Segmental Spinal Dysgenesis (SSD)

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Status

Done

Parent item

Spinal anomalies

General

• A dysraphic condition

• When segments of the spine and spinal cord fail to develop properly.

Diagnostic criteria

• Paraparesis/paraplegia, including lower limb aberrations;

• More than one segment vertebral abnormality which may include kyphosis or kyphoscoliosis;

• The absence or malformation of a portion of the spinal cord, along with associated nerve roots anywhere from the cervical spine to the sacrum;

• The presence of spinal cord distal to the affected region of cord.

Mechanism

• Mishap in the development of the early neural tube that results in a nonclosure

Radiology

• Images show marked lumbar spine abnormalities;

• Vertebral body hypoplasia;

• Absent posterior segments in L3-L5;

• Anterior displacement of the sacrum relative to the upper lumbar spine, resulting in a focal z-shaped deformity and kyphosis;

• Diminutive sacrum;

• A low-lying, posteriorly tethered spinal cord adherent to the dura with distorted morphology.

• The arrows across all images indicate the z-shaped deformity and also highlight where the tethered cord is most evident in magnetic resonance images B and C

 

Reference

• https://www.ochsnerjournal.org/content/20/3/244