Kronenburg Annick, Han Kuo Sen, Gooskens Rob, Esposito Giuseppe, Cochrane Douglas, Woerdeman Peter
Department of Neurology and Neurosurgery, Division of Neuroscience, Rudolf Magnus Institute, University Medical Center Utrecht, Heidelberglaan 100, room G03.124, 3484 CX, Utrecht, The Netherlands.
Childs Nerv Syst. 2013 Nov;29(11):2131-5. doi: 10.1007/s00381-013-2156-y. Epub 2013 May 19.
We report about a spina bifida patient with myelomeningocele at the lumbar level L5, extensive Chiari malformation type II with vermal herniation reaching to C6 with downward pontine shift, and a severe hypoplastic cerebellum. Chromosomal diagnostic tests showed no abnormalities.
The infant experienced severe central apneas successfully treated with oxygen therapy and caffeine medication; functional motor level was established at L5 with sparse anal sphincter function.
After surgical intervention (myelomeningocele repair and ventriculoperitoneal shunt placement), these abnormalities significantly improved on radiological imaging; the preoperative hypoplastic, almost undetectable, cerebellum developed to a fair sized cerebellum. Apneas disappeared over time and the patient showed further developmental improvement. Herein, we illustrate and discuss the changes of the cerebellar volume before and after neurosurgical intervention.
我们报告了一名患有L5水平脊髓脊膜膨出的脊柱裂患者,广泛的II型Chiari畸形,伴有小脑蚓部疝至C6水平并伴有脑桥向下移位,以及严重发育不全的小脑。染色体诊断测试未显示异常。
该婴儿经历了严重的中枢性呼吸暂停,通过氧疗和咖啡因药物治疗成功治愈;功能运动水平确定在L5,肛门括约肌功能稀疏。
手术干预(脊髓脊膜膨出修复和脑室腹腔分流置入)后,这些异常在影像学上有显著改善;术前发育不全、几乎无法检测到的小脑发展为大小适中的小脑。呼吸暂停随时间消失,患者显示出进一步的发育改善。在此,我们阐述并讨论神经外科干预前后小脑体积的变化。