Diastematomyelia

Diagnosis: Diastematomyelia (also known as a split cord malformation) refers to a type of spinal dysraphism (spina bifida occulta) when there is a longitudinal split in the spinal cord

Findings: A frontal plain film of the the thoracolumbar spine demonstrated marked scoliosis, multiple low thoracic segmentation abnormalities, and a central bony spur. A frontal view from a myelogram shows contrast surrounding two hemicords. An axial postmyelogram CT scan through the lower thoracic region reveals the bony septum separating the two hemicords, each within its own dural tube.

The two hemicords rejoined below this bony spur and were tethered in the low lumbar region.

 

Discussion: Diastematomyelia is an occult dysraphic state in which the spinal cord is divided into two hemicords by a sagittal cledt. The congenital anomaly most commonly occurs in females (80-85%) and usually involves the low thoracic or upper lumbar region. It is associated with various other abnormalities including scoliosis, vertebral segmentation anomalies, cutaneous stigmata overlying the spine,, Chiari II malformation, tethered cord, and hydromyelia (Hydromyelia refers to an abnormal widening of the central canal of the spinal cord that creates a cavity in which cerebrospinal fluid (commonly known as spinal fluid) can accumulate. As spinal fluid builds up, it may put abnormal pressure on the spinal cord and damage nerve cells and their connections.) (which may be confined to one hemicord). In half of the cases the hemicords share a dural sac; in the other half each hemicord has its own duraol sac. A fibrous or bony spur separating the teo hemicords is present in 50% of cases.

Aunt Minnie’s Pearls

  • Diastematomyelia is an occult dysraphic state (Spinal dysraphism is a medical term that refers to neurological disorders related to malformations of the spinal cord. Tethered spinal cord syndrome is a type of spinal dysraphism) in which the spinal cord is divided into two hemicords by a sagittal cleft.

 

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