Original Article

Journal of Child Neurology

1-9
Infantile Idiopathic Intracranial Hypertension: ª The Author(s) 2019
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A Case Study and Review of the Literature DOI: 10.1177/0883073819860393
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Sama Boles, BHSc1 , Claudia Martinez-Rios, MD2 ,

Daniel Tibussek, MD3, and Daniela Pohl, MD, PhD4

Abstract
Idiopathic intracranial hypertension, or pseudotumor cerebri, is an increase in cerebrospinal fluid pressure of unknown etiology. It
is mostly seen in adults, less frequently in adolescents, rarely in younger children. Only 5 infants meeting idiopathic intracranial
hypertension criteria have been mentioned in the literature. We report a case of a previously healthy 9-month-old boy who
presented with irritability, decreased appetite, and a bulging fontanelle. Computed tomography (CT) head imaging and cere-
brospinal fluid studies revealed normal results. The patient’s symptoms transiently resolved after the initial lumbar puncture, but
11 days later, his fontanelle bulged again. A second lumbar puncture revealed an elevated opening pressure of 35 cmH2O and led
to a diagnosis of idiopathic intracranial hypertension in accordance with the modified Dandy Criteria. Treatment with acet-
azolamide at a dose of 25 mg/kg/d was initiated and the patient remained symptom-free for 6 weeks, followed by another relapse.
His acetazolamide dose was increased to 37 mg/kg/d, with no further relapses to date.
A diagnosis of idiopathic intracranial hypertension is challenging in infants, because the patients cannot yet verbalize typical
idiopathic intracranial hypertension–related symptoms such as positional headaches, diplopia, or pulsatile tinnitus. Furthermore, it
is more difficult to assess papilledema in that age group. If undetected and untreated, idiopathic intracranial hypertension may
result in permanent visual deficits. Little is known about idiopathic intracranial hypertension in infants, and age-specific treatment
guidelines are lacking. We discuss this rare case of infantile idiopathic intracranial hypertension and provide a review of the
literature, including an overview of disease characteristics and outcomes of idiopathic intracranial hypertension in this very young
age group.

Keywords
pseudotumor cerebri, idiopathic intracranial hypertension, infant, fontanelle, acetazolamide

Received April 27, 2019. Received revised May 29, 2019. Accepted for publication June 6, 2019.

Idiopathic intracranial hypertension, or pseudotumor cerebri, is
characterized by elevated cerebrospinal fluid pressures with no
evident cause.1-3 The incidence of pediatric idiopathic intracra-
nial hypertension is around 0.5 per 100 000 children per year.4
A manifestation in infancy has only rarely been reported. We
present a 9-month-old idiopathic intracranial hypertension
patient and provide a comprehensive review of the literature
on infantile idiopathic intracranial hypertension.
Case Report
A 9-month-old male presented to our emergency department
with a 1-day history of fever, irritability, decreased appetite,
and a bulging fontanelle. He had a normal examination and
neurologic status apart from a full and tense anterior fontanelle.
Medical history revealed that he was born at 42 weeks’
gestational age via cesarean section with no complications,
following an uneventful pregnancy. He developed a pruritic
rash at the age of 2 months and was treated intermittently with
hydrocortisone 1% topical cream. He had received his last
vaccinations at age 5 months. Family history revealed eczema
in the father, but no neurologic or autoimmune disorders.
Laboratory investigations illustrated a mildly elevated lym-
phocyte count (13.3  109/L) and an elevated platelet count
(523  109/L). Vitamin D 25-OH was slightly low (49 nmol/L).
1
University of Ottawa, Ottawa, Ontario, Canada
2
Department of Medical Imaging, Children’s Hospital of Eastern Ontario,
University of Ottawa, Ottawa, Ontario, Canada
3
Department of General Pediatrics, Neonatology and Pediatric Cardiology,
University Children’s Hospital, Heinrich-Heine-University, Düsseldorf,
Germany
4
Division of Neurology, Children’s Hospital of Eastern Ontario, University of
Ottawa, Ottawa, Ontario, Canada
Corresponding Author:
Daniela Pohl, MD, PhD, Division of Neurology, Children’s Hospital of Eastern
Ontario, 401 Smyth Road, Ottawa, Ontario, K1 H 8L1, Canada.
Email: dpohl@cheo.on.ca
2 Journal of Child Neurology XX(X)
Figure 1. Brain magnetic resonance imaging (MRI). Sagittal T1-
weighted image (A) shows pituitary gland flattening. Axial T2-weighted
image (B) demonstrates tortuous optic nerves with mild elevation of
the papilla on the left. Time-of-flight MR venography in coronal (C) and
axial (D) planes demonstrates narrowing of bilateral sigmoid sinuses
with absence of flow signal.
Thyroid-stimulating hormone, free thyroxine, and parathyroid
hormone levels were normal. He was negative for Epstein-Barr
virus antibodies, and nasopharyngeal suction for respiratory
syncytial viruses and influenza A and B were also negative. A
CT of the head was normal. A lumbar puncture revealed nor-
mal results including negative Gram stains and cultures, as
well as negative herpes simplex and enterovirus polymerase
chain reactions (PCRs). Opening pressure was not measured.
The patient was admitted for observation and discharged
3 days later after his bulging fontanelle and all his symptoms
had resolved.
Eleven days after the initial lumbar puncture, the patient
presented with recurrence of the bulging fontanelle and irrit-
ability. His physical examination was otherwise normal and
he was afebrile. No papilledema was visible; however, the
admitting pediatrician commented that he struggled to visua-
lize the fundi.
Magnetic resonance imaging (MRI) demonstrated mild nar-
rowing of bilateral sigmoid sinuses, slightly tortuous bilateral
optic nerves with mild elevation of the papillae mainly on the
left side, and mild flattening of the pituitary gland (Figure 1).
Lumbar puncture opening pressure measured with the child
sedated was increased at 35 cmH2O. Grade 2 papilledema,
characterized by a circumferential halo, was identified by
ophthalmology. Investigations were conducted to rule out
Table 1. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri
Syndrome) Diagnostic Criteria.5,a
Diagnostic criteria for IIH: A-E required
a. Papilledema
b. Normal neurologic examination except for cranial nerve
abnormalities
c. Neuroimaging: normal brain parenchyma without evidence of
hydrocephalus, mass, or structural lesion and no abnormal
meningeal enhancement on MRI, with and without gadolinium,
for typical patients (female and obese), and MRI, with and
without gadolinium, and magnetic resonance venography for
others; if MRI is unavailable or contraindicated, contrast-
enhanced CT may be used
d. Normal CSF composition
e. Elevated LP opening pressure (25 cmH2O in adults; 28
cmH2O in children; 25 cmH2O CSF if the child is not sedated
and not obese)
Diagnostic Criteria for IIH in the Absence of Papilledema
a. B-E from above AND unilateral or bilateral abducens palsy
Diagnosis may be suggested but not made in absence of sixth nerve
palsy or papilledema if:
a. B-E from above satisfied
AND at least 3 of the following neuroimaging criteria are satisfied:
a. Empty sella
b. Flattening of the posterior aspect of the globe
c. Distention of the perioptic subarachnoid space
d. Transverse venous sinus stenosis
Abbreviations: CSF, cerebrospinal fluid; IIH, idiopathic intracranial
hypertension; LP, lumbar puncture; MRI, magnetic resonance imaging.
a
A diagnosis of pseudotumor cerebri syndrome is definite if the patient fulfills
criteria A-E. The diagnosis is considered probable if criteria A-D are met but
the measured CSF pressure is lower than specified for a definite diagnosis.5
secondary idiopathic intracranial hypertension, revealing unre-
markable iron tests (iron, ferritin, and total iron-binding capac-
ity), normal thyroid stimulating hormone, parathyroid
hormone, and morning cortisol. Erythrocyte sedimentation
rate, C-reactive protein, anti-double-stranded DNA, and anti-
nuclear antibody measurements were also normal.
The patient was diagnosed with idiopathic intracranial
hypertension, in accordance with idiopathic intracranial
hypertension diagnostic criteria (Table 1). Treatment with
acetazolamide 10 mg/kg/d was started, and increased to
25 mg/kg/d 2 days later. Upon follow-up 2 weeks later, he
was asymptomatic with a leveled fontanelle, though with
persisting papilledema.
Two months post discharge, follow-up with ophthalmology
revealed no papilledema, optic disc pallor, or fourth or sixth
nerve palsy. However, 5 days later, the patient presented to the
emergency department with a relapse of idiopathic intracranial
hypertension. He was afebrile and alert but had increased irrit-
ability, recurrent emesis, and a bulging, pulsating fontanelle.
An attempt for fundoscopy was conducted at the emergency
department; however, the examining emergency physician did
not comment on the presence or absence of papilledema. A
therapeutic spinal tap was performed with no opening pressure
measurement and the patient discharged on an increased dose
of acetazolamide (37 mg/kg/d).
Boles et al
Figure 2. Time course of clinical symptoms and treatment.
On follow-up with neurology 2 weeks after this emergency
department presentation, the now 12-month-old infant was
asymptomatic, demonstrating a levelled fontanelle. However,
he demonstrated poor weight gain and increased fatigue, in
keeping with adverse effects of high-dose acetazolamide. He
had mildly elevated thyroid-stimulating hormone levels at
7.49 mIU/L and low free thyroxine at 9 pmol/L. Cortisol
levels remained normal. On follow-up with endocrinology,
hypothyroidism secondary to idiopathic intracranial hyperten-
sion was suspected and treatment with thyroxine 25 mg daily
was initiated.
Neurology follow-up 4 months later illustrated no idio-
pathic intracranial hypertension relapse and acetazolamide
dose was decreased to 30 mg/kg/d. A subsequent follow-up
2 months later showed maintained resolved idiopathic intra-
cranial hypertension with no optic disc edema or cranial neu-
ropathy (Figure 2).
Discussion
Our case highlights an infant meeting all diagnostic criteria for
idiopathic intracranial hypertension. 5 He presented with
papilledema, normal neurologic status, normal cerebrospinal
fluid composition, and an elevated lumbar puncture opening
pressure. MRI revealed normal brain parenchyma with no
evidence of any masses, hydrocephalus, structural lesions,
or meningeal enhancement.
Background
Idiopathic intracranial hypertension was first described in 1897
by Quincke.6,7 It is also referred to as pseudotumor cerebri
because of similarities in presentation to that of an intracranial
mass.6,8 Idiopathic intracranial hypertension can result in irre-
versible deficits in visual acuity and cranial nerve palsies,
because of compressive nerve injury.3 Till date, 25 infants with
idiopathic intracranial hypertension have been reported; how-
ever, only 5 of those infants met idiopathic intracranial hyper-
tension diagnostic criteria.9,10
3
Diagnosis
Idiopathic intracranial hypertension is being diagnosed in
accordance with the revised idiopathic intracranial hyperten-
sion diagnostic criteria (Table 1).5,11 In children, cerebrospinal
fluid opening pressures of 25 cm H2O and of 28 cm H2O if
a child is obese or sedated are considered to be diagnostic for
idiopathic intracranial hypertension.5
Opening pressures of reported infants with idiopathic
intracranial hypertension range from 25 to 77 cm H2O, with
an average of 39 cm H2O and a median of 35 cm H2O (Table 2).
Unfortunately, many publications lack key diagnostic informa-
tion, and only 5 infants met all diagnostic criteria. Of note,
many infants presented with a bulging fontanelle, but lack of
papilledema (18/25), potentially because the increase in pres-
sure may at least temporarily be compensated by the open
fontanelle. We suggest that the absence of papilledema should
not be seen as an exclusion criterion for infantile idiopathic
intracranial hypertension.
Head imaging with normal brain parenchyma and no evi-
dence of structural lesions, no meningeal enhancement, and no
masses or hydrocephalus is required to diagnose idiopathic
intracranial hypertension.5 Sinus venous thrombosis should
be ruled out. Flattened posterior globes, partially empty sella,
and intraocular protrusion, as well as enlargement with tortu-
osity of the optic nerves are typical findings in idiopathic intra-
cranial hypertension but absence of those features does not
exclude the diagnosis.12-15
Symptoms
The clinical picture of idiopathic intracranial hypertension
changes with age: adolescents typically present with head-
aches, diplopia, and tinnitus, whereas younger children may
have nonspecific symptoms like irritability, sleep disruption,
and head tilting, and less often have papilledema.16-19 How-
ever, children will more often present with cranial nerve pal-
sies, particularly abducens nerve palsy.20
The presentation of infantile idiopathic intracranial hyper-
tension, as published so far, varies, with a bulging fontanelle
and irritability being the most commonly reported initial
4
Table 2. Reports of Infantile Idiopathic Intracranial Hypertension.
Age at diagnosis Presenting signs and CSF opening Potential triggers/
Authors and sex symptoms pressure (cmH2O) Treatment Follow-up comorbidities IIH criteria met5

Matalia et al, 20189 11 mo Cataract 29 Acetazolamidea 30 mo: Normal development, NRc Yes
Papilledema 30 mg/kg/d for 3 mo, with resolved IIHb
Female tapered dosing over the
following 2 mo

Fernandez-Pol 7 mo Bulging fontanelle >35 Single LPd Resolved IIH Human herpesvirus-6 PCR No (no papilledema, or abducens
et al, 201626 Vomiting positive (blood) palsy, normal imaging)
Male No papilledema Topical hydrocortisone 2
Fever wk before presentation
Decreased activity
Ramkumar, 201610 10 mo Acute esotropia 50 (at Acetazolamide 6 wk: resolution of Hypervitaminosis A Yes
Vomiting presentation); papilledema associated with maternal
Female Bilateral papilledema 35 (2 wk after 25 mg/kg/d 10 mo: Cranial nerve palsy prenatal vitamin intake
Sixth cranial nerve palsy initial LP) improved with residual left
Tapered dosing over 3 mo abduction deficit

Masri et al, 201519 7 mo Bulging anterior 77 Acetazolamide Lost to follow-up Hypophosphatasia No (no papilledema or abducens
fontanelle (Dosages and duration not (normal vitamin D levels) palsy)
Female No papilledema reported)
Hypotonia
Normal neurologic
exam
Masri et al, 201519 10 mo Irritability 30 Acetazolamide 6 wk: normal CSF pressure Vitamin D deficiency No (no papilledema or abducens
Vomiting (Dosages and duration not 24 mo: normal (22 ng/mL) palsy)
Male No papilledema reported) ophthalmologic exam 18 h after measles
Decreased feeding vaccination
Sleep disturbances
Normal neurologic
exam
Masri et al, 201519 11 mo Irritability 49 Acetazolamide and steroids 1 y: Normal CSF pressure NR Yes
Vomiting (Dosages and duration not 25 mo: normal
Male Papilledema reported) ophthalmologic exam
Sleep disturbances
Bilateral sixth cranial
nerve palsy

Roy, 201343 <1 y old Bulging anterior Opening pressure Acetazolamide 1: asymptomatic NR Indeterminate; insufficient data to
(youngest ¼ 4 mo) fontanelle for infants not 1 mo: resolution of adequately assess (no opening
Irritability reported (Dosages and duration not papilledema pressures reported)
(n¼4) þ/– Papilledema (2 of reported) 2: no recurrence of symptoms
the infants did not
have papilledema)
Squint
Normal MRI and
magnetic resonance
venography

(continued)
 Table 2. (continued)

Age at diagnosis Presenting signs and CSF opening Potential triggers/

Authors and sex symptoms pressure (cmH2O) Treatment Follow-up comorbidities IIH criteria met5

Goldberg, 201339 7 mo Bulging fontanelle >36 Single LP 24 h post presentation: Human herpesvirus-6 PCR No (no papilledema or abducens
Irritability resolved fever and bulging positive (blood) palsy)
Male Fever fontanelle
Cough
Rhinorrhea
Hacifazlioglu et al, 9 mo Restlessness Acetazolamide 1 mo: no recurrence NR Yes
201244 Bilateral papilledema 60
Male Head tilting 25 mg/kg/ d for 3 mo
Normal neurologic
exam
Obeid et al, 201145 9 wk Bulging fontanelle NR Acetazolamide for 1 d 1 mo: no signs of IIH Cystic fibrosis No (no papilledema or abducens
Irritability 1 y: residual bilateral facial Vitamin A deficiency (0.08 palsy)
Male Unilateral facial palsye weakness mg/L)
Twin 1 Hypotoniaf
Lethargyf

10 wk Bulging, pulsatile 14, 25g, 19h, 25i Acetazolamide 4 wk: Resolved IIH Cystic Fibrosis No (no papilledema or abducens
anterior fontanelle 2 mo: facial palsy improving Vitamin A deficiency palsy)
Male Irritability Gradual increase to 100 mg/
Twin II Facial asymmetry kg/d for 2 wk
Feeding difficulties
2 LPs

Tibussek et al, 9 mo Abducens palsy 18, 27, 22 Acetazolamide 25-35 mg/kg/ NR No comorbidities Indeterminate; insufficient data to
20102 Impaired consciousness d Not on medication adequately assess
Female Specific dosages and duration
not specified
Distelmaier et al, 9 mo Isolated abducens palsy 27 Treatment not reported NR NR Indeterminate; insufficient data to
20073 adequately assess
Female
Freedman, 200146 9 mo Bulging fontanelle 45 Acetazolamide NR 10-d course of antibiotic Yes
Vomiting therapy for otitis media
Female Papilledema
Strabismus
Bilateral sixth cranial
nerve palsies
Youroukos et al, n¼4 Bulging fontanelle (4/4) NR NR NR Vitamin D deficiency (3/4) No (no papilledema)
199847 Irritability (4/4) Hypophosphatasia (1/4)
Vomiting (3/4)
No papilledema (4/4)
Macrocephaly (4/4)
Anorexia (3/4)
4 mo Pulsating, bulging Normal baseline Acetazolamide and NR NR No (no papilledema, normal CSF
Schoeman, 199436 fontanelle pressure furosemide pressure)
Female No papilledema reportedj

(continued)

5
6
Table 2. (continued)

Age at diagnosis Presenting signs and CSF opening Potential triggers/

Authors and sex symptoms pressure (cmH2O) Treatment Follow-up comorbidities IIH criteria met5

Baker et al, 198948 NR No papilledema NR NR NR Indeterminate; insufficient data to

adequately assess

Roussounis, 7 mo Bulging anterior NR Reintroduction of One wk: no bulging fontanelle Topical betamethasone for 3 No
197628 Male fontanelle betamethasone for 2 d 12 mo: normal development mok, stopped 1 wk prior
Vomiting
Irritability
Drowsiness
Neville et al, 8.5 mo Bulging fontanelle 24 Reintroduction of NR Hirschsprung disease No (no papilledema, opening
197027 Irritability prednisone 10 mg/d Systemic prednisone15 mg pressure too low)
Neck retraction withdrawn gradually over for 2.5 mo prior to
2 mo symptoms onset

Abbreviations: CSF, cerebrospinal fluid; IIH, idiopathic intracranial hypertension; LP, lumbar puncture; MRI, magnetic resonance imaging; NR, not reported; PCR, polymerase chain reaction.
a
Infant also underwent cataract surgery in both eyes.
b
Idiopathic intracranial hypertension.
c
Not reported.
d
Lumbar puncture.
e
Progressed to bilateral in 1 day.
f
Developed 8 days after initial presentation.
g
9 days after presentation.
h
Recurrence of IIH 14 days after initial presentation.
i
Recurrence of IIH 20 days after initial presentation.
j
see discussion in text.
k
A total of 300 g had been used over 3 months.
Boles et al
symptoms (16/25 reported infants). Vomiting was another
relatively frequent symptom at presentation (9/25 infants).
Papilledema was only present in 8 of the 25 infants reported
with idiopathic intracranial hypertension, and even less fre-
quently, abducens palsy was described (3/25).2,4,17
The inability to assess vision changes and take an accurate
history of classical idiopathic intracranial hypertension specific
symptoms such as vision changes, diplopia, tinnitus, headache
and nausea makes infants particularly susceptible to misdiag-
nosis.20,21 Furthermore, assessing for papilledema in infants is
known to be challenging even among experienced clinicians.22
Risk Factors
No clear-cut risk factors for the development of idiopathic
intracranial hypertension in younger children or infants have
been identified. This is in contrast to adolescent and adults who
illustrate risk factors such as obesity, female gender, post pub-
ertal status, polycystic ovarian syndrome, and medication-
related adverse events (eg, steroid withdrawal).6,23,24
Amongst the 25 infants reported with idiopathic intracranial
hypertension, 4 had vitamin D deficiency, 2 had positive serum
human herpesvirus-6 testing (PCR), and 2 had hypophosphata-
sia. Viral-induced intracranial hypertension has been found to
mimic symptoms of idiopathic intracranial hypertension.25
Despite initial presentation of mild fever in our infant, the long
duration of his idiopathic intracranial hypertension as well as
his relapses unrelated to infectious symptoms speak against a
diagnosis of post/para-infectious intracranial hypertension.
Similarly to our patient, 3 other patients were on topical
hydrocortisone prior to presentation with infantile idiopathic
intracranial hypertension.26-28 Idiopathic intracranial hyperten-
sion has previously been reported to be associated with topical
steroid withdrawal.29-32 Despite the exact mechanism remain-
ing elusive, this is thought to occur more often in children and
is hypothesized to be impacted by the duration of steroid
administration and rate of withdrawal.28,33
Treatment
Treatment principles for pediatric idiopathic intracranial
hypertension are based on adult guidelines, given the paucity
of idiopathic intracranial hypertension treatment trials in
children.34-36 Acetazolamide has been reported to be effective
in both adult and pediatric populations with idiopathic intra-
cranial hypertension.11,22,37,38
The majority (21/25) of infants with idiopathic intracranial
hypertension have been treated with acetazolamide, with doses
of 25 to 100 mg/kg/d, and a duration of treatment ranging from
a single day to 5 months. In 2 patients, only single lumbar
punctures were conducted, and no medication was adminis-
tered.26,39 In 2 other infants, who were on either oral or topical
corticosteroids prior to presenting with idiopathic intracranial
hypertension, treatment was the reintroduction and gradual
reduction of steroids.27,28
7
Disease Course
Idiopathic intracranial hypertension relapses are reported in up
to 22% of the pediatric population and often occur within the
first 18 months after diagnosis.40,41 Through close monitoring
and timely treatment normalizing cerebrospinal fluid pressures,
children have been found to have favorable outcomes with
regard to the preservation of visual acuity and visual fields.42
A review of the infantile patients reported with idiopathic
intracranial hypertension reveals that the disease course was
extremely variable, with resolution occurring between 24 hours
and 2 years after the initial presentation. The mean reported
duration of infantile idiopathic intracranial hypertension was
7.8 months, with a median of 5.4 months (Table 2). Of 14
patients with reported follow-up, 11 improved back to baseline
and 3 showed either residual left abduction deficits, bilateral
facial weakness, or a slowly improving facial palsy (Table 2).
Conclusion
Infantile idiopathic intracranial hypertension is a rare condition
that has not yet been well described in the literature. A bulging
fontanelle in an otherwise healthy infant with normal head
imaging should raise suspicion for infantile idiopathic intracra-
nial hypertension, and we strongly advocate for routinely mea-
suring cerebrospinal fluid opening pressures in those children.
Prolonged high-dose treatment with acetazolamide may be
required to prevent relapses and to safeguard visual acuity.
Acknowledgments
We thank the family of our patient for agreeing and providing written
informed consent to publishing their son’s case report and images.
Author Contributions
SB conceptualized, drafted the initial manuscript and reviewed and
revised the manuscript. MR contributed with data acquisition, analy-
sis, and interpretation, ensuring accuracy or integrity for this project,
and reviewed and revised the manuscript. DP conceptualized,
designed, coordinated, and supervised the project and reviewed and
revised the manuscript. DT assisted with analysis and interpretation of
data and reviewed and revised the manuscript. All authors approved
the final manuscript as submitted and agree to be accountable for all
aspects of the work.
Declaration of Conflicting Interests
The authors declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this articl
Funding
The authors received no financial support for the research, authorship,
and/or publication of this article.
ORCID iD
Sama Boles, BHSc https://orcid.org/0000-0002-9355-1690
Claudia Martinez-Rios, MD https://orcid.org/0000-0001-8591-
9822
Daniela Pohl, MD, PhD https://orcid.org/0000-0002-2996-7210
8 Journal of Child Neurology XX(X)
Ethical Approval
This project received approval from the Children’s Hospital of Eastern
Ontario Research Ethics Board (Approval # 20180376).
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