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Pseudotumor Cerebri Syndrome in Children: Current Diagnosis and Treatment

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Pediatric Ophthalmology

Part of the book series: Current Practices in Ophthalmology ((CUPROP))

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Abstract

Suspicion of papilledema or swelling of the optic nerves from elevated intracranial pressure (ICP) is a common reason for referral to pediatric neuro-ophthalmology. The pediatric neuro-ophthalmologist is tasked with confirming the presence or absence of papilledema and if present, determining the next steps in evaluation and management. Increased ICP, in the absence of a mass, has been labeled pseudotumor cerebri syndrome [1–4]. This designation encompasses idiopathic intracranial hypertension (IIH; used interchangeably with primary pseudotumor cerebri syndrome in this chapter) as well as elevated ICP secondary to etiologies including exposure to certain medications, in association with underlying systemic disease, and cerebral venous system abnormalities. This chapter outlines the epidemiology, pathophysiology, and etiologies of pseudotumor cerebri syndrome (PTCS). Further, this chapter reviews the most recent diagnostic criteria, disease monitoring methods, treatment approaches, and visual outcomes for PTCS. Having a systematic strategy in place to evaluate patients with papilledema and presumed pseudotumor cerebri is crucial for the timely identification of an underlying etiology, if present, and for the prevention of visual loss with effective treatment.

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References

  1. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013;81(13):1159–65.

    Article  PubMed  Google Scholar 

  2. Beres SJ, Digre KB, Friedman DI, Liu GT. Pseudotumor cerebri syndrome is the best term for this condition. Pediatr Neurol. 2018;87:9–10.

    Article  PubMed  Google Scholar 

  3. Mollan SP, Davies B, Silver NC, et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018;89(10):1088–100.

    Article  PubMed  Google Scholar 

  4. Aylward SC, Waslo CS, Au JN, Tanne E. Manifestations of pediatric intracranial hypertension from the intracranial hypertension registry. Pediatr Neurol. 2016;61:76–82.

    Article  PubMed  Google Scholar 

  5. Gillson N, Jones C, Reem RE, Rogers DL, Zumberge N, Aylward SC. Incidence and demographics of pediatric intracranial hypertension. Pediatr Neurol. 2017;73:42–7.

    Article  PubMed  Google Scholar 

  6. Gordon K. Pediatric pseudotumor cerebri: descriptive epidemiology. Can J Neurol Sci. 1997;24(3):219–21.

    Article  CAS  PubMed  Google Scholar 

  7. Matthews YY, Dean F, Lim MJ, et al. Pseudotumor cerebri syndrome in childhood: incidence, clinical profile and risk factors in a national prospective population-based cohort study. Arch Dis Child. 2017;102(8):715–21.

    Article  PubMed  Google Scholar 

  8. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neurol. 1988;45(8):875–7.

    Article  CAS  PubMed  Google Scholar 

  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(8):907–14.

    Article  CAS  PubMed  Google Scholar 

  10. Sheldon CA, Paley GL, **ao R, et al. Pediatric idiopathic intracranial hypertension: age, gender, and anthropometric features at diagnosis in a large, retrospective. Multisite Cohort Ophthalmol. 2016;123(11):2424–31.

    Google Scholar 

  11. 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(1):6–9.

    Article  PubMed  Google Scholar 

  12. Balcer LJ, Liu GT, Forman S, et al. Idiopathic intracranial hypertension: relation of age and obesity in children. Neurology. 1999;52(4):870–2.

    Article  CAS  PubMed  Google Scholar 

  13. Brara SM, Koebnick C, Porter AH, Langer-Gould A. Pediatric idiopathic intracranial hypertension and extreme childhood obesity. J Pediatr. 2012;161(4):602–7.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Shah V, Hoyos-Martinez A, Horne VE. Association of adrenal insufficiency with pediatric Pseudotumor cerebri syndrome. JAMA Ophthalmol. 2020;138(11):1187–91.

    Article  PubMed  Google Scholar 

  15. Beres SJ. Update in Pediatric Pseudotumor Cerebri Syndrome. Semin Neurol. 2020;40(3):286–93.

    Article  PubMed  Google Scholar 

  16. 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(2):282–9.

    Article  PubMed  Google Scholar 

  17. Kesler A, Fattal-Valevski A. Idiopathic intracranial hypertension in the pediatric population. J Child Neurol. 2002;17(10):745–8.

    Article  PubMed  Google Scholar 

  18. Aylward SC, Aronowitz C, Reem R, Rogers D, Roach ES. Intracranial hypertension without headache in children. J Child Neurol. 2015;30(6):703–6.

    Article  PubMed  Google Scholar 

  19. Bassan H, Berkner L, Stolovitch C, Kesler A. Asymptomatic idiopathic intracranial hypertension in children. Acta Neurol Scand. 2008;118(4):251–5.

    Article  CAS  PubMed  Google Scholar 

  20. Whitecross S, Heidary G. Asymptomatic pediatric idiopathic intracranial hypertension. J AAPOS. 2013;17(01):e31.

    Article  Google Scholar 

  21. Orme DR, Vegunta S, Miller MA, et al. A comparison between the clinical features of Pseudotumor cerebri secondary to tetracyclines and idiopathic intracranial hypertension. Am J Ophthalmol. 2020;220:177–82.

    Article  PubMed  Google Scholar 

  22. Liu KC, Bhatti MT, Chen JJ, et al. Presentation and progression of papilledema in cerebral venous sinus thrombosis. Am J Ophthalmol. 2020;213:1–8.

    Article  PubMed  Google Scholar 

  23. Thambisetty M, Lavin PJ, Newman NJ, Biousse V. Fulminant idiopathic intracranial hypertension. Neurology. 2007;68(3):229–32.

    Article  PubMed  Google Scholar 

  24. Aylward SC, Aronowitz C, Roach ES. Intracranial hypertension without papilledema in children. J Child Neurol. 2016;31(2):177–83.

    Article  PubMed  Google Scholar 

  25. Sibony PA, Kupersmith MJ, Kardon RH. Optical coherence tomography Neuro-toolbox for the diagnosis and Management of Papilledema, optic disc edema, and Pseudopapilledema. J Neuroophthalmol. 2021;41(1):77–92.

    Article  PubMed  Google Scholar 

  26. Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol. 2002;47(6):515–32.

    Article  PubMed  Google Scholar 

  27. Friedman AH, Gartner S, Modi SS. Drusen of the optic disc. A retrospective study in cadaver eyes. Br J Ophthalmol. 1975;59(8):413–21.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Malmqvist L, Li XQ, Eckmann CL, et al. Optic disc Drusen in children: the Copenhagen child cohort 2000 eye study. J Neuroophthalmol. 2018;38(2):140–6.

    Article  PubMed  Google Scholar 

  29. Malmqvist L, Bursztyn L, Costello F, et al. The optic disc Drusen studies consortium recommendations for diagnosis of optic disc Drusen using optical coherence tomography. J Neuroophthalmol. 2018;38(3):299–307.

    Article  PubMed  Google Scholar 

  30. Chang MY, Velez FG, Demer JL, et al. Accuracy of diagnostic imaging modalities for classifying pediatric eyes as papilledema versus Pseudopapilledema. Ophthalmology. 2017;124(12):1839–48.

    Article  PubMed  Google Scholar 

  31. Merchant KY, Su D, Park SC, et al. Enhanced depth imaging optical coherence tomography of optic nerve head drusen. Ophthalmology. 2013;120(7):1409–14.

    Article  PubMed  Google Scholar 

  32. Dandy WE. Intracranial pressure without brain tumor: diagnosis and treatment. Ann Surg. 1937;106(4):492–513.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Smith JL. Whence pseudotumor cerebri? J Clin Neuroophthalmol. 1985;5(1):55–6.

    CAS  PubMed  Google Scholar 

  34. Wall M, Kupersmith MJ, Kieburtz KD, et al. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol. 2014;71(6):693–701.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Kohli AA, Vossough A, Mallery RM, et al. Magnetic resonance imaging findings in pediatric Pseudotumor cerebri syndrome. Pediatr Neurol. 2019;99:31–9.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Hartmann AJ, Soares BP, Bruce BB, et al. Imaging features of idiopathic intracranial hypertension in children. J Child Neurol. 2017;32(1):120–6.

    Article  PubMed  Google Scholar 

  37. Gilbert AL, Vaughn J, Whitecross S, Robson CD, Zurakowski D, Heidary G. Magnetic resonance imaging features and clinical findings in pediatric idiopathic intracranial hypertension: a case-control study. Life (Basel). 2021;11(6):487.

    Google Scholar 

  38. Gulani V, Calamante F, Shellock FG, Kanal E, Reeder SB, International Society for Magnetic Resonance in M. Gadolinium deposition in the brain: summary of evidence and recommendations. Lancet Neurol. 2017;16(7):564–70.

    Article  PubMed  Google Scholar 

  39. Hollander JPS, Heidary G. Utilization of MRV to evaluate pediatric patients with papilledema. J AAPOS. 2014;18(04):2.

    Article  Google Scholar 

  40. 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(6):833–41.

    Article  PubMed  Google Scholar 

  41. Masri A, Jaafar A, Noman R, Gharaibeh A, Ababneh OH. Intracranial hypertension in children: etiologies, clinical features, and outcome. J Child Neurol. 2015;30(12):1562–8.

    Article  PubMed  Google Scholar 

  42. Delaney AC, Velarde A, Harper MB, et al. Predictors of primary intracranial hypertension in children using a newly suggested opening pressure cutoff of 280 mm H2O. Pediatr Neurol. 2019;91:27–33.

    Article  PubMed  Google Scholar 

  43. 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;83:50–5.

    Article  PubMed  PubMed Central  Google Scholar 

  44. Committee NIIHSGW, Wall M, McDermott MP, et al. 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):1641–51.

    Article  Google Scholar 

  45. Celebisoy N, Gokcay F, Sirin H, Akyurekli O. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand. 2007;116(5):322–7.

    Article  CAS  PubMed  Google Scholar 

  46. Jion YI, Raff A, Grosberg BM, Evans RW. The risk and management of kidney stones from the use of topiramate and zonisamide in migraine and idiopathic intracranial hypertension. Headache. 2015;55(1):161–6.

    Article  PubMed  Google Scholar 

  47. Gaier ED, Heidary G. Pediatric idiopathic intracranial hypertension. Semin Neurol. 2019;39(6):704–10.

    Article  PubMed  Google Scholar 

  48. Liu GT, Glaser JS, Schatz NJ. High-dose methylprednisolone and acetazolamide for visual loss in pseudotumor cerebri. Am J Ophthalmol. 1994;118(1):88–96.

    Article  CAS  PubMed  Google Scholar 

  49. Tovia E, Reif S, Oren A, Mitelpunkt A, Fattal-Valevski A. Treatment response in pediatric patients with Pseudotumor cerebri syndrome. J Neuroophthalmol. 2017;37(4):393–7.

    Article  PubMed  Google Scholar 

  50. Thuente DD, Buckley EG. Pediatric optic nerve sheath decompression. Ophthalmology. 2005;112(4):724–7.

    Article  PubMed  Google Scholar 

  51. Hanak BW, Bonow RH, Harris CA, Browd SR. Cerebrospinal fluid shunting complications in children. Pediatr Neurosurg. 2017;52(6):381–400.

    Article  PubMed  Google Scholar 

  52. Brune AJ, Girgla T, Trobe JD. Complications of ventriculoperitoneal shunt for idiopathic intracranial hypertension: a single-institution study of 32 patients. J Neuroophthalmol. 2021;41(2):224–32.

    Article  PubMed  Google Scholar 

  53. Jiramongkolchai K, Buckley EG, Bhatti MT, et al. Temporary lumbar drain as treatment for pediatric fulminant idiopathic intracranial hypertension. J Neuroophthalmol. 2017;37(2):126–32.

    Article  PubMed  Google Scholar 

  54. Ploof J, Aylward SC, Jordan CO, Drapeau AI. Case series of rapid surgical interventions in fulminant intracranial hypertension. J Child Neurol. 2021;36(11):1047–53.

    Article  PubMed  Google Scholar 

  55. Carter LM, Chakraborty AR, McCoy-Stephens TM, Strickland AE, Bohnstedt BN, Gross NL. Venous sinus stenosis treatment in pediatric idiopathic intracranial hypertension: illustrative case and literature review. World Neurosurg. 2021;149:2–7.

    Article  PubMed  Google Scholar 

  56. Dwyer CM, Prelog K, Owler BK. The role of venous sinus outflow obstruction in pediatric idiopathic intracranial hypertension. J Neurosurg Pediatr. 2013;11(2):144–9.

    Article  PubMed  Google Scholar 

  57. Ahmed RM, Zmudzki F, Parker GD, Owler BK, Halmagyi GM. Transverse sinus stenting for pseudotumor cerebri: a cost comparison with CSF shunting. AJNR Am J Neuroradiol. 2014;35(5):952–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  58. Schwarz J, Al Balushi A, Sundararajan S, et al. Management of idiopathic intracranial hypertension in children utilizing venous sinus stenting. Interv Neuroradiol. 2021;27(2):257–65.

    Article  PubMed  Google Scholar 

  59. Wan MJ, Adebona O, Benson LA, Gorman MP, Heidary G. Visual outcomes in pediatric optic neuritis. Am J Ophthalmol. 2014;158(3):503–7 e2.

    Article  PubMed  Google Scholar 

  60. Group OCTS-SCfNIIHS, Auinger P, Durbin M, et al. Baseline OCT measurements in the idiopathic intracranial hypertension treatment trial, part I: quality control, comparisons, and variability. Invest Ophthalmol Vis Sci. 2014;55(12):8180–8.

    Article  Google Scholar 

  61. Optical Coherence Tomography Substudy C, Group NIIHS. Papilledema outcomes from the optical coherence tomography substudy of the idiopathic intracranial hypertension treatment trial. Ophthalmology. 2015;122(9):1939–45 e2.

    Article  Google Scholar 

  62. Dreesbach M, Joachimsen L, Kuchlin S, et al. Optic nerve head volumetry by optical coherence tomography in papilledema related to idiopathic intracranial hypertension. Transl Vis Sci Technol. 2020;9(3):24.

    Article  PubMed  PubMed Central  Google Scholar 

  63. Group OCTS-SCfNIIHS, Auinger P, Durbin M, et al. Baseline OCT measurements in the idiopathic intracranial hypertension treatment trial, part II: correlations and relationship to clinical features. Invest Ophthalmol Vis Sci. 2014;55(12):8173–9.

    Article  Google Scholar 

  64. Lessell S, Rosman NP. Permanent visual impairment in childhood pseudotumor cerebri. Arch Neurol. 1986;43(8):801–4.

    Article  CAS  PubMed  Google Scholar 

  65. 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(2):279–83.

    Article  PubMed  Google Scholar 

  66. 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):1913–8.

    Article  PubMed  Google Scholar 

  67. Gospe SM 3rd, Bhatti MT, El-Dairi MA. Anatomic and visual function outcomes in paediatric idiopathic intracranial hypertension. Br J Ophthalmol. 2016;100(4):505–9.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Ophthalmology, Boston Children’s Hospital, Boston, MA, USA

    Ryan Gise, Eric D. Gaier & Gena Heidary

  2. Harvard Medical School, Boston, MA, USA

    Ryan Gise, Eric D. Gaier & Gena Heidary

  3. Picower Institute for Learning and Memory, Massachusetts Institute of Technology, Boston, MA, USA

    Eric D. Gaier

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Correspondence to Gena Heidary .

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  1. Phoenix Children’s Hospital, Phoenix, AZ, USA

    Aparna Ramasubramanian

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Gise, R., Gaier, E.D., Heidary, G. (2022). Pseudotumor Cerebri Syndrome in Children: Current Diagnosis and Treatment. In: Ramasubramanian, A. (eds) Pediatric Ophthalmology. Current Practices in Ophthalmology. Springer, Singapore. https://doi.org/10.1007/978-981-19-4963-0_9

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  • DOI: https://doi.org/10.1007/978-981-19-4963-0_9

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