Skip to main content

Papilledema

  • Chapter
  • First Online:
  • 615 Accesses

Abstract

Papilledema refers to optic disc edema due to elevated intracranial pressure (ICP), which should be bilateral although may be very asymmetric in 4% of patients. ICP can be elevated due to increased cerebrospinal fluid (increased production or decreased absorption), increased intracranial blood volume (hemorrhage, acute hypertension, vasodilation, arteriovenous malformation, or fistula), increased cerebral volume (edema or tumor), or decreased intracranial space (increased bone deposition). The most common cause is idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri. While the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) demonstrated class 1 evidence for use of acetazolamide in patients with mild visual field loss and high-grade papilledema, other treatments are available including alternative medications as well as surgeries, and optimal treatment is not yet clear.

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Kovarik JJ, Doshi PN, Collinge JE, et al. Outcome of pediatric patients referred for papilledema. J AAPOS. 2015;19:344–8.

    Article  Google Scholar 

  2. Yeh EA, Graves JS, Benson LA, Wassmer E, Waldman A. Pediatric optic neuritis. Neurology. 2016;87(9 Suppl 2):S53–8. https://doi.org/10.1212/WNL.0000000000002822.

    Article  PubMed  Google Scholar 

  3. Narula S, Liu GT, Avery RA, Banwell B, Waldman AT. Elevated cerebrospinal fluid opening pressure in a pediatric demyelinating disease cohort. Pediatr Neurol. 2015;52(4):446–9. https://doi.org/10.1016/j.pediatrneurol.2015.01.002.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Rhoades W, Heidary G. Etiologies and visual outcomes in secondary pediatric intracranial hypertension (abstract). Invest Ophthalmol Vis Sci. 2013;54:1.

    Article  Google Scholar 

  5. Christian EA, Imahiyerobo TA, Nallapa S, Urata M, McComb JG, Krieger MD. Intracranial hypertension after surgical correction for craniosynostosis: a systematic review. Neurosurg Focus. 2015;38(5):E6. https://doi.org/10.3171/2015.2.FOCUS14853.

    Article  PubMed  Google Scholar 

  6. Florisson JM, van Veelen ML, Bannink N, et al. Papilledema in isolated single-suture craniosynostosis: prevalence and predictive factors. J Craniofac Surg. 2010;21(1):20–4. https://doi.org/10.1097/SCS.0b013e3181c3465e.

    Article  PubMed  Google Scholar 

  7. Lee HJ, Phi JH, Kim SK, Wang KC, Kim SJ. Papilledema in children with hydrocephalus: incidence and associated factors. J Neurosurg Pediatr. 2017;19(6):627–31. https://doi.org/10.3171/2017.2.PEDS16561.

    Article  PubMed  Google Scholar 

  8. Liu Y, Abongwa C, Ashwal S, Deming DD, Winter TW. Referral for ophthalmology evaluation and visual sequelae in children with primary brain tumors. JAMA Netw Open. 2019;2(8):e198273. https://doi.org/10.1001/jamanetworkopen.2019.8273.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Jackson BF, Porcher FK, Zapton DT, Losek JD. Cerebral sinovenous thrombosis in children: diagnosis and treatment. Pediatr Emerg Care. 2011;27(9):874–83. https://doi.org/10.1097/PEC.0b013e31822c9ccc.

    Article  PubMed  Google Scholar 

  10. Kılıç B, Güngör S. Clinical features and the role of magnetic resonance imaging in pediatric patients with intracranial hypertension. Acta Neurol Belg. 2020; https://doi.org/10.1007/s13760-020-01415-1.

  11. Gaier ED, Heidary G. Pediatric idiopathic intracranial hypertension. Semin Neurol. 2019;39(6):704–10. https://doi.org/10.1055/s-0039-1698743.

    Article  PubMed  Google Scholar 

  12. Ferro JM, Bousser MG, Canhão P, et al. European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis – endorsed by the European Academy of Neurology. Eur J Neurol. 2017;24(10):1203–13. https://doi.org/10.1111/ene.13381.

    Article  CAS  PubMed  Google Scholar 

  13. Slasky SE, Rivaud Y, Suberlak M, et al. Venous sinus thrombosis in blunt trauma: incidence and risk factors. J Comput Assist Tomogr. 2017;41(6):891–7. https://doi.org/10.1097/RCT.0000000000000620.

    Article  PubMed  Google Scholar 

  14. Uluduz D, Kürtüncü M, Yapıcı Z, et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology. 2011;77(21):1900–5. https://doi.org/10.1212/WNL.0b013e318238edeb.

    Article  CAS  PubMed  Google Scholar 

  15. Gala NB, Agarwal N, Barrese J, Gandhi CD, Prestigiacomo CJ. Current endovascular treatment options of dural venous sinus thrombosis: a review of the literature. J Neurointerv Surg. 2013;5(1):28–34. https://doi.org/10.1136/neurintsurg-2011-010117.

    Article  PubMed  Google Scholar 

  16. 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. https://doi.org/10.1016/j.ajo.2019.12.022.

    Article  PubMed  Google Scholar 

  17. Morris PP, Black DF, Port J, Campeau N. Transverse sinus stenosis is the most sensitive MR imaging correlate of idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 2017;38(3):471–7. https://doi.org/10.3174/ajnr.A5055.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Saindane AM, Bruce BB, Desai NK, Roller LA, Newman NJ, Biousse V. Transverse sinus stenosis in adult patients with Chiari malformation type I. AJR Am J Roentgenol. 2014;203(4):890–6. https://doi.org/10.2214/AJR.14.12528.

    Article  PubMed  PubMed Central  Google Scholar 

  19. Favoni V, Pierangeli G, Cirillo L, et al. Transverse sinus stenosis in refractory chronic headache patients: an observational study. Front Neurol. 2019;10:1287. https://doi.org/10.3389/fneur.2019.01287.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Fargen KM, Velat GJ, Lewis SB, Hoh BL, Mocco J, Lawson MF. Concomitant intracranial pressure monitoring during venous sinus stenting for intracranial hypertension secondary to venous sinus stenosis. J Neurointerv Surg. 2013;5(4):e22. https://doi.org/10.1136/neurintsurg-2012-010371.

    Article  PubMed  Google Scholar 

  21. Ding D, Starke RM, Durst CR, Crowley RW, Liu KC. Venous stenting with concurrent intracranial pressure monitoring for the treatment of pseudotumor cerebri. Neurosurg Focus. 2014;37(1 Suppl):1. https://doi.org/10.3171/2014.V2.FOCUS14162.

    Article  PubMed  Google Scholar 

  22. Dinkin M, Oliveira C. Men are from Mars, idiopathic intracranial hypertension is from venous: the role of venous sinus stenosis and stenting in idiopathic intracranial hypertension. Semin Neurol. 2019;39(6):692–703. https://doi.org/10.1055/s-0039-3399506.

    Article  PubMed  Google Scholar 

  23. Dinkin MJ, Patsalides A. Venous sinus stenting in idiopathic intracranial hypertension: results of a prospective trial. J Neuroophthalmol. 2017;37(2):113–21. https://doi.org/10.1097/WNO.0000000000000426.

    Article  PubMed  Google Scholar 

  24. Elder BD, Goodwin CR, Kosztowski TA, et al. Venous sinus stenting is a valuable treatment for fulminant idiopathic intracranial hypertension. J Clin Neurosci. 2015;22(4):685–9. https://doi.org/10.1016/j.jocn.2014.10.012.

    Article  PubMed  Google Scholar 

  25. Hartmann AJPW, Latting MW, Lee MS, et al. Papilloedema from dural venous sinus compression by meningiomas. Neuroophthalmology. 2018;43(3):171. https://doi.org/10.1080/01658107.2018.1524499.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Levitt MR, Albuquerque FC, Gross BA, et al. Venous sinus stenting in patients without idiopathic intracranial hypertension. J Neurointerv Surg. 2017;9(5):512–5. https://doi.org/10.1136/neurintsurg-2016-012405.

    Article  PubMed  Google Scholar 

  27. Lu G, Shin JH, Song Y, Lee DH. Stenting of symptomatic lateral sinus thrombosis refractory to mechanical thrombectomy. Interv Neuroradiol. 2019;25(6):714–20. https://doi.org/10.1177/1591019919852168.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Elhammady MS, Ambekar S, Heros RC. Epidemiology, clinical presentation, diagnostic evaluation, and prognosis of cerebral dural arteriovenous fistulas. Handb Clin Neurol. 2017;143:99–105. https://doi.org/10.1016/B978-0-444-63640-9.00009-6.

    Article  PubMed  Google Scholar 

  29. Guo WY, Lee CJ, Lin CJ, et al. Quantifying the cerebral hemodynamics of dural arteriovenous fistula in transverse sigmoid sinus complicated by sinus stenosis: a retrospective cohort study. AJNR Am J Neuroradiol. 2017;38(1):132–8. https://doi.org/10.3174/ajnr.A4960.

    Article  PubMed  PubMed Central  Google Scholar 

  30. Friedman DI. Medication-induced intracranial hypertension in dermatology. Am J Clin Dermatol. 2005;6(1):29–37. https://doi.org/10.2165/00128071-200506010-00004.

    Article  PubMed  Google Scholar 

  31. Thon OR, Gittinger JW Jr. Medication-related pseudotumor cerebri syndrome. Semin Ophthalmol. 2017;32(1):134–43. https://doi.org/10.1080/08820538.2016.1228415.

    Article  PubMed  Google Scholar 

  32. Blanch RJ, Vasseneix C, Liczkowski A, et al. Differing presenting features of idiopathic intracranial hypertension in the UK and US. Eye (Lond). 2019;33(6):1014–9. https://doi.org/10.1038/s41433-019-0359-5.

    Article  CAS  Google Scholar 

  33. Subramaniam S, Fletcher WA. Obesity and weight loss in idiopathic intracranial hypertension: a narrative review. J Neuroophthalmol. 2017;37(2):197–205. https://doi.org/10.1097/WNO.0000000000000448.

    Article  PubMed  Google Scholar 

  34. Bidot S, Bruce BB, Saindane AM, Newman NJ, Biousse V. Asymmetric papilledema in idiopathic intracranial hypertension. J Neuroophthalmol. 2015;35(1):31–6. https://doi.org/10.1097/WNO.0000000000000205.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002;59(10):1492–5. https://doi.org/10.1212/01.wnl.0000029570.69134.1b.

    Article  PubMed  Google Scholar 

  36. Manfield JH, Yu KK, Efthimiou E, Darzi A, Athanasiou T, Ashrafian H. Bariatric surgery or non-surgical weight loss for idiopathic intracranial hypertension? A systematic review and comparison of meta-analyses. Obes Surg. 2017;27(2):513–21. https://doi.org/10.1007/s11695-016-2467-7.

    Article  PubMed  Google Scholar 

  37. Ottridge R, Mollan SP, Botfield H, et al. Randomised controlled trial of bariatric surgery versus a community weight loss programme for the sustained treatment of idiopathic intracranial hypertension: the Idiopathic Intracranial Hypertension Weight Trial (IIH:WT) protocol. BMJ Open. 2017;7(9):e017426. https://doi.org/10.1136/bmjopen-2017-017426.

    Article  PubMed  PubMed Central  Google Scholar 

  38. 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. https://doi.org/10.1001/jamaneurol.2014.133.

    Article  PubMed  PubMed Central  Google Scholar 

  39. NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee, Wall M, MP MD, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI, Katz DM, Keltner JL, Schron EB, Kupersmith MJ. 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. https://doi.org/10.1001/jama.2014.3312.

    Article  CAS  Google Scholar 

  40. ten Hove MW, Friedman DI, Patel AD, et al. Safety and tolerability of acetazolamide in the idiopathic intracranial hypertension treatment trial. J Neuroophthalmol. 2016;36(1):13–9. https://doi.org/10.1097/WNO.0000000000000322.

    Article  PubMed  Google Scholar 

  41. Wall M, Falardeau J, Fletcher WA, et al. Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension. Neurology. 2015;85(9):799–805. https://doi.org/10.1212/WNL.0000000000001896.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  42. Wall M, Johnson CA, Cello KE, et al. Visual field outcomes for the idiopathic intracranial hypertension treatment trial (IIHTT). Invest Ophthalmol Vis Sci. 2016;57(3):805–12. https://doi.org/10.1167/iovs.15-18626.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Bruce BB, Digre KB, McDermott MP, Schron EB, Wall M, NORDIC Idiopathic Intracranial Hypertension Study Group. Quality of life at 6 months in the idiopathic intracranial hypertension treatment trial. Neurology. 2016;87(18):1871–7. https://doi.org/10.1212/WNL.0000000000003280.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. Friedman DI, Quiros PA, Subramanian PS, Mejico LJ, Gao S, McDermott M, Wall M, the NORDIC IIHTT Study Group. Headache in idiopathic intracranial hypertension: findings from the idiopathic intracranial hypertension treatment trial. Headache. 2017;57(8):1195–205. https://doi.org/10.1111/head.13153. Epub 2017 Jul 28. PMID: 28752894; PMCID: PMC5799151.

    Article  PubMed  PubMed Central  Google Scholar 

  45. Keisu M, Wiholm BE, Ost A, Mortimer O. Acetazolamide-associated aplastic anaemia. J Intern Med. 1990;228(6):627–32. https://doi.org/10.1111/j.1365-2796.1990.tb00290.x.

    Article  CAS  PubMed  Google Scholar 

  46. 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(5):322–7. https://doi.org/10.1111/j.1600-0404.2007.00905.x.

    Article  CAS  PubMed  Google Scholar 

  47. Schoeman JF. Childhood pseudotumor cerebri: clinical and intracranial pressure response to acetazolamide and furosemide treatment in a case series. J Child Neurol. 1994;9(2):130–4. https://doi.org/10.1177/088307389400900205.

    Article  CAS  PubMed  Google Scholar 

  48. Antaraki A, Piadites G, Vergados J, Andreou A, Chlouverakis C. Octreotide in benign intracranial hypertension. Lancet. 1993;342(8880):1170. https://doi.org/10.1016/0140-6736(93)92150-r.

    Article  CAS  PubMed  Google Scholar 

  49. Panagopoulos GN, Deftereos SN, Tagaris GA, et al. Octreotide: a therapeutic option for idiopathic intracranial hypertension. Neurol Neurophysiol Neurosci. 2007;2007:1.

    Google Scholar 

  50. House PM, Stodieck SR. Octreotide: the IIH therapy beyond weight loss, carbonic anhydrase inhibitors, lumbar punctures and surgical/interventional treatments. Clin Neurol Neurosurg. 2016;150:181–4. https://doi.org/10.1016/j.clineuro.2016.09.016.

    Article  PubMed  Google Scholar 

  51. Falardeau J, Lobb BM, Golden S, Maxfield SD, Tanne E. The use of acetazolamide during pregnancy in intracranial hypertension patients. J Neuroophthalmol. 2013;33(1):9–12. https://doi.org/10.1097/WNO.0b013e3182594001.

    Article  PubMed  Google Scholar 

  52. Ibrahim A, Hussain N. Brief report: Metabolic acidosis in newborn infants following maternal use of acetazolamide during pregnancy. J Neonatal Perinatal Med. 2019;13(3):419–25. https://doi.org/10.3233/NPM-190333.

    Article  Google Scholar 

  53. Satti SR, Leishangthem L, Chaudry MI. Meta-analysis of CSF diversion procedures and dural venous sinus stenting in the setting of medically refractory idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 2015;36(10):1899–904. https://doi.org/10.3174/ajnr.A4377.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  54. 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. https://doi.org/10.1097/WNO.0000000000000457.

    Article  PubMed  Google Scholar 

  55. Hwang TN, Rofagha S, McDermott MW, Hoyt WF, Horton JC, McCulley TJ. Sunken eyes, sagging brain syndrome: bilateral enophthalmos from chronic intracranial hypotension. Ophthalmology. 2011;118(11):2286–95. https://doi.org/10.1016/j.ophtha.2011.04.031.

    Article  PubMed  Google Scholar 

  56. Hyde RA, Mocan MC, Sheth U, Kaufman LM. Evaluation of the underlying causes of papilledema in children. Can J Ophthalmol. 2019;54(6):653–8. https://doi.org/10.1016/j.jcjo.2019.02.007.

    Article  PubMed  PubMed Central  Google Scholar 

  57. 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.

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Andrew R. Carey .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Carey, A.R. (2021). Papilledema. In: Henderson, A.D., Carey, A.R. (eds) Controversies in Neuro-Ophthalmic Management. Springer, Cham. https://doi.org/10.1007/978-3-030-74103-7_6

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-74103-7_6

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-74102-0

  • Online ISBN: 978-3-030-74103-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics