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Current Pain and Headache Reports

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01-08-2019 | Idiopathic Intracranial Hypertension | Childhood and Adolescent Headache (S Evers, Section Editor)

Pseudotumor Cerebri Syndrome in Children

Authors: Rebecca Barmherzig, Christina L. Szperka

Published in: Current Pain and Headache Reports | Issue 8/2019

Abstract

Purpose of Review

Pseudotumor cerebri syndrome (PTCS) may affect both children and adults; however, the risk factors and clinical presentation vary greatly between these populations. This review aims to highlight the entity of PTCS in children and the unique considerations in this population; review the epidemiology and demographics; discuss the clinical presentation, revised diagnostic criteria, and approach to evaluation; review management strategies; and discuss the prognosis and long-term outcomes in children with PTCS.

Recent Findings

Clinical presentation can be variable in children and may be less obvious than in their adult counterparts. Papilledema can also be challenging to diagnose in this population. The upper limits for opening pressure on lumbar puncture differ in children, with a cut-off of 25 cm H20 (or 28 cm H2O in a sedated or obese child).

Summary

Morbidity related to visual loss, pain and reduced quality of life lends urgency towards accurately identifying, evaluating and managing children with PTCS. There are no randomised controlled studies to allow for evidence-based recommendations for the management of PTCS in children. Further studies are needed to clarify and consolidate management approaches in this population.

Friedman DI. The pseudotumor cerebri syndrome. Neurol Clin. 2014;32(2):363–96. https://doi.org/10.1016/j.ncl.2014.01.001.CrossRefPubMed

•• Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013;81(13):1159–65. https://doi.org/10.1212/WNL.0b013e3182a55f17 The most reently revised diagnostic criteria for PTCS, including criteria for assessing children both with and without the presence of papilledema. CrossRefPubMed

Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumour cerebri: population studies in Iowa and Louisiana. Arch Neurol. 1988;45:875–7.CrossRef

McCluskey G, Mulholland DA, McCarron P, McCarron MO. Idiopathic Intracranial Hypertension in the Northwest of Northern Ireland: Epidemiology and Clinical Management. Neuroepidemiology. 2015;45(1):34–9. https://doi.org/10.1159/000435919.CrossRefPubMed

Matthews YY, Dean F, Lim MJ, Mclachlan K, Rigby AS, Solanki GA, 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. https://doi.org/10.1136/archdischild-2016-312238.CrossRefPubMed

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. https://doi.org/10.1016/j.pediatrneurol.2017.04.021.CrossRefPubMed

Bursztyn LL, Sharan S, Walsh L, LaRoche GR, Robitaille J, De Becker I. Has rising pediatric obesity increased the incidence of idiopathic intracranial hypertension? Can J Opthalmol. 2014;49(1):87–91. https://doi.org/10.1016/j.jcjo.2013.09.015.CrossRef

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

Sheldon CA, Paley GL, Xiao R, Kesler A, Eyal O, Ko MW, et al. Pediatric Idiopathic Intracranial Hypertension: Age, Gender, and Anthropometric Features at Diagnosis in a Large, Retrospective. Multisite Cohort. Ophthalmology. 2016;123(11):2424–31. https://doi.org/10.1016/j.ophtha.2016.08.004.CrossRefPubMed

10.

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):1308–11. https://doi.org/10.1177/0883073808318056.CrossRefPubMed

11.

Aylward SC, Aronowitz C, Roach ES. Intracranial Hypertension Without Papilledema in Children. J Child Neurol. 2016a;31(2):177–83. https://doi.org/10.1177/0883073815587029.CrossRefPubMed

12.

Rangwala LM, Liu GT. Pediatric idiopathic intracranial hypertension. Surv Opthalmol. 2007;52(6):597–617.CrossRef

13.

Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, and outcome. AM J Opthalmol. 1999;127(2):178–82.CrossRef

14.

Balcer LJ, Liu GT, Forman S, Pun K, Volpe NJ, Galetta SL, et al. Idiopathic intracranial hypertension: relation of age and obesity in children. Neurology. 1999;52(4):870–2.CrossRef

15.

Paley GL, Sheldon CA, Burrows EK, Chilutti MR, Liu GT, McCormack SE. Overweight and obesity in pediatric secondary pseudotumor cerebri syndrome. Am J Ophthalmol. 2015;159(2):344–52.e1. https://doi.org/10.1016/j.ajo.2014.11.003.CrossRefPubMed

16.

Phillips PH, Repka MX, Lambert SR. Pseudotumor cerebri in children. J AAPOS. 1998;2(1):33–8.CrossRef

17.

• Aylward SC, Waslo CS, Au JN, Tanne E. Manifestations of Pediatric Intracranial Hypertension From the Intracranial Hypertension Registry. Pediatr Neurol. 2016b;61:76–82. https://doi.org/10.1016/j.pediatrneurol.2016.04.007 A review of the demographics, clinical features, and interventions in from a large registry database of pediatric patients with PTCS. CrossRefPubMed

18.

Fernándes-García MÁ, Cantarín-Extremera V, Andíon-Catalán M, Duat-Rodríguez A, Jiménez- Echevarría S, Bermejo-Arndeo I, et al. Secondary Intracranial Hypertension in Pediatric Patients With Leukemia. Pediatr Neurol. 2017;77:48–53. https://doi.org/10.1016/j.pediatrneurol.2017.08.013.CrossRef

19.

Sussman J, leach M, Greaves M, Malia R, Davies-Jones GA. Potentially prothrombotic abnormalities of coagulation in benign intracranial hypertension. J Neurol Neurosurg Psychiatry. 1997;62(3):229–33.CrossRef

20.

Sodi M, Sheldon CA, Carleton B, Etminan M. Oral fluoroquinolones and risk of secondary pseudotumor cerebri syndrome: Nested case-control study. Neurology. 2017;89(8):792–5. https://doi.org/10.1212/WNL.0000000000004247.CrossRef

21.

Bassari H, Berkner L, Stolovitch C, Kesaler A. Asymptomatic idiopathic intracranial hypertension in children. Acta Neurol Scand. 2008;118(4):251–5. https://doi.org/10.1111/j.1600-0404.2008.01007.x.CrossRef

22.

• 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. https://doi.org/10.1097/WNO.0000000000000516 A retrospective study of 60 pediatric PTCS cases, outlining demographics, clinical characteristics, and treatment outcomes. CrossRefPubMed

23.

Hamedani AG, Witonsky KFR, Cosico M, Rennie R, Xiao R, Sheldon CA, et al. Headache Characteristics in Children With Pseudotumor Cerebri Syndrome, Elevated Opening Pressure Without Papilledema, and Normal Opening Pressure: A Retrospective Cohort Study. Headache. 2018;58(9):1339–46. https://doi.org/10.1111/head.13362.CrossRefPubMed

24.

Moavero R, Sforza G, Papetti L, Battan B, Tarantino S, Vigevano F, et al. Clinical Features of Pediatric Idiopathic Intracranial Hypertension and Applicability of New ICHD-3 Criteria. Front Neurol. 2018;9:819. https://doi.org/10.3389/fneur.2018.00819.CrossRefPubMedPubMedCentral

25.

Mathew NT, Ravishankar K, Sanin LC. Coexistence of migraine and idiopathic intracranial hypertension without papilledema. Neurology. 1996;46(5):1226–30.CrossRef

26.

Cleves-Bayon C. Idiopathic Intracranial Hypertension in Children and Adolescents: An Update. Headache. 2018;58(3):485–93. https://doi.org/10.1111/head.13236.CrossRefPubMed

27.

Dessardo NS, Dessardo S, Sasso A, Sarunic AV, Dezulovic MS. Pediatric idiopathic intracranial hypertension: clinical and demographic features. Coll Antropol. 2010;34(Suppl 2):217–21.PubMed

28.

• Smith SV, Friedman DI. The idiopathic intracranial hypertension treatment trial: a review of the outcomes. Headache. 2017;57(8):1303–10. https://doi.org/10.1111/head.13144 A review of the literature and summary of all outcome results from the IIHTT, supporting the use of acetazolamide and weight loss to improve visual outcomes and quality of life in adults. CrossRefPubMed

29.

Reid JE, Reem RE, Aylward SC, Rogers DL. Sixth nerve palsy in paediatric intracranial hypertension. Neuroophthalmology. 2016;40(1):23–7.CrossRef

30.

Sachdeva V, Vasseneix C, Hage R, et al. Optic nerve head edema among patients presenting to the emergency department. Neurology. 2018;90(5):e373–9. https://doi.org/10.1212/WNL.0000000000004895.CrossRefPubMedPubMedCentral

31.

Wall M, Kupersmith MJ, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI, et al. NORDIC Idiopathic Intracranial Hypertension Study Group. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol. 2014a;71(6):693–701. https://doi.org/10.1001/jamaneurol.2014.133.CrossRefPubMedPubMedCentral

32.

Marcelis J, Silberstein SD. Idiopathic intracranial hypertension without papilledema. Arch Neurol. 1991;48(4):392–9.CrossRef

33.

Fisayo A, Bruce BB, Newman NJ, Biousse V. Overdiagnosis of idiopathic intracranial hypertension. Neurology. 2016;86(4):341–50. https://doi.org/10.1212/WNL.0000000000002318.CrossRefPubMedPubMedCentral

34.

Aylward SC, Way AL. Pediatric Intracranial Hypertension: a Current Literature Review. Curr Pain Headache Rep. 2018;22(2):14. https://doi.org/10.1007/s11916-018-0665-9.CrossRefPubMed

35.

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. 2010a;363(9):891–3. https://doi.org/10.1056/NEJMc1004957.CrossRefPubMedPubMedCentral

36.

Inger HE, Rogers DL, McGregor ML, Aylward SC, Reem RE. Diagnostic criteria in pediatric intracranial hypertension. J AAPOS. 2017;21(6):492–495.e2. https://doi.org/10.1016/j.jaapos.2017.08.003.CrossRefPubMed

37.

Gerstl L, Schoppe N, Albers L, Ertl-Wagner B, Alperin N, Ehrt O, 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. https://doi.org/10.1016/j.ejpn.2017.08.002.CrossRefPubMed

38.

Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1):1–211. https://doi.org/10.1177/0333102417738202.CrossRef

39.

Vieira DS, Masruha MR, Goncalves AL, et al. Idiopathic intracranial hypertension with and without papilloedema in a consecutive series of patients with chronic migraine. Cephalalgia. 2008;28(6):609–13. https://doi.org/10.1111/j.1468-2982.2008.01564.x.CrossRefPubMed

40.

Aylward SC, Reem RE. Pediatric Intracranial Hypertension. Pediatr Neurol. 2017;66:32–43. https://doi.org/10.1016/j.pediatrneurol.2016.08.010.CrossRefPubMed

41.

Liu B, Murphy RK, Mercer D, Tychsen L, Smyth MD. Pseudopapilledema and association with idiopathic intracranial hypertension. Childs Nerv Syst. 2014;30(7):1197–200. https://doi.org/10.1007/s00381-014-2390-y.CrossRefPubMed

42.

Kovarik JJ, Doshi PN, Collinge JE, Plager DA. Outcome of pediatric patients referred for papilloedema. J AAPOS. 2015;19(4):344–8. https://doi.org/10.1016/j.jaapos.2015.05.007.CrossRefPubMed

43.

Gilbert AL, Heidary G. Update on the evaluation of pediatric idiopathic intracranial hypertension. Curr Opin Ophthalmol. 2016;27(6):493–7.CrossRef

44.

Lee YA, Tomsak RL, Sadikovic Z, Bahl R, Sivaswamy L. Use of Ocular Coherence Tomography in Children With Idiopathic Intracranial Hypertension-A Single-Center Experience. Pediatr Neurol. 2016;58:101–106.e1. https://doi.org/10.1016/j.pediatrneurol.2015.10.022.CrossRefPubMed

45.

Phillips PH, Sheldon CA. Pediatric Pseudotumor Cerebri Syndrome. J Neuroophthalmol. 2017;37(Suppl 1):S33–40. https://doi.org/10.1097/WNO.0000000000000548.CrossRefPubMed

46.

Chen JJ, Thurntell MJ, Longmuir RA, Garvin MK, Wang JK, Wall M, et al. Causes and prognosis of visual acuity loss at the time of initial presentation in idiopathic intracranial hypertension. Invest Opthalmol Vis Sci. 2015;56(6):3850–9. https://doi.org/10.1167/iovs.15-16450.CrossRef

47.

Mollan SP, Davies B, Silver NC, Shaw S, Mallucci CL, Wakerley BR. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018;89(10):1088–100. https://doi.org/10.1136/jnnp-2017-317440.CrossRefPubMedPubMedCentral

48.

Lim MJ, Pushparajah k JW, Jan W, Calver D, Lin JP. Magnetic resonance imaging changes in idiopathic intracranial hypertension in children. J Child Neurol. 2010;25(3):294–9. https://doi.org/10.1177/0883073809338874.CrossRefPubMed

49.

Görkem SB, Doğanay S, Canpolat M, Koc G, Dogan MS, Per H, et al. MR imaging findings in children with pseudotumor cerebri and comparison with healthy controls. Childs Nerv Syst. 2015;31(3):373–80. https://doi.org/10.1007/s00381-014-2579-0.CrossRefPubMed

50.

Kohli AA, Vossough A, Mallery R, Woo J, Sheldon CA, Paley GL, McCormack S, Liu G, Szperka CL. MRI Findings in Children with Pseudotumor Cerebri Syndrome (PTCS), Intracranial Hypertension, and Normal Opening Pressure without Papilledema. North American Neuro-Ophthalmology Society 42^nd Annual Meetin (poster), Tucson, Arizona. February-March 2016.

51.

Aylward SC. Pediatric idiopathic intracranial hypertension: a need for clarification. Pediatr Neurol. 2013;49(5):303–4. https://doi.org/10.1016/j.pediatrneurol.2013.05.019.CrossRefPubMed

52.

Avery RA, Mistry RD, Shah SS, Boswinkel J, Huh JW, Ruppe MD, et al. Patient position during lumbar puncture has no meaningful effect on cerebrospinal fluid opening pressure in children. J Child Neurol. 2010b;25(5):616–9. https://doi.org/10.1177/0883073809359198.CrossRefPubMedPubMedCentral

53.

Lim MJ, Lin JP. The effects of carbon dioxide on measuring cerebral spinal fluid pressure. Child's Nerv Syst. 2009;25(7):783–4. https://doi.org/10.1007/s00381-009-0902-y.CrossRef

54.

McLaren SH, Monuteaux MC, Delaney AC, Landschaft A, Kimia AA. How Much Cerebrospinal Fluid Should We Remove Prior to Measuring a Closing Pressure? J Child Neurol. 2017;32(4):356–9. https://doi.org/10.1177/0883073816681352.CrossRefPubMed

55.

Beres SJ, Sheldon CA, Boisvert CJ, Szperka CL, Paley GL, Burrows EK, et al. Clinical and Prognostic Significance of Cerebrospinal Fluid Opening and Closing Pressures in Pediatric Pseudotumor Cerebri Syndrome. Pediatr Neurol. 2018;83:50–5. https://doi.org/10.1016/j.pediatrneurol.2018.02.011.CrossRefPubMed

56.

Sinclair AJ, Burdon MA, Nightingale PG, et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: prospective cohort study. BMJ. 2010;341:c2701. https://doi.org/10.1136/bmj.c2701.CrossRefPubMedPubMedCentral

57.

Johnson LN, Krohel GB, Madsen RW, March GA Jr. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology. 1998;105(12):23137. https://doi.org/10.1016/S0161-6420(98)91234-9.CrossRef

58.

Spear BA, Barlow SE, Ervin C, et al. Recommendations for treatment of child and adolescent overweight and obesity. Pediatrics. 2007;120(Suppl 4):S254–88.CrossRef

59.

Piper RJ, Kalyvas AV, Young AM, et al. Interventions for idiopathic intracranial hypertension. Cochrane Database Syst Rev 2015:CD003434. doi:https://doi.org/10.1002/14651858.CD003434.pub3

60.

Wall M, McDermott MP, Kieburtz KD, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss. J Am Med Assoc. 2014b;311(16):1641–51. https://doi.org/10.1001/jama.2014.3312.CrossRef

61.

Bruce BB, Digre KB, McDermott MP, et al. Quality of life at 6 months in the idiopathic intracranial hypertension treatment trial. Neurology. 2016;87:1871–7. https://doi.org/10.1212/WNL.0000000000003280.CrossRefPubMedPubMedCentral

62.

Celebisoy N, Gokcay F, Sirin H, Aykyurekli O. Treatment of idiopathic intracranial hypertension: Topiramate vs acetazolamide, an open-label study. Acta Neurol Scand. 2007;116:322–7.CrossRef

63.

Sheldon CA, Paley GL, Beres SJ, McCormack SE, Liu GT. Pediatric Pseudotumor Cerebri Syndrome: Diagnosis, Classification, and Underlying Pathophysiology. Semin Pediatr Neurol. 2017;24(2):110–5. https://doi.org/10.1016/j.spen.2017.04.002.CrossRefPubMed

64.

Bersani TA, Meeker AR, Sismanis DN, Carruth BP. Pediatric and adult vision restoration after optic nerve sheath decompression for idiopathic intracranial hypertension. Orbit. 2016;35:132–9.CrossRef

65.

El-Dairi MA, Holgado S, O’Donnell T, et al. Optical coherence tomography as a tool for monitoring pediatric pseudotumor cerebri. J AAPOS. 2007;11:564–70.CrossRef

66.

Gospe SM 3rd, Bhatti MT, El-Dairi MA. Anatomic and visual function outcomes inpaediatric idiopathic intracranial hypertension. Br J Ophthalmol. 2016;100(4):505–9. https://doi.org/10.1136/bjophthalmol-2015-307043.CrossRefPubMed

67.

Agarwal A, Vibha D, Prasad K, Bhatia R, Singh MB, Garg A, et al. Predictors of poor visual outcome in patients with idiopathic intracranial hypertension (IIH): an ambispective cohort study. Clin Neurol Neurosurg. 2017;159:13–8. https://doi.org/10.1016/j.clineuro.2017.05.009.CrossRef

68.

• Ravid S, Shahar E, Schif A, Yehudian S. Visual outcome and recurrence rate in children with idiopathic intracranial hypertension. J Child Neurol. 2015;30(11):1448–52. https://doi.org/10.1177/0883073815569306 A retrospective study evaluating the visual outcome and recurrence rate of idiopathic intracranial hypertension in children and clinical predictors for poorer visual outcomes. CrossRefPubMed

69.

Wong R, Madill SA, Pandey P, Riordan-Eva P. Idiopathic intracranial hypertension: the association between weight loss and the requirement for systemic treatment. BMC Opthalmol. 2007;7:15.CrossRef

70.

Ko MW, Chang SC, Ridha MA, et al. Weight gain and recurrence in idiopathic intracranial hypertension: A case-control study. Neurology. 2011;76(18):1564–7. https://doi.org/10.1212/WNL.0b013e3182190f51.CrossRefPubMed

Title: Pseudotumor Cerebri Syndrome in Children
Authors: Rebecca Barmherzig
Christina L. Szperka
Publication date: 01-08-2019
Publisher: Springer US
Keywords: Idiopathic Intracranial Hypertension
Headache
Published in: Current Pain and Headache Reports / Issue 8/2019
Print ISSN: 1531-3433
Electronic ISSN: 1534-3081
DOI: https://doi.org/10.1007/s11916-019-0795-8

At a glance: The STEP trials

Springer Medicine

Pseudotumor Cerebri Syndrome in Children

Abstract

Purpose of Review

Recent Findings

Summary

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose of Review

Recent Findings

Summary

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