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.

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.

Aim:

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

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:

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.

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.

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.