Thoracolumbar kyphoscoliosis with unilateral subluxation of the spine and postoperative lumbar spondylolisthesis in Hunter syndrome

Abstract

<jats:p>Surgical correction for kyphoscoliosis is increasingly being performed for patients with mucopolysaccharidosis (MPS). Reported case series have predominantly included patients with Type I (Hurler) and Type IV (Morquio) MPS. To their knowledge, the authors describe the first case report of surgical management of thoracolumbar kyphoscoliosis in Hunter syndrome (MPS Type II) and the rare occurrence of lumbar spondylolisthesis following surgical stabilization. A 12-year-old boy with Hunter syndrome presented with severe thoracolumbar kyphoscoliosis and no associated symptoms. Spinal radiographs demonstrated kyphosis of 48° (T11-L3) and scoliosis of 22° (T11-L3) with an anteriorly hypoplastic L-1 vertebra. The deformity progressed to kyphosis of 60° and scoliosis of 42° prior to surgical intervention. Spinal CT scans identified left T12-L1 facet subluxation, causing anterior rotatory displacement of the spine proximal to L-1 and bilateral L-5 isthmic spondylolysis with no spondylolisthesis. A combined single-stage anterior and posterior instrumented spinal arthrodesis from T-9 to L-4 was performed. Kyphosis and scoliosis were corrected to 4° and 0°, respectively. Prolonged ventilator support and nasogastric feedings were required for 3 months postoperatively. At 2.5 years following surgery, the patient was asymptomatic, mobilizing independently, and had achieved a solid spinal fusion. However, he had also developed a Grade II spondylolisthesis at L4–5; this was managed nonoperatively in the absence of symptoms or further deterioration of the spondylolisthesis to the 3.5-year postoperative follow-up visit. Satisfactory correction of thoracolumbar kyphoscoliosis in Hunter syndrome can be achieved by combined anterior/posterior instrumented arthrodesis. The risk of developing deformity or instability in motion segments adjacent to an instrumented fusion may be greater in patients with MPS related to the underlying connective tissue disorder.</jats:p>

Orientation of the facet joints in degenerative rotatory lumbar scoliosis: an MR study on 52 patients

“…An asymmetry in the orientation of facet joint surfaces has been previously discussed as a potential cause of spinal degeneration [2,35,42] and instability [1,7,17,21,30,33,53]. This abnormality has been also suggested as an underlying cause of rotatory displacement of the spine in both anteroposterior [47] and axial directions [46].…”

Does orthopaedic surgery improve quality of life and function in patients with mucopolysaccharidoses?

“…Thoracolumbar kyphosis is a classic finding in MPS I and has also been reported in MPS II, 38 IV and VI. 10 , 39 - 41 Scoliosis can occur in association with kyphosis or in isolation, but surgery for scoliosis is uncommon in the absence of significant kyphosis.…”

Mucopolysaccharidoses and Orthopedic Management (Focused also on Craniocervical Junction)

“…A 12-year-old boy with kyphoscoliosis was successfully treated with combined anterior/posterior instrumented arthrodesis. 32 Based on available imaging, the evaluation of 39 patients enrolled in the Hunter Outcome Survey showed that in 87% any spine involvement was detected: almost 80% had thoracic vertebral deformity and 3.4% spinal compression, while 94% had lumbar vertebral deformity and 3% spinal cord compression. Spine fusion or decompression was performed in less than 1% of patients.…”