Asymptomatic Versus Symptomatic Idiopathic Intracranial Hypertension in Children

Abstract

Background: Idiopathic intracranial hypertension is a rare neurologic condition characterized by elevated intracranial pressure with normal cerebrospinal fluid analysis and neuroimaging. A subset of pediatric idiopathic intracranial hypertension patients are coincidentally found to have papilledema and elevated intracranial pressure without symptoms (eg, headache, visual blurring, tinnitus). This study aims to investigate the features of asymptomatic pediatric idiopathic intracranial hypertension. Methods: Retrospective case-control study of patients aged 0 to 18 years who received idiopathic intracranial hypertension diagnosis from 2005 to 2016. Subjects were included if they met established diagnostic criteria for idiopathic intracranial hypertension diagnosis. Subjects were classified as symptomatic if they presented with 1 symptom related to elevated intracranial pressure, and asymptomatic if no symptoms were present. Statistical analysis was performed to compare the 2 groups. Results: 12 (22.6%) of 53 pediatric idiopathic intracranial hypertension subjects were asymptomatic. Compared to symptomatic idiopathic intracranial hypertension, asymptomatic idiopathic intracranial hypertension had younger age of onset, lower initial opening pressure on lumbar puncture, lower optic nerve edema grades bilaterally, lower likelihood of globe flattening on magnetic resonance imaging (MRI), and smaller required dose of acetazolamide for resolution of papilledema (all P < .05). Conclusion: Asymptomatic idiopathic intracranial hypertension is common among pediatric patients with papilledema and is an important disease entity that requires special clinical management. It may exist as a milder version of idiopathic intracranial hypertension that occurs in younger children, or as a precursor state that later evolves into symptomatic disease.

Incidental idiopathic intracranial hypertension

“…Asymptomatic onset is less frequent and it has been reported in children. In these cases, diagnosis is possible due to the incidental finding of a bilateral optic disc swelling on a routine eye examination [ 6 ]. Thus, the ophthalmologist plays an important role making an early diagnosis, as in our case.…”

“…Asymptomatic patients may present specific anatomic or physiologic variances (a greater number of arachnoid granulations or a better cerebrospinal fluid leak, respectively) that allow a more effective compensation of elevated ICP. Moreover, it has been reported that nutritional, electrolyte and hormonal abnormalities exacerbate the pathology, so in patients without decompensated pathologies, the symptomatic onset is less frequent [ 6 ].…”

“…Gondi et al suggested that asymptomatic IIH may be a latent period of the disease in patients with better compensatory mechanisms against an increase in ICP, highlighting the importance of the early treatment in these cases [ 6 ]. Most patients show only mild visual field defects (such as an enlarged blind spot or arcuate defects) that regress after the reduction of the ICP, whereas some patients suffer a rapid progression of visual field defects.…”

Pseudotumor Cerebri Syndrome: From Childhood to Adulthood Risk Factors and Clinical Presentation

“…Patients were defined as overweight if body mass index was above 25 or body weight was above the 90th percentile, and obese if body mass index was 30.0 or higher, or over the 97th percentile for age. Subsequently, we merged and combined their clinical data with the information retrieved from 15 previous clinical studies published since 2001 3,14 -32 and performed a pooled analysis. In addition to patient’s weight and gender, data that were analyzed for that purpose included the finding of papilledema and the symptoms of headache, vomiting, visual impairment, dizziness, tinnitus, and associated drug therapies regarded as precipitating factors.…”

“…12,13 The fact that pseudotumor cerebri syndrome is associated with male gender and normal body mass in children raises the possibility that the underlying mechanisms are different at a younger age. Most previous studies on pediatric pseudotumor cerebri syndrome 14 -32 included only children, which made the comparison between pediatric and adult patients challenging.…”