Brainstem Tethering with Ondine's Curse

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.