- 作者：Zhan Liu ; Yanyang He ; Song Li ; Lihua Wu ; Qiangfang Jiao ; tomliuz2009@gmail.com
- 关键词：Brainstem tethering ; Occipital encephalocele ; Ondine's curse ; Ventriculoperitoneal shunt
- 刊名：World Neurosurgery
- 出版年：2011
- 出版时间：December 2011
- 年：2011
- 卷：76
- 期：6
- 页码：592.e11-592.e14
- 全文大小：930 K
Background
Brainstem tethering is a rare disease.Case Description
We report and discuss a 20-year-old patient who experienced paroxysmal apnea (a symptom of Ondine's curse) during sleeping, causing him to wake up and control his breathing consciously. A magnetic resonance imaging study revealed that his medulla oblongata was twisted and displaced posteriorly by an abnormal tissue cord. An operation was performed to detether the tethered brainstem, with a satisfying result reached.
Conclusion
Brainstem tethering is a rare but late complication of occipital encephalocele with insufficient operation. The symptoms of this disease are related to the dysfunction of the medulla oblongata and their adjunctive nerves. Magnetic resonance imaging can be used to identify the abnormal region and distinguish it from other medulla oblongata diseases. Surgery in the early stage of the brainstem tethering is helpful, but ventriculoperitoneal shunting is unnecessary or cannot be performed before detethering, although these patients usually have ventricular dilation.