A three-dimensional analysis of scoliosis progression in non-idiopathic scoliosis: is it similar to adolescent idiopathic scoliosis?

PURPOSE: To evaluate the three-dimensional (3D) characteristics of spine deformity in patients with non-idiopathic scoliosis compared with those observed in patients with adolescent idiopathic scoliosis (AIS). METHODS: A retrospective chart review was conducted to identify patients with non-idiopathic scoliosis. Twenty-eight patients with neural axis (NA) abnormalities (Chiari 1, syrinx) and 20 patients with connective tissue disorder (CTD) (Marfan's, Beal's, Ehlers-Danlos syndrome, mixed) were identified. The 3D parameters of the coronal, sagittal, and axial plane were compared with 284 AIS patients with a similar range of coronal deformity. RESULTS: The average coronal curve was similar between all three groups (AIS 48 ± 15°, CTD 43 ± 22°, and NA 49 ± 18°; p = 0.4). The NA patients had significantly greater 3D thoracic kyphosis (20 ± 18° vs 10 ± 15°, p = 0.001) and less thoracic apical vertebral rotation (- 5 ± 18° vs - 12 ± 10°, p = 0.003) when compared with AIS. The CTD group's 3D thoracic kyphosis (p = 0.7) and apical vertebral rotation (p = 0.09) did not significantly differ from AIS. Significant negative correlations were found in all three groups between thoracic kyphosis and coronal curve magnitude (AIS r = - 0.49, CTD r = - 0.772, NA r = -0.677, all p < 0.001). CONCLUSIONS: Scoliotic patients with NA abnormalities have a more kyphotic, less-rotated 3D profile than patients with AIS, while scoliosis patients with CTD have 3D features similar to AIS. Irrespective of the underlying diagnosis, however, greater scoliotic curves were associated with a greater loss of intersegmental kyphosis, suggesting a similar biomechanical pathophysiology for curve progression.

Paediatric syndromic scoliosis: proceedings of the half-day course at the 57th annual meeting of the Scoliosis Research Society.

There are some syndromes that present with unique manifestations pertaining to the spinal column. A good working understanding of these common syndromes is useful for the spinal deformity surgeons and related healthcare providers. This review attempts to encompass these unique features and discuss them in three broad groups: hypermobility syndromes, muscle pathology-related syndromes, and syndromes related to poor bone quality. This review explores the features of these syndromes underpinning the aspects of surgical and medical management. This review represents the proceedings of the Paediatric Half-Day Course at the 57th Annual Meeting of the Scoliosis Research Society.

The effect of antibiotic-impregnated calcium sulfate beads and Medical Optimization Clinic attendance on the acute surgical site infection rate in high-risk pediatric neuromuscular and syndromic scoliosis patients.

BACKGROUND: Neuromuscular and syndromic (NMS) scoliosis patients are at higher risk of acute surgical site infections (SSIs). Despite following POSNA's endorsed consensus-based guidelines for SSI prevention, our institutional rates of acute SSI have varied dramatically. This variability drove simultaneous strategies to lower SSI rates: the creation of a preoperative Medical Optimization Clinic (MOC) and use of antibiotic-impregnated (Abx-I) calcium sulfate beads. METHODS: Patients undergoing index PSF at a single institution between 2016 and 2022 were retrospectively reviewed. Patients with ≥ 2 risk factors were included: (1) BMI < 18.5 or > 25; (2) incontinence; (3) instrumentation to pelvis; (4) non-verbal; (5) GMFCS IV/V. SSI was defined as deep infection within 90 days. We compared patients who attended MOC and received Abx-I (MOC + Abx-I) to those receiving neither intervention (control) nor a single intervention. RESULTS: 282 patients were included. The overall infection rate was 4.26%. Higher GMFCS (p = 0.0147), non-verbal status (p = 0.0048), and longer fusions (p = 0.0298) were independently associated with infection rate. Despite the MOC + Abx-I group having larger Cobb angles (88° ± 26°), higher GMFCS levels (4.5 ± 0.9), ASA class (3 ± 0.4), and more frequent instrumentation to the pelvis (85%), they had the lowest infection rate (2.13%) when compared to the control (4.2%) or single intervention groups (5.7%, 4.6%) (p = 0.9). CONCLUSION: The study examined the modern infection rate of NMS patients following the implementation of two interventions: MOC and Abx-I. Despite having higher risk factors (curves (88°), GMFCS level (4.5), ASA class (3), higher % instrumentation to the pelvis (85%)), the patients treated with both interventions demonstrated the lowest infection rate (2.13%).

Vertebral body tethering for non-idiopathic scoliosis: initial results from a multicenter retrospective study.

PURPOSE: Vertebral body tethering (VBT) has been described for patients with idiopathic scoliosis. Results of the technique for non-idiopathic scoliosis have not yet been reported. METHODS: An international multicenter registry was retrospectively queried for non-idiopathic scoliosis patients who underwent VBT with minimum 2-year follow-up. Success at 2 years was defined as Cobb angle < 35 degrees and no fusion surgery. RESULTS: Of the 251 patients treated with VBT, 20 had non-idiopathic scoliosis and minimum 2-year follow-up. Mean age at surgery was 12.4 years (range 10 to 17 years). Mean major Cobb angle at enrollment was 56 degrees. Of those, 18 patients had a major thoracic curve and two had a major lumbar curve. Of the 20 patients, nine met criteria for success (45%). Eight of the 20 patients had poor outcomes (four fusions, four with curve > 50 degrees). Success was associated with smaller preoperative Cobb angle (50 vs. 62 degrees, p = 0.01) and smaller Cobb angle on initial postop imaging (28 degrees vs. 46 degrees, p = 0.0007). All patients with Cobb angle < 35 degrees on 1st postop imaging had a successful result, with the exception of one patient who overcorrected and required fusion. Syndromic vs. neuromuscular patients had a higher likelihood of success (5 of 7, 71%, 2 of 10, 20%, p = 0.03). CONCLUSION: Selected non-idiopathic scoliosis can be successfully treated with VBT, but failure rates are high and were associated with large curves, inadequate intraoperative correction and neuromuscular diagnosis. Achieving a Cobb angle less than 35 degrees on 1st standing radiograph was associated with a successful outcome which was achieved in 45% of patients. LEVEL OF EVIDENCE: Level IV (retrospective review study).

Long-Term Health-Related Quality of Life (QOL) after Paediatric Spinal Deformity Surgery and Comparison with the General Population.

QOL questionnaires assess patients' perception on surgical outcomes. We reviewed 1354 patients with spinal deformity. Four hundred and twenty-eight patients had >10 years of follow-up. The SRS-22r questionnaire was completed before surgery, at 6/12/24 months, 5-10 years and >10 years postoperatively. Patients with >10 years of follow-up completed the EQ-5D VAS/index and the VAS for back/leg pain. We used QOL data reporting in the general population of 20-29 and 30-39 years of age to compare against our patient cohort. Among the patients, 993 had AIS, 80 congenital scoliosis, 102 syndromic or secondary scoliosis, 105 Scheuermann kyphosis and 40 low-grade and 34 high-grade spondylolisthesis. SRS-22r total and domain scores improved from preoperative to follow-up in all diagnosis categories. At >10 years after surgery, patients with congenital scoliosis and Scheuermann kyphosis had better SRS-22r total/domain and EQ-5D (index/VAS) scores along with lower VAS back/leg pain scores compared to the other groups. Patients with congenital scoliosis and Scheuermann kyphosis had comparable SRS-22r total/domain, EQ-5D (index/VAS) and VAS back/leg pain scores to the general population in the 20-29 year category and better scores than the 30-39 year group. Patients with AIS, syndromic/secondary scoliosis and low/high-grade spondylolisthesis had reduced SRS-22r total/domain and EQ-5D (index/VAS) scores and higher VAS back/leg pain scores compared to the 20-29 year group but comparable scores to the 30-39 year group. Patients with spinal deformity reported improved QOL and high satisfaction after surgery which was maintained at >10 years of follow-up. Patients with congenital scoliosis and Scheuermann kyphosis had better QOL outcomes (comparable to the general population of similar age) as opposed to other types of scoliosis or lumbosacral spondylolisthesis.

Preoperative Base Excess as a Predictor of Perioperative Complications in Patients with Nonidiopathic Scoliosis who Have High Risk Associated with General Anesthesia.

Introduction: Patients with nonidiopathic scoliosis often have a high risk associated with general anesthesia because of cardiac or pulmonary dysfunction secondary to underlying diseases. Base excess has been reported as a predictor in the management of trauma and cancer, although not yet in scoliosis. This study was performed to clarify the surgical outcomes and the association of perioperative complications with base excess in patients with nonidiopathic scoliosis who have a high risk associated with general anesthesia. Methods: Patients with nonidiopathic scoliosis who were referred to our institution from 2009 to 2020 because of their high risk associated with general anesthesia were retrospectively enrolled. High-risk factors for anesthesia were determined by a senior anesthesiologist and categorized into circulatory or pulmonary dysfunction. Perioperative complications were analyzed using the Clavien-Dindo classification; severe complications were defined as grade ≥III. We investigated high-risk factors for anesthesia, underlying diseases, preoperative and postoperative Cobb angle, surgery-related factors, base excess, and postoperative management. These variables were statistically compared between patients with and without complications. Results: Thirty-six patients (mean age, 17.9 years old; range, 11-40 years old) were enrolled (two patients declined surgery). High-risk factors were circulatory dysfunction in 16 patients and pulmonary dysfunction in 20 patients. The mean Cobb angle improved from 85.1° (36°-128°) preoperatively to 43.6° (9°-83°) postoperatively. Three intraoperative complications and 23 postoperative complications occurred in 20 (55.6%) patients. Severe complications occurred in 10 (27.8%) patients. All patients underwent postoperative intensive care unit management after posterior all-screw construction. A large preoperative Cobb angle (p=0.021) and base excess outliers (>3 or <-3 mEq/L) (p=0.005) were significant risk factors for complications. Conclusions: Patients with nonidiopathic scoliosis who have a high risk associated with general anesthesia have a higher complication rate. Preoperative large deformity and base excess (>3 or <-3 mEq/L) could be predictors of complications.

Marfan syndrome and adolescent idiopathic scoliosis patients have similar 90-day postoperative outcomes and 5-year reoperation rates after spinal deformity surgery.

PURPOSE: Outcomes after spinal deformity surgery in patients with Marfan syndrome (MFS) are poorly characterized given the rarity of the condition. Updated analyses from nationally representative samples, and comparison to outcomes after more commonly performed procedures for conditions such as adolescent idiopathic scoliosis (AIS) could help define the relative risks. METHODS: Using the 2010-2020 PearlDiver administrative databases, patients who underwent posterior spinal fusion for > 7 segments were extracted. MFS patients were matched 1:4 to AIS patients based on age, sex, and Elixhauer comorbidity index (ECI). Ninety-day outcomes and 5-year reoperation rates were compared. Significance was set at p < 0.05. RESULTS: In total, 206 MFS patients were matched to 825 AIS patients. After adjusting for age, sex, and ECI, multivariate odds ratios (OR) for 90-day any, serious, and minor adverse events, as well as readmissions, were not significantly different for those with MFS compared to those with AIS (p > 0.05 for each). Five-year reoperation rates were also not significantly different (13.1% for the MFS cohort and 13.0% for the AIS cohort (no difference by log-rank, p = 0.9). CONCLUSION: While deformity surgery is much less commonly performed for MFS than AIS, it is not uncommonly considered for patients with this condition. Despite some known technical challenges for MFS deformity surgery, the current study leveraged a large, national database to find that 90-day adverse events and 5-year reoperations were not different for matched MFS and AIS patients undergoing deformity surgery. For select patients, these findings should be useful for surgical planning and patient counseling.

Outcome of Vertical Expandable Prosthetic Titanium Rib (VEPTR) Instrumentation in Scoliosis Associated With 1p36 Deletion Syndrome: A Case Report.

Monosomy 1p36 deletion is a rare syndrome that consists of developmental delay, intellectual disability, seizures, hearing and vision defects, brain anomalies, orofacial clefting, congenital heart defects, cardiomyopathy, renal anomalies, and scoliosis. We report the case of an eight-year-old boy who presented to the orthopedic clinic with spinal deformity with a background of 1p36 deletion syndrome. The treatment modalities at this age include growing rods, vertical expandable prosthetic titanium rib (VEPTR), or posterior spinal fusion. Keeping in view the challenges in this case due to multi-organ involvement and severe intellectual disability, we decided to manage this patient with a VEPTR device to prevent the progression of scoliosis and allow spinal growth. Vertical expandable prosthetic titanium rib (VEPTR) instrumentation for progressive scoliosis in p36 deletion syndrome is an effective mode of treatment and leads to favorable outcomes.

Improved Accuracy and Safety of Pedicle Screw Placement by Using a Probe with an Electrical Conductivity-Measuring Device during Severe Syndromic and Neuromuscular Scoliosis Spine Surgery.

An electrical conductivity-measuring device (ECD) has recently been developed to support pedicle screw placement. However, no evidence exists regarding its efficacy for syndromic/neuromuscular scoliosis with extremely difficult screwing. We retrospectively reviewed 2010-2016 medical records of 21 consecutive syndromic/neuromuscular scoliosis patients undergoing free-hand segmental fixation surgery at our institution and compared the pedicle screw insertion accuracy and safety between 10 with a conventional non-ECD probe (2010-2013) and 11 with an ECD probe (2014-2016). We analyzed preoperative pedicle shape and postoperative screw placement in computed tomography. There were no significant differences between ECD and non-ECD groups in demographic, clinical, and treatment characteristics including scoliosis severity and pedicle diameter. The abandonment rate due to liquorrhea or perforation was lower in ECD (12.3%) than in non-ECD (26.7%) (p < 0.01). Acceptable insertion without perforation or <2-mm lateral/cranial position was more frequent in ECD (67.1%) than in non-ECD (56.9%) (p = 0.02). Critical ≥5-mm medial/caudal malposition was not seen in ECD (0.0%) but in non-ECD (2.4%) (p = 0.02). The perforation distance was shorter in ECD (2.2 ± 1.1 mm) than in non-ECD (2.6 ± 1.7 mm) (p = 0.01). Results involve small sample size, selection, performance, and learning curve biases; nevertheless, ECD could be useful for more accurate and safer pedicle screw placement in severe syndromic/neuromuscular scoliosis.

Tranexamic acid use is associated with reduced intraoperative blood loss during spine surgery for Marfan syndrome.

PURPOSE: The utility of tranexamic acid (TXA) in patients with Marfan syndrome (MFS) is uncertain given associated aberrations within the vasculature and clotting cascade. Therefore, this study aimed to assess the association of TXA use with intraoperative blood loss and allogeneic blood transfusions in patients with MFS who underwent spinal arthrodesis. METHODS: We queried our institutional database for MFS patients who underwent spinal arthrodesis for scoliosis between 2000 and 2020 by one surgeon. We excluded procedures spanning < 4 vertebral levels, those using anterior or combined anterior/posterior approaches, and those involving growing rods, postoperative infection, or spondylolisthesis. Fifty-two patients met our criteria, of whom 22 were treated with TXA and 30 were not. Mean differences in blood loss, transfusion volume, and proportions receiving transfusion were compared between TXA and the control groups using Student t, chi-squared, or Fisher exact tests. Alpha = 0.05. RESULTS: MFS patients treated with TXA experienced less mean (± standard deviation) intraoperative blood loss (1023 ± 534 mL) compared to the control group (1436 ± 1022 mL) (p = 0.01). The TXA group had estimated blood volume loss of 27% ± 16% compared to 36% ± 21% for controls (p = 0.05). No differences were found in allogeneic transfusion rate (p = 0.66) or transfusion volume (p = 0.15). CONCLUSIONS: We found an association between TXA use and reduced blood loss during surgical treatment of MFS-associated scoliosis, suggesting that the connective tissue deficiency in MFS does not interfere with TXA's mechanism of action. LEVEL OF EVIDENCE: III.

Correcting Scoliosis in Rett Syndrome.

Objectives Rett syndrome is a rare disorder characterised by severe scoliosis in 80% of cases. In this retrospective case series, we analysed the radiographic, clinical, and functional outcomes of consecutive patients treated for scoliosis associated with Rett syndrome. We sought to understand the results of the treatment of scoliosis in Rett syndrome and evaluate the need to fuse to the pelvis. Methods A retrospective case series was used to analyse the radiographic, clinical, and functional outcomes of consecutive patients treated for Rett syndrome scoliosis between the ages of 10 and 8 years in a single tertiary paediatric spinal unit. Cases were identified through departmental and neurophysiological records, and patients were excluded if the diagnosis of Rett syndrome was not confirmed. Results Seven eligible cases were identified. At presentation, the mean coronal Cobb angle was 90.9°, mean sagittal Cobb 72.0°, and pelvic obliquity 24.5°. The mean post-operative improvement in coronal Cobb was 53.2° and pelvic obliquity reduced to 5.8°. These did not change during a mean follow up of 3.5 years. None showed any post-operative complications. Three patients with a mean 16.1° pelvic obliquity underwent a fusion to L5. The postoperative result in those cases remained stable at 3.5 years mean follow-up and full skeletal maturity. Conclusion Our data suggests that with modern technology, severe curves can be safely treated. Fusion to the pelvis is not necessary in patients with mild, flexible pelvic obliquity.

Emergent Cesarean Delivery in a Patient With Freeman-Sheldon Syndrome Complicated by Preeclampsia, Acute Pulmonary Embolism, and Pulmonary Edema: A Case Report.

Freeman-Sheldon syndrome (FSS) is an exceedingly rare congenital disorder with an unspecified prevalence. FSS is caused by a mutation in the embryonic skeletal muscle myosin heavy chain 3 gene. Patients may have facial abnormalities that put them at risk of difficult airway intubation. These facial abnormalities include micrognathia, macroglossia, high-arched palate, prominent forehead, and mid-face hypoplasia. Additionally, skeletal abnormalities such as joint contractures, scoliosis with resultant restrictive lung disease, and camptodactyly (bent fingers) can be noted. These features played an important role in the anesthetic management of our FSS patient. Perioperative planning and optimization were crucial in her anesthetic management as she underwent an urgent cesarean section due to preeclampsia with severe features.

Idiopathic stroke after syndromic and neuromuscular scoliosis surgery: a case report and literature review.

Children with syndromic and neuromuscular scoliosis undergoing corrective surgery experience higher complication rates than those with idiopathic scoliosis. A rare but devastating complication is stroke, which occurs in 0.57% of operations for neuromuscular scoliosis but accounts for 7.6% of deaths. Comorbidities associated with non-idiopathic scoliosis that increase risk of stroke include hypercoagulability, cardiac dysfunction, and contractures, which interfere with positioning and risk vascular compromise. Syndromic and neuromuscular diseases may predispose patients to intraoperative dural tears, either primarily (e.g., dural ectasia) or secondarily, in particular due to severity of deformity. We present the case of a perioperative, idiopathic stroke in a 15-year-old boy with scoliosis in the setting of neurofibromatosis type I, reviewing possible causes as well as strategies for preoperative assessment and postoperative monitoring.

Delayed Tetraplegia After Thoracolumbar Scoliosis Surgery in Stuve-Wiedemann Syndrome.

OBJECTIVE: We present 2 patients with Stuve-Wiedemann syndrome (SWS) who suffered delayed tetraparesis following posterior spinal surgery for scoliosis. BACKGROUND SUMMARY: Delayed tetraparesis after a syndromic thoracic scoliosis correction has never been reported. A cord injury distant from the surgical site is rare, and intraoperative neuromonitoring should be used to detect and prevent neurologic impairment. METHODS: Review of medical charts. RESULTS: Two patients with SWS suffered delayed tetraparesis 20 and 40 hours respectively after thoracolumbar posterior surgery. In one patient distal motor evoked potentials fell and recovered partially during surgery. In both patients, early postoperative neurologic examination was normal (in one of them except for the extensor hallucis 2/5). CT scan showed correct instrumentation placement and no compressive haematoma. MRI ruled out cord anomalies, but revealed in both patients identical cervical edema that was most likely secondary to ischemia. Angiogram revealed an absence of anterior cord vascular supply. CONCLUSIONS: Correction of severe deformities in syndromic patients may lead to stretch injuries of the spinal cord and its vascular supply. This in turn may lead to a neurological deficit extending beyond the limits of the spinal instrumentation. Abrupt postoperative neck pain may be an alert to this impending development. Close surveillance in the early postoperative period should be maintained in patients with SWS because a delayed neurological deficit can be present even hours afterwards and may be cranial to the surgical level.