Abstract:
Introduction. Circumferential fusion of kyphosis in patients with MPS is currently accepted as the most effective surgical approach. However, long-term results remain debatable. This study assesses the effectiveness
of posterior-only compared to circumferential fusion.
Methods. Eleven patients (7 male, 4 female) with MPS and thoracolumbar kyphosis underwent surgical
treatment. Hurler Syndrome (type I) was diagnosed in 5 patients, Morquio Syndrome (type IV) in 2, and Maroto-Lamy (type VI) in 4 patients.
Indications for surgical treatment included more than 40° kyphosis, sagittal spinal imbalance, progressive
neurological symptoms and severe pain. In 3 cases, patients underwent circumferential arthrodesis combining
anterior and posterior approaches. In 8 cases, instrumentation included hooks and/or pedicular screws, placed
two levels above and two levels below the deformity apex. The follow-up period ranged from 2 to 5 years.
Results. In 8 cases solid spinal fusion was achieved. Complications after surgical treatment were observed
in 4 patients (36%). PJK developed in one case 2 years after surgery, pseudarthrosis was observed in one case,
wound suppuration was observed in one case, and a broken metal rod in one case.
Conclusions. Surgical treatment of MPS patients with thoracolumbar kyphosis is accompanied by a high
risk of complications when circumferential stabilization is performed. Most authors and our data show that the
most optimal method of surgical treatment of thoracolumbar deformation is dorsal correction and fixation
in combination with a wide laminectomy at the level of stenosis. The second stage includes the anterior decompression and interbody fusion. However, if the patient’s lung function is dramatically compromised, and a
high risk of respiratory complications exists, surgery may be limited to only posterior correction and fixation in
conjunction with a wide laminectomy, which allows to achieve a comparable level of fixation with a lower risk
of complications.
