Pediatric cervical kyphosis in the MRI era (1984–2008) with long-term follow up: literature review

Objective Cervical kyphosis is rare in the pediatric population. It may be syndromic or acquired secondary to laminectomy, neoplasia, or trauma. Regardless, this should be avoided to prevent progressive spinal deformity and neurological deficit. Long-term follow-up is needed to evaluate fusion status, spine growth, potential instability, and neurological function. Methods and materials A retrospective review of 27 children (6 months to 16 years) with cervical kyphotic deformity was performed and limited to the MRI era until 2008, to provide a long-term follow-up after which complex instrumentation was available. There were 27 patients, 19 syndromic (average age 5.36 years), and 8 non-syndromic (average age 14 years). Syndromes encountered were spondyloepiphyseal dysplasia (SED) 4, spondylometaphyseal dysplasia 1, unnamed collagen abnormality syndrome 1, osteogenesis imperfecta (OI) 2, Aarskog syndrome 1, Weaver syndrome 1, Larsen syndrome 1, multiple cervical level disconnection syndrome 1, Klippel-Feil 3, congenital absence of C2 pars 4. Non-syndromic cases; 2 with neurofibromatosis (NF1) and prevertebral tumors, fibromatosis 1, spontaneous kyphosis 1, and postlaminectomy 4. Factors considered were age, pathology, flexibility on cervical spine dynamic films, reduction with traction and spinal cord compression. Patients with flexible kyphosis underwent dorsal fixation. Children with non-flexible ventral compression/kyphosis had crown halo traction. Irreducible kyphosis had ventral decompression and fusion as well as dorsal fusion. Eleven of 19 syndromic children with flexible and reducible kyphosis underwent dorsal fixation alone. Four of 8 non-syndromic (2 NF1) needed ventral and dorsal approaches. Results The preoperative deformity (global and local Cobb angles) as well as neurological status improved. Growth during follow-up was not impaired, and we did not encounter instability or junctional kyphosis. The only complications were seen in syndromic patients. One patient with SED showed delayed cantilever bending of the ventral fusion mass requiring reoperation, and 1 other OI child had left C5 and C6 nerve root weakness after anterior C4 and C5 decompression which resolved over 1 year. One child with SED developed cervicothoracic junction scoliosis 18 years later after thoracic scoliosis surgery. Conclusions Syndromic pathology presented early with neurological dysfunction and 24% had rigid kyphosis. An attempt at traction/reduction was successful as in