Ulrich Nils Harry-Bert, Maier Matthias, Bernays Rene-Ludwig, Krayenbuhl Niklaus, Kollias Spyros
University Hospital of Zurich, Department of Neurosurgery, Zurich, Switzerland.
Turk Neurosurg. 2013;23(3):410-4. doi: 10.5137/1019-5149.JTN.5604-11.0.
We present a rare cause of cervical myelopathy produced by an engorged suboccipital epidural venous plexus due to chronic cerebrospinal fluid (CSF) overdrainage. A 17-year-old boy with obstructive hydrocephalus due to a retrocerebellar cyst and secondary implantation of a ventricloperitoneal shunt (VP-shunt) presented with progressive spastic tetraparesis. MRI imaging revealed myelopathy due to significant compression of the cervical spinal cord by engorged epidural veins. Further assessment at a low-pressure setting revealed a broken shunt valve. The VP-shunt valve was changed with an additional anti-siphon device leading to a gradual increase of the intracranial pressure (ICP). After intensive physiotherapy, the patient showed slight clinical improvement. Follow-up imaging within nine days showed distinct regression of the dilated venous plexus at the cranial-cervical junction (CCJ) with the resolution of cord compression. Engorgement of the epidural venous plexus should always be considered in the differential diagnosis of myelopathy in long-term shunt patients even when classical clinical and radiological signs of overshunting are missing.
我们报告了一例因慢性脑脊液过度引流导致枕下硬膜外静脉丛充血而引起的颈髓病的罕见病例。一名17岁男孩,因小脑后囊肿导致梗阻性脑积水并继发脑室腹腔分流术(VP分流),出现进行性痉挛性四肢轻瘫。MRI成像显示,硬膜外静脉充血导致颈髓明显受压,从而引起脊髓病。在低压状态下进一步评估发现分流阀损坏。更换了VP分流阀并增加了一个抗虹吸装置,导致颅内压(ICP)逐渐升高。经过强化物理治疗,患者临床症状略有改善。九天内的随访成像显示,颅颈交界区(CCJ)扩张的静脉丛明显消退,脊髓压迫解除。即使长期分流患者没有典型的过度分流临床和放射学征象,在颈髓病的鉴别诊断中也应始终考虑硬膜外静脉丛充血的情况。