1. Therapeutic and diagnostic protocol for the treatment of scoliosis associated with Syringomyelia Francesco Lolli, Konstantinos Martikos, Francesco Vommaro, Alfredo Cioni, Mario Di Silvestre, Stefano Giacomini, Tiziana Greggi Surgery of Spinal Deformities Rizzoli Orthopaedic Institute Bologna, Italy

2. The purpose of this study is provide a safe diagnostic and therapeutic procedure to treat scoliosis in syringomyelia. INTRODUCTION Scoliosis can be associated with syringomyelia … reported incidence varies between 25%-85% On the other hand, syringomyelia is associated with surgical scoliosis in 2% of the cases

3. Materials and Methods  Thirty seven patients with scoliosis in syringomyelia were treated from 1975 to 2010  24 females and 13 males  Age from 1 to 52 years  Mean follow-up 42.6 months (range 4 to 180 months)

4.  Scoliosis was diagnosed before the age of 10 in 22 out of 37 cases  The main curve of was left thoracic or thoracolumbar  Syringomyelia was most often detected in the thoracic region Materials and Methods

5.  Peripheral neurological deficits (19 subjects)  Pain (14 subjects)  13 cases presented no symptoms Frequently observed clinical manifestations correction and posterior instrumented arthrodesis in 10 patients anterior arthrodesis in 1 patient arthrodesis by double approach in 1 patient Surgery  Syringomyelia was surgically treated in 12 patients  Scoliosis was operated in 12 cases spino-costal distractor from the age of 9 until the age of 11 in 1 case

6.  Evaluation of clinical and radiographical data of the patients with scoliosis in syringomyelia showed associated malformations in 27 patients Results Arnold Chiari in 13 patients Congenital malformations of the spine in 11 patients Tethered cord in 7 patients Kidney dysplasia in 2 patients Hydrocephalus 2 patients  9 patients had more than 1 associated malformation

7. Results  At follow up, excellent outcome in one patient instrumented with spino-costal distractor from the age of 9 until the age of 11, when he was instrumented posteriorly with pedicle screws  Very good out come in 4 cases who underwent posterior arthrodesis in the adolescence  Good out come with stable correction in 6 cases, although over time revision of instrumentation was necessary in 4 patients  Junctional kyphosis due to C6 subluxation with onset of spastic tetraparesis was observed in 1 patient.  One patient with hydrocephalus showed worsening of neurological impairement in the postoperative.

8.  MRI can provide surgeons with useful information for the preoperative planning of a scoliosis and becomes mandatory when facing either a rapidly progressive curve in juvenile age  Neurological evaluation should be recommended to complete the preoperative clinical picture, as well as pre- and intraoperative SSEP (somato-sensitive evoced potentials) and MEP (motor evoced potentials)  If MRI shows myeloradicular malformations, a neurosurgical assessment becomes mandatory.  Our experience allows us to state that the diagnostic-therapeutic must be part of the scientific and cultural background of the surgeon who deals with spinal deformity of the spine Conclusions

9. None of the authors has any potential conflict of interest