Kurschel Senta, Maier Richard, Gellner Verena, Eder Hans Georg
Department of Neurosurgery, Medical University, Auenbruggerplatz 29, 8036, Graz, Austria.
Childs Nerv Syst. 2007 Aug;23(8):901-5. doi: 10.1007/s00381-007-0355-0. Epub 2007 May 8.
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.
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.
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.
介绍一名患有Chiari I型畸形（CMI）和颅内高压（IH）儿童手术前后的临床和形态学表现。回顾相关文献并讨论病理生理因素。
一名13岁肥胖男孩因头痛、颈部疼痛、斜颈和进行性视力下降3周入院。在先前诊断为CMI的情况下，发现双侧慢性视乳头水肿和视力下降。
颅内压监测显示压力水平升高，随后进行枕下减压、C1椎板切除术和硬脑膜成形术。术后，男孩明显好转，6个月的随访眼科检查显示视乳头水肿消退，但随后出现双侧视神经萎缩。
伴有进行性视力下降的IH是CMI不同临床表现之一，可归类为特发性IH的继发形式。建议对所有CMI患者进行神经眼科检查，以确定这种表现的实际发生率。脑脊液动力学改变、静脉高压和肥胖作为共同因素可能是致病的病理生理因素。
