Semin Neurol 2019; 39(06): 704-710
DOI: 10.1055/s-0039-1698743
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pediatric Idiopathic Intracranial Hypertension

Eric D. Gaier
1   Department of Ophthalmology, Boston Children's Hospital, Boston, Massachusetts
2   Harvard Medical School, Boston, Massachusetts
,
Gena Heidary
1   Department of Ophthalmology, Boston Children's Hospital, Boston, Massachusetts
2   Harvard Medical School, Boston, Massachusetts
› Author Affiliations
Further Information

Publication History

Publication Date:
17 December 2019 (online)

Abstract

The presentation of idiopathic intracranial hypertension (IIH) in pediatric populations has several important distinctions from that in adults, especially among prepubertal patients, in which there is no apparent association with gender or obesity. Pediatric patients are more likely to be asymptomatic or present with atypical symptoms than their adult counterparts, posing a diagnostic challenge in some cases. It is important to be aware of the ways in which diagnostic criteria for IIH are modified from that of adults. Ideal treatment practices and the natural history of pediatric IIH remain unclear. Acetazolamide is the mainstay of medical treatment, but some patients with significant visual loss may require surgical intervention. Multicenter studies to accrue a large number of cases and future prospective studies will help to better define pediatric IIH and to formulate consensus guidelines for treatment and management of these patients.

 
  • References

  • 1 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013; 81 (13) 1159-1165
  • 2 Gillson N, Jones C, Reem RE, Rogers DL, Zumberge N, Aylward SC. Incidence and demographics of pediatric intracranial hypertension. Pediatr Neurol 2017; 73: 42-47
  • 3 Aylward SC, Waslo CS, Au JN, Tanne E. Manifestations of pediatric intracranial hypertension from the intracranial hypertension registry. Pediatr Neurol 2016; 61: 76-82
  • 4 Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. population studies in Iowa and Louisiana. Arch Neurol 1988; 45 (08) 875-877
  • 5 Gordon K. Pediatric pseudotumor cerebri: descriptive epidemiology. Can J Neurol Sci 1997; 24 (03) 219-221
  • 6 Balcer LJ, Liu GT, Forman S. , et al. Idiopathic intracranial hypertension: relation of age and obesity in children. Neurology 1999; 52 (04) 870-872
  • 7 Glatstein MM, Oren A, Amarilyio G. , et al. Clinical characterization of idiopathic intracranial hypertension in children presenting to the emergency department: the experience of a large tertiary care pediatric hospital. Pediatr Emerg Care 2015; 31 (01) 6-9
  • 8 Sheldon CA, Paley GL, Xiao R. , et al. Pediatric idiopathic intracranial hypertension: age, gender, and anthropometric features at diagnosis in a large, retrospective, multisite cohort. Ophthalmology 2016; 123 (11) 2424-2431
  • 9 Tepe D, Demirel F, Seker ED. , et al. Prevalence of idiopathic intracranial hypertension and associated factors in obese children and adolescents. J Pediatr Endocrinol Metab 2016; 29 (08) 907-914
  • 10 Brara SM, Koebnick C, Porter AH, Langer-Gould A. Pediatric idiopathic intracranial hypertension and extreme childhood obesity. J Pediatr 2012; 161 (04) 602-607
  • 11 Stiebel-Kalish H, Serov I, Sella R, Chodick G, Snir M. Childhood overweight or obesity increases the risk of IIH recurrence fivefold. Int J Obes 2014; 38 (11) 1475-1477
  • 12 Sheldon CA, Kwon YJ, Liu GT, McCormack SE. An integrated mechanism of pediatric pseudotumor cerebri syndrome: evidence of bioenergetic and hormonal regulation of cerebrospinal fluid dynamics. Pediatr Res 2015; 77 (02) 282-289
  • 13 Kesler A, Fattal-Valevski A. Idiopathic intracranial hypertension in the pediatric population. J Child Neurol 2002; 17 (10) 745-748
  • 14 Aylward SC, Aronowitz C, Reem R, Rogers D, Roach ES. Intracranial hypertension without headache in children. J Child Neurol 2015; 30 (06) 703-706
  • 15 Bassan H, Berkner L, Stolovitch C, Kesler A. Asymptomatic idiopathic intracranial hypertension in children. Acta Neurol Scand 2008; 118 (04) 251-255
  • 16 Whitecross S, Heidary G. Asymptomatic pediatric idiopathic intracranial hypertension. J AAPOS 2013; 17 (01) e31
  • 17 Aylward SC, Aronowitz C, Roach ES. Intracranial hypertension without papilledema in children. J Child Neurol 2016; 31 (02) 177-183
  • 18 Liu B, Murphy RK, Mercer D, Tychsen L, Smyth MD. Pseudopapilledema and association with idiopathic intracranial hypertension. Childs Nerv Syst 2014; 30 (07) 1197-1200
  • 19 Dandy WE. Intracranial pressure without brain tumor: diagnosis and treatment. Ann Surg 1937; 106 (04) 492-513
  • 20 Smith JL. Whence pseudotumor cerebri?. J Clin Neuroophthalmol 1985; 5 (01) 55-56
  • 21 Wall M, McDermott MP, Kieburtz KD. , et al; NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA 2014; 311 (16) 1641-1651
  • 22 Standridge SM, O'Brien SH. Idiopathic intracranial hypertension in a pediatric population: a retrospective analysis of the initial imaging evaluation. J Child Neurol 2008; 23 (11) 1308-1311
  • 23 Hollander JN, Prabhu S, Heidary G. Utilization of MRV to evaluate pediatric patients with papilledema. J AAPOS 2014; 18 (04) 2
  • 24 Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 1998; 105 (09) 1686-1693
  • 25 Gilbert AL, Vaughn J, Robson C, Whitecross S, Heidary G. Radiographic features in pediatric idiopathic intracranial hypertension. J AAPOS 2016; 20 (04) 1
  • 26 Görkem SB, Doğanay S, Canpolat M. , et al. MR imaging findings in children with pseudotumor cerebri and comparison with healthy controls. Childs Nerv Syst 2015; 31 (03) 373-380
  • 27 Hartmann AJ, Soares BP, Bruce BB. , et al. Imaging features of idiopathic intracranial hypertension in children. J Child Neurol 2017; 32 (01) 120-126
  • 28 Dwyer CM, Prelog K, Owler BK. The role of venous sinus outflow obstruction in pediatric idiopathic intracranial hypertension. J Neurosurg Pediatr 2013; 11 (02) 144-149
  • 29 Stienen A, Weinzierl M, Ludolph A, Tibussek D, Häusler M. Obstruction of cerebral venous sinus secondary to idiopathic intracranial hypertension. Eur J Neurol 2008; 15 (12) 1416-1418
  • 30 Avery RA, Shah SS, Licht DJ. , et al. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med 2010; 363 (09) 891-893
  • 31 Gerstl L, Schoppe N, Albers L. , et al. Pediatric idiopathic intracranial hypertension - Is the fixed threshold value of elevated LP opening pressure set too high?. Eur J Paediatr Neurol 2017; 21 (06) 833-841
  • 32 Tibussek D, Distelmaier F, Kummer S, von Kries R, Mayatepek E. Sedation of children during measurement of CSF opening pressure: lack of standardisation in German children with pseudotumor cerebri. Klin Padiatr 2012; 224 (01) 40-42
  • 33 El-Dairi MA, Holgado S, O'Donnell T, Buckley EG, Asrani S, Freedman SF. Optical coherence tomography as a tool for monitoring pediatric pseudotumor cerebri. J AAPOS 2007; 11 (06) 564-570
  • 34 Irazuzta JE, Brown ME, Akhtar J. Bedside optic nerve sheath diameter assessment in the identification of increased intracranial pressure in suspected idiopathic intracranial hypertension. Pediatr Neurol 2016; 54: 35-38
  • 35 Shuper A, Snir M, Barash D, Yassur Y, Mimouni M. Ultrasonography of the optic nerves: clinical application in children with pseudotumor cerebri. J Pediatr 1997; 131 (05) 734-740
  • 36 Beres SJ, Sheldon CA, Boisvert CJ. , et al. Clinical and prognostic significance of cerebrospinal fluid opening and closing pressures in pediatric pseudotumor cerebri syndrome. Pediatr Neurol 2018; 83: 50-55
  • 37 Tovia E, Reif S, Oren A, Mitelpunkt A, Fattal-Valevski A. Treatment response in pediatric patients with pseudotumor cerebri syndrome. J Neuroophthalmol 2017; 37 (04) 393-397
  • 38 Celebisoy N, Gökçay F, Sirin H, Akyürekli O. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand 2007; 116 (05) 322-327
  • 39 Chern JJ, Tubbs RS, Gordon AS, Donnithorne KJ, Oakes WJ. Management of pediatric patients with pseudotumor cerebri. Childs Nerv Syst 2012; 28 (04) 575-578
  • 40 Hanak BW, Bonow RH, Harris CA, Browd SR. Cerebrospinal fluid shunting complications in children. Pediatr Neurosurg 2017; 52 (06) 381-400
  • 41 Jiramongkolchai K, Buckley EG, Bhatti MT. , et al. Temporary lumbar drain as treatment for pediatric fulminant idiopathic intracranial hypertension. J Neuroophthalmol 2017; 37 (02) 126-132
  • 42 Thuente DD, Buckley EG. Pediatric optic nerve sheath decompression. Ophthalmology 2005; 112 (04) 724-727
  • 43 Gupta AK, Gupta K, Sunku SK, Modi M, Gupta A. Endoscopic optic nerve fenestration amongst pediatric idiopathic intracranial hypertension: a new surgical option. Int J Pediatr Otorhinolaryngol 2014; 78 (10) 1686-1691
  • 44 Lessell S, Rosman NP. Permanent visual impairment in childhood pseudotumor cerebri. Arch Neurol 1986; 43 (08) 801-804
  • 45 Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome. Am J Ophthalmol 1999; 127 (02) 178-182
  • 46 Stiebel-Kalish H, Kalish Y, Lusky M, Gaton DD, Ehrlich R, Shuper A. Puberty as a risk factor for less favorable visual outcome in idiopathic intracranial hypertension. Am J Ophthalmol 2006; 142 (02) 279-283
  • 47 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 (11) 1913-1918