Congenital scoliosis

The human spine is formed by individual vertebrae and connective tissue discs in between. The vertebrae form the spinal canal. There are seven cervical, twelve thoracic, and five lumbar vertebrae. The intervertebral discs are the link between the individual vertebral bodies.

The little joints that link the vertebrae together are known as facet joints. They help to stabilize the spine and, together with the intervertebral discs, allow a certain degree of mobility of the spinal cord. The spinal canal should be wide enough to allow nerve roots to float freely in cerebrospinal fluid. The front border of the spinal canal is built by the vertebral bodies and intervertebral discs, the side by the intervertebral joints (facets) and back by the ligamentum flavum (yellow band) and vertebral arches. Discs consist of an outer fibrous ring (annulus fibrosus), which surrounds an inner gel-like center (nucleus pulposus). The spinal cord and nerve roots lie within the spinal canal. The spinal cord extends downwards approx. to the 1st lumbar vertebra. Below, only nerve roots are present in the spinal canal. At the level of the intervertebral disc the nerve roots pass through the neural root foramina to exit the spinal canal. The spinal cord and nerve roots conduct electric-like signals from the skin and joints to the brain, and process of movement is initiated from the brain to the muscles.

Congenital scoliosis is a spinal deformity ocurring during pregnancy in the first trimester (PMID 30078055). Most cases occur spontaneously. May be related to maternal diseases such as diabetes or intrauterine exposure to alcohol, valproic acid, etc. These factors can lead to the formation of vertebrae of irregular shape, thus disrupting the physiological form of the whole spine. Often associated with Syringomyelia, Diastematomyelia, Arnold-Chiari malformation, Tethered-cord Syndrome.

Gradually progressing deformation of the spine

Gradually progressing inflammation and degeneration in the intervertebral joints

Gradually progressing compression of nerve roots

Total/partial paralysis and numbness from the site of the spinal cord damage downwoads

Visible spine deformity Chest deformity (protrusion on one side and concavity on the other) Waist asymmetry and pelvic tilt Shoulder height differences Inclined head position

Pain and stiffness along the spine

Due to unfavorable courses, surgical treatment is almost always required.

The scoliosis angle increases

Correct deformity

Anterior/posterior spinal fusion +/vertebrectomy in younger patients (girls < 10 yrs, boys < 12 yrs) with significant progression, neurologic deficits, or declining respiratory function Posterior fusion +/osteotomies and correction in older patients with significant progression, neurologic deficits, or declining respiratory function Distraction based growing rod construct adapting to spinal growth and delaing arthrodesis Hemi-Vertebrectomy Osteotomies between ribs if more than 4 fused ribs to avoid thoracic insufficiency syndrome