Review ArticleIdiopathic Intracranial Hypertension in Children: A Review and Algorithm
Introduction
Increased intracranial pressure is classified into two main categories, depending on its idiopathic or secondary etiology. In the literature, the two categories have not always been clearly delineated, leading to difficulties in understanding associations or drawing inferences about the syndrome. Categorizing etiologies is necessary, because characteristics, management, and outcomes may vary considerably. Idiopathic intracranial hypertension is defined increased intracranial pressure without a space-occupying lesion or hydrocephalus and normal cerebrospinal fluid composition [1]. Initially termed “serous meningitis,” this syndrome was first recognized by Quincke in 1897 [2]. With its unclear etiology, the syndrome has assumed different names, including pseudotumor cerebri and benign intracranial hypertension. Especially in children, questions continue to surround the diagnostic criteria and medical management.
A review of the literature indicates that the diagnostic evaluation of these patients often involves several medical subspecialties. Articles have been written by different physicians in many subspecialties, including general practice, emergency medicine, radiology, psychiatry, neurology, ophthalmology, neuro-ophthalmology, and neurosurgery [3], [4], [5], [6], [7], [8]. Although the approach to patients with idiopathic intracranial hypertension is often similar among subspecialties, differences do exist. Coordinating the patient’s care from presentation to follow-up is an enormous challenge. Because a risk of permanent visual loss exists with idiopathic intracranial hypertension, patients require a prompt and thorough collaborative approach in their management.
Wide diversity is evident in the published approaches, and little has been written about children. Therefore, an urgent need exists for a coherent approach to the evaluation and management of children. This updated review of idiopathic intracranial hypertension offers a focus on children, including epidemiology and pathogenesis, clinical presentations, common clinical course, diagnostic criteria and challenges, and treatments. An algorithm for the initial diagnostic evaluation of children with intracranial hypertension is proposed. This algorithm provides a systematic approach to management, identifies important decision-making factors, and illustrates the importance of a coordinated multidisciplinary approach.
Section snippets
Epidemiology
The true incidence of idiopathic intracranial hypertension in children is unknown. In the past, children were often included within adult studies, leaving the pediatric incidence unclear. Moreover, several previous pediatric studies did not exclude children in whom a secondary etiology was discovered for their intracranial hypertension signs. Hence the number of patients with idiopathic intracranial hypertension may have been falsely elevated [9], [10]. Although the incidence of idiopathic
Diagnostic Challenges
Diagnostic criteria were originally outlined by Smith [30] (Table 1), based on characteristics described by Dandy in 1937 [31]. Over the past several years, physicians have proposed further expansions of the criteria, primarily as a result of further research and knowledge, as well as advances in diagnostic technology such as magnetic resonance imaging. For example, physicians have recommended further clarification that a patient is not required to exhibit signs of idiopathic intracranial
Clinical Presentations
In children, the most common presenting symptom is headache [11], [16], [58]. However, the characteristics of these headaches are variable. A headache may begin as moderate and episodic and progress to severe, chronic pain [62]. Patients often report a headache to be exacerbated by Valsalva and postural changes [62]. Patients may describe a headache similar to a migraine, with a pulsatile quality as well as photophobia, nausea, and vomiting [62]. Differentiating between headaches caused by
Approach
The need exists for a systematic approach to the initial diagnostic evaluation of a child with signs of intracranial hypertension. The proposed algorithm provides a systematic approach to initial evaluation and management, and identifies important decision-making points (Figure 1, Figure 2). It also illustrates the importance of a coordinated multidisciplinary approach. Although Class I evidence is lacking, this algorithm could support more consistent practice and provide a foundation for
Collaboration
Collaboration among physicians within the algorithm is very important. The speed of the initial diagnostic evaluation and management depends on a well-coordinated multidisciplinary approach. If urgent outpatient cranial imaging and lumbar puncture are not possible, the neurologist may need to coordinate with a nearby, appropriately equipped facility for a rapid evaluation. This procedure may be particularly beneficial if sedation is necessary to complete cranial imaging and lumbar puncture.
Discussion and Limitations
One limitation of the algorithm pertains to a lack of recommended time frames for diagnostic procedures. At present, no evidence-based data provide guidance for a specific timeline of evaluation. Because visual loss can occur any time during the course of idiopathic intracranial hypertension, the physician should undertake a diagnostic evaluation promptly, to initiate management for preserving vision and reducing signs.
Another limitation involves the lack of evidence regarding magnetic
Chronic Management
The goal of treatment for idiopathic intracranial hypertension is primarily twofold: to prevent visual loss and to eliminate symptoms. At present, no randomized, controlled, double-blind, prospective studies have evaluated possible treatments for children. Because the etiology of idiopathic intracranial hypertension remains unclear, a specific treatment tailored to the cause has not yet developed. Therefore, current treatments are tailored to treat signs. Further studies are desperately needed
Prognosis
Permanent loss of visual function, regardless of treatment, was reported in children. Permanent decreased visual acuity occurs in up to 10% of children [15], [58], [107]. Permanent visual field defects occur in up to 17% [58]. In mixed adult and pediatric studies, blindness in one or both eyes developed in up to 5% of patients [35], [65]. Findings of visual examinations or complaints such as transient visual obscurations, severity of papilledema, duration of signs, or the presence of cranial
Future Directions and Conclusions
Although the etiology of idiopathic intracranial hypertension currently eludes the medical profession, the continued search for that etiology is imperative, because identifying the cause could lead to better treatments. Idiopathic intracranial hypertension is an uncommon problem, and an algorithm to outline an efficient management strategy for children should be helpful. This algorithm serves as a first step toward the consistent management of children with this condition. The lack of
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