Skip to main content

Advertisement

SpringerLink
Log in

How do presentation age and CSF opening pressure level affect long-term prognosis of pseudotumor cerebri syndrome in children? Experience of a single tertiary clinic

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Background

Diagnosis and treatment of pseudotumor cerebri syndrome in children is still a challenge for clinicians. The aim of this study is to reveal the influence of presentation age and CSF opening pressure on long-term prognosis of pseudotumor cerebri and share our clinical data of the very young age (≤ 5-year) group.

Method

This retrospective study includes the patients followed by the Marmara University Pediatric Neurology Clinic between years 2012 and 2020 diagnosed with definite, probable, or suggestive pseudotumor cerebri syndrome according to modified Friedman criteria. Patients were classified into three groups according to presentation age: group 1: ≤ 5 years old; group 2: 6–10 years; and group 3 > 10 years old. CSF opening pressure was also categorized into three groups as CSF < 20 cmH20; CSF 20–30 cmH20; and CSF > 30 cmH20.

Results

One hundred three patients, 62.1% female (n = 64), were enrolled in the study. Group 1 consisted of 16 patients (60% male), group 2 consisted of 30 patients (63.3% female), and group 3 consisted of 57 patients (66.7% female). The mean CSF opening pressure did not differ between the three age groups in our study (p > 0.05). Treatment response was not correlated with CSF opening pressure. Papilledema presence and level of CSF opening pressure were independent of age (p > 0.05).

Conclusions

Age at presentation and CSF opening pressure at diagnosis are not any predictive factors that influence long-term prognosis of pseudotumor cerebri syndrome in children. Evaluation and follow-up of children should be done in personalized approach.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Availability of data and materials

All raw data included in this study will be freely available from the corresponding author on reasonable request without breaching patient confidentiality.

References

  1. Balbi GGM, Matas SL, Len CA, Frage MM, Sousa IO, Terreri MT (2018) Pseudotumor cerebri in childhood and adolescence: data from a specialized service. Arq Neuropsiquiatr 76(11):751–755. https://doi.org/10.1590/0004-282X20180131

    Article  PubMed  Google Scholar 

  2. Per H, Canpolat M, Gümüş H, Poyrazoğlu HG, Yıkılmaz A, Karaküçük S et al (2013) Clinical spectrum of the pseudotumor cerebri in children: etiological, clinical features, treatment and prognosis. Brain Dev 35(6):561–568. https://doi.org/10.1016/j.braindev.2012.08.008

    Article  PubMed  Google Scholar 

  3. Wall M, Kupersmith M, Kieburtz K, Corbett J, Feldon S, Friedman D et al (2014) Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA 311(16):1641–1651. https://doi.org/10.1001/jama.2014.3312

    Article  CAS  PubMed  Google Scholar 

  4. Gersti L, Schoppe N, Albers L, Erti-Wagner B, Alperin N, Ehrt O et al (2017) Pediatric idiopathic intracranial hypertension - is the fixed threshold value of elevated LP opening pressure set too high? - Eur J Paediatr Neurol 21(6):833–841. https://doi.org/10.1016/j.ejpn.2017.08.002

  5. Spitze A, Lam P, Al-Zubidi N, Yalamanchili S, Lee AG (2014) Controversies: optic nerve sheath fenestration versus shunt placement for the treatment of idiopathic intracranial hypertension. Indian J Ophtalmol 62(10):1015–1021. https://doi.org/10.4103/0301-4738.146012

    Article  Google Scholar 

  6. Friedman DI, Liu GT, Digre KB (2013) Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 81:1–7. https://doi.org/10.1212/WNL.0b013e3182a55f17

    Article  Google Scholar 

  7. Sheldon CA, Paley GL, Beres SJ, McCormack SE, Liu GT (2017) Pediatric pseudotumor cerebri syndrome: diagnosis, classification, and underlying pathophysiology. Semin Pediatr Neurol 24(2):110–115. https://doi.org/10.1016/j.spen.2017.04.002

    Article  PubMed  PubMed Central  Google Scholar 

  8. Burkett JG, Ailani J (2018) An up to date review of pseudotumor cerebri syndrome. Curr Neurol Neurosci Rep 18(6):33. https://doi.org/10.1007/s11910-018-0839-1

    Article  CAS  PubMed  Google Scholar 

  9. Rangwala LM, Lui GT (2007) Pediatric idiopathic intracranial hypertension. Surv Ophtalmol 52(6). https://doi.org/10.1016/j.survophthal.2007.08.018. https://doi.org/10.1007/s00701-016-3050-7

  10. Portelli M, Papageorgiou PN (2017) An update on idiopathic intracranial hypertension. Acta Neurochir (Wien) 159(3):491–499. https://doi.org/10.1007/s00701-016-3050-7

    Article  Google Scholar 

  11. Barmberzig R, Szperka CL (2019) Pseudotumor cerebri syndrome in children. Curr Pain Headache Rep 23(8):58. https://doi.org/10.1007/s11916-019-0795-8

  12. Hilely A, Hecht I, Cohen NG, Leiba H (2019) Long term follow-up of pseudotumor cerebri syndrome in pre-pubertal children, adolescents and adults. Pediatr Neurol 101(57):63. https://doi.org/10.1016/j.pediatrneurol.2019.04.018

    Article  Google Scholar 

  13. Aylward SC (2013) Pediatric idiopathic intracranial hypertension: a need for clarification. Pediatr Neurol 49. https://doi.org/10.1016/j.pediatrneurol.2013.05.019

  14. Dandy WE (1937) Intracranial pressure without brain tumor. Ann Surg 106:492–513. https://doi.org/10.1097/00000658-193710000-00002

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Kohli AA, Vossough A, Mallery RM, Woo JH, Sheldon CA, Paley GL, Digre KB, Friedman DI, Farrar JT, Mc Cormack SE, Liu GT, Szperka CL (2019) Magnetic resonance imaging findings in pediatric pseudotumor cerebri syndrome. Pediatr Neurol 99:31–39. https://doi.org/10.1016/j.pediatrneurol.2019.04.010

    Article  PubMed  PubMed Central  Google Scholar 

  16. Mollan SP, Markey KA, Benzimra JD, Jacks A, Matthews TD, Burdon MA, Sinclair AJ (2014) A practical approach to diagnosis, assesment and management of idiopathic intracranial hypertension. Pract Neurol 14:380–390. https://doi.org/10.1136/practneurol-2014-000821

    Article  PubMed  PubMed Central  Google Scholar 

  17. Lenfeldt N, Koskinen LOD, Berenheim AT, Malm J, Eklund A (2007) CSF pressure assesed by lumbar puncture agrees with intracranial pressure. Neurology 68(2):155–158. https://doi.org/10.1212/01.wnl.0000250270.54587.71

    Article  CAS  PubMed  Google Scholar 

  18. Mahajnah M, Genizi J, Zahalka H, Andreus R, Zelnik N (2020) Pseudotumor cerebri syndrome: from childhood to adulthood risk factors and clinical presentation. J Child Neurol 35(5):311–316. https://doi.org/10.1177/0883073819895179

    Article  PubMed  Google Scholar 

  19. Bar-Joseph G, Guilburd Y, Tamir A, Guilburd JN (2009) Effectiveness of ketamine in decreasing intracranial pressure in children with intracranial hypertension. J Neurosurg Pediatr 4:40–46. https://doi.org/10.3171/2009.1.PEDS08319

    Article  PubMed  Google Scholar 

  20. Inger H, Rogers DL, McGregor ML, Aylward AC, Reem RE (2017) Diagnostic criteria in pediatric intracranial hypertension. J AAPOS 21(6):492-495.e2. https://doi.org/10.1016/j.jaapos.2017.08.003

    Article  PubMed  Google Scholar 

  21. Philips PH, Sheldon CA (2017) Pediatric pseudotumor cerebri syndrome. J Neuro-Ophtalmol 37(Suppl):S33–S40. https://doi.org/10.1097/WNO.0000000000000548

    Article  Google Scholar 

  22. Bole S, Martinez-Rios C, Tibussek D, Pohl D (2019) Infantile idiopathic intracranial hypertension: a case study and review of the literature. J Child Neurol 34(13):806–814. https://doi.org/10.1177/0883073819860393

    Article  Google Scholar 

  23. Kattah JC, Pula JH, Mejico LJ, McDermott MP, Kupersmith MJ, Wall M (2015) CSF Pressure, pilledema grade and response to acetozolamide in the idiopathic Intracranial Hypertension Treatment Trial. J Neurol 262(10):2271–2274. https://doi.org/10.1007/s00415-015-7838-9

    Article  CAS  PubMed  Google Scholar 

  24. Lim M, Kurian M, Penn A, Calver D, Lin JP (2005) Visual failure without headache in idiopathic intracranial hypertension. Arch Dis Child 90:206–210. https://doi.org/10.1136/adc.2003.039305

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Gondi KT, Chen KS, Gratton SM (2019) Asymptomatic versus symptomatic idiopathic intracranial hypertension in children. J Child Neurol 34(12):751–756. https://doi.org/10.1177/0883073819858455

    Article  PubMed  Google Scholar 

  26. Reid JE, Reem RE, Aylward SC, Rogers DL (2016) Sixth nerve palsy in pediatric intracranial hypertension. Neuroophtalmology 40(1):23–27. https://doi.org/10.3109/01658107.2015.1117498

    Article  Google Scholar 

  27. Mallery RM, Friedman DI, Liu GT (2014) Headache and the pseudotumor cerebri syndrome. Curr Pain Headache Rep 18:446. https://doi.org/10.1007/s11916-014-0446-z

    Article  PubMed  Google Scholar 

  28. Lin A, Foroozan R, Danesh-Meyer HV, De Salvo G, Savino PJ, Sergott RC (2006) Occurrence of cerebral venous sinus thrombosis in patients with presumed idiopathic intracranial hypertension. Ophtalmology 113(12):2281–2284. https://doi.org/10.1016/j.ophtha.2006.05.065

    Article  Google Scholar 

  29. Fonseca PL, Rigamonti D, Miller NR, Subramanian PS (2014) Visual outcomes of surgical intervention for pseudotumor cerebri:optic nerve sheath fenestration versus cerebrospinal fluid diversion. Br J Ophtalmol 98:1360. https://doi.org/10.1136/bjophthalmol-2014-304953

    Article  Google Scholar 

  30. Bruce BB, Digre KB, McDermott MP, Schron EB, Wall M (2016) NORDIC Idiopathic Intracranial Hypertension Study Group. Quality of life at 6 months in the idiopathic intracranial hypertension treatment trial. Neurology 87:18717. https://doi.org/10.1212/WNL.0000000000003280

  31. Soiberman U, Stolovitch C, Balcer LJ, Regenbogen M, Constantini S, Kesler A (2011) Idiopathic intracranial hypertension in children:visual outcome and risk recurrence. Childs Nerv Syst 27(11):1913–8. https://doi.org/10.1007/s00381-011-1470-5

  32. Masri A, Jaafar A, Noman R, Gharaibeh A, Ababneh OH (2015) Intracranial hypertension in children: etiologies, clinical features and outcome. J Child Neurol 30(12):1562–8. https://doi.org/10.1177/0883073815574332

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Neurology, Marmara University Medical Faculty Pendik Education and Research Hospital, Istanbul, Turkey

    Gulten Ozturk, Dilşad Turkdogan & Olcay Unver

  2. Department of Ophtalmology, Marmara University Pendik Education and Research Hospital, Istanbul, Turkey

    Volkan Dericioglu

  3. Department of Radiology, Marmara University Pendik Education and Research Hospital, Istanbul, Turkey

    Bulent Aslan

  4. Department of Neurosurgery, Marmara University Medical Faculty Pendik Education and Research Hospital, Istanbul, Turkey

    Adnan Dagcinar

Authors
  1. Gulten Ozturk
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Dilşad Turkdogan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Olcay Unver
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Volkan Dericioglu
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Bulent Aslan
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Adnan Dagcinar
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

GO designed the study, collected and analyzed data and wrote the manuscript. OU and DT read and critically revised the manuscript. VD performed the ophtalmologic examination of the patients, BA performed the radiological evaluation of cranial MRIs, and AD performed the surgical procedures to the patients when necessary.

Corresponding author

Correspondence to Gulten Ozturk.

Ethics declarations

Ethical approval

Ethics committee approval was obtained from the local ethics committee of the university with approval number 674 (12.06.2020). Informed consent was obtained from the parents of patients involved in the study. The authors also affirm that the participants gave consent for their data in a journal article.

Conflict of interest

The authors declare no potential conflicts of interest.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This manuscript presents a comprehensive study about Pediatric Pseudotumor Cerebri Syndrome based on demonstrative characteristics according to age and CSF opening pressure. Long follow-up experience of a tertiary pediatric neurology clinic is given with specific emphasis on pitfalls on diagnosis, and correlation of our findings is done compared to the literature.

Supplementary Information

Below is the link to the electronic supplementary material.

Supplementary file1 (DOCX 18 KB)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ozturk, G., Turkdogan, D., Unver, O. et al. How do presentation age and CSF opening pressure level affect long-term prognosis of pseudotumor cerebri syndrome in children? Experience of a single tertiary clinic. Childs Nerv Syst 38, 95–102 (2022). https://doi.org/10.1007/s00381-021-05365-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-021-05365-8

Keywords

Search

Navigation