Pettorini Benedetta Ludovica, Gao Anna, Rodrigues Desiderio
Department of Paediatric Neurosurgery, Birmingham Children's Hospital, Steelhouse Lane, B4 6NH, Birmingham, UK.
Childs Nerv Syst. 2011 Jun;27(6):857-60. doi: 10.1007/s00381-011-1442-9. Epub 2011 Apr 7.
Report and discuss acute neurological deterioration of Chiari I malformation (CMI), surgical treatment and timing, clinical outcome and literature review.
We report a recent case of a 15-year-old girl admitted for an acute neurological deterioration caused by an undiagnosed hindbrain herniation that required an urgent foramen magnum decompression and external ventricular drainage insertion. Moreover, we report and discuss the literature. To the authors' knowledge, this is only the third paediatric patient identified in this setting to be treated for an acute neurological deterioration due to a previous asymptomatic hindbrain herniation. The patient made an uncomplicated recovery and was discharged home on the seventh postoperative day and has remained well at review.
Acute deterioration of CMI in completely asymptomatic patients in absence of concomitant pathological findings is rarely reported in the literature, and exceptional in children.
CMI is typically treated electively, but as this case illustrates, it can present with rapidly deteriorating neurological signs. Symptoms usually respond well to surgical intervention with rapid improvement.
报告并讨论 Chiari I 型畸形（CMI）的急性神经功能恶化、手术治疗及时机、临床结果并进行文献综述。
我们报告了一例近期收治的 15 岁女孩，因未确诊的后脑疝导致急性神经功能恶化，需要紧急进行枕骨大孔减压和置入脑室外引流。此外，我们报告并讨论相关文献。据作者所知，这是该情况下第三例因先前无症状的后脑疝而接受急性神经功能恶化治疗的儿科患者。患者恢复顺利，术后第七天出院，复查时情况良好。
文献中很少报道完全无症状且无伴随病理发现的患者出现 CMI 急性恶化的情况，儿童中更是罕见。
CMI 通常采用择期治疗，但如本病例所示，它可能表现为神经体征迅速恶化。症状通常对手术干预反应良好，可迅速改善。
