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Long-term visual outcomes of craniopharyngioma in children

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Abstract

Visual function is a critical factor in the diagnosis, monitoring, and prognosis of craniopharyngiomas in children. The aim of this study was to report the long-term visual outcomes in a cohort of pediatric patients with craniopharyngioma. The study design is a retrospective chart review of craniopharyngioma patients from a single tertiary-care pediatric hospital. 59 patients were included in the study. Mean age at presentation was 9.4 years old (range 0.7–18.0 years old). The most common presenting features were headache (76%), nausea/vomiting (32%), and vision loss (31%). Median follow-up was 5.2 years (range 1.0–17.2 years). During follow-up, visual decline occurred in 17 patients (29%). On Kaplan Meier survival analysis, 47% of the cases of visual decline occurred within 4 months of diagnosis, with the remaining cases occurring sporadically during follow-up (up to 8 years after diagnosis). In terms of risk factors, younger age at diagnosis, optic nerve edema at presentation, and tumor recurrence were found to have statistically significant associations with visual decline. At final follow-up, 58% of the patients had visual impairment in at least one eye but only 10% were legally blind in both eyes (visual acuity 20/200 or worse or < 20° of visual field). Vision loss is a common presenting symptom of craniopharyngiomas in children. After diagnosis, monitoring vision is important as about 30% of patients will experience significant visual decline. Long-term vision loss occurs in the majority of patients, but severe binocular visual impairment is uncommon.

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References

  1. Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM (1998) The descriptive epidemiology of craniopharyngioma. J Neurosurg 89:547–551. https://doi.org/10.3171/jns.1998.89.4.0547

    Article  CAS  PubMed  Google Scholar 

  2. Ostrom QT, de Blank PM, Kruchko C, Petersen CM, Liao P, Finlay JL, Stearns DS, Wolff JE, Wolinsky Y, Letterio JJ, Barnholtz-Sloan JS (2015) Alex’s lemonade stand foundation infant and childhood primary brain and central nervous system tumors diagnosed in the United States in 2007–2011. Neuro Oncol 16(Suppl 10):x1–x36. https://doi.org/10.1093/neuonc/nou327

    Article  PubMed  Google Scholar 

  3. Miller DC (1994) Pathology of craniopharyngiomas: clinical import of pathological findings. Pediatr Neurosurg 21(Suppl 1):11–17

    Article  PubMed  Google Scholar 

  4. Weiner HL, Wisoff JH, Rosenberg ME, Kupersmith MJ, Cohen H, Zagzag D, Shiminski-Maher T, Flamm ES, Epstein FJ, Miller DC (1994) Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery 35:1001–1010 (discussion 1010–1001)

    Article  CAS  PubMed  Google Scholar 

  5. Steinbok P (2015) Craniopharyngioma in children: long-term outcomes. Neurol Med Chir 55:722–726. https://doi.org/10.2176/nmc.ra.2015-0099

    Article  Google Scholar 

  6. Chen C, Okera S, Davies PE, Selva D, Crompton JL (2003) Craniopharyngioma: a review of long-term visual outcome. Clin Exp Ophthalmol 31:220–228

    Article  PubMed  Google Scholar 

  7. Mallucci C, Pizer B, Blair J, Didi M, Doss A, Upadrasta S, Newman W, Avula S, Pettorini B (2012) Management of craniopharyngioma: the Liverpool experience following the introduction of the CCLG guidelines: introducing a new risk assessment grading system. Childs Nerv Syst 28:1181–1192. https://doi.org/10.1007/s00381-012-1787-8

    Article  PubMed  Google Scholar 

  8. Buch H, Vinding T, Nielsen NV (2001) Prevalence and causes of visual impairment according to World Health Organization and United States criteria in an aged, urban Scandinavian population: the Copenhagen city eye study. Ophthalmology 108:2347–2357

    Article  CAS  PubMed  Google Scholar 

  9. Congdon N, O’Colmain B, Klaver CC, Klein R, Munoz B, Friedman DS, Kempen J, Taylor HR, Mitchell P, Eye Diseases Prevalence Research Group (2004) Causes and prevalence of visual impairment among adults in the United States. Arch Ophthalmol 122: 477–485 https://doi.org/10.1001/archopht.122.4.477

    Article  PubMed  Google Scholar 

  10. Lateef TM, Merikangas KR, He J, Kalaydjian A, Khoromi S, Knight E, Nelson KB (2009) Headache in a national sample of American children: prevalence and comorbidity. J Child Neurol 24:536–543. https://doi.org/10.1177/0883073808327831

    Article  PubMed  PubMed Central  Google Scholar 

  11. Lin LL, El Naqa I, Leonard JR, Park TS, Hollander AS, Michalski JM, Mansur DB (2008) Long-term outcome in children treated for craniopharyngioma with and without radiotherapy. J Neurosurg Pediatr 1:126–130. https://doi.org/10.3171/PED/2008/1/2/126

    Article  PubMed  Google Scholar 

  12. Villani RM, Tomei G, Bello L, Sganzerla E, Ambrosi B, Re T, Giovanelli Barilari M (1997) Long-term results of treatment for craniopharyngioma in children. Childs Nerv Syst 13:397–405

    Article  CAS  PubMed  Google Scholar 

  13. Van Effenterre R, Boch AL (2002) Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97:3–11. https://doi.org/10.3171/jns.2002.97.1.0003

    Article  PubMed  Google Scholar 

  14. Habrand JL, Ganry O, Couanet D, Rouxel V, Levy-Piedbois C, Pierre-Kahn A, Kalifa C (1999) The role of radiation therapy in the management of craniopharyngioma: a 25-year experience and review of the literature. Int J Radiat Oncol Biol Phys 44:255–263

    Article  CAS  PubMed  Google Scholar 

  15. Karavitaki N, Brufani C, Warner JT, Adams CB, Richards P, Ansorge O, Shine B, Turner HE, Wass JA (2005) Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clin Endocrinol 62:397–409. https://doi.org/10.1111/j.1365-2265.2005.02231.x

    Article  CAS  Google Scholar 

  16. Gautier A, Godbout A, Grosheny C, Tejedor I, Coudert M, Courtillot C, Jublanc C, De Kerdanet M, Poirier JY, Riffaud L, Sainte-Rose C, Van Effenterre R, Brassier G, Bonnet F, Touraine P, Craniopharyngioma Study G (2012) Markers of recurrence and long-term morbidity in craniopharyngioma: a systematic analysis of 171 patients. J Clin Endocrinol Metab 97:1258–1267. https://doi.org/10.1210/jc.2011-2817

    Article  CAS  PubMed  Google Scholar 

  17. Abrams LS, Repka MX (1997) Visual outcome of craniopharyngioma in children. J Pediatr Ophthalmol Strabismus 34:223–228

    CAS  PubMed  Google Scholar 

  18. Winkfield KM, Tsai HK, Yao X, Larson E, Neuberg D, Pomeroy SL, Ullrich NJ, Cohen LE, Kieran MW, Scott RM, Goumnerova LC, Marcus KJ (2011) Long-term clinical outcomes following treatment of childhood craniopharyngioma. Pediatr Blood Cancer 56:1120–1126. https://doi.org/10.1002/pbc.22884

    Article  PubMed  Google Scholar 

  19. Sorva R, Heiskanen O, Perheentupa J (1988) Craniopharyngioma surgery in children: endocrine and visual outcome. Childs Nerv Syst 4:97–99

    CAS  PubMed  Google Scholar 

  20. Reddy GD, Hansen D, Patel A, Lin Y, Jea A, Lam S (2016) Treatment options for pediatric craniopharyngioma. Surg Neurol Int 7:S174–S178. https://doi.org/10.4103/2152-7806.178570

    Article  PubMed  PubMed Central  Google Scholar 

  21. Chia EM, Mitchell P, Rochtchina E, Foran S, Wang JJ (2003) Unilateral visual impairment and health related quality of life: the Blue mountains eye study. Br J Ophthalmol 87:392–395

    Article  PubMed  PubMed Central  Google Scholar 

  22. Wang JJ, Mitchell P, Smith W (2000) Vision and low self-rated health: the Blue mountains eye study. Invest Ophthalmol Vis Sci 41:49–54

    CAS  PubMed  Google Scholar 

  23. Taylor HR, McCarty CA, Nanjan MB (2000) Vision impairment predicts five-year mortality. Trans Am Ophthalmol Soc 98:91–96 (discussion 96–99)

    CAS  PubMed  PubMed Central  Google Scholar 

  24. Lee DJ, Gomez-Marin O, Lam BL, Zheng DD (2002) Visual acuity impairment and mortality in US adults. Arch Ophthalmol 120:1544–1550

    Article  PubMed  Google Scholar 

  25. Poretti A, Grotzer MA, Ribi K, Schonle E, Boltshauser E (2004) Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev Med Child Neurol 46:220–229

    Article  PubMed  Google Scholar 

  26. van Dijk K (2007) Providing care for children with low vision. Community Eye Health 20:24–25

    PubMed  PubMed Central  Google Scholar 

  27. Huang D, Swanson EA, Lin CP, Schuman JS, Stinson WG, Chang W, Hee MR, Flotte T, Gregory K, Puliafito CA et al (1991) Optical coherence tomography. Science 254:1178–1181

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Mediero S, Noval S, Bravo-Ljubetic L, Contreras I, Carceller F (2015) Visual outcomes, visual fields, and optical coherence tomography in paediatric craniopharyngioma. Neuroophthalmology 39:132–139. https://doi.org/10.3109/01658107.2015.1039549

    Article  PubMed  PubMed Central  Google Scholar 

  29. Bialer OY, Goldenberg-Cohen N, Toledano H, Snir M, Michowiz S (2013) Retinal NFL thinning on OCT correlates with visual field loss in pediatric craniopharyngioma. Can J Ophthalmol 48:494–499. https://doi.org/10.1016/j.jcjo.2013.05.001

    Article  PubMed  Google Scholar 

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Acknowledgements

Assistance with statistical analysis: Derek Stephens, MSc. Consulting Biostatistician, The Hospital for Sick Children. Manager, Biostatistics, Design & Analysis, The Hospital for Sick Children. Lecturer, Department of Public Health Sciences, University of Toronto.

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Authors and Affiliations

  1. Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children and University of Toronto, 555 University Ave., Toronto, ON, M5G 1X8, Canada

    Michael J. Wan

  2. Division of Haematology/Oncology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada

    Michal Zapotocky, Eric Bouffet & Ute Bartels

  3. Division of Neurosurgery, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada

    Abhaya V. Kulkarni & James M. Drake

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  1. Michael J. Wan
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  2. Michal Zapotocky
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Correspondence to Michael J. Wan.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Wan, M.J., Zapotocky, M., Bouffet, E. et al. Long-term visual outcomes of craniopharyngioma in children. J Neurooncol 137, 645–651 (2018). https://doi.org/10.1007/s11060-018-2762-3

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  • DOI: https://doi.org/10.1007/s11060-018-2762-3

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