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Table of Contents    
Year : 2013  |  Volume : 61  |  Issue : 5  |  Page : 488-490

Idiopathic intracranial hypertension in pediatric population: Case series from India

Department of Neurology, Amrita Institute of Medical Sciences, Cochin, Kerala, India

Date of Submission16-Sep-2013
Date of Decision07-Oct-2013
Date of Acceptance23-Oct-2013
Date of Web Publication22-Nov-2013

Correspondence Address:
Arun Grace Roy
Department of Neurology, Amrita Institute of Medical Sciences, Ponekkara Lane, Cochin
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.121915

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 » Abstract 

Background: Idiopathic intracranial hypertension (IIH) is a well described entity in adults. In pediatric age group the presentation of disease can vary depending on the age of patients and is less frequently reported. Aim: The aim of this study is to describe the clinical features, investigations, treatment and outcome of IIH in pediatric population (age <18 years). Materials and Methods: This retrospective hospital based study was carried out on 25 children with diagnosis of IIH based on modified Dandys criteria. Their clinical, investigation, treatment, outcome and follow-up for 2 year period were analyzed. Results: Out of the 25 children, the youngest child was 4-month-old infant. The commonest symptom was headache (76%) followed by vomiting and papilledema (72%). The mean cerebrospinal fluid (CSF) pressure was 330 mm of H 2 O. In Infants irritability and bulging anterior fontanelle was seen. A total of 24 patients showed a complete resolution of symptom. None of patient had recurrence over a period of 2 years follow-up. Conclusion: IIH can present at any age group. This is the largest series of IIH reported in pediatric population in India. The clinical features are similar to adult patients except in infants. Absence of papilledema does not exclude the diagnosis of IIH. CSF pressure monitoring is needed in suspected cases of IIH. Early and prompt treatment can prevent deficits.

Keywords: Idiopathic intracranial hypertension, intracranial pressure, papilledema

How to cite this article:
Roy AG, Vinayan KP, Kumar A. Idiopathic intracranial hypertension in pediatric population: Case series from India. Neurol India 2013;61:488-90

How to cite this URL:
Roy AG, Vinayan KP, Kumar A. Idiopathic intracranial hypertension in pediatric population: Case series from India. Neurol India [serial online] 2013 [cited 2022 Aug 18];61:488-90. Available from: https://www.neurologyindia.com/text.asp?2013/61/5/488/121915

 » Introduction Top

Idiopathic intracranial hypertension (IIH) is a syndrome characterized by raised intracranial pressure (ICP) with normal cerebrospinal fluid (CSF) components and no secondary cause detected on imaging. [1] Clinical features are secondary to raised ICP. Syndrome is well described in middle aged obese women. IIH can present in children of any age group. Clinical features in post-pubertal age group are similar to adult population while in pre-pubertal age group it can be varying. Here we studied 25 children (<18 years) with IIH to determine the clinical profile and outcome.

 » Materials and Methods Top

We reviewed the records of patients below the age of 18 years with the diagnosis of or pseudotumor cerebri with minimum 2 year follow-up from the day of diagnosis. The study period was between January 2005 and 2008. Inclusion criteria included children aged less than 18 years and satisfying the modified Dandys criteria. Children were divided into pre-pubertal (12 years or <12 years) and pubertal (>12 years of age). Exclusion criteria were cerebral venous sinus thrombosis, intracranial space occupying lesions and bleed, meningitis and renal failure.

Demographic features, presenting symptoms, duration of symptoms, presence of any systemic disease, relevant drug history (corticosteroids, vitamins) were noted. All patients had detailed ophthalmology evaluation including optic disc examination and Humphrey's 30-2 visual field analysis. Magnetic resonance imaging (MRI) scan with magnetic resonance venography (MRV) and CSF analysis and pressure recording was performed for all patients. Thyroid function test was done in all children and serum cortisol assay was done in children with obesity.

 » Results Top

During the study period, 25 children fulfilled the inclusion and exclusion criteria of IIH. The age wise distribution is shown in [Table 1] with youngest being a 4-month-old infant. The sex distribution was equal in pre-pubertal age group and female predominance was seen in pubertal age group. The most common presenting symptom was headache (76%) followed by papilledema (72%), vomiting (72%), irritability (37%), transient visual obscurations (31%), diplopia (16%), bulging anterior fontanelle 16%) squint (16%) and one (4%) child had optic atrophy.
Table 1: Age wise distribution of patients

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Headache was continuous in majority of children and nocturnal aggravation was reported by four children. Bilateral papilledema was seen in 18 children and one of them developing optic atrophy. Transient visual obscuration was seen in pubertal age. In infants, irritability, bulging anterior fontanelle and squint were the presenting symptoms and two infants had papilledema. The duration of symptom before presentation was less than 1 month in all the children except for three children where it was less than 4 months. Obesity was seen in only one patient. Possible etiology for the disorder was vitamin drop intake in two infants.

Of the 25 children, 13 children were cooperative for formal visual acuity testing by Snellens chart and field assessment. Visual acuity was normal in 11 children and two had 6/6 corrected vision with pinhole. Field assessment showed enlargement of blind spot in six, constriction of peripheral field in one and rest six had normal field. Six children with papilledema had normal field. CSF pressure showed a range of 280-400 mm of H 2 O, with mean CSF pressure of 330 mm of H 2 O. CSF biochemistry and cytology were normal. Brain MRI and MRV were normal in all patients. Thyroid hormone assay was normal in all children and serum cortisol level was normal in obese children whom it was done.

Acetazolamide was started in all the children. Topiramate and frusemide were also tried as combination with acetazolamide in eight patients who had CSF pressure on higher side. Corticosteroid was given to the child with optic atrophy, who later underwent thecoperitoneal shunt surgery for refractory symptoms. All patients were asymptomatic within 1 week of treatment. Papilledema resolved by 1 month in all patients. Drugs were tapered and stopped over 6 months and none of the patients had relapse.

 » Discussion Top

IIH is a rare entity in pediatric age group. This is probably the largest series reported from India. In our series, 12 children were pre-pubertal with four cases in infancy and 13 were post-pubertal. The youngest was 4-month-old infant. Previous reports have shown that incidence is high in post-pubertal children and the incidence in infancy is rare. [2],[3] The male female ratio in pre-pubertal age group was equal with a higher incidence in females in post-pubertal age group. These findings are similar to previous studies.

In children, the most common presenting symptom is headache, which was generalized with classical features of raised ICP seen in only four children. [3],[4] In infants where headache is not a symptom, irritability, squint and bulging anterior fontanelle were the presenting symptom. This finding was noted in previous studies also. [2],[5] Papilledema was not seen in seven children of whom two were infants. Papilledema is the hallmark finding in IIH, however, there have been case descriptions where papilledema can be absent in IIH. [6],[7] Child with optic atrophy had CSF pressure of 380 mm of H 2 O and symptoms of 3 months duration. Secondary optic atrophy can develop if prompt control of ICP is not done.

In adults, obesity is proven risk factor for IIH, but it has been shown previously that children with IIH are not generally obese and our findings were consistent with this since only child had obesity. [7],[8] Vitamin intake were seen in our series, which could be a risk factor for IIH [9] and vitamin level assay was not done to look for elevated levels in these patients.

Increased CSF pressure with normal CSF cytology and biochemical study is prerequisite for diagnosis of IIH. In adults, the cut-off value of CSF pressure to diagnose IIH is clear, but in children the upper limit of normal CSF pressure is not well defined. In general, CSF pressure more than 200 mm of H 2 O in young children and above 250 mm of H 2 O in older children is taken. The opening CSF pressure was between 280 and 400 mm of H 2 O (mean value of 330 mm of H 2 O) with normal CSF content.

Field assessment is technically difficult in younger children. In this study, the field defects noted included: Enlargement of blind spot in six and constriction of peripheral visual fields in one. Various field defects has been reported in IIH, the most common defect is an enlarged blind spot, which has been reported to occur in virtually all eyes with papilledema, [10],[11] but normal field in patients who has papilledema has been previously reported. [7],[11] On follow-up field, defects became normal in all children except in the child with peripheral constriction of field.

Normal imaging is a prerequisite for the correct diagnosis of IIH. MRI brain can show various signs of raised ICP in children such as empty sella, distension of the perioptic subarachnoid space, venous tortuosity and elongation of the optic nerve and flattening of the posterior aspect of the globe and compressed ventricles, [12] we looked for each of the above findings, but all patients had normal MRI. Reversal of the imaging findings are seen with treatment and can be used as an indicator of response to therapy also.

Most of the cases of pediatric IIH respond well to adequate medical therapy. [13] In our series, all except one child responded to medical therapy, mainly acetazolamide. One child who had optic atrophy underwent thecoperitoneal shunting. Thecoperitoneal shunting is preferred for those children who failed to respond medically or after repeated lumbar taps. [14] Headache was the first symptom to improve followed by sixth nerve palsy with 95% of children showing resolution of features within 1 week of treatment. At 3 months, all patients were asymptomatic and had resolution of papilledema. One child who had optic atrophy had persistent field defect on follow-up. These findings were similar to previous studies, which showed resolution of headaches with reduction in cerebrospinal opening pressure and majority of children having complete resolution of disc swelling and visual abnormalities after treatment, however 25% can have visual field deficit and acuity loss. [15],[16] None of our patients had recurrence of symptom on follow-up of 2 year, which is most likely to occur during the first 18 months after diagnosis. [16]

 » References Top

1.Dandy WE. Intracranial pressure without brain tumor: diagnosis and treatment. Ann Surg 1937;106:492-513.  Back to cited text no. 1
2.Babikian P, Corbett J, Bell W. Idiopathic intracranial hypertension in children: The Iowa experience. J Child Neurol 1994;9:144-9.  Back to cited text no. 2
3.Standridge SM. Idiopathic intracranial hypertension in children: A review and algorithm. Pediatr Neurol 2010;43:377-90.  Back to cited text no. 3
4.Distelmaier F, Sengler U, Messing-Juenger M, Assmann B, Mayatepek E, Rosenbaum T. Pseudotumor cerebri as an important differential diagnosis of papilledema in children. Brain Dev 2006;28:190-5.  Back to cited text no. 4
5.Grant DN. Benign intracranial hypertension. A review of 79 cases in infancy and childhood. Arch Dis Child 1971;46:651-5.  Back to cited text no. 5
6.Faz G, Butler IJ, Koenig MK. Incidence of papilledema and obesity in children diagnosed with idiopathic "benign" intracranial hypertension: Case series and review. J Child Neurol 2010;25:1389-92.  Back to cited text no. 6
7.Kesler A, Fattal-Valevski A. Idiopathic intracranial hypertension in the pediatric population. J Child Neurol 2002;17:745-8.  Back to cited text no. 7
8.Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: Characteristics, treatment, and outcome. Am J Ophthalmol 1999;127:178-82.  Back to cited text no. 8
9.Soler D, Cox T, Bullock P, Calver DM, Robinson RO. Diagnosis and management of benign intracranial hypertension. Arch Dis Child 1998;78:89-94.  Back to cited text no. 9
10.Corbett JJ. The 1982 Silversides lecture. Problems in the diagnosis and treatment of pseudotumor cerebri. Can J Neurol Sci 1983;10:221-9.  Back to cited text no. 10
11.Ambika S, Arjundas D, Noronha V, Anshuman. Clinical profile, evaluation, management and visual outcome of idiopathic intracranial hypertension in a neuro-ophthalmology clinic of a tertiary referral ophthalmic center in India. Ann Indian Acad Neurol 2010;13:37-41.  Back to cited text no. 11
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12.Degnan AJ, Levy LM. Pseudotumor cerebri: Brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol 2011;32:1986-93.  Back to cited text no. 12
13.Warman R. Management of pseudotumor cerebri in children. Int Pediatr 2000;15:147.  Back to cited text no. 13
14.Corbett JJ, Thompson HS. The rational management of idiopathic intracranial hypertension. Arch Neurol 1989;46:1049-51.  Back to cited text no. 14
15.Salman MS, Kirkham FJ, MacGregor DL. Idiopathic "benign" intracranial hypertension: Case series and review. J Child Neurol 2001;16:465-70.  Back to cited text no. 15
16.Soiberman U, Stolovitch C, Balcer LJ, Regenbogen M, Constantini S, Kesler A. Idiopathic intracranial hypertension in children: Visual outcome and risk of recurrence. Childs Nerv Syst 2011;27:1913-8.  Back to cited text no. 16


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