Journal of Neuro-Oncology

, Volume 120, Issue 3, pp 651–656 | Cite as

The ophthalmic natural history of paediatric craniopharyngioma: a long-term review

  • Evangelos DrimtziasEmail author
  • Kevin Falzon
  • Susan Picton
  • Irfan Jeeva
  • Danielle Guy
  • Olwyn Nelson
  • Ian Simmons
Clinical Study


We present our experience over the long-term of monitoring of visual function in children with craniopharyngioma. Our study involves an analysis of all paediatric patients with craniopharyngioma younger than 16 at the time of diagnosis and represents a series of predominantly sub-totally resected tumours. Visual data, of multiple modality, of the paediatric patients was collected. Twenty patients were surveyed. Poor prognostic indicators of the visual outcome and rate of recurrence were assessed. Severe visual loss and papilledema at the time of diagnosis were more common in children under the age of 6. In our study visual signs, tumour calcification and optic disc atrophy at presentation are predictors of poor visual outcome with the first two applying only in children younger than 6. In contrast with previous reports, preoperative visual field (VF) defects and type of surgery were not documented as prognostic indicators of poor postoperative visual acuity (VA) and VF. Contrary to previous reports calcification at diagnosis, type of surgery and preoperative VF defects were not found to be associated with tumour recurrence. Local recurrence is common. Younger age at presentation is associated with a tendency to recur. Magnetic resonance imaging (MRI) remains the recommended means of follow-up in patients with craniopharyngioma.


Craniopharyngioma Visual acuity Visual field Tumour recurrence 


Conflict of interest

The authors declare that they have no conflict of interest.

Ethical standards

The study complies with the current laws of the country.


  1. 1.
    Parisi JE, Mena H (1993) Non glial tumours. In: Nelson JS, Parisi JE, Schochet SSJ (eds) Principles and practice of neuropathology. Mosby, St Louis, pp 203–266Google Scholar
  2. 2.
    Van Effenterre R, Boch AL (2002) Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97:3–11PubMedCrossRefGoogle Scholar
  3. 3.
    Honegger J, Buchfelder M, Fahlbusch R (1990) Surgical treatment of craniopharyngiomas: endocrinological results. J Neurosurg 90:251–257CrossRefGoogle Scholar
  4. 4.
    Müller HL (2011) Consequences of craniopharyngioma surgery in children. J Clin Endocrinol Metab 96:1981–1991PubMedCrossRefGoogle Scholar
  5. 5.
    Pereira AM, Schmid EM, Schutte PJ et al (2005) High prevalence of long-term cardiovascular, neurological and psychosocial morbidity after treatment for craniopharyngioma. Clin Endocrinol Oxf 62:197–204PubMedCrossRefGoogle Scholar
  6. 6.
    Chen C, Okera S, Davies PE et al (2003) Craniopharyngioma: a review of long-term visual outcome. Clin Exp Ophthalmol 31:220–228CrossRefGoogle Scholar
  7. 7.
    Vinchon M, Weill J, Delestret I et al (2009) Craniopharyngioma and hypothalamic obesity in children. Childs Nerv Syst 25:347–352PubMedCrossRefGoogle Scholar
  8. 8.
    Pierre-Kahn A, Recassens C, Pinto G et al (2005) Social and psycho-intellectual outcome following radical removal of craniopharyngiomas in childhood. A prospect series. Childs Nerv Syst 21:817–824CrossRefGoogle Scholar
  9. 9.
    Khafaga Y, Jenkin D, Kanaan I et al (1998) Craniopharyngioma in children. Int J Radiat Oncol Biol Phys 42:601–606PubMedCrossRefGoogle Scholar
  10. 10.
    Repka MX, Miller NR, Miller M (1989) Visual outcome after surgical removal of craniopharyngiomas. Ophthalmology 96:195–199PubMedCrossRefGoogle Scholar
  11. 11.
    Fisher PG, Jenab J, Gopldthwaite PT et al (1998) Outcomes and failure patterns in childhood craniopharyngiomas. Childs Nerv Syst 14:558–563PubMedCrossRefGoogle Scholar
  12. 12.
    Abrams LS, Repka MX (1997) Visual outcome of craniopharyngioma in children. J Pediatr Ophthalmol Strabismus 34:223–228PubMedGoogle Scholar
  13. 13.
    Avery RA, Ferner RE, Listernick R et al (2012) Visual acuity in children with low grade gliomas of the visual pathway: implications for patient care and clinical research. J Neurooncol 110:1–7PubMedCrossRefGoogle Scholar
  14. 14.
    Defoort-Dhellemmes S, Moritz F, Bouacha I et al (2006) Craniopharyngioma: ophthalmological aspects at diagnosis. J Pediatr Endocrinol Metab 19:321–324PubMedGoogle Scholar
  15. 15.
    Karavitaki N, Brufani C, Warner JT et al (2005) Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clin Endocrinol Oxf 62:397–409PubMedCrossRefGoogle Scholar
  16. 16.
    Lee MJ, Hwang JM (2012) Initial visual field as a predictor of recurrence and postoperative visual outcome in children with craniopharyngioma. J Pediatr Ophthalmol Strabismus 49:38–42PubMedCrossRefGoogle Scholar
  17. 17.
    Albright AL, Hadjipanayis CG, Lunsford LD et al (2005) Individualized treatment of pediatric craniopharyngiomas. Childs Nerv Syst 21:649–654PubMedCrossRefGoogle Scholar
  18. 18.
    Kalapurakal JA, Goldman S, Hsieh YC et al (2003) Clinical outcome in children with craniopharyngioma treated with primary surgery and radiotherapy deferred until relapse. Med Pediatr Oncol 40:214–218PubMedCrossRefGoogle Scholar
  19. 19.
    Puget S, Garnett M, Wray A et al (2007) Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg 106:3–12PubMedGoogle Scholar
  20. 20.
    Anderson VA, Godber T, Smibert E et al (2004) Impairments of attention following treatment with cranial irradiation and chemotherapy in children. J Clin Exp Neuropsychol 26:684–697PubMedCrossRefGoogle Scholar
  21. 21.
    Spiegler BJ, Bouffet E, Greenberg ML et al (2004) Change in neurocognitive functioning after treatment with cranial radiation in childhood. J Clin Oncol 22:706–713PubMedCrossRefGoogle Scholar
  22. 22.
    Neglia JP, Robison LL, Stovall M et al (2006) New primary neoplasms of the central nervous system in survivors of childhood cancer: a report from the childhood cancer survivor study. J Natl Cancer Inst 98:1528–1537PubMedCrossRefGoogle Scholar
  23. 23.
    Elliott RE, Wisoff JH (2009) Successful surgical treatment of craniopharyngioma in very young children. J Neurosurg Pediatr 3:397–406PubMedCrossRefGoogle Scholar
  24. 24.
    Vinchon M, Dhellemmes P (2008) Craniopharyngiomas in children: recurrence, reoperation and outcome. Childs Nerv Syst 24:211–217PubMedCrossRefGoogle Scholar
  25. 25.
    Thompson D, Phipps K, Hayward R (2005) Craniopharyngioma in childhood: our evidence-based approach to management. Childs Nerv Syst 2:660–668CrossRefGoogle Scholar
  26. 26.
    Tamasauskas A, Bunevicius A, Matukevicius A et al (2014) Extended pterional approach for initial surgical management of craniopharyngiomas: a case series. Turk Neurosurg 24:174–183PubMedGoogle Scholar
  27. 27.
    Elliott RE, Jane JA Jr, Wisoff JH (2011) Surgical management of craniopharyngiomas in children: meta-analysis and comparison of transcranial and transsphenoidal approaches. Neurosurgery 69:630–643PubMedCrossRefGoogle Scholar
  28. 28.
    Komotar RJ, Starke RM, Raper DM et al (2012) Endoscopic endonasal compared with microscopic transsphenoidal and open transcranial resection of craniopharyngiomas. World Neurosurg 77:329–341PubMedCrossRefGoogle Scholar
  29. 29.
    Elliott RE, Moshel YA, Wisoff JH (2009) Minimal residual calcification and recurrence after gross-total resection of craniopharyngioma in children. J Neurosurg Pediatr 3:276–283PubMedCrossRefGoogle Scholar
  30. 30.
    Spoudeas HA (2005) Paediatric endocrine tumours. A multi-disciplinary consensus statement of best practice from a working group convened under the auspices of the BSPED and UKCCSG. Novo Nordisk Ltd, CrawleyGoogle Scholar
  31. 31.
    Quinn GE, Fea AM, Minguini N (1991) Visual fields in 4- to 10-year-old children using Goldmann and double-arc perimeters. J Pediatr Ophthalmol Strabismus 28:314–319PubMedGoogle Scholar
  32. 32.
    Safran AB, Laffi GL, Bullinger A et al (1996) Feasibility of automated visual field examination in children between 5 and 8 years of age. Br J Ophthalmol 80:515–518PubMedCentralPubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  • Evangelos Drimtzias
    • 1
    Email author
  • Kevin Falzon
    • 1
  • Susan Picton
    • 2
  • Irfan Jeeva
    • 1
  • Danielle Guy
    • 1
  • Olwyn Nelson
    • 1
  • Ian Simmons
    • 1
  1. 1.Department of Ophthalmology, Leeds Teaching HospitalsSt. James University HospitalLeedsUK
  2. 2.Division of Oncology, Leeds Teaching HospitalsSt. James University HospitalLeedsUK

Personalised recommendations