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Orthopaedic Proceedings
Vol. 97-B, Issue SUPP_17 | Pages 6 - 6
1 Dec 2015
Carter T Tsirikos A
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Scheuermann's kyphosis is a structural deformity of the thoracic or thoracolumbar spine, which can result in severe pain, neurological compromise and cosmetic dissatisfaction. Modern surgical techniques have improved correction through a posterior-only or antero-posterior approach but can result in significant morbidity.

We present our results of the surgical management of severe Scheuermann's kyphosis by a single surgeon with respect to deformity correction, global balance parameters, functional outcomes and complications at latest follow-up. We included 49 patients, of which 46 had thoracic and 3 had thoracolumbar kyphosis. Surgical indications included persistent back pain, progressive deformity, neurological compromise and poor self-image. Fourty-seven patients underwent posterior-only and 2 antero-posterior spinal arthrodesis utilising Chevron-type osteotomies and hybrid instrumentation. Mean age at surgery was 16.0 years with mean postoperative follow-up of 4.5 years. Mean kyphosis corrected from 92.1o to 46.9o (p<0.001). Concomitant scoliosis was eliminated in all of the 28 affected patients. Coronal and sagittal balance was corrected in all patients. Mean blood loss was 24% total blood volume. Mean operation time was 4.3 hours with mean inpatient stay of 9 days. SRS-22 questionnaire improved from a mean preoperative score of 3.4 to 4.6 at 2 years, with high treatment satisfaction rates. Complications included one toxic septicaemia episode but otherwise no wound infections, no junctional deformity, no loss of correction and no requirement for re-operation.

Posterior spinal arthrodesis with the use of hybrid instrumentation can safely achieve excellent correction of severe Scheuermann's kyphosis helping to relieve back pain, improve functional outcomes and enhance self-image.


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_15 | Pages 11 - 11
1 Oct 2014
Tsirikos A Hathorn C Fall A McGurk S Urquhart D
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There are limited data on scoliosis in cystic fibrosis (CF), and the two most recent studies came to opposite conclusions. Reported prevalence ranges from 2% (within the normal range for the general population) to 15.5%. We felt that a recent study under-estimated the prevalence due to a very young population (mean age 10.9 years), since scoliosis develops most commonly in adolescents. We hypothesised that scoliosis is more prevalent in adolescents with CF compared to the general population. The aim of our study was to determine the incidence of scoliosis in adolescents with CF followed to and beyond skeletal maturity and describe the type of spinal deformity. We included all patients in our CF clinic aged >10 years, and those who have transitioned to adult services in the last 10 years. Patients with a co-existent neuromuscular condition were excluded.

We conducted a retrospective observational study. Most recent chest radiographs at end of spinal growth, or those taken at transition to adult services, were reviewed by a Consultant Radiologist and a Consultant Spine Surgeon. Scoliosis was defined as a Cobb angle of >10° in the coronal plane. Demographics and characteristics of the curves were recorded.

Our cohort included 143 CF patients (48% male) with a mean age at the time of chest radiograph of 18 years (range 15–22 years). 16 (6 male) subjects were noted to have scoliosis with a mean (range) Cobb angle of 14° (10–38°) giving a prevalence of 11%. 13 were single thoracic curves, 2 double and 1 triple. The majority were non-progressive short mid-thoracic curves, convex to the right. 5 curves were progressive, only one of which was significant and required bracing to the end of growth but no surgical treatment.

We found a prevalence of scoliosis in our adolescent CF population that is significantly greater than the general population. Only one curve was significant and progressive requiring bracing, the remainder being minor and non-progressive. A strength of our study is that all patients had achieved skeletal maturity at the time of latest X-ray and, therefore, development or further progression of scoliosis is unlikely. The negative effect of scoliosis on lung function is well-documented. With the progressive nature of CF lung disease, scoliosis may have further deleterious effects. Bone disease is increasingly recognised in CF patients, with osteopenia and osteoporosis occurring earlier and more frequently than in the general population (38% & 24% respectively in 18–32 year old CF patients). To date, studies have failed to show a correlation between scoliosis, lung function and bone mineral density. The paradigm of a radiologically significant (Cobb angle >10°) versus a clinically important scoliosis remains.


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The SRS-22 instrument was developed to assess quality of life in adolescent idiopathic scoliosis patients (AIS). The aim of our study was to evaluate the impact of surgical treatment on quality of life in neurologically normal patients with complex spinal deformity of a range of aetiologies using the SRS-22 questionnaire.

We analysed prospectively collected SRS-22 data on 407 consecutive patients (315 females-92 males) treated from 2006–2012 under the care of the senior author. 97.6% of patients had a posterior (PSF), 1.3% an anterior (ASF) and 1.1% an anterior-posterior (A/PSF) spinal fusion. Questionnaires were completed at 4 stages of treatment: preoperatively, 6-months, 12-months and 24-months postoperatively. Other variables included age at surgery, gender, diagnosis and year of surgery. 9 diagnoses were included in the study: 271 patients had AIS; 39 Scheuermann's kyphosis; 31 spondylolisthesis; 16 congenital scoliosis; 13 JIS; 6 IIS; 13 scoliosis associated with intraspinal anomalies; 11 syndromic scoliosis; 7 scoliosis associated with congenital cardiac disease. Mean age at surgery was 15.14±2.07 years. Age at surgery was divided in 3 groups to compare outcomes against previous studies: 10–12, 13–15, and 15–19 years.

Mean total SRS-22 scores for the whole group were: preoperative 3.62±0.66; 6-month 4.12±0.44; 12-month 4.39±0.40; and 24-month 4.52±0.37 (p<0.0001). Individual preoperative scores for the whole group were: function 3.77±0.75; pain 3.7±0.97; self-image 3.14±0.66; mental 3.86±0.77. Mean 24-month postoperative scores for the whole group were: function 4.39±0.42; pain 4.59±0.56; self-image 4.39±0.51; mental 4.43±0.56; satisfaction 4.81±0.40. All changes observed at 2-year follow-up were statistically significant (p<0.0001). Male preoperative total score (3.64±0.66) did not have a significant difference compared to female (3.62±0.66); however 24-month total score improvement was significant, with males (4.62±0.25) performing better than females (4.49±0.39) including satisfaction (p=0.004). Spondylolisthesis patients performed worse preoperatively (2.93±0.26) compared to other diagnoses (p<0.0001); AIS 3.67±0.64, congenital 3.81±0.57, syndromic 3.80±0.54, Scheuermann's 3.48±0.75, JIS 3.90±0.63, scoliosis with congenital cardiac disease 4.04±0.41 or intraspinal anomalies 3.71±0.66, and IIS 3.58±0.80. No change in total and individual scores was observed at 24-month review across diagnoses (p>0.05). There were no significant changes between each year of surgery in the study period and the total values at each stage (p>0.05). There were no significant changes between the 3 age ranges at each stage (p>0.05). Patients undergoing ASF performed significantly better at preoperative function (4.7±0.27) compared to PSF (3.76±0.74) and A/PSF (3.68±0.72), (p=0.19). All values at 2-years were not significant in comparison regarding type of operation (p>0.05).

All individual domains and total SRS-22 scores improved after surgical correction of spinal deformity in our young patient cohort with an incremental change between preoperative, 6-month, 12-month and 24-month postoperative. Our 24-month postoperative outcomes in all diagnoses compare favourably to reported SRS-22 scores in healthy adolescents (function: 4.31±0.54; pain: 4.44±0.67; image: 4.41±0.64; mental health: 3.96±0.81; total 4.26±0.54). We are planning to continue this study with longer follow-up in order to allow comparison of our SRS-22 outcomes after surgical treatment to those obtained in patients treated with bracing or patients who were followed untreated (previous reports SRS-22 total scores: 4.2 for braced patients and 4.1 for observed patients).


To present the results of surgical correction in patients with double or triple thoracic/lumbar AIS (Lenke types 2,3,4) with the use of a novel convex/convex unilateral segmental screw correction technique in a single surgeon's prospective series.

We reviewed the medical records and spinal radiographs of 92 consecutive patients (72 female-20 male). We measured scoliosis, thoracic kyphosis, lumbar lordosis, scoliosis flexibility and correction index, coronal and sagittal balance before and after surgery, as well as at minimum 2-year follow-up. SRS-22 data was available preoperatively, 6-month, 12-month and 2-year postoperatively for all patients. Surgical technique. All patients underwent posterior spinal fusion using pedicle screw constructs. Unilateral screws were placed across the convexity of each individual thoracic or lumbar curve to allow for segmental correction. ‘Corrective rod’ was the one attached to the convexity of each curve with the correction performed across the main thoracic scoliosis always before the lumbar. Maximum correction of main thoracic curves was always performed, whereas the lumbar scoliosis was corrected to the degree required to achieve a balanced effect across the thoracic and lumbar segments and adequate global coronal spinal balance. Concave screws were not placed across any deformity levels. Bilateral screws across 2 levels caudally and 1–2 levels cephalad provided proximal/distal stability of the construct.

Mean age at surgery was 14.9 years with mean Risser grade 2.8. The distribution of scoliosis was: Lenke type 2–26 patients; type 3–43 patients; type 4–23 patients. Mean preoperative Cobb angle for upper thoracic curves was 45°. This was corrected by 62% to mean 17° (p<0.001). Mean preoperative Cobb angle for main thoracic curves was 70°. This was corrected by 69% to mean 22° (p<0.001). Mean preoperative Cobb angle for lumbar curves was 56°. This was corrected by 68% to mean 18° (p<0.001). No patient lost >2° correction at follow-up. Mean preoperative thoracic kyphosis was 34° and lumbar lordosis 46°. Mean postoperative thoracic kyphosis was 45° (p<0.001) and lumbar lordosis 46.5° (p=0.69). Mean preoperative coronal imbalance was 1.2 cm. This corrected to mean 0.02 cm at follow-up (p<0.001). Mean preoperative sagittal imbalance was −2 cm. This corrected to mean −0.1 cm at follow-up (p<0.001). Mean theatre time was 187 minutes, hospital stay 6.8 days and intraoperative blood loss 0.29 blood volumes (1100 ml). Intraoperative spinal cord monitoring was performed recording cortical and cervical SSEPs and transcranial upper/lower limb MEPs and there were no problems. None of the patients developed neurological complications, infection or detected non-union and none required revision surgery to address residual or recurrent deformity. Mean preoperative SRS-22 score was 3.6; this improved to 4.6 at follow-up (p<0.001). All individual parameters also demonstrated significant improvement (p<0.001) with mean satisfaction rate at 2-year follow-up 4.9.

The convex-convex unilateral pedicle screw technique can reduce the risk of neurological injury during major deformity surgery as it does not require placement of screws across the deformed apical concave pedicles which are in close proximity to the spinal cord. Despite the use of a lesser number of pedicle fixation points compared to the bilateral segmental screw techniques, in our series it has achieved satisfactory scoliosis correction and restoration of global coronal and sagittal balance with improved thoracic kyphosis and preserved lumbar lordosis. These results have been associated with excellent patient satisfaction and functional outcomes as demonstrated through the SRS-22 scores.


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_6 | Pages 31 - 31
1 Apr 2014
Keenan A Henderson L Michaelson C Tsirikos A
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Aim:

To present the results of multi-modal IOM in 298 patients who underwent spinal deformity correction.

Method:

We reviewed the notes, surgical and IOM charts of all patients who underwent spinal surgery with the use of cortical and cervical SSEPs, as well as upper/lower limb transcranial electrical MEPs under the senior author. We recorded IOM events which we categorised as true, transient true and false (+) or (−). We correlated the IOM events with surgical or anaesthetic incidents.


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_6 | Pages 6 - 6
1 Apr 2014
Urquhart D Gallella S Brady E Blacklock S Tsirikos A
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Background:

Long-Term Follow-Up Of Lung Function And Quality Of Life (Qol) In Those Undergoing Combined Anterior And Posterior Spinal Fusion (A/Psf) For Ais Is Not Well Documented With Only One Study Extending Beyond 2 Years.

Aim:

To Evaluate Long-Term Change In Pulmonary Function And Qol Following A/Psf For Ais.


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_6 | Pages 25 - 25
1 Apr 2014
Duckworth A Mitchell M Tsirikos A
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Aim:

Our aim was to report the rate and risk factors for post-operative complications in Duchenne Muscular Dystrophy (DMD) patients undergoing spinal arthrodesis for scoliosis, with a comparison to neuromuscular scoliosis of other aetiology.

Methods:

From a prospective single surgeon spinal deformity database, we identified all patients with neuromuscular disorders who underwent surgical correction for progressive scoliosis. We recorded demographic and peri-operative data, including complications and subsequent procedures. The rate and risk factors for complications was determined, with a sub-analysis of the DMD group to determine any peri-operative factors predictive of overall complication rates.


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_6 | Pages 26 - 26
1 Apr 2014
Karampalis C Tsirikos A
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Aim:

To present 11 patients with quadriplegia who developed severe lordoscoliosis or hyperlordosis. This is a rare deformity in children with CP, treatment is challenging and there are less than 20 patients ever reported.

Method:

All patients underwent posterior spinal arthrodesis at mean age 14.6 years with mean follow-up 3.5 years. We measured all radiographic parameters including coronal and sagittal balance and sacral slope before and after surgery.


Aim:

To determine radiographic variables that predict the need for distal extension of the fusion beyond Cobb-to-Cobb levels in treating thoracolumbar/lumbar (TL/L) scoliosis (Lenke 5) in adolescent patients.

Method:

We reviewed the medical notes and radiographs of the senior author's consecutive series of 53 adolescent patients with TL/L scoliosis treated by posterior instrumented spinal arthrodesis using an all-pedicle screw construct. Our patients were categorised into 2 groups: patients with instrumented fusion between Cobb-to-Cobb levels of the TL/L curve (Group 1), and patients that required distal extension beyond the caudal Cobb level (Group 2). Pearson correlation and binary logistic regression analyses (significance p<0.05) were performed to identify variables that predict the need for distal extension.


Lowest instrumented vertebra (LIV) selection is critical to preventing complications following posterior spinal arthrodesis (PSA) for thoracolumbar/lumbar adolescent idiopathic scoliosis (TL/L AIS), but evidence guiding LIV selection is limited.

This study aimed to investigate the efficacy of PSA using novel unilateral convex segmental pedicle screw instrumentation (UCS) in correcting TL/L AIS, to identify radiographic parameters correlating with distal extension of PSA, and to develop a predictive equation for distal fusion extension using these parameters.

We reviewed data (demographic, clinical, radiographic, and SRS-22 questionnaires) preoperatively to 2-years' follow-up for TL/L AIS patients treated by PSA using UCS between 2006 to 2011. 53 patients were included and divided into 2 groups: Group-1 (n=36) patients had PSA between Cobb-to-Cobb levels; Group-2 (n=17) patients required distal fusion extension.

A mean curve correction of 80% was achieved. Mean postoperative LIV angle, TL/L apical vertebra translation (AVT), and trunk shift were lower than previous studies. Six preoperative radiographic parameters significantly differed between groups and correlated with distal fusion extension: thoracic curve size, thoracolumbar curve size, LIVA, AVT, lumbar flexibility index, and Cobb angle on lumbar convex bending. Regression analysis optimised an equation (incorporating the first five parameters) which is 81% accurate in predicting Cobb-to-Cobb fusion or distal extension. SRS-22 scores were similar between groups.

We conclude that TL/L AIS is effectively treated by PSA using UCS, six radiographic parameters correlate with distal fusion extension, and a predictive equation incorporating these parameters reliably informs LIV selection and the need for fusion extension beyond the caudal Cobb level.


Orthopaedic Proceedings
Vol. 88-B, Issue SUPP_II | Pages 231 - 231
1 May 2006
Tsirikos A McMaster M
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Background: Congenital anomalies of the chest wall and Sprengel’s shoulder are often associated with congenital deformities of the spine. It has been suggested that extensive rib fusions on the concavity of a congenital scoliosis may adversely affect progression of the spine deformity, thoracic function and growth of the lungs, which can lead to a thoracic insufficiency syndrome.

Methods: This is a retrospective study of the medical records and spine radiographs of 620 consecutive patients with congenital spine deformities. The rib anomalies were classified into simple and complex and the presence of a Sprengel’s shoulder was recorded.

Subjects: There were 497 patients (80%) with scoliosis, 88 patients (14%) with kyphoscoliosis, and 35 patients with kyphosis (6%). The mean rate of scoliosis deterioration without treatment, age and curve size at surgery was compared for the different types of vertebral abnormalities in patients with and without rib anomalies.

Results: Rib anomalies occurred in 124 patients. The rib anomalies were simple in 97 patients (78%) and complex in 27 (22%). The most common simple rib anomaly (70 patients; 72%) was a fusion of two or three ribs. The most common complex rib anomaly (20 patients; 74%) was a fusion of multiple ribs associated with a large chest wall defect. Rib anomalies were most commonly associated with a congenital scoliosis (116 patients; 93.6%), and much less frequently with a congenital kyphoscoliosis or kyphosis (8 patients). In those patients with a scoliosis, the rib anomalies were simple in 91 patients (78.5%) and complex in 25 patients (21.5%). Eighty-eight of the 124 patients (76%) with rib abnormalities had a scoliosis due to a unilateral unsegmented bar with or without contra-lateral hemivertebrae at the same level, and 8 patients had mixed unclassifiable vertebral anomalies. In contrast, only 17 patients with a scoliosis and rib anomalies had hemivertebrae alone. The rib anomalies were most frequently associated with a thoracic or thoracolumbar scoliosis (107 patients; 92.2%) and occurred on the concavity in 81 patients (70%), convexity in 27 patients (23%), and were bilateral in 8 patients. Sprengel’s deformity occurred in 45 patients; 43 patients with congenital scoliosis (8.6%), and 2 with kyphoscoliosis or kyphosis. A Sprengel’s shoulder occurred most frequently in association with a thoracic scoliosis due to a unilateral failure of vertebral segmentation (28 patients). The elevated shoulder was on the concavity of scoliosis in 26 patients and on the convexity in 16 patients. We compared the mean rate of curve progression without treatment, the age and curve size at spine surgery for the different types of spine deformities in patients with and without rib anomalies either simple or complex and found no significant difference (p> 0.05); the only statistical difference was that the mean age at surgery was higher for patients with a unilateral unsegmented bar without rib anomalies (p=0.005).

Conclusion: Congenital rib anomalies and Sprengel’s shoulder occur most commonly on the concavity of a thoracic or thoracolumbar congenital scoliosis due to a unilateral failure of vertebral segmentation. However, these anomalies do not have an adverse effect on curve size or rate of progression.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 182 - 182
1 Feb 2004
Tsirikos A Chang W Shah S Miller F
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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.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 182 - 182
1 Feb 2004
Tsirikos A Chang W Dabney K Miller F Glutting J
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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.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 117 - 118
1 Feb 2004
Tsirikos A Chang W Dabney K Miller F
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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.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 181 - 181
1 Feb 2004
Tsirikos A Carr L Noordeen H
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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.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 118 - 118
1 Feb 2004
Tsirikos A Chang W Dabney K Miller F
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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.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 120 - 120
1 Feb 2004
Huynh T Tsirikos A Tucker S
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Objective: To compare the surgical outcome of patients with right thoracic adolescent idiopathic scoliosis undergoing: 1). Single-stage anterior spinal release and posterior instrumented fusion with costoplasty, 2). Staged anterior spinal release and posterior instrumented fusion with costoplasty.

Study Design-Material: A retrospective study was performed including 20 consecutive patients for each surgical group.

Summary of Background Data: Scoliosis surgery in our institution has evolved over the past decade with respect to the interval between the anterior and posterior procedure. It was typical in the 1980’s/early 1990’s for the patients who required combined anterior-posterior spinal fusion to have 2 weeks between stages. This interval has now been reduced to one week by all our scoliosis surgeons. The authors recently endeavor to perform single-stage anterior-posterior procedures with costoplasty for all right thoracic curves in patients with adolescent idiopathic scoliosis.

Methods: The medical records and radiographs of the 40 patients were reviewed.

Results: The patients in both groups have been assessed for: 1). Correction of spinal deformity achieved with the operation, 2). Incidence of perioperative complications, 3). In-patient stay, 4). Postoperative course. The results were comparative between the 2 groups. Considerable curve correction was obtained with the combined anteroposterior spinal surgery and maintained at follow-up in both groups. No difference in the incidence of complications was recorded between the same-day and the staged groups. Less hospitalization time and time spent in the intensive care unit were required for the patients who underwent same-stage sequentially performed anterior-posterior spinal procedures.

Conclusions: This study showed that single-stage anterior-posterior surgery for right thoracic adolescent idiopathic scoliotic curvatures is a safe and efficacious procedure with no documented adverse effects for the patients. We believe that when a combined anteroposterior spinal approach is necessitated, the one-stage surgery could be considered the procedure of choice for the management of this type of scoliotic deformity.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 181 - 182
1 Feb 2004
Tsirikos A Aderinto J Tucker S Noordeen H
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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.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 182 - 182
1 Feb 2004
Tsirikos A Chang W Dabney K Miller F
Full Access

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.


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 182 - 182
1 Feb 2004
Tsirikos A Chang W Shah S Dabney K Miller F
Full Access

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.