gms | German Medical Science

Objectives: Patients with mucopolysaccharidosis type I nowadays have a good life expectancy due to early therapeutic options such as stem cell therapy. Basically, MPS leads to a variety of musculoskeletal developmental disorders. The stem cell therapy can prevent the progression of some malformations of the skeleton e.g. odontoid hypoplasia. In contrast, the progression of thoracolumbar kyphoscoliosis, genua vara and hip dysplasia cannot be influenced. We present three cases of children suffering MPS I with thoracolumbar kyphosis/ kyphoscoliosis treated with growing rod systems.

Methods: The medical records and radiological imaging of three children (mean age 9.7 years) with the diagnosis of MPS I and kyphosis/ kyphoscoliosis of the lumbar spine treated from 2007 to 2017 were retrospectively analyzed. Radiological evaluation included radiographs in two planes (antero-posterior and lateral) and MRI-scans of the whole spine. All patients underwent frequent postoperative follow-up.

Results and conclusion: Two children presented with a kyphoscoliosis, one case showed a combination of severe anterolisthesis with kyphoscoliosis. Surgery with correction of the kyphosis and dorsal stabilization was performed in all patients after exhausted conservative treatment. Initial fusion was not necessary. There were no neurological complications. Postoperative treatment and aftercare included a manufactured corset for four months and physical therapy. In all three cases distraction surgery of the lumbar stabilization was done twice with a mean interval of one year.

If conservative treatment fails and surgery is necessary an individual approach is needed. Dorsal stabilization with pedicle screws using growing rod techniques is an option for the correction of thoracolumbar/lumbar kyphosis in children with MPS I. However, fusion should be prevented initially and or should be kept as short as possible. We achieved acceptable correction of the spinal deformity using the growing rod technique. A corset and physical therapy are necessary for aftercare. Follow-up should be done at frequent intervals. Finally, surgery with correction and fusion is needed after exhausted correction potential.