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

Childs Nerv Syst. 2007 Aug;23(8):901-5. doi: 10.1007/s00381-007-0355-0. Epub 2007 May 8.


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.

Publication types

  • Case Reports

MeSH terms

  • Arnold-Chiari Malformation / complications*
  • Arnold-Chiari Malformation / physiopathology
  • Arnold-Chiari Malformation / surgery
  • Atrophy
  • Child
  • Humans
  • Hypertension / complications
  • Intracranial Hypertension / etiology*
  • Intracranial Hypertension / physiopathology
  • Intracranial Hypertension / surgery
  • Intracranial Pressure / physiology
  • Magnetic Resonance Imaging
  • Male
  • Neurosurgical Procedures
  • Obesity / complications
  • Optic Nerve Diseases / complications
  • Optic Nerve Diseases / pathology
  • Papilledema / complications
  • Papilledema / pathology
  • Vision Disorders / etiology
  • Visual Acuity