Endocrine, cognitive and visual outcomes following treatment for Craniopharyngioma at a single institution: a prospective observational study
Esieza Clare Ikazoboh, Carly Redington, Adam Kuczynski, Dominic Thompson, Faraneh Vadgha Khadem & Helen Spoudeas
We prospectively assessed endocrine, cognitive and visual outcomes in eight (three males) craniopharyngioma (CP) patients aged 7.98 (range 2.4514.15) years presenting to our centre over the last 18 months, according to initial risk-based surgical strategy. Four patients had incomplete debulking (GpA), and four had conservative cyst decompression surgery (GpB). One patient (GpB) recurred 8 months later and needed repeat cyst decompression surgery. Four patients (two from each Gp) had adjuvant radiotherapy (DXT) delivered as protons in two cases.
BMI increased (mean increase of +1.05 SDS and 0.56 SDS by 29 months and >9 months respectively) post operatively in all but was worse for GpA. All GpA patients were rendered panhypopitiutary (with DI) by surgery, before DXT, whereas 3/4 GpB patients currently have intact HPA function, and one developed panhypopitiutarism and DI shortly after conservative cyst decompression surgery (and before DXT). 3/8 patients (GpA=2; GpB=1) presented with visual field defects, one (GpA) progressing to unilateral blindness post-operatively. All our patients attend mainstream schools with normal neuro-development.
Despite the rarity of CP we have managed eight new patients over 18 months on a risk-based surgical strategy and demonstrated that conservative cyst decompression surgery demonstrably preserves pituitary (and visual) function but does not prevent a rapid post-op increase in BMI, although this is less marked than after debulking surgery. Longer-term follow up will serve to show if recurrence is also minimalised and intellectual function preserved.