diastematomyelia; diastomyelia; split cord syndrome

Epidemiology

• rare
• females affected much more frequently than males

Pathology

• longitudinal splitting of the spinal cord generally in the region of the upper lumbar spine
• a bone spur in the central part of the spinal canal produces a complete or incomplete sagittal division of the spinal cord into two hemicords
• when the split does not reunite distally, the condition is referred to as a diplomyelia (true duplication of the spinal cord)

Clinical manifestations

• usually presents in childhood
• hairy patch, dimple, hemangioma, lipoma o teratoma override the affected area of the spine in > 50% of cases
• symptoms are caused by tissue attachments that limit the movement of the spinal cord within the spinal column
• the disorder is progressive
• variable manifestations
  • foot deformities
  • spinal deformities
  • weakness in the legs
  • low back pain; scoliosis
  • urinary incontinence

Radiology

• magnetic resonance imaging of spinal cord

More general terms

• spinal cord malformation; myelatelia