Objective: To present clinical and morphological findings before and after surgery in a child with Chairi I malformation (CMI) and intra-cranial hypertension (IH). The literature is reviewed and pathophysiologic factors are discussed.
Clinical presentation: A 13-year-old obese boy with a 3-week history of headaches, neck pain, torticollis and progressive visual deterioration was admitted. Bi-lateral chronic papilloedema and decrease in visual acuity were found in the presence of a previously diagnosed CMI. INTERVENTION AND FOLLOW-UP: Intra-cranial pressure monitoring demonstrating increased pressure levels was followed by a sub-occipital decompression, C1 laminectomy and duroplasty. Post-operatively, the boy improved markedly, the 6 months follow-up opthalmological examination demonstrated resolution of papilloedema, but consecutive bi-lateral optic nerve atrophy.
Conclusion: IH with progressive visual deterioration represents one of the varying clinical presentations of CMI and may be classified as a secondary form of idiopathic IH. Neuro-ophthalmological examination in all patients with CMI is recommended to identify the real incidence of this presentation. Altered CSF dynamics, venous hypertension and obesity as co-factors may be causative pathophysiologic factors.