Elsevier

Pediatric Neurology

Volume 66, January 2017, Pages 32-43
Pediatric Neurology

Topical Review
Pediatric Intracranial Hypertension

https://doi.org/10.1016/j.pediatrneurol.2016.08.010Get rights and content

Abstract

Primary (idiopathic) intracranial hypertension has been considered to be a rare entity, but with no precise estimates of the pediatric incidence in the United States. There have been attempts to revise the criteria over the years and adapt the adult criteria for use in pediatrics. The clinical presentation varies with age, and symptoms tending to be less obvious in younger individuals. In the prepubertal population, incidentally discovered optic disc edema is relatively common. By far the most consistent symptom is headache; other symptoms include nausea, vomiting tinnitus, and diplopia. Treatment mainstays include weight loss when appropriate and acetazolamide. Furosemide may exhibit a synergistic benefit when used in conjunction with acetazolamide. Surgical interventions are required relatively infrequently, but include optic nerve sheath fenestration and cerebrospinal fluid shunting. Pain and permanent vision loss are the two major complications of this disorder and these manifestations justify aggressive treatment. Once intracranial hypertension has resolved, up to two thirds of patients develop a new or chronic headache type that is different from their initial presenting headache.

Section snippets

History

Primary intracranial hypertension (idiopathic intracranial hypertension, pseudotumor cerebri) is generally considered to be a rare entity. Although the annual incidence in the United States adult population is estimated at 0.9 per 100,000, there are no precise incidence estimates in the pediatric population.1 In Germany the annual pediatric incidence is estimated to be 0.47 per 100,000, and in Croatia, 1.2 per 100,000.2, 3 In the provinces of Nova Scotia and Prince Edward Island, the annual

Diagnostic criteria

The diagnostic criteria for adult PIH stems from a series of 22 patients reported by Dandy in 1937.15 His report resulted in the initial criteria bearing his name, although he did not propose the criteria in this paper. The main limitation of the Dandy criteria is that imaging was at that time limited to pneumoencephalography, which sometimes provided evidence of an intracranial mass by distortion or compression of the ventricles. This method did not allow the identification of nonmass causes

Demographics

In pediatrics, PIH is often divided into prepubertal or pubertal groups. Pubertal patients have the same risk factors as adults, whereas sex and weight are not prominent risk factors in prepubertal patients. The pediatric female-to-male ratio ranges from 1:1 to 13:6, and concurrent obesity ranges from 10% to 78%, largely depending on whether or not patients are divided by pubertal status (Table 1).3, 4, 5, 37, 38, 39, 40, 41 Balcer et al.42 found that obesity correlated with an increased risk

Signs and symptoms

The clinical presentation of intracranial hypertension in pediatric patients can vary with age, with symptoms tending to be less evident in younger individuals. In the prepubertal population, incidentally discovered optic disc edema is relatively common and reported in up to 33% of cases.3, 38, 39, 40, 46, 47 When compared with children with symptomatic PIH, asymptomatic cases are often younger, have a lower percentage of obesity, and a male predominance.47 Asymptomatic cases typically require

Mimickers of papilledema

Papilledema is defined as optic disc edema in the presence of increased intracranial pressure. Optic disc edema in the absence of increased intracranial pressure can result from optic neuritis, neuroretinitis, anterior ischemic optic neuropathy, and infiltration of the optic nerve head by tumor cells. There are also conditions that can have the appearance of optic nerve edema to those unfamiliar with the fundus examination (and even the experienced at times). These include anomalous optic

Secondary intracranial hypertension

As technology advances, more conditions are recognized that result in classification of increased pressure as secondary (see Table 2). Conditions such as traumatic brain injury, hydrocephalus, intracranial masses, subarachnoid hemorrhage, and meningitis have a high propensity to develop intracranial hypertension. There have been previous reviews of these conditions and treatment guidelines developed for some. For the purposes of this review, we have chosen to focus on conditions associated with

Diagnosis

The ophthalmic examination is a critical component in the diagnosis and subsequent management of intracranial hypertension. Key components include visual acuity, pupillary and motility assessments, color vision evaluation, and detailed funduscopic examination with special attention to the optic nerve. Humphrey or Goldmann visual field testing, optical coherence tomography of the retinal nerve fiber layer, and fundus autofluorescence are all helpful testing modalities for initial evaluation as

Treatment

A multidisciplinary team is optimal to address the needs of a patient with intracranial hypertension. At a minimum, that team should consist of a neurologist and ophthalmologist. If the situation dictates, a neurosurgeon, dietitian, physical therapist, psychologist, hematologist, and/or endocrinologist should be included.

Adult patients often report a recent weight gain during the 12 months preceding diagnosis.79, 126 There are no pediatric studies, but numerous studies of obese adults have

Outcome

Pain and permanent vision loss are the two major complications of intracranial hypertension, both of which can have a major impact on quality of life.150, 151 Headache is typically the first symptom to resolve within the first few weeks after starting treatment; papilledema resolves in 4.2 to 5 months.3, 40, 152 Postpubertal status was associated with a worse visual outcome in one study.153 Another study found grade 3 or higher optic disc edema was a predictive marker of permanent vision

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