Abstract
BACKGROUND
Pediatric patients with craniopharyngioma have a high rate of survival but are at risk for vision loss. The epidemiology of long term vision impairment has not been well-described in the literature in patients diagnosed in the recent decades.
METHODS
We reviewed medical records and abstracted information on clinical presentation, treatment and sequential vision function in 44 patients diagnosed and treated between 1997 and 2014 at a large tertiary children’s hospital. We assessed visual impairment using a combined metric of visual acuity and visual fields using an adapted version of the International Statistical Classification of Diseases standards for vision.
RESULTS
Sixteen patients underwent a gross total resection (GTR, 6 received radiotherapy) and 22 were treated with radiation therapy at diagnosis or recurrence in addition to subtotal resection, biopsy or cyst-directed therapy. Eighteen (41%) had a recurrence and with a median follow up time of 59 months overall survival rate was 93%. At diagnosis 18 (51%) patients were classified as having normal vision, 9 (26%) impaired and 8 (23%) blind (9 uncooperative), whereas at latest follow-up 15 (35%) had normal vision, 9 (21%) were impaired and 19 (44%) were blind (1 uncooperative) (p = 0.13). Upfront treatment modality (GTR vs any XRT) and recurrence were not associated with visual impairment.
CONCLUSIONS
Substantial number of patients with craniopharyngioma have poor vision at long-term follow up. Larger multicenter studies are needed to determine whether upfront treatment modality has an impact on vision in long term survivors.