The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients’ quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction.

Neuromuscular scoliosis patients face rates of major complications of up to 49%. Along with pre-operative risk reduction strategies (including nutritional and bone health optimization), intra-operative strategies to decrease blood loss and decrease surgical time may help mitigate these risks. A major contributor to blood loss and surgical time is the insertion of instrumentation which is challenging in neuromuscular patient given their abnormal vertebral and pelvic anatomy. Standard pre-operative radiographs provide minimal information regarding pedicle diameter, length, blocks to pedicle entry (e.g. iliac crest overhang), or iliac crest orientation. To minimize blood loss and surgical time, we developed an “ultra-low dose” CT protocol without sedation for neuromuscular patients. Our prospective quality improvement study aimed to determine: if ultra-low dose CT without sedation was feasible given the movement disorders in this population; what the radiation exposure was compared to standard pre-operative imaging; whether the images allowed accurate assessment of the anatomy and intra-operative navigation given the ultra-low dose and potential movement during the scan. Fifteen non-ambulatory surgical patients with neuromuscular scoliosis received the standard spine XR and an ultra-low dose CT scan. Charts were reviewed for etiology of neuromuscular scoliosis and medical co-morbidities. The CT protocol was a high-speed, high-pitch, tube-current modulated acquisition at a fixed tube voltage. Adaptive statistical iterative reconstruction was applied to soft-tissue and bone kernels to mitigate noise. Radiation dose was quantified using reported dose indices (computed tomography dose index (CTDIvol) and dose-length product (DLP)) and effective dose (E), calculated through Monte-Carlo simulation. Statistical analysis was completed using a paired student's T-test (α = 0.05). CT image quality was assessed for its use in preoperative planning and intraoperative navigation using 7D Surgical System Spine Module (7D Surgical, Toronto, Canada). Eight males and seven females were included in the study. Their average age (14±2 years old), preoperative Cobb angle (95±21 degrees), and kyphosis (60±18 degrees) were recorded. One patient was unable to undergo the ultra-low dose CT protocol without sedation due to a co-diagnosis of severe autism. The average XR radiation dose was 0.5±0.3 mSv. Variability in radiographic dose was due to a wide range in patient size, positioning (supine, sitting), number of views, imaging technique and body habitus. Associated CT radiation metrics were CTDIvol = 0.46±0.14 mGy, DLP = 26.2±8.1 mGy.cm and E = 0.6±0.2 mSv. CT radiation variability was due to body habitus and arm orientation. The radiation dose differences between radiographic and CT imaging were not statistically significant. All CT scans had adequate quality for preoperative assessment of pedicle diameter and orientation, obstacles impeding pedicle entry, S2-Alar screw orientation, and intra-operative navigation. “Ultra-low dose” CT scans without sedation were feasible in paediatric patients with neuromuscular scoliosis. The effective dose was similar between the standard preoperative spinal XR and “ultra-low dose” CT scans. The “ultra-low dose” CT scan allowed accurate assessment of the anatomy, aided in pre-operative planning, and allowed intra-operative navigation despite the movement disorders in this patient population

Neuromuscular scoliosis patients face rates of major complications of up to 49%. Along with pre-operative risk reduction strategies (including nutritional and bone health optimization), intra-operative strategies to decrease blood loss and decrease surgical time may help mitigate these risks. A major contributor to blood loss and surgical time is the insertion of instrumentation which is challenging in neuromuscular patient given their abnormal vertebral and pelvic anatomy. Standard pre-operative radiographs provide minimal information regarding pedicle diameter, length, blocks to pedicle entry (e.g. iliac crest overhang), or iliac crest orientation. To minimize blood loss and surgical time, we developed an “ultra-low dose” CT protocol without sedation for neuromuscular patients. Our prospective quality improvement study aimed to determine:. if ultra-low dose CT without sedation was feasible given the movement disorders in this population;. what the radiation exposure was compared to standard pre-operative imaging;. whether the images allowed accurate assessment of the anatomy and intra-operative navigation given the ultra-low dose and potential movement during the scan. Fifteen non-ambulatory surgical patients with neuromuscular scoliosis received the standard spine XR and an ultra-low dose CT scan. Charts were reviewed for etiology of neuromuscular scoliosis and medical co-morbidities. The CT protocol was a high-speed, high-pitch, tube-current modulated acquisition at a fixed tube voltage. Adaptive statistical iterative reconstruction was applied to soft-tissue and bone kernels to mitigate noise. Radiation dose was quantified using reported dose indices (computed tomography dose index (CTDIvol) and dose-length product (DLP)) and effective dose (E), calculated through Monte-Carlo simulation. Statistical analysis was completed using a paired student's T-test (α= 0.05). CT image quality was assessed for its use in preoperative planning and intraoperative navigation using 7D Surgical System Spine Module (7D Surgical, Toronto, Canada). Eight males and seven females were included in the study. Their average age (14±2 years old), preoperative Cobb angle (95±21 degrees), and kyphosis (60±18 degrees) were recorded. One patient was unable to undergo the ultra-low dose CT protocol without sedation due to a co-diagnosis of severe autism. The average XR radiation dose was 0.5±0.3 mSv. Variability in radiographic dose was due to a wide range in patient size, positioning (supine, sitting), number of views, imaging technique and body habitus. Associated CT radiation metrics were CTDIvol = 0.46±0.14 mGy, DLP = 26.2±8.1 mGy.cm and E = 0.6±0.2 mSv. CT radiation variability was due to body habitus and arm orientation. The radiation dose differences between radiographic and CT imaging were not statistically significant. All CT scans had adequate quality for preoperative assessment of pedicle diameter and orientation, obstacles impeding pedicle entry, S2-Alar screw orientation, and intra-operative navigation. “Ultra-low dose” CT scans without sedation were feasible in paediatric patients with neuromuscular scoliosis. The effective dose was similar between the standard preoperative spinal XR and “ultra-low dose” CT scans. The “ultra-low dose” CT scan allowed accurate assessment of the anatomy, aided in pre-operative planning, and allowed intra-operative navigation despite the movement disorders in this patient population

Children undergoing posterior spinal fusion (PSF) for neuromuscular and syndromic scoliosis were admitted to the paediatric intensive care (PIC) until about 6 years ago, at which time we created a new unit, a hospital floor-based spinal high-dependency unit-plus (SHDU-plus), in response to frequent bed-shortage cancellations. This study compares postoperative management on PIC with HDU-plus for these non-hospital floor suitable children with syndromic and neuromuscular scoliosis undergoing PSF. Retrospective review of 100 consecutive children with syndromic and neuromuscular scoliosis undergoing PSF between June 2016 and January 2022. Inclusion criteria were: 1) diagnosis of syndromic or neuromuscular scoliosis, 2) underwent PSF, 3) not suitable for immediate postoperative hospital floor-based care. Exclusion criteria were children with significant cardio-respiratory co-morbidity requiring PIC postoperatively. 55 patients were managed postoperatively on PIC and 45 on SHDU-plus. No significant difference between groups was found with respect to age, weight, ASA grade, preoperative Cobb angles, operative duration, number of levels fused and estimated blood loss. 4 patients in the PIC group and 1 in the SHDU-plus group were readmitted back to PIC or HDU following step-down to the hospital floor. Average length of stay was 2 days on PIC and 1 day on SHDU-plus. Average total length of hospital stay was 16.5 days in the PIC group and 10.5 days in the HDU-plus group. 19 (35%) patients developed complications in the PIC group, compared to 18 (40%) in SHDU-plus. Mean specialist unit charge per day was less on SHDU-plus compared with PIC. There were no bed-shortage cancellations in the SHDU-plus group, compared to 11 in the PIC group. For children with neuromuscular or syndromic scoliosis undergoing PSF and deemed not suitable for post-operative care on the hospital floor, creation of a SHDU-plus was associated with fewer readmissions back to PIC or HDU, shorter hospital stays, an equivalent complication rate, significant cost-saving and fewer cancellations. Level of Evidence: Therapeutic Level III

Neuromuscular scoliosis is a difficult condition to treat. Curve severity, secondary pelvic obliquity and poor respiratory function can make operative treatment and post operative care challenging. The benefits to the child in terms of improved sitting position and trunk posture can be considerable. We present a large consecutive series of patients with neuromuscular scoliosis treated surgically at our institution. The aim of this work was to study the clinical and radiographic impact of surgery for neuromuscular scoliosis. Data was gathered from patient records and radiographs for all cases of neuromuscular scoliosis treated surgically between April 2002 and Feb 2005. 52 cases were identified. They fell into 2 surgical groups: single stage posterior correction and two stage anterior and posterior correction. All posterior instrumentation was transpedicular. Complications, length of stay, and change in severity of sagittal and coronal plane deformity were recorded. Average pre-operative Cobb angle was 85°. There were 16 patients with additional sagittal plane deformity. Average percentage improvement of Cobb angle was 59%. The correction was better in two stage procedures. Pelvic obliquity was improved in those who were obligatory sitters. Fusion rate was 83% for those followed up more than 1 year. ITU stay was longer in single stage procedures. Complication rate was 58%. We have shown that with appropriate patient selection the correction of neuromuscular scoliosis can achieve good results with high fusion rates. Two stage correction confers correctional advantage on those who have sufficient respiratory reserve to tolerate it

SUMMARY. A retrospective cohort study of 19 patients of EOS who underwent MCGR rod instrumentation with subsequent serial distractions, measured using ultrasonography. The degree of distraction achieved during each session were analyzed and subgroup analysis done. HYPOTHESIS. The degree of distraction/ distractibility of the MCGR rod is closely linked to the etiology of EOS. The degree of distraction achieved at each session progressively diminishes with successive distractions. STUDY DESIGN. Retrospective cohort study. INTRODUCTION. MCGR rods have gained popularity as an alternative to traditional growing rods for the treatment of Early-onset scoliosis (EOS), serially distracting with an aim to mimic the normal spinal growth, which can be achieved in an outpatient setting using external remote controller. The use of ultrasound as a tool to measure the degree of distraction achieved has been validated previously. However the association between the etiological diagnosis of EOS and the degree of distraction achieved has not been studied previously. METHODS. We performed a retrospective cohort study of 19 patients with EOS of varying etiologies who underwent MCGR rod instrumentation at our institution since 2016. Their hospital records were analyzed to assess the demographics, distraction interval and the degree of distraction achieved at each session. RESULTS. 19 patients (12 females and 7 males) were included in the study. The average age of the study population was 7 years (4–13 years). The average follow up was 2.1 years (1–4). The following were the etiological diagnoses: Idiopathic EOS(8), Neuromuscular scoliosis(5), Syndromic(5), Congenital(1). The average interval between each distraction was 109.8days (88.2–140.6). The overall mean distraction was 1.66mm (0.6–3.427). The mean distraction achieved for the concave rod was 1.69mm (0.6–3.03) as against 1.59mm (0.2–3.427) for the convex rod. The degree of distraction achieved per session was the greatest in patients with neuromuscular scoliosis 2.79mm (0.94–4.62), while it was the least in Congenital scoliosis 0.99mm (0.8–2.93). The degree of distraction in Syndromic and Idiopathic scoliosis groups were 2.19mm (0.1–4.2) and 1.50mm (0.2–4.45) respectively. The mean distraction achieved during the first session after MCGR instrumentation was 2.82mm (0.4–9.8) as against 1.98mm (0.1–4.2) and 1.18mm (0.2–2.3), achieved during the 5. th. and 10. th. distractions respectively. CONCLUSIONS. The average distraction of the MCGR rod achieved per session depends upon the etiological diagnosis of EOS. Neuromuscular curves are the most amenable to MCGR distractions, while the congenital curves are the least. The degree of distraction achieved progressively diminishes with each successive distractions. The distraction achieved slumps to 70% of the initial distraction by the 5. th. session and further declines to 41% of the initial distraction by the 10. th. session. TAKE HOME MESSAGE. The distractibility of the MCGR rod is closely linked to the etiology of EOS. MCGR rods are most effective in cases of neuromuscular scoliosis, while least effective in congenital scoliosis. The law of diminishing returns holds true even with MCGR rods

Objective: To describe Sniff Nasal Inspiratory Pressure (SNIP) a new way of assessing the global respiratory function in scoliosis patients and explain it’s particular relevance in neuromuscular scoliosis. To correlate of SNIP with conventional lung function tests in scoliosis patients. To compare SNIP values between patients with neuromuscular and idiopathic scoliosis. Design: A prospective observational study of 36 scoliosis patients. All patients underwent conventional lung function tests and SNIP during their pre-operative assessment. The SNIP values were correlated with FVC and FEV1 values obtained by spirometry. The SNIP values of those with neuromuscular scoliosis were compared to those with idiopathic scoliosis and also with the normative data of general population. Subjects: Thirty-six patients (13M, 23F) with scoliosis. Of them, 17 (1M, 16F) had idiopathic scoliosis and 19 (12M, 7F) had neuromuscular scoliosis. The mean age was 16.5 years (7–54). Outcome Measures: Comparison of the mean SNIP, FEV1, FVC and FEV1/FVC between the idiopathic and neuromuscular group. Assessing the degree of correlation between SNIP and spirometry data for all patients. Results: Overall mean SNIP was 50.22 cm H. 2. O. Average SNIP in the neuromuscular group was 40.92±11.68 and 58.72±21.96 in the idiopathic group (p=0.0127). While the mean FEV1 (p=0.183), FVC (p=0.191) and FEV1/FVC (p=0.721) values were not significantly different between the two groups. The correlation coefficient for spirometry values and SNIP was −0.577 (mean x=−48.86, mean y=1.87) (p=0.0002), indicating very good correlation. Conclusion: SNIP is the best measure of global inspiratory muscle strength and less subjective than spirometry. It is easier to administer to children. SNIP is better able to distinguish between idiopathic and neuromuscular scoliosis patients, than spirometry and shows good correlation with spirometry values. It can be a useful test in assessment and follow-up of breathing in neuromuscular scoliosis patients

Introduction and Aims: Surgical correction of pelvic obliquity is an important component of spinal instrumentation for neuromuscular scoliosis, though instrumentation to the pelvis has high reported complication rates. This study evaluates the results of pelvic fixation during surgical correction of neuromuscular scoliosis in a consecutive series of 62 children and adolescents. Method: A retrospective chart and radiographic review of 62 consecutive patients treated with spinal fusions to the pelvis as treatment for neuromuscular scoliosis was performed. Follow-up ranged from two to seven years. Diagnoses included cerebral palsy (36 patients), muscular dystrophy (16 patients), myelomeningocele (three patients), spinal muscular atrophy (three patients) and other disorders (four patients). Mean age at surgery was 13.5 years. Pelvic fixation techniques used included Luque-Galveston or iliosacral screw fixation. Correction of deformity in each patient was assessed with Cobb angle measurements of scoliosis, thoracic kyphosis, and lumbar lordosis. Pelvic obliquity and coronal decompensation was also assessed. Results: The Luque-Galveston spinal instrumentation technique was used in 54 patients and iliosacral screw fixation was used in eight patients. Seventeen patients had an additional anterior release and fusion without instrumentation. The mean Cobb angle measured 73 degrees pre-operatively and 31 degrees (mean correction 59%) post-operatively. The mean Cobb angle on latest follow-up was 33 degrees (loss of correction 12%). Thoracic kyphosis remained essentially unchanged, as did lumbar lordosis (56 pre-op and 61 on follow-up). Pelvic obliquity corrected from a mean of 16 degrees pre-operatively to eight degrees on most recent follow-up. Mean pre-operative coronal decompensation measured 135mm, and follow-up decompensation measured 46mm. Eleven patients with Galveston fixation exhibited the ‘windshield-wiper’ sign, with a radiolucency of 2mm or more, though most were asymptomatic. Wound infection was observed in 6% (3/54) of the patients who underwent Galveston instrumentation and 50% (4/8) who had iliosacral screws. In patients treated with Galveston fixation, three had symptomatic prominant hardware and one had hardware breakage for an overall mechanical failure rate of 7% (4/54). In contrast, two patients with iliosacral screws had construct breakage and pseudoarthrosis for a mechanical failure rate of 25% (2/8), though the numbers in the iliosacral screw group are small. Conclusions: In this series, Galveston pelvic fixation during spinal instrumentation treatment of neuromuscular scoliosis was associated with satisfactory results and with less complications than generally reported in the literature. This technique is recommended as the preferred method for pelvic fixation in severe neuromuscular scoliosis associated with pelvic obliquity

Aim: This study measures the outcomes of surgery for neuromuscular scoliosis using patient/ carer goal setting techniques. Method and Results: In neuromuscular scoliosis, the objective of surgery is to maintain or improve sitting ability and to improve overall function. Measuring the outcome of surgery by patient/carer grading of patient/ carer set goals has not been used in this patient group. A group of 20 children who underwent spinal fusion for neuromuscular scoliosis were assessed using a postural and functional measure pre-op, post-op, and at 3 and 12 months post-op. In addition, each patient was asked to record three goals for undergoing the surgery. At one year post op, patient/carers were asked to grade on a scale of 0 – 10, how satisfied they were that the goals had been achieved. Nineteen patients had clear pre-op goals for the surgery relating to functional activities. The most frequent goals stated for the non-ambulant children were- sitting for longer periods (7/46), making dressing easier (7/46) and sitting more upright (6/38). There were 15 other functional goals stated. The ambulant children stated- appearing straighter (3/12), increase in confidence (2/12), reducing pain (2/12) and maintaining respiratory function (2/12). There were 3 other functional goals stated. Seventeen patients completed the study, 2 were lost to follow up, 1 died. The average satisfaction rate from goals achieved 1 year post-op was 7.9/10. Conclusion: Establishing goals that are realistic and contribute positively to the functional ability or practical management of the child with neuromuscular scoliosis undergoing spinal surgery, encourages the family to be central in the decision making process. It also allows unrealistic expectations to be discussed pre-op and represents the most patient centred method of outcome assessment

The purpose of this study was to first determine if neuromuscular scoliosis results in greater peri-operative transfusion requirements compared to idiopathic scoliosis, and secondly to compare the effects of tranexamic acid (TXA) dosing on reducing transfusion requirements in scoliosis surgery. Previous studies have suggested that patients with neuromuscular scoliosis tend to have more blood loss during scoliosis corrective surgery as compared to patients with idiopathic scoliosis. Tranexamic acid has not been studied extensively in these populations and consensus regarding appropriate dosing has not yet been elucidated. A retrospective chart review of all patients who underwent posterior instrumentation and fusion for scoliosis for the years 1999 to 2006 was performed. Peri-operative transfusion requirements for idiopathic and neuromuscular scoliosis patients were compared and grouped according to TXA use. Transfusion requirements for those patients receiving either a low (10mg/kg loading, 1mg/kg/h infusion) or high (20mg/kg loading, 10mg/kg/h infusion) dose TXA were also compared. Idiopathic patients had significantly decreased transfusion requirements overall (no TXA: idiopathic 1028.3 ± 558.7ml vs. neuromuscular 1400.7 ± 911.3ml, p = 0.02; with TXA: idiopathic 1082.9 ± 1005.5ml vs. neuromuscular 2043.8 ± 1397.5ml, p = 0.03). In the idiopathic group, high dose TXA resulted in a significant reduction in peri-operative transfusion requirements compared to low dose TXA (687.9 ± 778.1ml vs. 1355.0 ± 965.8ml, p = 0.04). Neuromuscular scoliosis patients have significantly higher transfusion requirements as compared to idiopathic patients. For patients with idiopathic scoliosis, the use of the high dose TXA is suggested over low dose TXA given the relative reduction in transfusion requirements for the high dose group

Surgical site infections following spinal surgery profoundly influence continued treatment, significantly impacting psychological and economic dimensions and clinical outcomes. Its reported incidence varies up to 20%, with the highest incidence amongst neuromuscular scoliosis and metastatic cord compression patients. We describe the first reported biphasic osteoconductive scaffold (Cerament G) with a logarithmic elution profile as a cumulative strategic treatment modality for adjacent spinal surgery infections. All patients who developed surgical site infections following instrumented fusion (May 2021-December 2021) had their demographics (age, sex), type and number of procedures, isolated organism, antibiotics given, comorbidities, and WHO performance status analysed. The infected wound was debrided to healthy planes, samples taken, and Cerament g applied. Thirteen patients were treated for deep SSI following spinal instrumentation and fusion procedures with intraoperative Cerament G application. There were four males and nine females with an average age of 40 ranging between 12 and 87. Nine patients underwent initial surgery for spinal deformity, and four were treated for fractures as index procedure. 77% of infections were attributable to MSSA and Cutibacteriousm acnes; others included Klebsiella, Pseudomonas and Streptococcus and targeted with multimodal cumulative therapy. A WHO performance score improved in 11 patients. In addition, there was no wound leak, and infection was eradicated successfully in 12/13 with a single procedure. This series shows the successful eradication of the infection and improved functional outcomes with Cerament G. However, the low numbers of patients in our series are an essential consideration for the broader applicability of this device

Introduction: Sniff nasal inspiratory pressure has become a valuable tool in assessing respiratory muscle weakness. Its role in the scoliosis population is still being defined. Aim: To assess significance of Sniff nasal inspiratory pressure in paediatric patients with scoliosis. Eighty-nine paediatric patients were investigated with SNIP at the time of preoperative assessment for scoliosis surgery from Jan, 2000 to Dec, 2006. Patients were divided into neuromuscular(24) and idiopathic(55). Other causes and revision were excluded(10). SNIP was evaluated with respect to curve pattern and curve degree. This included radiograph and case note review. The mean SNIP value for the idiopathic and neuromuscular groups was 70cmH2O and 44cmH2O respectively. This was significantly different (P=0.006). The mean cobb angle for idiopathic pattern was 58°. For the neuromuscular group it was 73°. There was no correlation between SNIP value and curve severity in either the idiopathic or neuromuscular group. SNIP value does not correlate with cobb angle severity. SNIP can differentiate idiopathic from neuromuscular scoliosis. Low SNIP values are found in neuromuscular scoliosis. Its role in non neuromuscular scoliosis does not appear to be significant

Objective: The purpose of this study is to determine the accuracy of the push-pull radiographs in predicting the surgical correction in neuromuscular scoliosis. Study Design: Retrospective radiographic review. Subjects: Radiographs of 26 patients with neuromuscular scoliosis secondary to Duchennes Muscular Dystrophy were reviewed. All the patients had a posterior instrumented correction of the deformity by the same surgeon with the Universal Spinal System. Duchennes Muscular Dystrophy was chosen as a model for neuromuscular scoliosis since it represents a homogenous group with regards to the spinal deformity. Outcome measures: The Cobb angle, the translation of the apex of the deformity from the central sacral line, pelvic tilt and the number of motion segments in the curve were compared between pre-operative erect, push-pull view and the post-operative radiographs. Results were analysed using student’s t test for significance and Pearson’s coefficient for correlation with the SPSS software. Results: A correlation was seen in the form of an improvement in the Cobb angle, pelvic tilt and the apical translation when comparing the push-pull views and the post-operative radiographs. The improvement was statistically significant. Conclusion: The push-pull view provides an adequate assessment of the fl exibility that guides a safe deformity correction

Introduction: Surgery in patients with neuromuscular scoliosis is aimed at improving truncal balance, facilitating sitting, prevention of progression of the curve and preservation of respiratory function. Patients and Methods: This was a retrospective study of surgical results in a group of 24 patients with minimum follow up of 2 years an average post-operative follow up of 5 years (2–9 years) with neuromuscular scoliosis due to varying aetiologies. The aetiologies included SMA (7), CP (6), Duchenne (5), Congenital Myopathies (3), Spina Bifida (2) and Paraspinal Neuroblastoma (1). Results: The average age at surgery was 10.6 years, the average duration was 4hrs 25mins with an average blood loss pf 1.1 liters. An average ICU stay was 1–2 days and stay in hospital is 11 days. The curves ranged from 25–103° (average 75.6°) pre-operative and we were able to obtain a correction of 56%. In all but 2 of our patients we were successful in preventing deterioration of respiratory function and all our patients could at least sit without support post-operatively. Our complications included only one deep infection (necessitating implant removal), 1 rod breakage and 2 patients with UTI. There were no significant respiratory complications in post-operation. Conclusion: Spinal surgery in patients with neuromuscular scoliosis is safe and prevents deterioration of respiratory and improve truncal balance and hence quality of life

Objective: To evaluate per-operative and postoperative complications following surgical correction of neuromuscular scoliosis and assess the amount of radiological correction of Cobb’s angle and pelvic obliquity angle. Design: Retrospective study of 25 consecutive patients with neuromuscular scoliosis (10 Duchenne Muscular Dystrophy, 8 Cerebral palsy, 2 Neurofibromatosis, and 1 each of Spinal Muscular Atrophy, Friedrich’s Ataxia, Spina Bifida, Rett’s Syndrome and incarcerated hemi-vertibrae) who had surgical correction for their spinal disorders between 1999 and 2002. Background: Aims of surgical correction in neuromuscular scoliosis include improving sitting ability, cardio-respiratory function and cosmesis. Patients studied underwent a combination of anterior release, posterior spinal fusion and pelvic fixation. Due to the length and complexity of procedures many of these patients need two stage surgical procedures on two separate occasions to achieve this goal. However our practice is performing these two stage procedures in one sitting with post operative ITU support. Results: Patients were aged between 4 and 16 years of age with mean preoperative Cobb angles of 75.9° and pelvic obliquity of 18°. A mean correction of 55.8° of Cobb angle and 12° of Pelvic obliquity were achieved. We had mean operating time of 413 minutes with a mean blood loss of 50.2 ml/ Kg body weight. All patients were admitted to ITU post operatively with a mean stay in ITU of 5.8 days. All patients achieved clinical and radiological spinal fusion. Instrumentation had to be removed from one patient after fusion because of persistent infection. Conclusion: Surgery for Neuromuscular Scoliosis is time consuming and complicated. With anticipation of blood loss, complications and ITU support, we can successfully perform the two-stage procedure in one sitting

Introduction: Pelvic obliquity is a constant problem in neuromuscular scoliosis. Galveston and Luque L rod techniques are well described and achieve good correction of pelvic obliquity. We describe a sacral and iliac screw construct integrated with double-rod, pedicle screws and hook system, for correction of pelvic obliquity. Method: 44 patients underwent posterior or combined anterior and posterior fusion to pelvis, for correction of neuromuscular scoliosis and pelvic obliquity. Average age at the time of surgery was 13.8 years. All patients were wheelchair-bound and nine of them were therapeutic walkers. Average follow-up was 44 months (range 24–69 months). Twenty-six patients had combined anterior and posterior surgery. All patients had posterior instrumentation to pelvis and 18 had anterior instrumentation as well. Eighteen patients had posterior instrumented fusion alone. Anterior instrumentation (when used) was Synergy and posterior instrumentation was Synergy or Colorado for all patients. Result: Average time for surgery was 5 hours and 20 minutes and average blood loss 3600 ml. The average pre-operative Cobb angle was 69° and pelvic obliquity 23°. Post-operative average Cobb angle was 29° and pelvic obliquity 7.5°. At the latest follow-up the average Cobb angle was 36° and pelvic obliquity 10°. There were three deep wound infections. Two of the sacral screws have become prominent and two patients had de-linking of the iliac screw with the rod on one side. None showed significant loss of correction. Conclusion: The sacraliliac screw construct with double rod segmental instrumentation achieved good correction of pelvic obliquity in patients with neuromuscular scoliosis. Implant related problems were infrequent

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 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

Homoplastic bone has been produced and used at the Rizzoli Orthopaedic Institute since 1998. Bone grafts are treated following the “freeze-dry” technique developed by the Rizzoli Bone Bank. Up to now, 111 patients have been surgically managed by using lyophilised bone grafts at the Division of Spine Surgery. With a follow-up of more than 12 months, the authors reviewed 81 subjects affected with scoliosis (45 cases – group A), lumbar diseases (34 cases – group B) and segmental cervical degenerative stenosis (two cases – group C). Group A consisted of 20 patients, mean age 14 years, affected with progressive or congenital idiopathic scoliosis; 12 patients, mean age 34.5 years, affected with adult symptomatic scoliosis; 10 patients, mean age 14.4 years, affected with neuromuscular scoliosis; and the remaining three, mean age 37 years, who had already undergone surgery) presenting with pseudarthrosis. Surgical technique was circumferential arthrodesis in paediatric congenital scoliosis and posterior arthrodesis in adult and adolescent idiopathic scoliosis. Fusion was extended to the sacrum and iliac crests in five cases of neuromuscular scoliosis. Patients of group B (16 spondylolisthesis and 18 degenerative lumbar instability), aged 42 years on average, underwent posterior arthrodesis with pedicle fixation. Only two patients in group C were treated with bone graft anteriorly placed at the cervical level to supplement the intersomatic fusion with cage. At a mean follow-up of 28 months, solid fusion was observed in 79 cases (97%); a clearly visible pseudarthrosis was seen only in two cases (3%) (an infantile scoliosis and reintervention for neuromuscular scoliosis in an adult patient). A deep infection with Staphlylococcus aureus was encountered in one patient (1.2%) with degenerative lumbar instability treated with arthrodesis and L2-S1 pedicle fusion: the infection resolved after surgical reintervention (leaving the instrumentation in situ) and drainage. Lyophilised bone, either used as wedge grafts anteriorly or morcellised grafts posteriorly, provides good resistance and integration. Safety, ready availability and possibility to be stored at environmental temperature are further advantages offered by these bone grafts, which make them particularly suitable for application in spinal surgery, above all when surgical times are long and the risk of complications is high

Aims:. Identifying and scoring risk factors that predict early wound dehiscence and progression to metalwork infection. Results of wound healing, eradication of infection and union of with the use of vacuum dressing. Compare results of serial washouts against early vacuum dressing in this group of children with significant medical co-morbidities. Method:. A retrospective review of 300 patients with neuromuscular scoliosis who underwent posterior instrumented correction and fusion between 2008 and 2012 at two institutions. 10 patients had an early wound dehiscence which progressed to deep seated infection requiring wound washout(s) and subsequent vacuum dressing. Medical notes, clinical photographs and imaging were reviewed. Minimum follow up period was 14 months. Results:. This study shows that readmission to PICU, presence of viscero-cutaneous devices (e.g. PEG, tracheostomy), restrictive lung disease, concomitant infection, polymicrobial (gram negative) organism and hyperlordotic deformities are cumulative risk factors to developing early wound dehiscence leading to deep seated infections. All patients in our series went on to have a sealed, non infected wound with union confirmed on CT. Patients who had multiple serial washouts (n=3–7), eventually required vacuum dressing. Discussion:. By stratifying risk factors in patients with neuromuscular scoliosis, the occurrence of wound dehiscence can be predicted. With higher risk factor scores, early vacuum dressing is recommended as multiple serial wound washouts have poor results and with added morbidity to the patient. Conflicts of Interest: No conflicts of interest