Background: It has been previously reported that with current management, 9/10 children with craniopharyngioma will survive 10 years. Though most suffer both chronic neuroendocrine and cognitive impairments from disease and/or treatment, few prospective cognitive assessments have been reported which might separate the contributions of each.
Objective and hypotheses: i) To prospectively evaluate the prevalence of cognitive dysfunction, before and after a conservative surgical and radiation strategy, avoiding hypothalamic morbidity. ii) To determine aetiology, incidence and severity of any deficits.
Method: 21 children (14 males, seven females) presenting with craniopharyngioma between 09/07/2009 and 25/02/2014, of median age 7.8 (range 1.917.18) years underwent Wechsler assessments of IQ (full scale (FSIQ), verbal (VIQ), performance (PIQ) and working memory (WMI) and processing speed index (PSI)), after conservative surgery and before adjuvant proton (n=7) or IMRT (n=7) radiation to any residual. eight subjects underwent a repeat assessment 13 years after radiotherapy. Tumours were graded for hypothalamic involvement (Paris 02). There were no baseline demographic, tumour or cognitive differences between those who did and did not undergo reassessment (P=0.16). Data are shown as means and 95% CI and analysed using non parametric statistics.
Results: Baseline IQ data on all parameters was available for only 16/21 patients assessed at 9.40 (2.8017.50) years of age. At diagnosis, the majority (12/16) had hypothalamic Grade 2 tumour involvement, but two had Grade 1 involvement and two had none. The overall median score for FSIQ was 105.5 (73128), no different from age and sex standardised norms, and without any difference between individual domains (VIQ, PIQ, PSI, WMI, P=0.121). A significant number of patients, 13/16 (81%) had behavioural problems (limited adaptation, peer interaction and emotional distress) at diagnosis. Eight patients-7 Grade 2 and one Grade 1 assessed 2.05 (0.603.30) years later, tended to a decline though still normal FSIQ (105.5 vs 97.0 95 ci; P=0.48) not seen in VIQ P=0.26), PIQ (P=0.916), WMI (0.292) or PSI (P=0.528) Behaviour remained unchanged, only one out of eight patients seem to be coping better.
Conclusion: Children with craniopharyngioma are of average intelligence at diagnosis but experience significant behavioural difficulties, which precede radiotherapy and are not changed by it, possibly due to hypothalamic tumour involvement. The trend to decline, seen after radiotherapy, can be found in FSIQ, but not in VIQ or other parameters, even in the youngest children. Proactive targeted schooling support may prevent significant decline, arguably due as much to behavioural difficulties, failed community reintegration and lost schooling, as to brain injury of multiple aetiology.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology