Evaluation of Syringomyelia in Children

Examination

• Motor and sensory including tone: Comprehensive motor and sensation examinations should be performed. Myelopathy signs such as spasticity and hyperreflexia should be sought. In central cord syndrome, dissociated sensory impairment is usually evident on examination.

• Seek signs of brainstem involvement: If syringomyelia extends into the brainstem (syringobulbia) or when it is associated with Chiari malformation, there is often involvement of cranial nerve or cerebellar function.

Laboratory Tests

• Routine preoperative studies only

• CSF analysis unusual: CSF analysis is not routinely recommended. When it is obtained, the cell count is usually normal and the protein count might be mildly elevated.

Radiologic Tests

Regular x-rays

• Only for assessment of scoliosis: There is no role for plain radiographs in the diagnosis of syringomyelia or associated conditions. Plain radiographs are primarily used for assessment of scoliosis.

Plain AP x-ray of spine: Note the scoliosis

CT scans

• None indicated

MRI

• CSF cavity on T2-weighted imaging: Imaging may show a dilated cavity with the same intensity as CSF on T2-weighted imaging.

T2-weighted sagittal MRI of cervical syrinx: Note the large upper cervical syrinx expanding the cord diameter and an associated Chiari I malformation.

• Evaluate entire CNS axis: If a syrinx is found on routine MRI, further imaging studies should be sought to determine the primary etiology of the syrinx. This should include a complete brain and spinal MRI with and without gadolinium.

• Cine MRI: Cine MRI may also help in diagnosing abnormal CSF flow patterns, but so far results have been conflicting.

Nuclear Medicine Tests

• None

Electrodiagnostic Tests

• Not routine: Neurophysiology tests such as SSEPs can show low-amplitude or delayed responses in cases of myelopathy. Motor evoked potentials may be more sensitive than SSEPs in the evaluation of spinal cord dysfunction. Neither is routinely obtained.

Neuropsychological Tests

• None

Correlation of Tests

• Imaging for size and symptoms: The most difficult problem is to distinguish between symptoms related to the syrinx and those related to the underlying etiology. Nonetheless, the presence of a large syrinx warrants treatment regardless of symptoms. Small syrinxes may be followed with serial imaging.