Combined spinal arthrodesis with instrumentation for the management of progressive thoracolumbar kyphosis in children with mucopolysaccharidosis.
OBJECTIVE
To evaluate the outcome and complications of a novel technique for the treatment of progressive thoracolumbar kyphosis in children with mucopolysaccharidosis (MPS).


METHODS
The medical records and spinal imaging of four consecutive paediatric patients who underwent a single stage anteroposterior spinal fusion with segmental pedicle screw instrumentation were reviewed.


RESULTS
Patients underwent spinal deformity correction at the mean age of 3 years (2.4-3.7) with mean clinical follow-up of 3.2 years (2.1-4.5) and mean postoperative radiographic follow-up was 2.4 years (0.8-3). Preoperative kyphosis was corrected from a mean angle of 65º (63º-70º) to 6.5º (-12º-13º). Vertebral subluxation at the apex of the deformity was corrected from an average 64% (56-83%) to 12% (0-24%). Spinal cord monitoring with somatosensory evoked potentials (SSEP) was successfully obtained and stable throughout surgery. No instrumentation failure, loss of correction or junctional problems occurred at final follow-up.


CONCLUSIONS
Anterior and posterior spinal arthrodesis with segmental pedicle screw instrumentation is a safety and reliable technique for the treatment of severe thoracolumbar kyphosis in children with MPS. This technique achieves excellent correction of the deformity with adequate decompression of the spinal canal. The fusion is limited to the thoracolumbar junction and interferes minimally with the longitudinal growth of the thorax. No neurological complications or intraoperative spinal cord monitoring events occurred. No loss of correction or junctional kyphosis was observed.
Scottish National Spine Deformity Centre, Royal Hospital for Sick Children, Sciennes Road, Edinburgh, EH9 1LF, UK, enrique.garrido@luht.scot.nhs.uk.
Enrique
E
Garrido
Félix
F
Tomé-Bermejo
Christopher I
CI
Adams
D002675:Child, Preschool; D019468:Disease Management; D018450:Disease Progression; D005260:Female; D006801:Humans; D004204:Joint Dislocations / Q000150:complications / Q000601:surgery*; D007738:Kyphosis / Q000150:complications / Q000601:surgery*; D008297:Male; D009083:Mucopolysaccharidoses / Q000150:complications*; D065289:Pedicle Screws; D012189:Retrospective Studies; D013123:Spinal Fusion / Q000295:instrumentation / Q000379:methods*; D016896:Treatment Outcome