children: analysis of 49 con- Neurochir (Wien) 158(2):341–7. https://d​ oi.o​ rg/1​ 0.1​ 007/s​ 00701-​
secutive cases. Neurosurgery 62(6):1307–13. https://​doi.​org/​10.​ 015-​2669-0 (discussion 6–7)
1227/​01.​neu.​00003​33302.​72307.​3b
128. Doberstein CA, Torabi R, Klinge PM (2017) Current concepts Publisher’s Note Springer Nature remains neutral with regard to
in the pathogenesis, diagnosis, and management of type I Chiari jurisdictional claims in published maps and institutional affiliations.
malformations. Rhode Island Med J 100(6):47–9
129. Frič R, Eide PK (2015) Comparison of pulsatile and static pres-
sures within the intracranial and lumbar compartments in patients

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