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Traitement et pronostic de l’hypertension intracrânienne idiopathique de l’enfant. Étude rétrospective (1995–2009) et revue de la littérature
Abstract
Aim
Idiopathic intracranial hypertension (IIH) may cause severe visual loss due to the optic nerve damage. Routine management involves mainly medical treatment. The aim of this study was to improve diagnosis and management of IIH in children.
Methods
The medical records of all patients with definite IIH seen at the children's hospital of Toulouse between 1995 and 2009 were reviewed. Cases of secondary intracranial hypertension were included because they did not present any cerebral lesions and underwent a similar therapeutic approach. The clinical and ophthalmological data at the beginning and at the end of their treatment was collected.
Results
Eighteen children were included in this study. The average age was 10 years and the sex-ratio was equal to 1. There were 3 cases of secondary idiopathic intracranial hypertension in this pediatric group. The main features encountered were headache (15 children) and diplopia (8 children). Abnormal neurological examination was found for 11 patients with abducens nerve paresis in 8 cases, rachialgia in 6 cases, and neurogenic pains (neuralgia, dysesthesia, paresthesia, hyperesthesia) in the other cases. Papilledema was noted in 16 patients. At the initial phase, loss of visual acuity was documented in 6 patients and altered visual field in nine patients. All patients had a medical treatment. When recurrence occurred, each new treatment was documented, for a total of 23 treatments analyzed. Lumbar puncture was the only treatment for 2 patients. In 16 cases, first-line treatment was acetazolamide and it was the second choice in 1 case, with an average dosage of 11.2 mg/kg and a mean duration of 2.5 months (15 treatments could be analyzed). This treatment was effective in 11 cases out of 15. Steroids were the initial treatment in 4 cases and second-line treatment in 4 cases (after failed acetazolamide therapy). The dosage was 1.5–2 mg/kg for a mean duration of 1.5 months (6 treatments could be analyzed). This treatment was effective in 5 patients out of 6. One patient had dual therapy. No surgical procedure was necessary in this pediatric cohort. Three patients presented relapses of IIH. The outcome was good with no residual visual impairment in the 13 patients analyzed. One patient was still under medication.
Comments
Therapeutic management of IIH in a pediatric population is essentially medical, in some cases limited to lumbar puncture. The first-line treatment is acetazolamide, but this study shows that low doses and short duration are usually chosen. Doses must be increased and treatment prolonged to avoid the use of corticosteroids as a second-line treatment and prevent possible relapses that require close monitoring of visual function.
Conclusion
The visual prognosis is generally better for this age group compared to adults and no risk factors for visual sequelae were identified. A standardized protocol for management of IIH was proposed.
... A papilledema and unilateral or bilateral VIth nerve palsy are frequently found during an ophthalmological examination. It affects 9 to 48% of children with IIH, making it the most prevalent cranial nerve deficiency [4]. Other cranial nerve palsies are rarely involved. ...
Idiopathic intracranial hypertension is rare in the pediatric population. It’s characterized by increased intracranial pressure in the absence of any evident underlying neurologic disease. Abducens nerve palsy is the most common cranial nerve deficit related to that condition. The association of sixth and seventh cranial nerve damage is uncommon. In this report, we describe the case of an 8-year-old girl who presented with headache, diplopia and peripheral facial palsy related to idiopathic intracranial hypertension
Concepto: aumento de la presión intracraneal sin altera-ciones en la composición del líquido cefalorraquídeo (LCR) ni lesiones ocupantes de espacio. Etiopatogenia: desconocida a pesar de las numerosas hipótesis. Incidencia: estimada entre 1-2/100 000, con mayor inci-dencia en mujeres obesas de 15 a 44 años. Clínica: lo más frecuente es la cefalea, papiledema, di-plopia y otras alteraciones visuales. En lactantes puede cursar únicamente con fontanela abombada e irritabili-dad. Diagnóstico: clínico mediante los criterios de Dandy-Smith modificados y excluyendo lesiones ocupantes de espacio intracraneales. Tratamiento: hay tres escalones terapéuticos; el primero son las medidas de soporte, el segundo es farmacológico (acetazolamida de elección) y el tercero es quirúrgico in-vasivo. Pronóstico: bueno con evolución favorable y tendencia a la autorresolución sin precisar tratamiento en la mayoría de los casos Seguimiento: es necesario un estrecho seguimiento clíni-co y oftalmológico, siendo la periodicidad individualizada en función de la gravedad y el tratamiento precisado. RESUMEN La hipertensión intracraneal idiopática (HII) es una patolo-gía de etiología desconocida cuyo diagnóstico es clínico (crite-rios de Dandy-Smith modificados) y de exclusión, incluyendo signos y síntomas producidos por el aumento de la presión in-tracraneal sin alteraciones en la composición del líquido cefalo-rraquídeo, y excluyendo otras causas de hipertensión intracra-neal mediante neuroimagen y otras evaluaciones. Las manifestaciones clínicas más frecuentes son cefalea, papilede-ma y diplopía u otras alteraciones visuales. El diagnóstico dife-rencial ha de realizarse con los procesos expansivos intracra-neales y las hidrocefalias obstructivas. El pronóstico es generalmente bueno con evolución favorable y tendencia a la autorresolución sin precisar tratamiento en la mayoría de los casos. Existen tres escalones terapéuticos: el primero consiste en medidas no farmacológicas para aliviar el aumento de presión intracraneal, el segundo asocia el uso de fármacos (acetazolamida de elección), y el tercero consiste en tratamiento quirúrgico invasivo. INTRODUCCIÓN La hipertensión intracraneal idiopática (HII) es una patolo-gía definida por criterios clínicos, que incluye signos y síntomas producidos por el aumento de la presión intracraneal (cefalea, papiledema y afectación visual) sin alteraciones en la composi-ción del LCR, y por la exclusión de otras causas de hipertensión Puesta al día en… Hipertensión intracraneal idiopática en el niño (pseudotumor cerebri)
The pseudotumor cerebri syndrome (PTCS), also known as idiopathic intracranial hypertension (IIH), is characterized by signs and symptoms of increased intracranial pressure such as headache and cranial nerve palsies, in the absence of any space-occupying mass. This condition commonly affects overweight women of childbearing age, even if it is also frequent in men and children. Children with PTCS may present with atypical signs and symptoms, with a different prognosis compared to adults. However, the treatment is the same for both children and adults, even if there are no strict treatment guidelines in regards. All treatment strategies in children are based on retrospective studies and none has been evaluated in prospective or randomized controlled trial studies. This review focuses on literature data on PTCS treatment in children.
Idiopathic Intracranial Hypertension is less common in children, predominantly being a diagnosis of middle aged women1. This entity is exceedingly uncommon in infants, and, so far, although it has been reported with the use or withdrawal of steroids by other routes (apart from other medications) it has rarely been reported with the use of topicalsteroids. We report here a case of 3 month old baby who developed idiopathic intracranial hypertension in association with the use of a topical steroid Fluticasone.
Headache is one of the most common symptoms in neurology. The Croatian Society of Pediatric Neurology Headache Task Force suggests the following guidelines for evaluation of children with headaches including diagnostic criteria for headache classification (International Classification of Headache Disorders, ICHDII) according to the International Headache Society (IHS), which are based on data analysis of the available literature and treatment principles based on facts and evidence. The goal of the guidelines is good clinical practice. Guidelines are available on the CSPN web page (www.hddn.hlz.hr). According to the IHS classification, headaches are divided into primary and secondary headaches. Migraine, psychogenic or tension headache, trigeminal neuralgia and Horton-Bing's (cluster) headache are primary headaches. Acute headaches are primary headaches in most cases. Chronic headaches are a large group of frequent or everyday long-lasting headaches (more than 4 hours per day) when compared to short-term headaches. Every child with headache needs individual approach. The evaluation of a child with headache starts with detailed physical examination (blood pressure measurement included), neurological examination and thorough history. Secondary headache must be excluded, which means that other causes must be sought. Focal neurologic symptoms and signs suggest the presence of secondary headache that needs urgent diagnostic procedure. Headache characteristics that present an indication for urgent neuro-radiological examination are intense acute sudden headache in a previously healthy child, acute or chronic headache including migraine and progressive psychogenic headache, positive meningeal signs, focal neurological signs and papilledema of the optic nerve. If neurological examination is normal in a child with recurrent headaches, there is no need for (urgent) neuroradiological diagnostic procedure (brain CT/MRI scans). If brain CT is normal in a child with intense headache, brain MRI has to be done along with lumbar tap and cerebrospinal fluid (CSF) examination in order to exclude subarachnoid hemorrhage or meningoencephalitis. Neuroradiological procedures are absolutely necessary to find the cause of secondary headaches. CSF examination has to be done in a febrile immunocompromised child to confirm or exclude meningitis. Routine EEG is not necessary in a child with headache. It has to be performed if there is the possibility of epileptic seizure or secondary headache (caused by a tumor, head trauma, encephalitis, intracranial hemorrhage or ischemia). EEG changes are neither specific nor pathognomonic for a special cause of headache. When treating headache (after secondary causes have been excluded), the child must be placed in a dark quiet room, and only drugs with known mechanism of action can be prescribed. It is always necessary to ask for parents' informed consent to treatment with a certain specific drug for a migraine considering the possible side effects and whether the drug is approved by the Croatian Institute of Health Insurance for treating children. Analgesics and nonsteroidal antirheumatics are used in the treatment of acute headache. Acetaminophen is used in analgesic dosage (15-20 mg/kg) and can be repeated in 4 hours. The dosage of ibuprofen is 10 mg/kg up to twice a day. Ondansetron can be prescribed if there is recurrent vomiting accompanied with migraine. Beta blockers are used as prophylactic drugs in patients with migraine as well as antiepileptic drugs and nonpharmacological procedures. Clinical trials have not proved to be of value in pharmacological migraine prophylaxis. Multidisciplinary approach in headache treatment includes parent and children education along with psychosocial approach.
Background:
Idiopathic intracranial hypertension (IIH) is a rare condition in children. VIth nerve palsy is the most common cranial nerve deficit related to that condition. Other cranial nerve dysfunctions have also been described but remain rare in paediatric daily practice.
Case presentation:
We here report the case of a 13-year-old girl who presented with VIth and contralateral VIIth nerve palsy due to IIH.
Conclusion:
Although rarely encountered, paediatricians should be familiar with the possible association of VIth and contralateral VIIth nerve palsy in children suffering from IIH. Moreover, other cranial nerve deficits may also be affected.
Objectives:
Headaches are a common problem among adolescents, and malocclusion is a possible risk factor. The purpose of this study was to investigate the association between malocclusion and headache among Japanese adolescents aged 12-15 years using a population-based sample.
Methods:
A total of 938 adolescents (94.7% of the target population in Koshu City) participated. A modified version of the Index of Orthodontic Treatment Need (IOTN) was used by orthodontists to evaluate occlusal characteristics, and the frequency of headaches (none, rarely, sometimes) was assessed via questionnaire (N = 938). Ordered logistic regression analyses were used to analyze the association between malocclusion and headache.
Results:
The prevalence of malocclusion diagnosed using the modified version of the IOTN was 44.9%. Multiple ordered logistic regression analyses showed that the odds ratio (OR) of having malocclusion for headache was 1.38 (95% confidence interval [CI]: 1.06-1.80), after adjustment for demographics, lifestyle, and dental status including orthodontic treatment history and tooth size. Moreover, among occlusal traits, lower crowding was independently associated with headache (OR: 1.64, 95% CI: 1.07-2.51).
Conclusions:
We found that malocclusion, especially lower crowding, was associated with headache in a population-based sample of adolescents aged 12-15 years in Japan. Further study is needed to elucidate the mechanisms by which malocclusion affects headache.
Overweight and obesity are less closely associated with idiopathic intracranial hypertension (IIH) in young children than in post-pubescent children and adults. We examined the hypothesis that being overweight or obese (body mass index [BMI] ≥85(th) percentile) in children is a risk factor for IIH recurrence. A total of 43 children with IIH who were followed for an average of 9±3.4 years were evaluated in a retrospective case-control series. The rate of IIH recurrence was compared between children of healthy weight and children presenting with overweight or obesity, using survival-curve analysis. The overall risk for long-term IIH recurrence in children is ~20%. Following weight stratification, the risk for IIH recurrence in our cohort was 5-fold higher in children with a BMI ≥85th percentile (57%) than in healthy-weight children (11% log-rank test P=0.04). Pediatricians may consider counseling families that weight control may be a means of decreasing the risk of IIH recurrence.International Journal of Obesity accepted article preview online, 18 March 2014; doi:10.1038/ijo.2014.44.
Our aim was to improve diagnosis and management of pseudotumor cerebri (PTC; also known as idiopathic intracranial hypertension) in children.
We performed a comprehensive analysis of epidemiology, diagnostic work-up, therapy, and clinical follow-up in 53 consecutive patients.
We identified several important aspects to be considered in the management of these children. First, patients may present without obvious symptoms at diagnosis. Second, bilateral papilledema might not or not yet be present in symptomatic patients. Third, measurement of cerebrospinal fluid (CSF) opening pressure may not always be reliable due to drugs used for sedation, which may alter intracranial pressure. Fourth, normal CSF pressure values in childhood are not well established and diagnosis might even be justified if pressure is <20 cm H(2)O. Fifth, associated conditions are frequent (at least in our cohort); however, in most cases, a causative link cannot be proven. Finally, disease relapse is a serious problem (20% in our group), which stresses the importance of standardized follow-up programs.
PTC constitutes an important and possibly underrecognized disorder in children and adolescents. Considering the high percentage of possibly associated conditions in our study, a detailed diagnostic work-up is crucial to identify treatable underlying conditions.
Acetazolamide (ACTZ), a carbonic anhydrase inhibitor, has been shown to decrease cerebrospinal fluid (CSF) production in both in vivo and in vitro animal models. We report two children with hydrocephalus who experienced multiple shunt failures, and who had externalised ventriculostomy drains (EVD) prior to ventriculopleural shunt placement. The effects of increasing doses of ACTZ on CSF production and subsequent tolerance to ventriculopleural shunts were evaluated. The patients had a 48% and a 39% decrease in their EVD CSF output when compared to baseline with maximum ACTZ dose of 75 mg/kg/day and 50 mg/kg/day, respectively (p < 0.05). This is the first report of change in CSF volume in children after extended treatment with ACTZ. ACTZ treatment in mechanically ventilated paediatric patients with hydrocephalus may improve tolerance of ventriculopleural shunts and minimise respiratory compromise. Potassium and bicarbonate supplements are required to correct metabolic disturbances.
To determine the differences in outcome in a group of children with idiopathic intracranial hypertension (IIH) who do not present with headaches.
Differences in epidemiological and aetiological factors, clinical presentation, and visual outcome were investigated in children with a diagnosis of IIH presenting with and without headaches to the Paediatric Neurology and Paediatric Ophthalmology Services at Guy's & St Thomas' Hospitals NHS Trust between 1997 and 2002.
Compared to the 29 children with headaches, the 12 children in the non-headache group were younger (7.3 v 9.5 years), presented with more neurological signs (33% v 10%), and were more likely to present with severe visual failure (33% v 4%), with a tenfold increased risk of an enlarged blind spot or field defects (50% v 5%). Permanent visual failure affected a third of all children in the non-headache group, but was rare in children presenting with headaches (33% v 3%), with one patient registered blind and two severely visually impaired.
The management of IIH is difficult in the absence of headache. Visual surveillance is vital. These children were treated with an aggressive management programme to reduce cerebrospinal fluid pressure by repeated lumbar puncture, medication, and early surgical intervention if required. Non-invasive monitoring techniques might contribute to a better understanding of the natural history of IIH, improved management, and visual outcome.
Idiopathic intracranial hypertension is characterised by increased intracranial pressure in the presence of normal cerebrospinal fluid, normal neuroimaging and no localising signs on neurologic exam except cranial nerve VI palsy. It is common in adults, particularly obese young women, but also occurs in children and adolescents. We discuss the clinical presentation of idiopathic intracranial hypertension in the pediatric population and how the presenting signs and symptoms may be different from those seen in adult patients. We discuss the differential diagnosis for idiopathic intracranial hypertension, as well as underlying conditions that may be associated with it. As in adults, therapy most often involves the use of carbonic anhydrase inhibitors, diuretics, and steroids. Children with vision loss due to idiopathic intracranial hypertension who not respond to aggressive medical therapy should be promptly referred for surgical intervention, including optic nerve fenestration and cerebrospinal (CSF) shunting procedures such as ventriculoperitoneal or lumboperitoneal shunting.
Neuroimaging is useful in benign intracranial hypertension (BIH), contributing to its diagnosis criteria and ruling out other causes of intracranial hypertension. CT and/or MR results may be normal or may show small ventricles, an empty sella, or more frequently optic nerve sheath dilatation. Diffusion as well as perfusion MR studies show conflicting results. Venous sinus imaging plays a growing role in BIH evaluation, because impaired cranial venous outflow is a common factor in the pathogenesis of BIH and venous sinus stenosis is frequently observed in this condition. Angiography is not the gold standard for this evaluation: angio-MR (with dynamic sequences) clearly demonstrates venous sinus stenosis (mostly located in the transverse sinus). We believe that this investigation must be carried out in every case of BIH. For medically refractory cases of BIH with associated sinus venous stenosis, sinus pressure recording may be necessary, sometimes leading to endovascular treatment of the stenosis (stenting). The results are promising, but long-term follow-up of these patients is needed.
IntroductionBenign intracranial hypertension (BIH) is a rare condition, especially in childhood. The aim of this study was to analyze retrospectively pediatric cases that were diagnosed and managed in the same institution during the 2002–2006 period.
The goal of medical treatment in benign intracranial hypertension (BIH) is to treat intracranial hypertension symptoms as well as to preserve vision. Reducing the production rate of cerebrospinal fluid can be achieved using acetazolamide and/or furosemide (carbonic anhydrase inhibitors), although acetazolamide is the most effective drug. The use of steroids is debatable in BIH. This review focuses on the pathophysiology of these medications, followed by the report of a series of 16 pediatric patients suffering from BIH (1996–2006). BIH was idiopathic for eight children. Depletive lumbar punctures were effective, but this result was often transient. All children were treated with acetazolamide. Doses of acetazolamide (10–20mg/kg per day) must be given every 8h to respect its kinetics. This treatment has to be continued for at least several months and decreasing the dosage must be progressive. Hypokalemia is always prevented with oral potassium. There was only one true treatment failure requiring surgery. The authors therefore advise acetazolamide as a first-line treatment (combined with etiologic treatment, if available, in cases of nonidiopathic situations) in BIH.
In patients presenting with intracranial hypertension without hydrocephalus, mass lesions, and with normal cerebrospinal fluid (CSF) composition (pseudotumor cerebri syndrome), the diagnosis of intracranial sinus venous thrombosis (ISVT) has crucial etiological, therapeutic and prognostic implications. Utilizing two well-defined groups of pseudotumor cerebri patients, one with magnetic resonance imaging (MRI) or angiography confirmed ISVT (17 patients) and the other in whom ISVT has been excluded (idiopathic intracranial hypertension [IIH], 27 patients), we investigated the characteristics that might be helpful in distinguishing them. No clinical or auxiliary findings differed between the ISVT and IIH groups except for female gender and lower CSF protein level, which were significantly associated with the latter. While the syndrome pseudotumor cerebri could be due to multiple causes including ISVT, the term IIH should be restricted for patients with isolated intracranial hypertension attributable to no other neurological or systemic disease. Since CT frequently misses ISVT, patients with pseudotumor cerebri syndrome should undergo MRI and MR venography before being labeled as IIH. We conclude that Modified Dandy's Diagnostic Criteria of pseudotumor cerebri, formulated prior to MRI era, can no longer be applied for the diagnosis of IIH.
To compare clinical features, visual characteristics, and treatment of idiopathic intracranial hypertension patients with and without papilledema.
Idiopathic intracranial hypertension does not often occur without papilledema. This study estimates the prevalence and compares the clinical characteristics of idiopathic intracranial hypertension patients with and without papilledema.
We performed a cross-sectional analysis of all idiopathic intracranial hypertension patients diagnosed at the University of Utah Neuro-Ophthalmology Unit between 1990 and 2003. Patient records were reviewed for presence of papilledema and other signs, symptoms, and treatment characteristics. Each patient without papilledema was matched to the patient with papilledema who was closest to his/her age and sex. McNemar's and Wilcoxon-signed rank sum tests were used to compare characteristics between matched pairs.
Among all patients (n = 353), the prevalence of those without papilledema was 5.7% (n = 20). Patients without papilledema reported photopsias (20%), and were found to have spontaneous venous pulsations (75%) and non-physiologic visual field constriction (20%) more often than did those with papilledema. Mean opening pressure, although above normal, was lower in patients without papilledema (mean = 309 mm cerebrospinal fluid) compared with those with papilledema (mean = 373 mm cerebrospinal fluid, P = .031). Idiopathic intracranial hypertension patients without papilledema had more frequent diagnostic lumbar punctures than did patients with papilledema. Visual acuities and treatment were similar between groups.
The clinical presentation of idiopathic intracranial hypertension without papilledema is only somewhat different from that of idiopathic intracranial hypertension with papilledema. The lower opening pressure in patients without papilledema may explain variations in symptoms and signs between the 2 groups. When there are visual field changes in idiopathic intracranial hypertension without papilledema, non-physiologic visual loss should be considered.
To identify the symptoms and coexisting medical conditions associated with idiopathic intracranial hypertension (IIH), we administered an 83-item questionnaire at the time of diagnosis to 50 IIH patients and 100 aged-matched controls. Ninety percent of the IIH patients were women; the mean age was 33. Obesity and recent weight gain were much more common among patients than controls. Symptoms most commonly reported by IIH patients were headache (94%), transient visual obscurations (TVO) (68%), and intracranial noises (ICN) (58%). Daily occurrence of these symptoms was much more common among patients than controls. Controls also reported these and other IIH symptoms, but at lower frequencies. Several conditions previously associated with IIH were no more common in patients than controls including iron deficiency anemia, thyroid disease, pregnancy, antibiotic intake, and use of oral contraceptives. We conclude that previous studies of IIH, mostly uncontrolled and retrospective, have underestimated the frequency of symptoms in IIH patients and reported chance and spurious associations with common medical conditions and medications. The profile of a young obese woman with headaches and either TVO or ICN should alert the clinician to the diagnosis of IIH, especially when the symptoms occur daily.
The prognosis for vision in most patients with pseudotumor cerebri is excellent; however, visual loss, which is the only serious complication, may occur either early or late in the course of the disease. A group of 57 patients was followed up five to 41 years with visual fields, visual acuity, and fundus photographs. Blinding visual loss or severe visual impairment in one or both eyes occurred in 14 patients, and in seven patients, this occurred months to years after the initial symptoms. Systemic hypertension was a significant risk factor for visual loss in patients with pseudotumor cerebri, and blindness occurred in eight of 13 patients who were hypertensive. Despite suggestions that blind spot measurement is useful for following up patients with this condition, we believe that sequential quantitative perimetry gives more complete information and is essential to rational decision making in the treatment of pseudotumor cerebri.
To investigate sex distribution, frequency of obesity, and other associated conditions among children and adolescents with idiopathic intracranial hypertension.
We conducted a retrospective chart review of patients aged 18 years or younger diagnosed with idiopathic intracranial hypertension between 1988 and 1995 at two medical centers. Meta-analyses were performed using our data pooled with published information.
Of 374 patients, 175 (46.8%) were male and 199 (53.2%) were female. Obesity was noted in 50 (29.6%) of the 169 patients for whom relevant data are available, and other associated conditions were noted in 185 (53.2%) of the 348 patients.
Idiopathic intracranial hypertension among children and adolescents affects boys and girls equally; concurrent obesity occurs less frequently than in adults; and other associated conditions or secondary causes are common.
To determine the weight loss associated with resolution of papilledema from idiopathic intracranial hypertension (IIH).
A retrospective study.
Fifteen consecutive female patients with IIH associated with obesity were studied.
Patients underwent weight loss and treatment with acetazolamide during a 24-week period.
The severity of papilledema was graded: absent (grade 0), mild (grade 1), moderate (grade 2), and marked (grade 3), based on a predetermined grading system ("gold standard") using stereoscopic photographs and the Frisén classification.
The 15 patients, with mean age of 31.3+/-8.8 years, had a mean weight of 110.5+/-28.7 kg and mean body mass index of 40.7+/-13.0 kg/m2. Eleven (73.3%) patients had improved papilledema during the 24-week study period, of which 10 (66.7%) had complete resolution of papilledema within a median time of 8.5 weeks. An average of 3.3% weight loss (+/-0.5% standard error of the mean) was observed among patients having a one-grade change in papilledema. Weight loss of 6.2%+/-0.6% standard error of the mean was associated with a three-grade change in papilledema (i.e., complete resolution of marked papilledema). Nine of the ten patients with complete resolution of papilledema also took acetazolamide. However, none (26.7%) of the four patients without weight loss had improvement in papilledema despite similar treatment with acetazolamide.
Approximately 6% weight loss was associated with resolution of marked papilledema in these authors' patients. The benefit of acetazolamide in IIH is questioned since weight loss, rather than acetazolamide, appeared to have been the catalyst for reducing the severity of papilledema.
To report the features of idiopathic intracranial hypertension in prepubertal children, with emphasis on presentation, treatment, and outcome.
We retrospectively reviewed the charts of all patients 11 years and younger diagnosed with idiopathic intracranial hypertension at two university-affiliated medical centers. Inclusion critera included papilledema, normal brain computed tomography or magnetic resonance imaging, cerebrospinal fluid pressure greater than 200 mm H2O, normal cerebrospinal fluid content, and a nonfocal neurologic examination except for sixth nerve palsy. Patients with concomitant systemic illness were excluded.
Of the 10 patients, four were girls and six were boys. Only one patient was obese. The most common presenting symptoms were stiff neck (four patients) and diplopia (four patients), and the most common presenting sign was strabismus (eight patients). Six of eight patients with strabismus had abducens nerve palsy (four bilateral), one patient had a sensory exotropia, and one had a comitant esotropia. Visual field abnormalities were present in 11 of 13 eyes (85%), and severe visual loss resulting in no light perception vision occurred in one eye of one patient. Nine patients were treated medically, four with a combination of prednisone or dexamethasone and acetazolamide and five with acetazolamide alone. One patient in this group also required a lumboperitoneal shunt. One patient was treated with lumbar puncture only. Resolution of papilledema occurred rapidly in all patients, with a mean of 4.7 +/- 2.6 months. Resolution of sixth nerve palsy also occurred rapidly in four of six patients in a mean of 1.6 +/- 1.2 months. One patient required strabismus surgery for persistent esotropia and one was lost to follow-up.
Idiopathic intracranial hypertension in prepubertal children is rare and is different than the disease in adults. In our series, there appeared to be no sex predilection, and obesity was uncommon. Children are likely to present with strabismus and stiff neck rather than headache or pulsatile tinnitus. Both papilledema and sixth nerve palsy resolved rapidly with treatment. However, children can sustain loss of visual field and visual acuity despite treatment.
Idiopathic "benign" intracranial hypertension is an uncommon but important cause of headache that can lead to visual loss. This study was undertaken to review our experience in the diagnosis and management of idiopathic intracranial hypertension, giving special attention to treatments used. A retrospective chart review was conducted on 32 patients diagnosed with idiopathic intracranial hypertension between 1984 and 1995. Subjects included 23 females and ranged in age from 2 to 17.5 years. Headache was the most common symptom, followed by nausea and vomiting, double vision, and visual loss. Papilledema was the most common sign. Others were VIth cranial nerve palsy and compromised visual acuity at or within 3 months of presentation. Management included administration of acetazolamide or corticosteroids, lumboperitoneal shunt, optic nerve fenestration, and repeat lumbar puncture. Treatment combinations were used in 40% of cases. During follow-up, headache, papilledema, and decreased visual acuity persisted for longer than 10 months in a significant number of patients. We conclude that idiopathic intracranial hypertension causes significant short- and long-term morbidity with no proven effective treatment available. A prospective study is needed to establish the indications for treatment and the efficacy of the treatments used.
The syndrome of increased intracranial pressure without hydrocephalus or mass lesion and with normal CSF composition, previously referred to as pseudotumor cerebri, is a diagnosis of exclusion now termed idiopathic intracranial hypertension (IIH). Diagnostic criteria of this disorder have not been updated since the Modified Dandy Criteria were articulated in 1985. Since then, new developments, including advances in neuroimaging technology and recognition of additional secondary causes of intracranial hypertension, have further enhanced the ability to diagnose conditions that may mimic IIH. These factors are not addressed in the Modified Dandy Criteria. This report describes updated diagnostic criteria for IIH that may be used for routine patient management and for research purposes.
The aim of this study was to determine the features of pseudotumor cerebri or idiopathic intracranial hypertension in prepubertal and pubertal children. We retrospectively reviewed patient charts of those 16 years and younger, diagnosed with pseudotumor cerebri/idiopathic intracranial hypertension. Our study group consisted of 27 patients; the mean age was 10.9 years, and there was a male-to-female ratio of 13 to 14. In the prepubertal group (n = 13), the male-to-female ratio was 8 to 5; in the pubertal group, (n = 14), the ratio was 5 to 9. Overweight or obesity was found in 16 (59%) patients. Outcome was favorable except for one who remained symptomatic. Pseudotumor cerebri/idiopathic intracranial hypertension in children is rare. Its characteristics differ from adults. We found the prepubertal group to be a distinct group since pseudotumor cerebri/idiopathic intracranial hypertension did not occur predominantly in females and was not associated with obesity.
The history, diagnosis, and therapy of idiopathic intracranial hypertension (IIH) (pseudotumor cerebri) are reviewed. Theories of pathogenesis are considered, the clinical presentation is described, and potential diagnostic and therapeutic challenges are explored.
An extensive literature review of IIH and related conditions (secondary pseudotumor syndromes) was performed. The history of and rationale for the diagnosis and medical and surgical approaches to treatment are reviewed. Available outcome studies are presented.
Diagnosis of IIH requires that the modified Dandy criteria be satisfied. Multiple potential contributing causes of intracranial hypertension must be identified or excluded. The clinical presentation most often includes headaches and papilledema, but many other findings have been described. The most important goal of therapy is to prevent or arrest progressive visual loss. Medical therapies include alleviation of associated systemic diseases, discontinuation of contributing medications, provision of carbonic anhydrase inhibitors, and weight loss. Surgical therapies include lumboperitoneal shunting, ventriculoperitoneal shunting, and optic nerve sheath fenestration. On the basis of the advantages and disadvantages of these treatment modalities, a suggested treatment paradigm is presented.
Idiopathic intracranial hypertension is the term to be adopted instead of pseudotumor cerebri. IIH remains an enigmatic diagnosis of exclusion. However, prompt diagnosis and thorough evaluation and treatment are crucial for preventing visual loss and improving associated symptoms.
To report a high frequency of idiopathic intracranial hypertension (IIH) in patients with cystinosis and to speculate on the relationship between these two disorders.
Retrospective case series and review of the literature regarding risk factors for the development of IIH in cystinosis.
Eight patients with cystinosis had documented papilledema, normal neuroimaging of the brain, cerebrospinal fluid (CSF) opening pressure greater than 200 mm of H2O, and normal CSF composition. No common medication, condition, or disease except cystinosis was found in these persons. Six of the patients had received prednisone, growth hormone, cyclosporine, oral contraceptives, vitamin D, or levothyroxine at the time of onset of IIH. Five patients had previous renal transplants.
No single risk factor for the development of IIH linked IIH to cystinosis in our patients. However, thrombosis susceptibility as a result of renal disease or impaired CSF reabsorption in the arachnoid villi as a result of cystine deposition might lead to the development of IIH in cystinosis.
Long-term prognosis and visual outcome of 54 patients with idiopathic intracranial hypertension (IIH) was studied. Mean observation period was 6.2 years; 33 patients had two or more recurrences. Visual acuity was preserved in all patients without recurrence and in 86% of patients with recurrences. Recurrences did not occur while patients were maintained on acetazolamide. No statistical difference was found between IIH patients who had only one event, compared to the recurrent group.
To review our experience with optic nerve sheath decompression for pediatric pseudotumor cerebri.
Retrospective chart review.
Seventeen eyes in 12 children younger than 16 years of age. All patients were either unresponsive or intolerant to medication. INTERVENTION, METHODS, OR TESTING: An optic nerve sheath fenestration was performed.
Optic nerve appearance, visual acuity, color vision, and visual fields.
The average age at surgery was 10.1 years of age. The average follow-up was 39.6 months. Headache was the most common presenting symptom. All patients showed improvement in optic nerve edema. Visual acuity improved or stayed the same in all surgical eyes (P = 0.0078). One patient required a neurosurgical lumbar peritoneal shunt, and 2 patients required acetazolamide on the last follow-up appointment. No patient had postoperative infection, loss of vision, or strabismus develop. Five of the patients in this study required sheath decompression on the other eye.
Optic nerve sheath decompression in children is safe, and the results are similar to those obtained in adults. Close follow-up is required, because 5 of 12 patients in this study required a contralateral optic nerve sheath decompression.
Idiopathic intracranial hypertension (IIH) is the syndrome of raised intracranial pressure without clinical, laboratory or radiological evidence of intracranial pathology. IIH is a relatively rare disease but rapidly increasing incidence is reported due to a global increasing incidence of obesity. Disease course is generally said to be self-limiting within a few months. However, some patients experience a disabling condition of chronic severe headache and visual disturbances for years that limit their capacity to work. Permanent visual defects are serious and not infrequent complications. The pathophysiology of IIH is still not fully understood. Advances in neuroimaging techniques have facilitated the exclusion of associated conditions that may mimic IIH. No causal treatment is yet known for IIH and existing treatment is symptomatic and rarely sufficient. The aim of this review is to provide an updated overview of this potentially disabling disease which may show a future escalating incidence due to obesity. Theories of pathogenesis, diagnostic criteria and treatment strategies are discussed.
Idiopathic intracranial hypertension is common in obese women and can lead to significant visual impairment. First described more than 100 years ago, the cause of the disorder remains unknown. Despite a multitude of proposed links, the aetiology has never been established. Impairment of cerebrospinal-fluid reabsorption is the most likely underlying pathophysiological cause of the raised pressure, but this notion has yet to be proven. Cerebral venous sinus abnormalities associated with the disorder need further exploration. Although the major symptoms of headache and visual disturbance are well documented, most data for disease outcome have been from small retrospective case series. No randomised controlled trials of treatment have been done and the management is controversial. The importance of weight loss needs clarification, the role of diuretics is uncertain, and which surgical intervention is the most effective and safe is unknown. Prospective trials to examine these issues are urgently needed.
To test the hypothesis that puberty is a risk factor for poorer visual outcome in idiopathic intracranial hypertension (IIH).
Retrospective chart review case series.
Setting: Tertiary referral center, neuro-ophthalmology unit. Patient population: Ninety-six patients with IIH followed for a minimum of one year. Observation: Age (grouped into prepubertal, pubertal, teenage, or adult), obesity, initial intracranial pressure (ICP), measurements and presence of hypertension, anemia, or renal failure were correlated with final visual outcome using chi(2), stepwise logistic regression, and model-selection log linear analyses. Main outcome measures: Visual outcome was graded into "excellent" -- no evidence of an optic neuropathy or any permanent visual field defect in either eye, "moderate"-- evidence of an optic neuropathy and/or a mild (nasal constriction) visual field defect, or "poor outcome" (peripheral constriction) -- permanent visual field defect.
Outcome data were complete for 96 patients. Moderate to poor visual outcome, as opposed to excellent, was significantly associated with puberty (P = .007 using the gender-specific definition of puberty, .0002 using the broad definition). Moderate-poor visual outcome occurred in none of seven IIH patients of prepubertal age (<9 years), in 15 of 26 patients presenting between nine to 16 years, in two of six patients aged 17 to 22 years, and in seven of 57 adult patients over the age of 23 years.
In this series of 96 patients with IIH, visual outcome was less favorable in pubertal patients than in prepubertal, teenage, and adult patients. We recommend that clinicians maintain a high index of awareness when caring for pubescent children with IIH.
Idiopathic intracranial hypertension is an enigmatic disorder of elevated cerebrospinal fluid pressure. In adulthood, patients are typically obese women of childbearing age; however, in young children the clinical picture is strikingly different, indicating age-related differences in the aetiology of idiopathic intracranial hypertension. To investigate this phenomenon, we analysed the clinical details of 15 pre-pubertal children with the diagnosis of idiopathic intracranial hypertension. Evaluating the date of initial presentation, we discovered a distinct seasonal variation. Ten patients presented between November and March, thus coinciding with the typical season of paediatric viral and bacterial infections in Germany. Therefore, we suggest an association between intracranial hypertension and possibly concurrent infections in these children. Moreover, eight children presented only with ophthalmologic findings without any other obvious symptoms, raising questions regarding the incidence of undetected cases, particularly in this age group.
Our understanding of pediatric idiopathic intracranial hypertension has been refined since Dr. Simmons Lessell's review in 1992. The use of rigorous methodologies and standard definitions in recent studies has demonstrated distinct demographic trends. Specifically, the incidence of idiopathic intracranial hypertension seems to be increasing among adolescent children, and among older children its clinical picture is similar to that of adult idiopathic intracranial hypertension (female and obese). Within younger age groups there are more boys and nonobese children who may develop idiopathic intracranial hypertension. The pathogenesis of the disease has yet to be elucidated. Idiopathic intracranial hypertension among young children has been associated with several new etiologies, including recombinant growth hormone and all-trans-retinoic acid. More modern neuroimaging techniques such as MRI and MRI-venograms are being used to exclude intracranial processes. Although most cases of pediatric idiopathic intracranial hypertension improve with medical treatment, those who have had visual progression despite medical treatment have undergone optic nerve sheath fenestration and lumboperitoneal shunting. Because idiopathic intracranial hypertension in young children appears to be a different disorder than in adolescents and adults, separate diagnostic criteria for younger children are warranted. We propose new criteria for pediatric idiopathic intracranial hypertension in which children should have signs or symptoms consistent with elevated intracranial pressure, be prepubertal, have normal sensorium, can have reversible cranial nerve palsies, and have an opening cerebrospinal fluid pressure greater than 180 mm H(2)O if less than age 8 and papilledema is present, but greater than 250 mm H(2)0 if age 8 or above or less than 8 without papilledema.
Idiopathic intracranial hypertension (IIH) is a rare condition of increased intracranial pressure (ICP) without any identifiable pathology. Despite intervention, the clinical course of IIH is often prolonged and recurring with potentially serious complications of distressing headache and blindness.1–10 Various therapeutic measures either alone or in combination have been used widely in children with IIH, however, their therapeutic efficacy has not been proven in controlled studies. This article provides a comprehensive review of clinical knowledge in childhood IIH and its various treatment modalities with an emphasis on the drugs used. It also highlights that current management is not evidence based and that well-designed multicentre randomised controlled trials are urgently needed.
Idiopathic intracranial hypertension was first described in 189711 as “serous meningitis”, followed by various names such as otitic hydrocephalus, toxic hydrocephalus, etc, pseudotumour cerebri,12 and the commonly known benign intracranial hypertension.13 Subsequently, IIH was introduced14 and is now a preferred term due to its identified risk (up to 40%) of visual impairment.2 6 7 15–19
The overall (including children and adult) annual incidence of IIH is estimated at 1–3 per 100 000 population.20–22 To date, the epidemiological data on childhood IIH, which are limited to hospital-based retrospective case series, vary greatly from 0.1–0.9 per 100 000 children.23 24 Both genders are equally affected in childhood IIH, whereas it is most common in obese young women in adult IIH. However, obesity has become increasingly dominant in recent paediatric reports, affecting 43–91% of teenage IIH cases.1 7 25 This raises the concern that the recent rise in the prevalence of childhood obesity will be associated with an increased incidence of paediatric IIH.
Although the key features of IIH are headache and papilloedema (table 1), neither of them is a prerequisite …
In this prospective study, we report fifty consecutive cases of bilateral papilledema without neurosurgical or obvious ophthalmologic etiology, referred to our institution between January 2005 and March 2007. Lumbar puncture with opening CSF pressure measurement distinguished two groups of patients: Group 1 (n=39) with and Group 2 (n=11) without intracranial hypertension. In Group 1, 9/39 patients presented secondary intracranial hypertension mainly due to cerebral venous thrombosis. In 30 patients, after complete investigations, a diagnosis of idiopathic intracranial hypertension was made: as commonly reported, patients were predominantly overweight (96.7% with body mass index>25kg/m2) young (mean age=27.6 years) and women (96.7%). Eleven patients with intracranial hypertension had no headaches. In Group 2, the most common diagnosis was bilateral non-arteritic anterior ischemic optic neuropathy, but rare causes have been identified.
To define characteristics of pediatric asymptomatic idiopathic intracranial hypertension (IIH).
We retrospectively reviewed our Neuro-Ophthalmology database (2000-2006) for all cases of symptomatic and asymptomatic pediatric IIH.
Out of 45 IIH cases, 14 (31.1%) were asymptomatic (incidental examination). When compared with children with symptomatic IIH, asymptomatic cases were younger [5.6 (1.8-15) vs 11.0 (5-17) years, P = 0.007], had lower percentage of obesity (14.3% vs 48.4%, P = 0.046), and had male predominance (71.4% vs 38.7%, P = 0.06). Asymptomatic cases required shorter duration of acetazolamide treatment [3 (0-8), vs 6 (0-20) months, P = 0.021], and resulted in complete resolution of swollen discs.
We speculate that asymptomatic IIH may be more common in young children and could represent a milder form or a presymptomatic phase before evolving into classic symptomatic IIH. Further studies to assess the clinical significance of asymptomatic IIH are warranted.
Vieira DSS, Masruha MR, Gonçalves AL, Zukerman E, Senne Soares CA, da Graça Naffah-Mazzacoratti M & Peres MFP. Idiopathic intracranial hypertension with and without papilloedema in a consecutive series of patients with chronic migraine. Cephalalgia 2008; 609–613. London. ISSN 0333-1024
Chronic migraine (CM) has been associated with idiopathic intracranial hypertension without papilloedema (IIHWOP), a significant percentage of these cases occurring in obese patients with intractable headache. A prospective study from February 2005 to June 2006 was made of 62 CM patients who fulfilled International Headache Society diagnostic criteria and had cerebral magnetic resonance venography (MRV) and lumbar puncture (LP) done. Two patients were excluded, six (10%) with elevated cerebrospinal fluid (CSF) open pressure (OP), five with body mass index (BMI) > 25. None of the patients had papilloedema or abnormal MRV. BMI and CSF OP were significantly correlated (r = 0.476, P < 0.001, Pearson's correlation test). Obesity (defined as BMI > 30) was a predictor of increase in intracranial pressure (defined as OP > 200 mmH2O) (f = 17.26, 95% confidence interval 6.0, 8.6; P < 0.001). From our study we strongly recommend that not only intractable CM patients with high BMI, but also first diagnosed patients with BMI > 30 should be systematically evaluated by a LP to rule out IIHWOP.
OEdèmes papillaires bilaté : e ´tude prospective de 50 patients
- Dehais C F Heran
Deschamps R, Dehais C, Heran F, et al. OEdèmes papillaires bilaté : e ´tude prospective de 50 patients. Rev neurol 2008;164:42–6.