Posterior spinal fusion for scoliosis in mucopolysaccharidosis I (Hurler syndrome)

Abstract

Spinal deformity is a characteristic feature of mucopolysaccharidosis (MPS), for which surgical correction is indicated when the deformity is progressive to avoid neurological deficits and respiratory impairment. However, there exist few reports on the surgical treatment of spinal deformity in MPS and no treatment standard has been established. We herein describe the clinical and radiological outcomes of a patient with MPS-I receiving surgery for severe thoracolumbar (TL) kyphoscoliosis. The patient was a 12-year-old girl with MPS-I who underwent posterior spinal fusion with an all pedicle screw construct from T9 to L4 for a preoperative TL curve Cobb angle of 50° and TL kyphotic angle of 71°. Postoperative Cobb angle of the TL curve and TL kyphotic angle improved to 5 and 12°, respectively. Although her lumbar lordosis decreased slightly due to mild back-out of the pedicle screws in L4, bony union was achieved 3 years after surgery without perioperative complications. Based on the present case, posterior spinal fusion represents a strong treatment option for severe spinal deformity in MPS. Keywords: Posterior spinal fusion, Pedicle screw, Thoracolumbar kyphoscoliosis, Radiological findings, Mucopolysaccharidosis