The aim of this study was to investigate upper limb botulinum toxin A (BTX-A) injections in children with spastic hemiplegia.

Ten children with hemiplegia, aged 10–17 years, received upper limb BTX-A injections and 6 weeks therapy. BTX-A was injected using EMG guidance into elbow and wrist flexors, and forearm pronators (dose 1–2 units/kg body wt (Botox®) per muscle). Follow-up assessments continued to 24 weeks post BTX-A. Outcome measures included three-dimensional (3-D) upper limb analysis of functional tasks, Melbourne Assessment; passive range of motion (PROM), and muscle tone.

There were no serious adverse effects. Elbow flexor muscle tone was reduced to 12 weeks post BTX-A (p < 0.05). Mean passive elbow supination increased by 19 degrees (not significant, p= 0.3). Pre-injection 3-D analysis showed that, compared to controls, children with hemiplegia were slower at performing upper limb reaching tasks, using less elbow extension and supination, and utilising increased compensatory trunk forward flexion. Post BTX-A, the time to complete upper limb tasks did not change (p> 0.15). However, at least six subjects had increased elbow extension (average 17 degrees) and decreased trunk forward flexion (average 16 degrees) during upper limb reaching tasks. Five subjects improved their Melbourne Assessment score by 5% or greater.

Decreased tone and individual improvements in upper limb functional tasks were seen post BTX-A and therapy. However deficits in timing of upper limb movements did not change post BTX-A.

Objective: To report the development of acquired atlan-toaxial instability in pediatric patients with spastic cerebral palsy and no previous history of traumatic incident.

Study Design-Material: We present three patients with severe spastic quadriplegia who developed C1–C2 instability and cervical myelopathy at mean age 12.6 years.

Results: These patients demonstrated similar clinical picture with symptoms attributed to cervical myelopathy in varied severity including apneic episodes, opisthotonus, alteration in muscle tone, torticollis, respiratory problems, hypereflexia and bradycardia. Patient 1 was scheduled for surgery but died due to an apneic episode. Patient 2 denied surgery and has been followed for 3 years while his neurological condition remains unchanged. Patient 3 underwent decompression through a C1 laminectomy and occiput-C4 posterior spinal fusion, recovered neurologically and resumed his previous functional skills.

Conclusions: The cases included in our study serve to illustrate the numerous difficulties related to the diagnosis and treatment of severely involved children with spastic cerebral palsy complicated with acquired atlan-toaxial instability and myelopathy. A high index of suspicion in patients who demonstrate a significant functional deterioration or a sudden change in their previous neurological status may lead to the diagnosis of cervical myelopathy at an early stage, when the possibility of recovery is higher. The management of spastic patients with documented cervical instability and myelopathy remains controversial, mainly due to the complexity of the medical problems that affect the general condition of these patients. We believe that even in severely involved pediatric patients appropriate management, including spinal cord decompression and fusion, may alter the natural history of myelopathy if the treatment is performed when the neurologic damage is not long standing. If the etiology of myelopathy is not addressed it may result to fatal complications, although clinical progression is not documented in all patients.

Objective: The aim of this study was to document rate of survival among 288 severely affected pediatric patients with spasticity and neuromuscular scoliosis who underwent spinal fusion and to identify exposure variables that could significantly predict survival times.

Methods: Kaplan-Meier survivorship analysis was performed and Cox’s proportional hazards model was used to evaluate predictive efficacy of exposure variables such as gender, age at surgery, level of ambulation, mental ability, degree of coronal and sagittal plane spinal deformity, intraoperative blood loss, surgical time, days in the hospital, and days in the intensive care unit (ICU).

Results: The statistical analysis demonstrated a mean predicted survival of 134.3 months (11.2 years) after surgical correction of spinal deformities for this group of globally involved children with cerebral palsy. The number of days in the ICU after surgery and the presence of severe preoperative thoracic hyperkyphosis were the only factors affecting survival rates. ICU stay of greater than five days, which was usually associated with respiratory problems, substantially increasing the risk of death. Thoracic hyperkyphosis of greater than 70o caused a considerable increase in the predicted mortality rate.

Conclusions: Our study demonstrated a relatively long mean predicted survivorship for pediatric patients with severe spastic cerebral palsy and neuromuscular scoliosis who underwent spinal surgery, which is consistent with the current concept of increased life expectancy even for the total-body involved patients. The most accurate determinants for survival rates among this population group were the number of days the patient had to spend postoperatively in the intensive care unit, and the presence of excessive preoperative thoracic hyperkyphosis.

Objective: To evaluate the outcomes and complications of one-stage and two-stage combined anterior-posterior spine fusion, and to document which procedure is more efficacious and provides better results.

Study Design-Material: A retrospective study was performed including 45 pediatric patients with spastic quadriplegic cerebral palsy and neuromuscular scoliosis who underwent anterior-posterior spinal fusion.

Summary of Background Data: Circumferential spinal arthrodesis has been proven to achieve better scoliotic curve correction, decreasing significantly the risk of pseudarthrosis and progression of the deformity. There have been a few studies comparing same-day versus staged anterior-posterior spinal surgery in mixed populations with neuromuscular scoliosis, but not in an isolated group of pediatric patients with spastic cerebral palsy.

Methods: The medical records and radiographs of all patients were reviewed, and the results were statistically analyzed. The complications were divided into medical, subcategorized into major and minor, and technical.

Results: There was no statistically significant difference (p> .05) between one-stage (group 1) and two-stage (group 2) patients, considering age at surgery, preoperative scoliosis angle, pelvic obliquity, kyphosis angle, lordosis angle, levels of anterior release, percentage of scoliosis correction, radiographic follow-up, hospitalization time and intensive care unit stay. Sequentially performed spinal procedures (group 1) were associated with increased intraoperative blood loss, prolonged operative time, and a considerably higher incidence of medical and technical complications, including two perioperative deaths.

Conclusions: Two-stage anteroposterior spinal fusion provides safer and more consistent results with several advantages over the single-stage procedure in the management of patients with cerebral palsy and neuromuscular scoliosis. Since this study cannot separate risk based on disease severity, staging the procedures on different days is likely more important in individuals with very large curves and concomitant medical illness.

Objective: To evaluate the outcome of spinal fusion with unit rod in pediatric patients with cerebral palsy who were treated by the two senior authors using the same operative technique.

Study Design-Methods: This is a retrospective study of 288 patients with mean age at surgery 13.9 years (SD: 3.26), whose medical charts and radiographs were reviewed. A questionnaire including 14 questions assessing patients’ functional improvement was given to the caretakers.

Results: Mean radiographic follow up of 3.2 years (range: 1–9.9) was available in 213 patients. In 46 patients anterior-posterior fusion was performed and in 242 only posterior. The preop. Cobb angle was 74°(range: 6–176°) corrected by 68% to 23° (p< .01) and increased by 2° in the last follow-up (p< .01). The preop. pelvic obliquity was 17°(range: 0–57°), corrected by 73% to 4.7° (p< .01) and increased by 0.4° at follow-up. The preop. kyphosis angle was 56°(range: −44–130°), corrected by 37% to 35° (p< .01) and increased by 2° at follow-up. The preop. lordosis angle was 38°(range: −50–140°), corrected by 14% to 43° (p< .05) and increased by 1.4° at follow-up. There was a strong correlation between hyperlordosis and days of hospitalization, blood loss and surgical time (r= −.22, .23, .24). Patients with lordotic angle > 60° developed 15.1% technical problems related to pelvic fixation, whereas only 3.4% of those with < 60°. The mean days of hospitalization were 19.6, the mean blood loss 2.9lt (1.2BV), the mean surgical time 4.4h (Anterior-Posterior: 7h, Posterior only: 3.9h) and the mean ICU stay 5 days. There was a difference only in surgical time (p< .01) if an anterior procedure was required. The major complications included 3 perioperative deaths, 14 deep infections, 13 (4.5%) reoperations for mechanical problems and no detected pseudarthrosis. The answers received by the caretakers were 99% positive, emphasizing the improvement in child’s appearance, sitting ability and respiratory function.

Conclusions: Spine surgery in patients with cerebral palsy was accompanied by a considerable blood loss and multiple medical complications. A very satisfactory correction of spinal curvatures was achieved and maintained in follow up. Excessive lumbar lordosis was associated with a high incidence of technical problems and an increased morbidity. There were no pseudarthrosis and the overall number of reoperations for technical reasons was very low. The caretakers were extremely pleased with the outcome of this procedure. Unit rod is a very effective instrumentation system, providing excellent results and a low mechanical complication rate in the treatment of cerebral palsy patients with spinal deformities.

Objective: The aim of this study was to delineate parents’ and professional caretakers’ satisfaction after spinal fusion in children with spasticity, and to determine differences in their perceptions.

Study Design-Methods: A questionnaire assessing patients’ functional improvement after spinal arthrodesis for correcting scoliosis was addressed to 190 parents. An expanded questionnaire was also addressed to 122 educators and therapists working exclusively in the care of children with cerebral palsy.

Results: The vast majority of parents and caregivers reported a very positive impact of the surgery on patients’ overall function, quality of life, and ease of care. Caretakers did not recognize effects of the scoliotic deformity on patients’ head control, hand use, and feeding ability. Parents had more appreciation of the benefits in the children’s appearance, while educators and therapists acknowledged more improvement in gross and oral motor function. Most parents (95.8%) and caretakers (84.3%) would recommend spinal surgery, considering that the benefits from scoliosis correction clearly outweigh the increased risk of surgical complications in this group of severely involved patients.

Conclusions: Correction of spinal deformity in children with cerebral palsy is associated with a very high parents’ and caretakers’ satisfaction rate, with both groups reporting excellent improvement in appearance, comfort, ease of care, and some functional gains. The answers received during the survey demonstrated clearly that the vast majority of parents and caretakers consider the surgical outcome of the spinal fusion very beneficial for the patients’ overall function and level of care. There were documented differences in the perceived positive impact of spine surgery in children with cerebral palsy between the two groups. Parents stated more consistent benefits from correcting scoliosis in the patients’ appearance, whereas therapists and educators delineated the improvement in the gross and oral motor function. However, both groups considered that the benefits from correcting scoliotic deformity undoubtedly offset the potential risks from surgery, and would have no hesitation in recommending this procedure for children with cerebral palsy who develop significant scoliosis interfering with their level of function and their overall quality of life.

Objective: To evaluate the effect of spinal fusion from T1, T2 to the sacrum with pelvic fixation using unit rod instrumentation on the ambulatory potential of pediatric patients with cerebral palsy.

Study Design: A retrospective study of 24 ambulatory pediatric patients with spastic cerebral palsy and neuro-muscular scoliosis was performed.

Summary of Background Data: Spinal deformities, occurring in patients with cerebral palsy and good ambulatory capacity, are infrequently associated with pelvic obliquity, and so instrumented spinal fusions traditionally do not extend to the pelvis.

Methods: The medical charts and radiographs were reviewed, and the patients’ ambulatory ability was assessed clinically, with video tape or complete gait analysis. A questionnaire assessing patients’ functional improvement was given to the caretakers.

Results: The study group included 17 female and 7 male patients, 19 quadriplegics and 5 diplegics. Follow-up evaluations for ambulatory function occurred at a mean of 2.86 years after surgery. Mean age at surgery was 15.4 years. Twenty patients underwent posterior spinal fusions and 4 patients combined anterior-posterior procedures. The patients were evaluated clinically pre-operatively, postoperatively and at follow-up with no alteration in their ambulatory status, except one patient who developed bilateral hip heterotopic ossification and gradually lost her ability to ambulate. Thirteen patients had both preoperative and postoperative gait analysis, showing no change in their ambulatory function. The surgical outcome survey demonstrated significant improvement in the child’s physical appearance, head and trunk balance, sitting ability, amount of back pain, respiration, and no change in ambulatory capacity.

Conclusions: Spine surgery with fusion extending to the pelvis in ambulatory patients with cerebral palsy provided excellent deformity correction and preserved their ambulatory function.

Objective: To evaluate the outcomes and complications of one-stage and two-stage combined anterior-posterior spine fusion, and to document which procedure is more efficacious and provides better results.

Study Design-Material: A retrospective study was performed including 45 pediatric patients with spastic quadriplegic cerebral palsy and neuromuscular scoliosis who underwent anterior-posterior spinal fusion.

Summary of Background Data: Circumferential spinal arthrodesis has been proven to achieve better scoliotic curve correction, decreasing significantly the risk of pseudarthrosis and progression of the deformity. There have been a few studies comparing same-day versus staged anterior-posterior spinal surgery in mixed populations with neuromuscular scoliosis, but not in an isolated group of pediatric patients with spastic cerebral palsy.

Methods: The medical records and radiographs of all patients were reviewed, and the results were statistically analyzed. The complications were divided into medical, subcategorized into major and minor, and technical.

Results: There was no statistically significant difference (p> .05) between one-stage (group 1) and two-stage (group 2) patients, considering age at surgery, preoperative scoliosis angle, pelvic obliquity, kyphosis angle, lordosis angle, levels of anterior release, percentage of scoliosis correction, radiographic follow-up, hospitalization time and intensive care unit stay. Sequentially performed spinal procedures (group 1) were associated with increased intraoperative blood loss, prolonged operative time, and a considerably higher incidence of medical and technical complications, including two perioperative deaths.

Conclusions: Two-stage anteroposterior spinal fusion provides safer and more consistent results with several advantages over the single-stage procedure in the management of patients with cerebral palsy and neuromuscular scoliosis. Since this study cannot separate risk based on disease severity, staging the procedures on different days is likely more important in individuals with very large curves and concomitant medical illness.

Objective: The aim of this study was to document rate of survival among 288 severely affected pediatric patients with spasticity and neuromuscular scoliosis who underwent spinal fusion and to identify exposure variables that could significantly predict survival times.

Methods: Kaplan-Meier survivorship analysis was performed and Cox’s proportional hazards model was used to evaluate predictive efficacy of exposure variables such as gender, age at surgery, level of ambulation, mental ability, degree of coronal and sagittal plane spinal deformity, intraoperative blood loss, surgical time, days in the hospital, and days in the intensive care unit (ICU).

Results: The statistical analysis demonstrated a mean predicted survival of 134.3 months (11.2 years) after surgical correction of spinal deformities for this group of globally involved children with cerebral palsy. The number of days in the ICU after surgery and the presence of severe pre-operative thoracic hyperkyphosis were the only factors affecting survival rates. ICU stay of greater than five days, which was usually associated with respiratory problems, substantially increasing the risk of death. Thoracic hyperkyphosis of greater than 70° caused a considerable increase in the predicted mortality rate.

Conclusions: Our study demonstrated a relatively long mean predicted survivorship for pediatric patients with severe spastic cerebral palsy and neuromuscular scoliosis who underwent spinal surgery, which is consistent with the current concept of increased life expectancy even for the total-body involved patients. The most accurate determinants for survival rates among this population group were the number of days the patient had to spend postoperatively in the intensive care unit, and the presence of excessive preoperative thoracic hyperkyphosis.

Objective: To evaluate the effect of spinal fusion from T1, T2 to the sacrum with pelvic fixation using unit rod instrumentation on the ambulatory potential of pediatric patients with cerebral palsy.

Study Design: A retrospective study of 24 ambulatory pediatric patients with spastic cerebral palsy and neuromuscular scoliosis was performed.

Summary of Background Data: Spinal deformities, occurring in patients with cerebral palsy and good ambulatory capacity, are infrequently associated with pelvic obliquity, and so instrumented spinal fusions traditionally do not extend to the pelvis.

Methods: The medical charts and radiographs were reviewed, and the patients’ ambulatory ability was assessed clinically, with video tape or complete gait analysis. A questionnaire assessing patients` functional improvement was given to the caretakers.

Results: The study group included 17 female and 7 male patients, 19 quadriplegics and 5 diplegics. Follow-up evaluations for ambulatory function occurred at a mean of 2.86 years after surgery. Mean age at surgery was 15.4 years. Twenty patients underwent posterior spinal fusions and 4 patients combined anterior-posterior procedures. The patients were evaluated clinically pre-operatively, postoperatively and at follow-up with no alteration in their ambulatory status, except one patient who developed bilateral hip heterotopic ossification and gradually lost her ability to ambulate. Thirteen patients had both preoperative and postoperative gait analysis, showing no change in their ambulatory function. The surgical outcome survey demonstrated significant improvement in the child’s physical appearance, head and trunk balance, sitting ability, amount of back pain, respiration, and no change in ambulatory capacity.

Conclusions: Spine surgery with fusion extending to the pelvis in ambulatory patients with cerebral palsy provided excellent deformity correction and preserved their ambulatory function.

Objective: To evaluate the outcome of spinal fusion with unit rod in pediatric patients with cerebral palsy who were treated by the two senior authors using the same operative technique.

Study Design-Methods: This is a retrospective study of 288 patients with mean age at surgery 13.9 years (SD: 3.26), whose medical charts and radiographs were reviewed. A questionnaire including 14 questions assessing patients’ functional improvement was given to the caretakers.

Results: Mean radiographic follow up of 3.2 years (range: 1–9.9) was available in 213 patients. In 46 patients anterior-posterior fusion was performed and in 242 only posterior. The preop. Cobb angle was 740(range: 6–176o) corrected by 68% to 230 (p< .01) and increased by 20 in the last follow-up (p< .01). The preop. pelvic obliquity was 170(range: 0–57o), corrected by 73% to 4.70 (p< .01) and increased by 0.40 at follow-up. The preop. kyphosis angle was 560(range: −44–130o), corrected by 37% to 350 (p< .01) and increased by 20 at follow-up. The preop. lordosis angle was 380(range: −50–140o), corrected by 14% to 430 (p< .05) and increased by 1.40 at follow-up. There was a strong correlation between hyperlordosis and days of hospitalization, blood loss and surgical time (r= −.22,. 23,. 24). Patients with lordotic angle > 600 developed 15.1% technical problems related to pelvic fixation, whereas only 3.4% of those with < 600. The mean days of hospitalization were 19.6, the mean blood loss 2.9lt (1.2BV), the mean surgical time 4.4h (Anterior-Posterior: 7h, Posterior only: 3.9h) and the mean ICU stay 5 days. There was a difference only in surgical time (p< .01) if an anterior procedure was required. The major complications included 3 perioperative deaths, 14 deep infections, 13 (4.5%) reoperations for mechanical problems and no detected pseudarthrosis. The answers received by the caretakers were 99% positive, emphasizing the improvement in child’s appearance, sitting ability and respiratory function.

Conclusions: Spine surgery in patients with cerebral palsy was accompanied by a considerable blood loss and multiple medical complications. A very satisfactory correction of spinal curvatures was achieved and maintained in follow up. Excessive lumbar lordosis was associated with a high incidence of technical problems and an increased morbidity. There were no pseudarthrosis and the overall number of reoperations for technical reasons was very low. The caretakers were extremely pleased with the outcome of this procedure. Unit rod is a very effective instrumentation system, providing excellent results and a low mechanical complication rate in the treatment of cerebral palsy patients with spinal deformities.

Objective: To identify the demographics, risk factors and guidelines for treatment in a population of cerebral palsy (CP) children with fractures.

Materials and Methods: One hundred and fifty children with CP received treatment for fractures from 1948 through 2000. To identify changes in demographics, patients treated before 1992 were compared to those treated after 1992. The latter group was matched by age and gender to a group of CP children without fractures and compared for: CP pattern, bone density, ambulatory status, presence of G-tube, weight for height-age Z score, surgical procedures during the previous year, presence of contractures in the proximity of the fracture and seizure medications. All data was analyzed statistically.

Results: 67% of patients had a spastic quadriplegic pattern of CP and 89% were non-ambulatory; 78% of fractures occurred in lower limbs, 48% of the fractures were delayed in diagnosis, and 62% of patients showed osteo-penia. Children treated after 1992 had higher incidence of multiple fractures, less contractures, and less surgical procedures. The risk factors identified for increased fracture risk were: the use of seizure medication (p=.001), quadriplegic CP pattern (p=.005), decreased ambulatory status (p=.001), and lower bone density (p=.001). Most fractures were treated with soft bulky dressing.

Conclusions: Ambulatory status and the presence of seizure medication are the greatest risk factors for fracture in children with CP. As a consequence, a low bone density occurs. Future research should focus on underlying fracture mechanisms and prevention.

Purpose. The aim of this study was to document rate of survival among 288 severely affected pediatric patients with spasticity and neuromuscular scoliosis who underwent spinal fusion and to identify exposure variables that could significantly predict survival times.

Methods. Kaplan-Meier survivorship analysis was performed and Cox’s proportional hazards model was used to evaluate predictive efficacy of exposure variables such as gender, age at surgery, level of ambulation, mental ability, degree of coronal and sagittal plane spinal deformity, intraoperative blood loss, surgical time, days in the hospital, and days in the intensive care unit (ICU).

Results. The statistical analysis demonstrated a mean predicted survival of 134.3 months (11.2 years) after spine surgery for this group of globally involved children with cerebral palsy (Figure 1). The number of days in the ICU after surgery and the presence of severe preoperative thoracic hyperkyphosis were the only factors affecting survival rates. ICU stay of greater than five days, which was usually associated with respiratory problems, substantially increased the risk of death. Thoracic hyperkyphosis of greater than 70° caused a considerable increase in the predicted mortality rate.

Conclusions. Our study demonstrated a relatively long mean predicted survivorship for children and adolescents with severe spastic cerebral palsy and neuromuscular scoliosis who underwent spine surgery, which is consistent with the current concept of increased life expectancy even for the total-body involved patients.

The most accurate determinants for survival rates among this population group were the number of days the patient had to spend postoperatively in the intensive care unit, and the presence of excessive thoracic hyper-kyphosis.

Purpose: To evaluate the outcome of spinal fusion with unit rod in pediatric patients with cerebral palsy who were treated by the two senior authors using the same operative technique.

Methods: This is a retrospective study of 288 patients with mean age at surgery 13.9 years (SD: 3.26), whose medical charts and radiographs were reviewed. A questionnaire including 14 questions assessing patients’ functional improvement was given to the caretakers.

Results: Mean radiographic follow up of 3.2 years (range: 1-9.9) was available in 213 patients. In 46 patients anterior-posterior fusion was performed and in 242 only posterior. The preop. Cobb angle was 740(range: 6-176°) corrected by 68% to 230 (p< .01) and increased by 20 in the last follow-up (p< .01). The preop. pelvic obliquity was 170(range: 0-57°), corrected by 73% to 4.70 (p< .01) and increased by 0.40 at follow-up. The preop. kyphosis angle was 560(range: -44-130°), corrected by 37% to 350 (p< .01) and increased by 20 at follow-up. The preop. lordosis angle was 380(range: -50-140°), corrected by 14% to 430 (p< .05) and increased by 1.40 at follow-up. There was a strong correlation between hyperlordosis and days of hospitalization, blood loss and surgical time (r= -.22, .23, .24). Patients with lordotic angle > 600 developed 15.1% technical problems related to pelvic fixation, whereas only 3.4% of those with < 600. The mean days of hospitalization were 19.6, the mean blood loss 2.9lt (1.2BV), the mean surgical time 4.4h (Anterior-Posterior: 7h, Posterior only: 3.9h) and the mean ICU stay 5 days. There was a difference only in surgical time (p< .01) if an anterior procedure was required. The major complications included 3 perioperative deaths, 14 deep infections, 13 (4.5%) reop-erations for mechanical problems and no detected pseud-arthrosis. The answers received by the caretakers were 99% positive, emphasizing the improvement in child’s appearance, sitting ability and respiratory function.

Conclusion: Spine surgery in patients with cerebral palsy was accompanied by a considerable blood loss and multiple medical complications. A very satisfactory correction of spinal curvatures was achieved and maintained in follow up. Excessive lumbar lordosis was associated with a high incidence of technical problems and an increased morbidity. There were no pseudarthrosis and the overall number of reoperations for technical reasons was very low. The caretakers were extremely pleased with the outcome of this procedure.