Abstract
Syringomyelia refers to the presence of cavities within the spinal cord or a dilatation of the central spinal cord canal. In 90% of cases, syringomyelia is associated with a Chiari I malformation. The association of syringomyelia with tethered cord is well known but syrinxes associated with these defects are usually below vertebral level T6. Holocord syrinx associated with tethered cord is rare and is almost always associated with Chiari 1 malformation. To the best of our knowledge, only a single case report of holocord syrinx with tethered cord has been reported, but this patient had multiple overt lumbosacral defects (tethered cord, meningocele and diastematomyelia). We are reporting a three year old child with holocord syrinx with tethered cord and diastematomyelia and no evidence of Chiari malformation, meningocele or any overt spinal malformation and minimal neurological abnormalities. J Nepal Paediatr Soc 2012;32(2):169-171 doi: http://dx.doi.org/10.3126/jnps.v32i2.6098
Highlights
Syringomyelia refers to presence of cavities within the spinal cord or a dilatation of the central spinal cord canal
In 90% of cases, syringomyelia is associated with a Chiari I malformation[1], where the cerebellar tonsils descend through the foramen magnum and disrupt the normal CSF flow[2]
Syringomyelia may be associated with other obstructive lesions at the foramen magnum as well as spinal cord trauma, intramedullary tumors, infection, cord tethering, and developmental anomalies
Summary
Syringomyelia refers to presence of cavities within the spinal cord or a dilatation of the central spinal cord canal. We are reporting a 3 year old child with holocord syrinx with tethered cord and diastematomyelia and no evidence of Chiari malformation, meningocele or any overt spinal malformation and minimal neurological abnormalities. A three year old male child was brought with gradual onset, progressively increasing clumsiness and frequent falls while walking since last three months. He was apparently well prior to this period and attained all developmental milestones at normal age. Child was appropriately toilet trained with normal bladder and bowel function. He had no difficulty in getting up from supine position or weakness of upper limbs. In view of progressively increasing symptoms, the child was referred to neurosurgeons for appropriate intervention
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