704

Pediatric Idiopathic Intracranial Hypertension

Eric D. Gaier, MD, PhD1,2 Gena Heidary, MD, PhD1,2

1 Department of Ophthalmology, Boston Children’s Hospital, Boston, Address for correspondence Gena Heidary, MD, PhD, Department of
Massachusetts Ophthalmology, Boston Children’s Hospital, 300 Longwood Avenue,
2 Harvard Medical School, Boston, Massachusetts Boston, MA 02115 (e-mail: Gena.Heidary@childrens.harvard.edu).

Semin Neurol 2019;39:704–710.

Abstract The presentation of idiopathic intracranial hypertension (IIH) in pediatric populations

has several important distinctions from that in adults, especially among prepubertal
patients, in which there is no apparent association with gender or obesity. Pediatric
patients are more likely to be asymptomatic or present with atypical symptoms than

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their adult counterparts, posing a diagnostic challenge in some cases. It is important to
be aware of the ways in which diagnostic criteria for IIH are modified from that of
adults. Ideal treatment practices and the natural history of pediatric IIH remain unclear.
Keywords Acetazolamide is the mainstay of medical treatment, but some patients with significant
► idiopathic intracranial visual loss may require surgical intervention. Multicenter studies to accrue a large
hypertension number of cases and future prospective studies will help to better define pediatric IIH
► pseudotumor cerebri and to formulate consensus guidelines for treatment and management of these
► papilledema patients.

Pseudotumor cerebri syndrome is a term that is currently
proposed to describe the condition of elevated intracranial
pressure without the presence of a mass. This new terminol-
ogy encompasses idiopathic intracranial hypertension (IIH),
the most common cause of pseudotumor cerebri syndrome
in adults and children,1–3 as well as secondary causes of
elevated intracranial pressure, including exposure to certain
medications, abnormalities of the cerebral venous system, or
predisposing systemic diseases.1 This review focuses on
pediatric IIH and will examine the most recent and reliable
data regarding the epidemiology, pathophysiology, risk fac-
tors, diagnostic testing, treatment, and outcomes, consider-
ing the differences from the adult condition. Relative to adult
IIH, pediatric IIH is an under-studied entity that would
benefit from more organized, multicenter prospective stud-
ies to guide evidence-based guidelines for diagnosis and
treatment.
Epidemiology
In adults, the incidence of IIH is 0.9 in 100,000 in the general
population and 19.3 in 100,000 among obese women aged
between 20 and 44 years.4 In children age <18 years, the
incidence is comparable but slightly less, at 0.63 to 0.90 in
100,000.2,5
In adults with IIH, there is a strong predilection for female
gender and obesity, but in children, this association varies
based on the pubertal status of the child. While pubertal
children have a strong female predominance that is similar to
adults, in prepubertal children, boys and girls are likely to be
affected similarly. Aylward et al3 analyzed 203 cases of
intracranial hypertension, including 142 cases of IIH from
the international Intracranial Hypertension Registry (formed
by the Intracranial Hypertension Research Foundation in
2003) and found a nearly 1:1 ratio of girls:boys among
prepubertal children.
Additionally, obesity is not a risk factor among pre-
pubertal children, but is among pubertal children.6,7 In a
multicenter, retrospective study of children aged between 2
and 18 years with IIH and papilledema, Sheldon et al8
analyzed 233 cases collected over 8 sites. Subdividing chil-
dren by age around typical pubertal onset, the authors found
that children with IIH who were younger than 7 years of age
had significantly lower body mass indices than older chil-
dren. However, when the investigators assessed weight as a
risk factor for IIH among the smaller group of patients, where

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 Pediatric Idiopathic Intracranial Hypertension
Tanner staging was specifically defined, the relationship did
not reach statistical significance.8 Likewise, Balcer et al6
performed a regression analysis to study the relationship
between age and body mass index in 45 consecutively
evaluated pediatric IIH patients, and demonstrated that
obesity becomes a risk factor for IIH at later ages in child-
hood. Tepe et al9 prospectively performed fundus examina-
tion on 1,058 obese children (ages 2–18 years) and found 14
cases of IIH (prevalence of 1.3%). When segregated by puber-
tal status (as assessed by Tanner staging), the prevalence was
0.9% and 1.5% for pre- and postpubertal patients respectively.
Brara et al10 showed that the risk of developing IIH among
children aged between 11 and 19 years increased signifi-
cantly with weight class. Obesity also increases the risk for
recurrence of elevated intracranial pressure among treated
patients.11 In a longitudinal retrospective case series of 43
children with IIH and an average 9-year follow-up, Stiebel-
Kalish et al showed a five-fold higher risk of IIH recurrence
among overweight or obese children. Therefore, obesity
becomes more relevant as a risk factor for IIH with increasing
age in childhood.
In their IIH prevalence study in 1,058 obese children, Tepe et
al9 found that obese patients with IIH had significantly higher
fasting insulin levels, measures of insulin resistance, cortisol
levels, and alanine transaminase levels. These findings support
a hormonal basis for pediatric IIH. Metabolic and hormonal
signaling may influence production of cerebrospinal fluid and
thus contribute to a common pathway of elevated intracranial
pressure in pseudotumor cerebri syndrome (for review see the
study by Sheldon et al12). Hormonal contributions are strongly
supported by the demographic and clinical associations with
IIH discussed below. Most notably, the influence of pubertal
status on IIH demographics suggests a role for the hypotha-
lamic-pituitary-gonadal axis.12,13
Symptoms
As in adults with IIH, pediatric patients with IIH typically
present with positional headaches, pulsatile tinnitus, tran-
sient visual obscurations, blurred vision, and diplopia. How-
ever, pediatric patients with IIH can present with more
varied or inconsistent symptoms compared with adult IIH
patients. For example, children with elevated intracranial
pressure who present with nausea or vomiting often under-
go negative abdominal/gastrointestinal evaluations before a
diagnosis of IIH is made. While there may be real differences
in the way that IIH is expressed in children compared with
adults, in part the difference in reported symptoms may
simply represent the difficulty some children experience in
expressing their symptoms (►Fig. 1).
A significant number of pediatric IIH patients present with
no headache. In their retrospective review, Aylward et al14
found that 22 (14%) patients did not have headaches. Patients
without headache were significantly younger (mean age: 9.7
vs. 13.4 years) and had significantly lower body mass index
(21.7 vs. 28.7 kg/m2). Similar results were found among pedi-
atric patients in an emergency room setting, with patients
younger than 11 years (prepubertal) less likely to report
headache.7 Among those who were symptomatic, blurred
Gaier, Heidary 705
vision was the most common complaint.14 The analysis seems
to have included patients with secondary causes along with
those meeting the criteria for IIH. Of note, patients with
secondary causes of elevated intracranial pressure were nearly
thrice more likely to present with headaches.
Several children with IIH are asymptomatic altogether. In
their series, Aylward et al14 reported 5% of their sample (36% of
those presenting without headache) were asymptomatic and
were only incidentally identified as having papilledema on
routine eye examinations. In a retrospective review of 45
pediatric IIH cases, Bassan et al15 found a substantially higher
proportion of asymptomatic patients (14/45, 31%). Similar to
Aylward et al,14 Bassan et al15 found that asymptomatic
patients were significantly younger (median age, 5.6 vs. 11.0
years) and less likely to be obese (14 vs. 48%). In contrast, they
found a significant male predominance among the asymptom-
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atic group (71%) compared with the symptomatic group (39%).
Our own retrospective review of 86 patients with pediat-
ric IIH revealed that 18 (21%) were asymptomatic.16 In
agreement with the above studies, these patients were
significantly younger (mean age, 9 vs. 13 years) and had
significantly lower body mass indices (22 vs. 30 kg/m2)
compared with symptomatic patients. It is important to
recognize the limitations of retrospective chart reviews,
especially when assessing the rate of symptom reporting.
Nevertheless, it appears that a substantial proportion of
pediatric patients with elevated intracranial pressure can
present with no headache or be asymptomatic altogether,
and this fraction is higher among younger children.
Examination Findings
On examination, manifestations of pediatric IIH mirror those
observed in adults, including decreased vision with visual
field deficits, dyschromatopsia, cranial neuropathies (VI, VII,
less commonly IV), papilledema, and the absence of other
neurologic deficits (►Fig. 1).1
Papilledema or optic disc edema is an important sign in
the setting of elevated intracranial pressure. However, a
subset of patients with pediatric IIH can manifest without
papilledema. In their retrospective review, Aylward et al17
found that 27 (17.7%) patients with elevated intracranial
pressure presented with no papilledema. These patients did
not differ from those with papilledema with regard to their
age, opening pressure on lumbar puncture, or body mass
index. A similar rate of elevated intracranial pressure with-
out papilledema 12/63 (19.0%) was reported by Glatstein et
al among patients aged between 2 and 16.5 years who
presented to the emergency department.7 These findings
suggest that consideration of pseudotumor cerebri should be
maintained in patients presenting with clinical features
suggestive of elevated intracranial pressure even in the
absence of papilledema.
As pseudopapilledema from hyperopia and optic disc
drusen can closely resemble papilledema from elevated
intracranial pressure, the distinction between papilledema
and pseudopapilledema is a frequent source of referrals to
neuroophthalmology among pediatric as well as adult
patients.18 These cases can often represent a diagnostic
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Fig. 1 Representative case initial presentation. A 13-year-old boy presented to the emergency department with a 3-week history of headaches and 1-week
history of double vision. His best-corrected visual acuities were 20/200 in the right eye and 20/80 in the left eye. His pupils were normally reactive to light
without a relative afferent pupillary defect. There was dyschromatopsia on Ishihara color plate testing. Sensorimotor examination revealed a mild abduction
limitation in the right eye with a corresponding incomitant esotropia worse in right gaze, consistent with a right sixth nerve palsy. (A) Dilated funduscopy
revealed papilledema with peripapillary hemorrhages and subretinal fluid tracking into the maculae in both eyes. (B) Kinetic Goldmann perimetry showed
severe global depression and constriction of the visual fields in the right eye greater than the left. (C) An MRI showed slightly low-lying cerebellar tonsils with
normal configuration. There were no intracranial masses or signal abnormalities. An MRV did not show signs of thrombosis. Lumbar puncture showed an
opening pressure of 55 cm H2O with normal CSF constituents.

challenge because a significant fraction of patients with IIH
can be asymptomatic and the features of headaches can be
similar irrespective of whether they relate to elevated intra-
cranial pressure. Importantly, optic nerve head drusen may
coexist with papilledema, and therefore a careful evaluation
is necessary to clarify the underlying etiology of the optic
nerve appearance.
imaging (MRI). These criteria are used in clinical trials for
IIH.1,21 With respect to pediatric IIH, current criteria for
diagnosis include papilledema, normal neuroimaging, elevated
intracranial pressure of 28 cm H2O with a sedated lumbar
puncture in the lateral decubitus position (25 cm H2O with a
nonsedated lumbar puncture or in an obese child), and normal
cerebral spinal fluid constituents.1

Diagnosis
Diagnostic Criteria
The diagnosis of IIH is predicated on signs, symptoms, and
objective evidence of elevated intracranial pressure. First de-
fined by Dandy in 1937 in a report of 22 patients, the original
criteria included signs and symptoms consistent with elevated
intracranial pressure and an opening pressure of >25 cm H2O,
the absence of localizing neurologic signs except for a sixth
nerve palsy, normal cerebrospinal fluid composition, and
normal to small ventricles without an intracranial mass on
pneumoencephalography.19 These criteria were updated in
1985 by Smith20 to reflect advancements in neuroimaging to
include computed tomography (CT) and magnetic resonance
Diagnostic Testing
If the clinical suspicion for elevated intracranial pressure is
sufficiently high, the first step in the work-up of papilledema
is neuroimaging. The modality of choice is an MRI of the brain
and orbits with gadolinium. There is a debate in the field
whether MR venogram (MRV) is necessary to evaluate dural
venous thrombosis in all cases. More rapid onset of symp-
toms, a history of recent head trauma, and/or coagulopathic
risk factors should raise suspicion for dural venous throm-
bosis. It has been suggested that cases with highly typical
demographics for IIH or another identified secondary cause
of pseudotumor cerebri may not require MRV imaging. On
the other hand, occult dural venous thrombosis can be
present in many circumstances,22 and its presence

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 Pediatric Idiopathic Intracranial Hypertension
significantly impacts the therapeutic approach to include
anticoagulation. A retrospective review of 360 patients with
IIH or papilledema revealed that of the 72 patients who were
imaged by MRV, 10 (14%) had dural venous thrombosis;23 of
those 10 with dural venous thrombosis, 6 (60%) were occult.
For this reason, when patients present to the pediatric
neuroophthalmology service with suspected elevated intra-
cranial pressure and optic disc edema, we include MRV as
part of the initial diagnostic work up.
Assuming no causative pathology is identified on MRI,
there are some subtle findings to support the presence of
elevated intracranial pressure. In adult patients, the most
well-known of these is the so-called “empty sella,” or a
depression of the pituitary gland secondary to compression.
Others include flattening of the posterior sclera, enlarge-
ment of the retrobulbar subarachnoid space, and intraocular
protrusion of the nerves into the globes (representing pap-
illedema).24 We performed a case-control study of 38 IIH
patients and 24 controls to examine the radiographic fea-
tures of IIH in pediatric patients.25 We found significant
differences in multiple structural measures including expan-
sion of the perioptic subarachnoid space, posterior globe
flattening, optic nerve protrusion, empty sella, and trans-
verse sinus narrowing. Skull base crowding and prominence
of arachnoid granulations were not significant. Görkem et
al26 reported similar findings in their retrospective review of
25 pediatric pseudotumor cerebri patients, reporting mod-
erate to high sensitivities and specificities for each of these
parameters. Hartmann et al27 also found similar results and
noted that compared with adolescents, prepubertal children
were less likely to exhibit flattening of the globe and more
likely to show perioptic arachnoid expansion, optic nerve
tortuosity, empty sella, and transverse sinus stenosis.
Dwyer et al28 found an increased incidence of venous
outflow obstruction (dural venous stenosis) of the dominant
side among patients with suspected IIH. Evidence of collateral
circulation in the IIH group was much more common than in
the control group. In most cases, these changes in the dural
venous system are likely to represent an effect of elevated
intracranial pressure rather than the major causative factor,
since these effects are frequently reversible with treatment.29
Following neuroimaging, the next essential diagnostic
step is a lumbar puncture. The primary purposes of the
lumbar puncture are to confirm the presence of elevated
intracranial pressure and sample the cerebrospinal fluid for
analysis. The threshold for what is considered an abnormally
elevated opening pressure on lumbar puncture is different
for children and dependent on the method used. Historically,
an opening pressure of greater than 20 cm H2O was consid-
ered elevated. Avery et al30 tested this notion by evaluating a
distribution of opening pressures for pediatric patients
undergoing lumber puncture for reasons other than sus-
pected elevated intracranial pressure. They found an upper
limit of normal (90th percentile) 28 cm H2O if the patient is
obese or sedation is required, and 25 cm H2O if the patient is
not obese or sedated. Opening pressure was not influenced
by age. These values are now incorporated in revised criteria
for pediatric pseudotumor cerebri syndrome.1 Opening pres-
Gaier, Heidary 707
sures have been reported to be similar among symptomatic
and asymptomatic patients with pseudotumor cerebri,14,15
and among patients with primary (IIH) and secondary
pseudotumor cerebri.3 It is important to remember that
some patients with truly elevated intracranial pressure
causing pseudotumor cerebri syndrome may not manifest
an opening pressure at or above the revised threshold.31,32
Therefore, the overall clinical context must always be taken
into consideration when interpreting opening pressure val-
ues with reference to a given numeric cut-off.
Development of adjunctive tools to assess intracranial
pressure will greatly facilitate our ability to more accurately
and reliably evaluate and monitor papilledema as well as
treatment response in pseudotumor cerebri. Optical coher-
ence tomography is a noninvasive tool to assess fundus
changes reflective of elevated intracranial pressure, which
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are not readily apparent on clinical examination.33 Another
imaging modality is B-scan ultrasonography, which can be
performed at the bedside and reveals changes relating to
fluid dynamics from elevated intracranial pressure.34,35 One
potential major advantage is that some of these changes may
be rapidly responsive to lowering of intracranial pressure,
unlike papilledema, allowing for more refined temporal
resolution and treatment response.
Treatment
Initial treatment for pediatric IIH focuses on management of
elevated intracranial pressure. Extracting larger volumes of
CSF and measurement of the closing pressure has no bearing
on reducing time to resolution of papilledema or headache.36
When pursuing medical management, we begin with acet-
azolamide with a dosing of 10 to 25 mg/kg divided BID or TID
(►Fig. 2). The IIH Treatment Trial (IIHTT) determined that
acetazolamide effectively reduced visual field loss in adults
with mild visual loss secondary to IIH. There is no equivalent
study in children, but a case series of 60 pediatric pseudo-
tumor cerebri patients suggested clinical benefit occured
from acetazolamide in 46 (76.6%) patients. Patients who
were younger at presentation were less likely to respond.
The question of when it is safe to taper patients off
acetazolamide is less clear in children than in adults. The
same factors that pose diagnostic challenges in pediatric
patients with IIH also pose challenges in effectively monitor-
ing their progress and treatment response. In their case
series of 60 pediatric patients with pseudotumor cerebri,
Tovia et al37 noted that 12/60 (26%) patients who were
treated with acetazolamide experienced a relapse following
discontinuation of the medicine. Those who relapsed were
significantly younger than those who remained in remission.
To date, there is no consensus on when and how to taper
pediatric patients, especially prepubertal patients, off acet-
azolamide. A comprehensive natural history study on this
subgroup of patients would provide invaluable context for
providers who care for these patients and provide a frame-
work for clinical decision making for these children.
Topiramate38 and furosemide have also been used in IIH to
lower intracranial pressure. Although their efficacy has not
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Fig. 2 Representative case treatment course. The patient was started on 250 mg bid of oral acetazolamide, which was quickly increased to
750 mg bid with minimal improvement in his visual acuity and fields. A new relative afferent pupillary defect on the right became apparent. The
patient was urgently referred for neurosurgical consultation to consider placement of a ventriculoperitoneal shunt, but the patient was
considered a poor candidate given his low-lying cerebellar tonsils. Therefore, the patient underwent an optic nerve sheath fenestration in the
right eye 9 days following his initial presentation. Following the procedure, the patient’s visual acuities improved to 20/25 in the right eye and
20/20 in the left eye without residual measureable dyschromatopsia. The relative afferent pupillary defect in the right eye persisted. (A) His optic
disc edema resolved, leaving secondary atrophic changes bilaterally. (B) His visual fields improved in both eyes, but with residual nasal
constriction more in the right eye than in the left.

been carefully studied in children, these medications may
serve an important role in patients who cannot tolerate
acetazolamide. Topiramate can be teratogenic and should be
avoided in female patients of childbearing age when possible.
Patients presenting with significant visual loss may require
a surgical intervention, typically a neurosurgical shunting
procedure, to quickly and effectively lower the intracranial
pressure.39 Although these procedures have considerable mor-
bidity and mortality, they can be justified in cases of fulminant
or progressive vision loss that can become irreversible without
emergent intervention. The utility of neurosurgical shunting
procedures in cases of severe visual loss secondary to IIH is the
focus of the SIGHT trial (NCT03501966), although this trial will
not include pediatric patients like the IIHTT. For use of a drug
like acetazolamide, parallels between adult and pediatric
patients can be relatively safely drawn; in contrast, outcomes
data from the SIGHT trial will require careful consideration and
analysis before its conclusions should be applied to children.
The risks of ventricular shunting procedures carry greater
weight in children because of the morbidity associated with

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 Pediatric Idiopathic Intracranial Hypertension
repetitive shunt failure and infection.40 Temporary lumbar
drains can also be considered as a way to lower the intracranial
pressure quickly, while secondary causes can be adequately
addressed and/or intracranial pressure lowering medications
can take effect.41
An alternative to the ventricular shunt is optic nerve
sheath fenestration, which provides a conduit for cerebro-
spinal fluid to escape the subarachnoid space around the
optic nerves.42 This approach should only be conducted by a
trained and experienced oculoplastic surgeon or neuro-
surgeon to reduce risk of the major potential complication
of irreversible, severe visual loss in the operated eye. This
approach can be an important alternative in patients who are
not good candidates for ventricular shunts. New surgical
approaches may improve the safety of this procedure.43
Although weight reduction may not be relevant for pre-
pubertal children, when obesity is present, weight loss is a
well-established, effective means to address IIH. The stan-
dard recommendation is that patients lose 6 to 10% of their
total weight. The control arm of the IIHTT included a rigorous
weight loss regimen that was effective in reducing visual
field loss secondary to papilledema. Comprehensive, multi-
specialty approaches to IIH, including nutritional and exer-
cise counseling, can be a powerful approach.
Outcomes
Historically, IIH was considered and even termed a “benign”
entity until cases of significant visual loss were reported.44
One study of prepubertal children found visual field abnor-
malities in 85% of eyes and no light perception vision in 9% of
eyes.45 In a retrospective case series of 96 patients, Stiebel-
Kalish et al46 identified a greater likelihood of poor visual
outcomes among 26 pubertal patients (grouped by age)
compared with pre-pubertal and adults patients with pseu-
dotumor cerebri. However, these results were obtained from
a single tertiary referral center, and differences in practice
could have highly influenced the results. Another retrospec-
tive study of 90 pediatric patients with IIH (the majority of
whom were younger than 10 years) conducted by Soiberman
et al47 showed significant improvement of visual acuity
following treatment.
As we standardize treatment approaches to pediatric IIH,
ways to improve outcomes for our patients will become
increasingly clear. These advances can only be achieved
through large, well-powered studies that allow for compar-
ative subgroup analyses. In addition, treatment practices
unique to a particular provider or institution can significant-
ly influence clinical data concerning disease features and
treatment response. Inter-institutional, multi-center collab-
orations to compile collections of patients will help to
mitigate these limitations and optimize the quality of clinical
studies on this topic.
Conclusion
Pediatric IIH is a condition of elevated intracranial pressure
with normal neuroimaging and CSF constituents that has the
Gaier, Heidary 709
potential to cause profound, irreversible vision loss. Prepu-
bertal pediatric IIH does not have a predilection for gender or
body habitus. In contrast, the clinical characteristics of IIH
presented after puberty are more similar to its presentation
in adults. Symptoms of pediatric IIH include headache, visual
changes, diplopia, and pulsatile tinnitus. However, it is
important to consider that many children may be asymp-
tomatic or may have difficulty recognizing or verbalizing
their symptoms. IIH carries the potential for profound,
permanent vision loss, and therefore prompt diagnosis and
treatment is essential. Recent strides in understanding the
pathogenesis and clinical characterization of pediatric IIH
will give way to a better understanding of the pathophysiol-
ogy and optimal treatment approaches for this significant
condition.
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Conflicts of Interest
The authors declare no relevant conflicts of interest.
References
1 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the
pseudotumor cerebri syndrome in adults and children. Neurology
2013;81(13):1159–1165
2 Gillson N, Jones C, Reem RE, Rogers DL, Zumberge N, Aylward SC.
Incidence and demographics of pediatric intracranial hyperten-
sion. Pediatr Neurol 2017;73:42–47
3 Aylward SC, Waslo CS, Au JN, Tanne E. Manifestations of pediatric
intracranial hypertension from the intracranial hypertension
registry. Pediatr Neurol 2016;61:76–82
4 Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor
cerebri. population studies in Iowa and Louisiana. Arch Neurol
1988;45(08):875–877
5 Gordon K. Pediatric pseudotumor cerebri: descriptive epidemiol-
ogy. Can J Neurol Sci 1997;24(03):219–221
6 Balcer LJ, Liu GT, Forman S, et al. Idiopathic intracranial hyper-
tension: relation of age and obesity in children. Neurology 1999;
52(04):870–872
7 Glatstein MM, Oren A, Amarilyio G, et al. Clinical characterization
of idiopathic intracranial hypertension in children presenting to
the emergency department: the experience of a large tertiary care
pediatric hospital. Pediatr Emerg Care 2015;31(01):6–9
8 Sheldon CA, Paley GL, Xiao R, et al. Pediatric idiopathic intracra-
nial hypertension: age, gender, and anthropometric features at
diagnosis in a large, retrospective, multisite cohort. Ophthalmol-
ogy 2016;123(11):2424–2431
9 Tepe D, Demirel F, Seker ED, et al. Prevalence of idiopathic intracra-
nial hypertension and associated factors in obese children and
adolescents. J Pediatr Endocrinol Metab 2016;29(08):907–914
10 Brara SM, Koebnick C, Porter AH, Langer-Gould A. Pediatric
idiopathic intracranial hypertension and extreme childhood obe-
sity. J Pediatr 2012;161(04):602–607
11 Stiebel-Kalish H, Serov I, Sella R, Chodick G, Snir M. Childhood
overweight or obesity increases the risk of IIH recurrence fivefold.
Int J Obes 2014;38(11):1475–1477
12 Sheldon CA, Kwon YJ, Liu GT, McCormack SE. An integrated
mechanism of pediatric pseudotumor cerebri syndrome: evi-
dence of bioenergetic and hormonal regulation of cerebrospinal
fluid dynamics. Pediatr Res 2015;77(02):282–289
13 Kesler A, Fattal-Valevski A. Idiopathic intracranial hypertension in
the pediatric population. J Child Neurol 2002;17(10):745–748
14 Aylward SC, Aronowitz C, Reem R, Rogers D, Roach ES. Intracranial
hypertension without headache in children. J Child Neurol 2015;
30(06):703–706
Seminars in Neurology Vol. 39 No. 6/2019
710 Pediatric Idiopathic Intracranial Hypertension Gaier, Heidary
15 Bassan H, Berkner L, Stolovitch C, Kesler A. Asymptomatic idio-
pathic intracranial hypertension in children. Acta Neurol Scand
2008;118(04):251–255
16 Whitecross S, Heidary G. Asymptomatic pediatric idiopathic
intracranial hypertension. J AAPOS 2013;17(01):e31
17 Aylward SC, Aronowitz C, Roach ES. Intracranial hypertension
without papilledema in children. J Child Neurol 2016;31(02):
177–183
18 Liu B, Murphy RK, Mercer D, Tychsen L, Smyth MD. Pseudopa-
pilledema and association with idiopathic intracranial hyperten-
sion. Childs Nerv Syst 2014;30(07):1197–1200
19 Dandy WE. Intracranial pressure without brain tumor: diagnosis
and treatment. Ann Surg 1937;106(04):492–513
20 Smith JL. Whence pseudotumor cerebri? J Clin Neuroophthalmol
1985;5(01):55–56
21 Wall M, McDermott MP, Kieburtz KD, et al; NORDIC Idiopathic
Intracranial Hypertension Study Group Writing Committee. Effect
of acetazolamide on visual function in patients with idiopathic
intracranial hypertension and mild visual loss: the idiopathic
intracranial hypertension treatment trial. JAMA 2014;311(16):
1641–1651
22 Standridge SM, O’Brien SH. Idiopathic intracranial hypertension
in a pediatric population: a retrospective analysis of the initial
imaging evaluation. J Child Neurol 2008;23(11):1308–1311
23 Hollander JN, Prabhu S, Heidary G. Utilization of MRV to evaluate
pediatric patients with papilledema. J AAPOS 2014;18(04):2
24 Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseu-
dotumor cerebri. Ophthalmology 1998;105(09):1686–1693
25 Gilbert AL, Vaughn J, Robson C, Whitecross S, Heidary G. Radio-
graphic features in pediatric idiopathic intracranial hypertension.
J AAPOS 2016;20(04):1
26 Görkem SB, Doğanay S, Canpolat M, et al. MR imaging findings in
children with pseudotumor cerebri and comparison with healthy
controls. Childs Nerv Syst 2015;31(03):373–380
27 Hartmann AJ, Soares BP, Bruce BB, et al. Imaging features of
idiopathic intracranial hypertension in children. J Child Neurol
2017;32(01):120–126
28 Dwyer CM, Prelog K, Owler BK. The role of venous sinus outflow
obstruction in pediatric idiopathic intracranial hypertension.
J Neurosurg Pediatr 2013;11(02):144–149
29 Stienen A, Weinzierl M, Ludolph A, Tibussek D, Häusler M.
Obstruction of cerebral venous sinus secondary to idiopathic
intracranial hypertension. Eur J Neurol 2008;15(12):1416–1418
30 Avery RA, Shah SS, Licht DJ, et al. Reference range for cerebrospinal
fluid opening pressure in children. N Engl J Med 2010;363(09):
891–893
31 Gerstl L, Schoppe N, Albers L, et al. Pediatric idiopathic intracra-
nial hypertension - Is the fixed threshold value of elevated LP
opening pressure set too high? Eur J Paediatr Neurol 2017;21(06):
833–841
32 Tibussek D, Distelmaier F, Kummer S, von Kries R, Mayatepek E.
Sedation of children during measurement of CSF opening pres-
Seminars in Neurology Vol. 39 No. 6/2019
sure: lack of standardisation in German children with pseudotu-
mor cerebri. Klin Padiatr 2012;224(01):40–42
33 El-Dairi MA, Holgado S, O’Donnell T, Buckley EG, Asrani S,
Freedman SF. Optical coherence tomography as a tool for moni-
toring pediatric pseudotumor cerebri. J AAPOS 2007;11(06):
564–570
34 Irazuzta JE, Brown ME, Akhtar J. Bedside optic nerve sheath
diameter assessment in the identification of increased intracra-
nial pressure in suspected idiopathic intracranial hypertension.
Pediatr Neurol 2016;54:35–38
35 Shuper A, Snir M, Barash D, Yassur Y, Mimouni M. Ultrasonogra-
phy of the optic nerves: clinical application in children with
pseudotumor cerebri. J Pediatr 1997;131(05):734–740
36 Beres SJ, Sheldon CA, Boisvert CJ, et al. Clinical and prognostic
significance of cerebrospinal fluid opening and closing pressures
in pediatric pseudotumor cerebri syndrome. Pediatr Neurol 2018;
83:50–55
37 Tovia E, Reif S, Oren A, Mitelpunkt A, Fattal-Valevski A. Treatment
response in pediatric patients with pseudotumor cerebri syn-
Downloaded by: Collections and Technical Services Department. Copyrighted material.
drome. J Neuroophthalmol 2017;37(04):393–397
38 Celebisoy N, Gökçay F, Sirin H, Akyürekli O. Treatment of idio-
pathic intracranial hypertension: topiramate vs acetazolamide,
an open-label study. Acta Neurol Scand 2007;116(05):322–327
39 Chern JJ, Tubbs RS, Gordon AS, Donnithorne KJ, Oakes WJ.
Management of pediatric patients with pseudotumor cerebri.
Childs Nerv Syst 2012;28(04):575–578
40 Hanak BW, Bonow RH, Harris CA, Browd SR. Cerebrospinal fluid
shunting complications in children. Pediatr Neurosurg 2017;52
(06):381–400
41 Jiramongkolchai K, Buckley EG, Bhatti MT, et al. Temporary
lumbar drain as treatment for pediatric fulminant idiopathic
intracranial hypertension. J Neuroophthalmol 2017;37(02):
126–132
42 Thuente DD, Buckley EG. Pediatric optic nerve sheath decompres-
sion. Ophthalmology 2005;112(04):724–727
43 Gupta AK, Gupta K, Sunku SK, Modi M, Gupta A. Endoscopic optic
nerve fenestration amongst pediatric idiopathic intracranial hyper-
tension: a new surgical option. Int J Pediatr Otorhinolaryngol 2014;
78(10):1686–1691
44 Lessell S, Rosman NP. Permanent visual impairment in childhood
pseudotumor cerebri. Arch Neurol 1986;43(08):801–804
45 Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial
hypertension in prepubertal pediatric patients: characteristics,
treatment, and outcome. Am J Ophthalmol 1999;127(02):178–182
46 Stiebel-Kalish H, Kalish Y, Lusky M, Gaton DD, Ehrlich R, Shuper A.
Puberty as a risk factor for less favorable visual outcome in
idiopathic intracranial hypertension. Am J Ophthalmol 2006;
142(02):279–283
47 Soiberman U, Stolovitch C, Balcer LJ, Regenbogen M, Constantini S,
Kesler A. Idiopathic intracranial hypertension in children: visual
outcome and risk of recurrence. Childs Nerv Syst 2011;27(11):
1913–1918