Spinal cord injury in children often occurs without evidence of fracture or dislocation. The mechanisms of neural damage in this syndrome of spinal cord injury without radiographic abnormality (SCIWORA) include flexion, hyperextension, longitudinal distraction, and ischemia. Inherent elasticity of the vertebral column in infants and young children, among other age-related anatomical peculiarities, render the pediatric spine exceedingly vulnerable to deforming forces. The neurological lesions encountered in this syndrome include a high incidence of complete and severe partial cord lesions. Children younger than 8 years old sustain more serious neurological damage and suffer a larger number of upper cervical cord lesions than children aged over 8 years. Of the children with SCIWORA, 52% have delayed onset of paralysis up to 4 days after injury, and most of these children recall transient paresthesia, numbness, or subjective paralysis. Management includes tomography and flexion-extension films to rule out incipient instability, and immobilization with a cervical collar. Delayed dynamic films are essential to exclude late instability, which, if present, should be managed with Halo fixation or surgical fusion. The long-term prognosis in cases of SCIWORA is grim. Most children with complete and severe lesions do not recover; only those with initially mild neural injuries make satisfactory neurological recovery.

Patients with tethered cord syndrome (TCS) rarely have symptomatic onset in adulthood. Twenty-three adult patients with TCS were studied with respect to the clinical, radiological, and pathological features of this syndrome. Specific circumstances involving either additional tugging of the already tight conus, narrowing of the spinal canal, or direct trauma to the back or buttocks precipitated symptomatic onset in 60% of patients. Diffuse and non-dermatomal leg pain, often referred to the anorectal region, was the most common presenting symptom. Progressive sensorimotor deficits in the lower extremities as well as bladder and bowel dysfunction were also common findings; but, unlike TCS in children, progressive foot and spinal deformities were not seen. As in TCS with onset in childhood, the most common tethering lesions were thickened filum, intradural lipoma, and fibrous adhesions. The degree of cord traction, rather than the type or distribution of the tethering lesions, probably determines the age of symptom onset: less severe traction remains asymptomatic in childhood but results in neurological dysfunction in later life due to repeated tugging of the conus during natural head and neck flexion, or when abnormal tension is aggravated by trauma or spondylotic canal stenosis. Metrizamide myelography revealed the diagnosis of tethered conus in most cases, but the addition of computerized tomographic imaging provided valuable structural details concerning the tethering lesion. The surgical outcome was gratifying in relation to pain and motor weakness but disappointing in the resolution of bowel and bladder dysfunction. Early diagnosis and adequate release of the tethered conus are the keys to successful management.

Injury prevention, prompt recognition, use of MRI and electrophysiological verification, and timely bracing of SCIWORA patients remain the chief measures to improve outcome.

No abstract

Much confusion still exists concerning the pathological definitions and clinical significance of double spinal cord malformations. Traditional terms used to describe the two main forms of these rare malformations, diastematomyelia and diplomyelia, add to the confusion by their inconsistent usage, ambiguities, and implications of their dissimilar embryogenesis. Based on the detailed radiographic and surgical findings of 39 cases of double cord malformations and the autopsy data on two other cases, this study endorses a new classification for double cord malformations and proposes a unified theory of embryogenesis for all their variant forms and features. The new classification recommends the term split cord malformation (SCM) for all double spinal cords. A Type I SCM consists of two hemicords, each contained within its own dural tube and separated by a dura-sheathed rigid osseocartilaginous median septum. A Type II SCM consists of two hemicords housed in a single dural tube separated by a nonrigid, fibrous median septum. These two essential features necessary for typing, the state of the dural tube and the nature of the median septum, do not ever overlap between the two main forms and can always be demonstrated by imaging studies so that accurate preoperative typing is always possible. All other associated structures in SCM such as paramedian nerve roots, myelomeningoceles manqué, and centromedian vascular structures frequently do overlap between types and are not reliable typing criteria. The unified theory of embryogenesis proposes that all variant types of SCMs have a common embryogenetic mechanism. Basic to this mechanism is the formation of adhesions between ecto- and endoderm, leading to an accessory neurenteric canal around which condenses an endomesenchymal tract that bisects the developing notochord and causes formation of two hemineural plates. The altered state of the emerging split neural tube and the subsequent ontogenetic fates of the constituent components of the endomesenchymal tract ultimately determine the configuration and orientation of the hemicords, the nature of the median septum, the coexistence of various vascular, lipomatous, neural, and fibrous oddities within the median cleft, the high association with open myelodysplastic and cutaneous lesions, and the seemingly unlikely relationship with fore and midgut anomalies. The multiple facets of this theory are presented in increasing complexity against the background of known embryological facts and theories; the validity of each facet is tested by comparing structures and phenomena predicted by the facet with actual radiographic, surgical, and histopathological findings of these 41 cases of SCM.