Background: To assess the use of subcutaneous rodding with sequential lengthening procedures to control progressive early onset curves not responding to conservative treatment modalities.

Methods: A retrospective study reviewing the notes and plain radiographs of all children with early onset scoliosis treated by growth rod insertion over a seven year period (two paediatric spinal surgeons using similar techniques at two major centres). Subjects were children with early onset scoliosis unresponsive to conservative management. Outcome measures – curves at time of instrumentation, curve progression, number of lengthenings, curve magnitude and age at time of definitive fusion, spinal growth achieved and complications encountered.

Results: Majority of children treated uneventfully with satisfactory control of curvature until age at which definitive fusion acceptable. However our results do suggest a number of cases and circumstances where simple growth rod instrumentation is not sufficient and augmentation with anterior apical fusion is required (will discuss these on an individual basis).

Conclusion: The management of early onset progressive scoliosis by means of growth rod instrumentation and sequential lengthenings is safe and effective.

Objective: To document an unusual pattern of clinical presentation simulating cerebral palsy and investigate the evolution of spinal deformity in relation to patients’ growth and responsiveness to levodopa therapy.

Study Design-Material: A retrospective study was performed including 3 pediatric patients with dopa-responsive dystonia who developed in the course of their disease spinal curvatures.

Summary of Background Data: Dopa-responsive dystonia has been recognized as a separate type of idiopathic dystonia with early onset, gradual generalized involvement, diurnal fluctuation of symptoms, spinal malalignment, and remarkable response to levodopa. Nevertheless, it can present with atypical features including prominent spastic elements and intrafamilial variability of expression.

Methods: The medical records and radiographs of the 3 patients were reviewed.

Results: All 3 siblings were normal at birth and had negative family history of neurological disease or spinal imbalance. Soon they developed progressive neurological impairment with exaggerated spasticity, underestimated dystonic patterns, and marked phenotypic variation, leading to the initial misdiagnosis of spastic-dystonic cerebral palsy of familial inheritance. With further growth, patient 1 and 3 developed spinal deformity, which responded dramatically to levodopa treatment and resolved spontaneously, while the neurological symptoms persistently ameliorated. Patient 2 developed a rigid C-shaped thoracolumbar scoliotic curve measuring at age 10 years 88o; even though she demonstrated considerable overall improvement with levodopa, the spinal curvature remained unresponsive and necessitated surgical correction through a combined anterior-posterior instrumented spinal fusion extending to the sacrum. However, her ambulatory function was preserved.

Conclusions: Spinal decompensation is a common manifestation of dopa-responsive dystonia with excellent prognosis if adequate diagnosis and initiation of levodopa treatment are made early. On the contrary, if diagnosis and levodopa treatment are delayed, spinal deformity may progress following the rapid evolution pattern of neuromuscular curves, necessitating surgical intervention. When spinal arthrodesis is required, inclusion of the lumbosacral joint does not preclude latter ambulatory function.

Objective-Study Design: Recognizing the value of intraoperative SEP monitoring in scoliosis and other spinal surgery, we applied prospectively continuous SEP recording during reconstructive procedures in 82 patients who sustained 20 cervical, 8 thoracic, 6 thoraco-lumbar, and 48 lumbar vertebral fractures or fractures-dislocations to investigate its efficacy in spinal trauma.

Material: Seventy-one patients underwent single anterior or posterior operations, and 11 combined anterior-posterior procedures. Forty patients had incomplete injuries, and 42 had no preoperative neurological deficit. SEP trace amplitude at insertion of electrode was considered as the baseline value, and was compared to the lowest intraoperative signal amplitude and the amplitude at completion of operation.

Results: Fifty-nine patients had a depression in wave amplitude of more than 25% during surgery; in 25 patients the trace fell by more than 50%, and in 7 cases a more than 75% diminution was recorded. A loss of 50% in SEP signal amplitude showed 67% sensitivity, and 71% specificity in predicting neurologic outcome. Patients with a fall in SEP amplitude of more than 50% that did not recover at completion of the surgical procedure demonstrated an increased risk of neurological compromise (p< .01). Increasing trace deterioration threshold from 50 to 60% improved specificity to 81% without compromising sensitivity. There was also 100% correlation between the side of the amplitude drop and the side of neurological loss in the trunk or limb (p< .001). A total number of 22 patients had improved SEP recordings before skin closure; 19 of these patients demonstrated an improved neurologic function after the operative procedure. In these 19 patients a positive statistical association could be documented between the signal changes and the neurological outcome (p< .05). Nevertheless, 2 of the patients with up to 20% improvement in the trace amplitude compared to the original control measurement presented deterioration in their neurological picture in the postoperative period. In 17 patients the SEP waveform amplitude was unchanged at conclusion of the operation; in those cases the neurological functional level post-surgery was equally unaltered. No significant difference was obtained when comparing the systolic blood pressures or the core temperatures at skin closure between the different outcome groups (p> .05). A loss of more than 50% in SEP amplitude occurred with significantly increased incidence during the anterior compared to the posterior spinal procedures (p< .001). More than 20% recovery in signal amplitude at conclusion of the procedure in patients with incomplete injuries was correlated with favorable neurological function.

Conclusions: Persistent intraoperative decrement in SEP amplitude and poor restitution at completion of surgery increase the risk for postoperative neurologic compromise. In this series, continuous intraoperative SEP monitoring appeared to be adequately reproducible, sufficiently reliable, and therefore a practical tool in monitoring operative procedures for spinal trauma. Even though compared to deformity surgery the method is less sensitive and specific, it may help reduce the incidence of devastating neurologic injury during the operation on an already compromised neural cord, and can provide good prediction in terms of postoperative neurological outcome. Thus, it could be considered a useful surgical adjunct in the management of patients with spinal trauma.