Chiari I malformation and intra-cranial hypertension:a case-based review.
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.


METHODS
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.


CONCLUSIONS
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.
Kurschel
Senta
S
Department of Neurosurgery, Medical University, Auenbruggerplatz 29, 8036, Graz, Austria. senta.kurschel@meduni-graz.at
Maier
Richard
R
Gellner
Verena
V
Eder
Hans Georg
HG
D001139:Arnold-Chiari Malformation / Q000150:complications* / Q000503:physiopathology / Q000601:surgery; D001284:Atrophy; D002648:Child; D006801:Humans; D006973:Hypertension / Q000150:complications; D019586:Intracranial Hypertension / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery; D007427:Intracranial Pressure / Q000502:physiology; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures; D009765:Obesity / Q000150:complications; D009901:Optic Nerve Diseases / Q000150:complications / Q000473:pathology; D010211:Papilledema / Q000150:complications / Q000473:pathology; D014786:Vision Disorders / Q000209:etiology; D014792:Visual Acuity