Acute deterioration of a Chiari I malformation: an uncommon neurosurgical emergency

作者：Pettorini Benedetta Ludovica^*; Gao Anna; Rodrigues Desiderio

来源：Child's Nervous System, 2011, 27(6): 857-860.

DOI：10.1007/s00381-011-1442-9

• Hindbrain tonsillar herniation
• Neurological deterioration
• Papilloedema
• Acute onset

Objectives Report and discuss acute neurological deterioration of Chiari I malformation (CMI), surgical treatment and timing, clinical outcome and literature review.
Case report 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.
Discussion Acute deterioration of CMI in completely asymptomatic patients in absence of concomitant pathological findings is rarely reported in the literature, and exceptional in children.
Conclusions 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.

• 出版日期2011-6