A case of cervical spinal cord injury in 12-year-old angular craniopagus twins is presented, with a description of the planning and execution of surgical treatment along with subsequent clinical outcome. The injury occurred following a fall from a standing position, resulting in quadriparesis in one of the twins. Imaging revealed severe craniocervical stenosis resulting from a C1–2 dislocation, and T2-weighted hyperintensity of the cervical spinal cord. After custom halo fixation was obtained, a posterior approach was utilized to decompress and instrument the occiput, cervical, and upper thoracic spine with intraoperative reduction of the dislocation. Early neurological improvement was noted during the acute postoperative phase, and 27 months of follow-up demonstrated intact instrumentation with continued neurological improvement to near baseline. The complexity of managing such an injury, inclusive of the surgical, anesthetic, biomechanical, and ethical considerations, is described in detail.
Michael P. Wemhoff, Kevin Swong, Daphne Li, Neal Mugve, Lisa A. Gramlich and Russ P. Nockels
Stephen J. Johans, Kevin N. Swong, Daniel J. Burkett, Michael P. Wemhoff, Sean M. Lew, Chirag R. Patel and Anand V. Germanwala
Superficial siderosis (SS) of the CNS is a rare and often unrecognized condition. Caused by hemosiderin deposition from chronic, repetitive hemorrhage in the subarachnoid space, it results in parenchymal damage in the subpial layers of the brain and spinal cord. T2-weighted MRI shows the characteristic hypointensity of hemosiderin deposition, classically occurring around the cerebellum, brainstem, and spinal cord. Patients present with progressive gait ataxia and sensorineural hearing impairment. Although there have been several studies, case reports, and review articles over the years, the clear pathophysiology of subarachnoid space hemorrhage remains to be elucidated. The proposed causes include prior intradural surgery, prior trauma, tumors, vascular abnormalities, nerve root avulsion, and dural abnormalities.
Surgical repair of a dural defect associated with SS has been shown to be efficacious at preventing symptomatic progression. There have been several reports of dural defects within the spinal canal treated with surgery. Here, the authors present the first known case of a dural defect of the ventral skull base, namely a clival meningocele, presumed to be causing SS. In this case report, a 10-year-old girl with a history of head trauma at the age of 3 years was found to have a clival meningocele 3 years after her original trauma. On follow-up imaging, the patient was found to have radiographic growth of the meningocele along with evidence of SS of the CNS. The patient was treated conservatively until she began to have progressive hearing loss. It was presumed that the growing meningocele was the source of her SS. An endoscopic endonasal transclival approach with a multilayer dural reconstruction was performed to fix the dural defect and repair the meningocele in hopes of mitigating the progression of her symptoms. At her 12-month postoperative follow-up, she was doing well, with audiometry showing a slightly decreased hearing threshold in the left ear but improved speech discrimination bilaterally. Postoperative MRI showed a stable level of hemosiderin deposition and meningocele repair. Long-term follow-up will be necessary to evaluate for continued clinical stabilization or possible improvement.