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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 506-510

Diagnostic criteria for pediatric idiopathic intracranial hypertension – A tale of two cases


Department of Pediatric Ophthalmology, Strabismus and Neuro-Ophthalmology, CL Gupta Eye Institute, Ram Ganga Vihar, Phase II (Ext), Moradabad, Uttar Pradesh, India

Date of Submission24-May-2021
Date of Acceptance18-Oct-2021
Date of Web Publication13-Apr-2022

Correspondence Address:
Veenu Maan
Department of Pediatric Ophthalmology, Strabismus and Neuro-Ophthalmology, CL Gupta Eye Institute, Ram Ganga Vihar, Phase II (Ext), Moradabad - 244 001, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1386_21

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  Abstract 


There is a diagnostic dilemma for pediatric Idiopathic intracranial hypertension (IIH), presenting rarely. Most clinicians use the Friedman et al. criteria. However, it is not appropriate because of the variability in the cerebrospinal fluid (CSF) opening pressure and other parameters between children less than 8 years and adults. We report two cases of IIH in children less than 8 years. Both indicate a chance of misdiagnosis using the Friedman et al. criteria in pediatric IIH. Based on our observation, a combination of Rangwala et al.'s recommendation and Friedman et al.'s criteria is suggested for diagnosing pediatric IIH in children less than 8 years of age.

Keywords: Diagnostic criteria, idiopathic intracranial hypertension, pediatric


How to cite this article:
Maan V, Agarwal P. Diagnostic criteria for pediatric idiopathic intracranial hypertension – A tale of two cases. Indian J Ophthalmol Case Rep 2022;2:506-10

How to cite this URL:
Maan V, Agarwal P. Diagnostic criteria for pediatric idiopathic intracranial hypertension – A tale of two cases. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Dec 3];2:506-10. Available from: https://www.ijoreports.in/text.asp?2022/2/2/506/342882



Idiopathic intracranial hypertension (IIH) is defined as elevated intracranial pressure without clinical, radiologic, or laboratory evidence of a secondary cause.[1] Overall, the incidence of IIH in children is from 0.5 to 0.9 cases per 100,000.[2] The increasing incidence of IIH is observed among adolescents (12–15 years) as compared to young (2–12) years.[3] The Friedman et al.[4] criteria have been used for diagnosing pediatric as well as adult IIH. However, in cases of pediatric IIH, using the Friedman criteria of CSF opening pressure ≥280 mm H20 can lead to a missed diagnosis of pediatric IIH.

There is a need for specific diagnostic criteria for pediatric IIH. Based on our observation and cases, we suggest diagnostic criteria for pediatric IIH by combining the Friedman et al.[4] criteria and Rangwala et al.[5] recommendations.


  Case Reports Top


Case 1

A female child aged 7 years presented with chief complaints of double vision, severe headache, and vomiting for 1 week. The birth history and family history were not significant. There was no history of drug intake or any systemic disease. There was no recent history of weight gain or weight loss. The body weight was 22 kg. The body mass index was normal for age. On examination, she had best-corrected visual acuity of 20/30 (OD) and 20/30 (OS) with Snellen's chart and N6 (OU) at 30 cm with Snellen's near vision chart. The anterior segment examination was normal. There was a limitation in the abduction of −3 (OS) [Figure 1]a. There was esotropia of 20 prism diopter in primary gaze, 40 prism diopter esotropia in levoversion, and no esotropia in dextroversion. The fundus examination showed disk edema of grade 4 (OU) [Figure 1]b. The child was clinically diagnosed with disk edema (OU) with Abducens nerve palsy (OS). Non-contrast and contrast-enhancedMagnetic Resonance Imaging (MRI) of the brain was done. Globe indentation along with dilation of bilateral optic nerve sheath was noted in the axial cut of the MRI of the brain showing orbits [Figure 1]c. The magnetic resonance venography (MRV) with contrast was normal. The child was referred to a neuro physician. The neurological examination was normal. Lumbar puncture without sedation in lateral decubitus position, legs in a flexed position, was performed by a neurologist which revealed an opening CSF pressure of 220 mm H20 (measured with a standard manometer) and normal CSF composition. The clinical and radiological findings implicated a diagnosis of probable pediatric IIH with left abducens nerve palsy as per the criteria proposed by Friedman et al.[4]. An opening CSF pressure of ≥280 mm H20 CSF in children is to be considered as a definite diagnosis of pediatric IIH.
Figure 1: Case 1 at presentation; (a) Nine gaze photo showing limitation in abduction -3 in OS, (b) Fundus photo showing grade 4 disk edema OU, (c) Contrast-enhanced MRI of the brain, normal sagittal section (right image), axial section showing orbits suggestive of globe indentation and flattening of the posterior sclera (left image)

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However as per the recommendations by Rangwala et al.,[5] that is, to consider an upper limit of 180 mm H20 as the normal opening pressure for children <8 years with papilledema, this was considered as a definite diagnosis of pediatric IIH and treated as a pediatric IIH under the supervision of a neuro physician. Tablet acetazolamide 250 mg, half tablet three times a day, and tablets furosemide and spironolactone combination 25 mg, half tablet twice a day, were also prescribed as the patient was having severe signs and symptoms (OU Grade 4 disk edema, OS Abducens nerve palsy with severe headache, vomiting). There are reports of combining acetazolamide with furosemide to produce additive effects than with just acetazolamide alone.[6] Tablet calcium 500 mg once daily was also added along with the monitoring of serum electrolytes. The child was advised weekly follow-up. At 4 weeks, the best-corrected visual acuity of 20/25 (OU) with Snellen's chart, N6 (OU) at 30 cm with Snellen's near vision chart were noted with no diplopia. The extraocular movements were full and free [Figure 2]a. No esotropia or esophoria was noted on examination. The fundus examination revealed resolving disk edema (OU). [Figure 2]b.
Figure 2: Case 1 at 4 weeks; (a) Nine gaze photo showing full and free extraocular movements, (b) Fundus photo showing resolving disk edema OU

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Case 2

A female child aged 12 months presented with chief complaints of inward deviation OD with a history of vomiting, no history of fever or trauma. There was no history of any drug intake or recent vaccination. The birth history and family history were not significant. All milestones were within normal limits. The body weight was 9.5 kg. The child was fixing following torch light OU. There was a limitation of abduction -1 OD with esotropia in OD suggestive of OD abducens nerve palsy [Figure 3]a. The anterior segment was normal. The fundus examination revealed mild blurring of the disk margins OU [Figure 3]b. The MRI of the brain scan with and without contrast was advised. Tortuous optic nerves, prominent subarachnoid space around the retrobulbar parts of the optic nerve, and flattening of the posterior sclera were noted in the axial cut of the MRI of the brain showing orbits [Figure 3]c. The MRV with contrast was normal. The neurological examination by a pediatric neurologist was normal. There was no sign of bulging of the anterior fontanelle. However, lumbar puncture without sedation in the lateral decubitus position, legs in flexed position was performed by a neurologist and revealed a CSF opening pressure of 200 mm H20 (measured with a standard manometer). The CSF composition was normal. As per the Rangwala et al.[5] recommendation, this patient was considered as a definite pediatric IIH case considering the upper limit of 180 mm H20 as the normal opening CSF pressure in children <8 years with papilledema. The child was further managed by a pediatric neurologist. Syrup acetazolamide (15 mg/kg/day in three divided doses) was prescribed for about 6 weeks. A weekly follow-up was done till there was a resolution of OU disk edema and full recovery of abduction limitation in OD. The complete recovery took approximately 6 weeks after the initiation of the treatment. Thereafter, the child was advised observation.
Figure 3: Case 2 at presentation; (a) Gaze photo showing limitation in abduction -1 in OD, (b) Fundus photo showing blurring of the disk margins OU, (c) MRI of the brain, axial section with orbits showing prominent subarachnoid space around the retrobulbar parts of the optic nerve and flattening of the posterior sclera

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In both cases, the other causes of disk edema like traumatic injury, atypical optic neuritis, anemia-iron deficiency, aplastic anemia, and sickle cell disease were also considered.[7] Hydrocephalous, encephalitis, meningitis, abscess, intracranial space-occupying lesion (ICSOL), structural abnormalities of the brain were also considered as differential diagnoses. All these differential diagnoses were ruled out by a thorough history taking, proper ophthalmic, general, and neurological workup, necessary hematological, laboratory investigations, and radiological investigations. Since this is an retrospective review of cases, the requirement of informed consent was waived of by the ethics committee. However consent for treatment was taken at the time of treatment provided. This case series was reviewed and approved by CL Gupta Eye Institute Ethics Committee.


  Discussion Top


Currently, the criteria used for the diagnosis of IIH is proposed by Friedman et al.[4] [Table 1]. It suggests that the opening CSF pressure of ≥280 mm H20 in children is to be considered for a definite diagnosis of pediatric IIH. Whereas, the CSF opening pressure recommended by Rangwala et al.[5]
Table 1: Diagnostic criteria for IIH proposed by Friedman et al.[4]

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  • In children <8 years with papilledema—the upper limit of 180 mm H20 is to be considered as the normal opening pressure.
  • In children <8 years without papilledema—the adult norm of 250 mm H20 is considered as a normal opening CSF pressure.
  • In children >8 years with or without papilledema—the adult norm of 250 mm H20 is considered as a normal opening CSF pressure.


Fishman et al.[8] also suggested that the normal opening CSF pressure in a young child may vary between 10 and 100 mm H20 and will approach adult levels only after the age of 8 years. A study by Avery et al.[9] established a normal opening pressure of 196 mm H20 in children with lower and upper limits of normal as 105 and 280 mm H20, respectively. The patients' reference group consisted of children between 1 and 18 years of age and none of them were taking medication or had signs of a disease or condition that would alter the opening pressure on lumbar puncture (e.g., papilledema, hydrocephalus, cerebral edema,  Chiari malformation More Details, meningitis, or the use of diuretics).[9] Based on the available data, our observations,[10] and cases, we recommend that the diagnostic criteria for pediatric IIH should take into consideration the opening CSF pressure in children as recommended by Rangwala et al.[5]


  Conclusion Top


As of now, there is a diagnostic dilemma for pediatric IIH. We suggest diagnostic criteria for pediatric IIH [Table 2]. These criteria are crucial as they will help in preventing the misdiagnosis of pediatric IIH, especially in children less than 8 years, who are diagnosed as probable IIH following the adult norm of opening the CSF pressure in the currently followed criteria by Friedman et al.[4] It will also help in a definite diagnosis and better management of pediatric IIH.
Table 2: Suggested diagnostic criteria for pediatric IIH (Maan's Criteria)

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Acknowledgments

Authors thank Mr. Lokesh Chauhan; Department of Clinical Research, CL Gupta Eye Institute, Moradabad (India) for his technical support.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neurol 1988;45:875-7.  Back to cited text no. 1
    
2.
Mercille G, Ospina LH. Pediatric idiopathic intracranial hypertension: A review. Pediatr Rev 2007;28:e77-86.  Back to cited text no. 2
    
3.
Gordon K. Pediatric pseudotumor cerebri: Descriptive epidemiology. Can J Neurol Sci 1997;24:219-21.  Back to cited text no. 3
    
4.
Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013;81:1159-65.  Back to cited text no. 4
    
5.
Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 2007;52:597-617.  Back to cited text no. 5
    
6.
Schoeman JF. Childhood pseudotumor cerebri: Clinical and intracranial pressure response to acetazolamide and furosemide treatment in a case series. J Child Neurol 1994;9:130-4.  Back to cited text no. 6
    
7.
Segal S, Discepola M. Idiopathic intracranial hypertension and sickle cell disease: Two case reports. Can J Ophthalmol 2005;40:764-7.  Back to cited text no. 7
    
8.
Fishman RA. Cerebrospinal Fluid in Diseases of the Nervous System. 2nd ed. Philadelphia, PA: WB Saunders; 1992.  Back to cited text no. 8
    
9.
Avery RA, Shah SS, Licht DJ, Seiden JA, Huh JW, Boswinkel J, et al. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med 2010;363:891-3.  Back to cited text no. 9
    
10.
Gupta S, Maan V, Agarwal P. Diagnostic criteria in pediatric intracranial hypertension. J AAPOS 2017;21:492-5.e2.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

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