Abstract
Purpose
Idiopathic intracranial hypertension (IIH) is a disorder associated with increased intracranial pressure without evidence of a space-occupying lesion and with normal cerebrospinal fluid constituents. The disease is rare in the pediatric population. In this study, we assessed the visual outcome of children with IIH and the risk of recurrence.
Methods
This single-center observational retrospective cohort study included 90 children younger than 18 years of age who satisfied the modified Dandy criteria for the diagnosis of IIH. Upon follow-up, the treatment was discontinued when patients were free of symptoms such as headaches, transient visual obscurations or tinnitus, and when examination revealed no evidence of papilledema. The main outcome measures were visual acuity and visual field outcomes as well as risk of recurrence.
Results
The mean follow-up was 30.65 months (range 1.15–172.6 months, standard deviation 27.47 months). Special grading scales were devised for visual acuity and visual field scores. The mean visual acuity score improved from 4.7 ± 0.62 to 4.87 ± 0.44 (p = 0.003).The mean visual field score improved from 3.41 ± 0.8 to 3.52 ± 0.75 (p = 0.21). The recurrence rate was 23.7%, and the risk of recurrence was highest within the first 18 months after diagnosis of IIH.
Conclusions
These study results suggest that pediatric patients with IIH have a favorable visual outcome in terms of both visual acuity and visual field. If there is any recurrence, it is most likely to occur during the first 18 months after diagnosis.
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References
Kesler A, Goldhamer Y, Gadoth N (2001) Do men with pseudotumor cerebri share the same characteristics as women? A retrospective review of 141 cases. J Neuroophthalmol 21:15–17
Digre KB, Corbett JJ (2001) Idiopathic intracranial hypertension (pseudotumor cerebri): a reappraisal. Neurologist 7:2–6
Radhakrishnan K, Ahlskog JE, Cross SH, Kurland LT, O'Fallon WM (1993) Idiopathic intracranial hypertension (Pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn. 1976 to 1990. Arch Neurol 50:78–80
Ahlskog JE, O'Neil BP (1982) Pseudotumor cerebri. Ann Intern Med 97:249–256
Radhakrishnan K, Ahlskog JE, Garrity JA, Kurland LT (1994) Idiopathic intracranial hypertension. Mayo Clin Proc 69:169–180
Friedman DI, Jacobson DM (2002) Diagnostic criteria for idiopathic intracranial hypertension. Neurology 59:1492–1505
Lessell S (1992) Pediatric pseudotumor cerebri (idiopathic intracranial hypertension). Surv Ophthalmol 37:155–166
Wall M, George D (1991) Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain 114(Pt 1A):155–180
Babikian P, Corbett J, Bell W (1994) Idiopathic intracranial hypertension in children: the Iowa experience. J Child Neurol 9:144–149
Baker RS, Baumann RJ, Buncic JR (1989) Idiopathic intracranial hypertension (pseudotumor cerebri) in pediatric patients. Pediatr Neurol 5:5–11
Digre KB, Corbett JJ (1988) Pseudotumor cerebri in men. Arch Neurol 45:866–872
Phillips PH, Repka MX, Lambert SR (1998) Pseudotumor cerebri in children. J AAPOS 2:33–38
McKiernan SP, DiFazio MP (2001) Index of suspicion. Case 3. Diagnosis: infantile pseudotumor cerebri. Pediatr Rev 22:211–215
Gordon K (1997) Pediatric pseudotumor cerebri: descriptive epidemiology. Can J Neurol Sci 24:219–221
Cinciripini GS, Donahue S, Borchert MS (1999) Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment and outcome. Am J Ophthalmol 127:178–182
Stiebel-Kalish H, Kalish Y, Lusky GDD, Ehrlich R, Shuper A (2006) Puberty as a risk factor for less favorable visual outcome in idiopathic intracranial hypertension. Am J Ophthalmol 142:279–283
Giuseffi V, Wall M, Siegel PZ, Rojas PB (1991) Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case–control study. Neurology 41:239–244
Brodsky MC, Baker RS, Hamed LM (1996) Pediatric neuro-ophthalmology. Springer, New York
Rangwala LM, Liu GT (2007) Pediatric idiopathic intracranial hypertension. Surv Ophthalmol 52:597–617
Avery RA, Shah SS, Licht DJ, Seiden JA, Huh JW, Boswinkel J, Ruppe MD, Chew A, Mistry RD, Liu GT (2010) Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med 363:891–893
Standridge SM (2010) Idiopathic intracranial hypertension in children: a review and algorithm. Pediatr Neurol 43:377–390
Kesler A, Hadayer A, Goldhammer Y, Almog Y, Korczyn AD (2004) Idiopathic intracranial hypertension: risk of recurrence. Neurology 63:1737–1739
Tibussek D, Schneider DT, Vandemeulebroecke N, Turowski B, Messing-Juenger M, Willems PH, Mayatepek E, Distelmaier F (2010) Clinical spectrum of the pseudotumor cerebri complex in children. Child Nerv Syst 26:313–321
Balcer LJ, Liu GT, Forman S, Pun K, Volpe NJ, Galetta SL, Maguire MG (1999) Idiopathic intracranial hypertension: relation of age and obesity in children. Neurology 52:870–872
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Soiberman, U., Stolovitch, C., Balcer, L.J. et al. Idiopathic intracranial hypertension in children: visual outcome and risk of recurrence. Childs Nerv Syst 27, 1913–1918 (2011). https://doi.org/10.1007/s00381-011-1470-5
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DOI: https://doi.org/10.1007/s00381-011-1470-5