The Effect of Proximal Anchor Choice During Distraction-based Surgeries for Patients With Nonidiopathic Early-onset Scoliosis: A Retrospective Multicenter Study.

BACKGROUND: It is unclear whether the type of proximal anchor affects the spine length achieved with distraction-based surgeries in patients with nonidiopathic early-onset scoliosis (EOS). Since distraction may produce kyphosis, spine length should be assessed in the sagittal plane using the sagittal spine length (SSL-curved arc length of the spine in the sagittal plane). Our purpose was to determine if the type of proximal anchor in distraction-based surgeries will affect final spine length. METHODS: Patients with nonidiopathic EOS treated with distraction-based systems (minimum 5 y follow-up, 5 lengthenings) were identified from 2 EOS registries. Radiographic analysis preoperative, postimplant (L1), and after each lengthening (L2-L5, L6-L10, L11-L15) was performed with the primary outcome of T1-S1 SSL. RESULTS: We identified 126 patients-70 had rib-based implants (52 congenital, 9 syndromic, 9 neuromuscular) and 56 had spine-based implants (15 congenital, 29 syndromic, 12 neuromuscular) with preoperative age 4.6 years, scoliosis 75 degrees, and kyphosis 48 degrees. After initial correction (P<0.05), scoliosis remained constant [58 degrees (13 to 104 degrees) at L11-L15] and kyphosis increased over time [38 degrees (9 to 108 degrees) at L1 to 60 degrees (17 to 134 degrees) at L11-L15] (P<0.05). Preoperative SSL was higher in the spine-based group (29.6 cm) when compared with the rib-based group (25.2 cm) (P<0.05). This difference was maintained after initial implantation (spine-based: 32.2 cm vs. rib-based: 26.7 cm, P<0.05) and at final follow-up (spine-based: 37.0 cm vs. rib-based: 34.4 cm, P<0.05). As preoperative SSL differed between groups, maximum SSL gains per interval were also normalized to preoperative SSL. There was no statistically significant difference between groups at L1, L2-L5, and L6-L10. However, at L11-L15, the rib-based group achieved a more relative increase in spine length compared with the spine-based group (45% vs. 31%, P<0.05). CONCLUSION: At minimum 5 year follow-up, distraction-based surgeries increased spine length for patients with nonidiopathic EOS; regardless of proximal anchor type.

The Effect of Spinopelvic Parameters on the Development of Proximal Junctional Kyphosis in Early Onset: Mean 4.5-Year Follow-up.

BACKGROUND: Proximal junctional kyphosis (PJK) is a major complication after posterior spinal surgery. It is diagnosed radiographically based on a proximal junctional angle (PJA) and clinically when proximal extension is required. We hypothesized that abnormal spinopelvic alignment will increase the risk of PJK in children with early-onset scoliosis (EOS). METHODS: A retrospective study of 135 children with EOS from 2 registries, who were treated with distraction-based implants. Etiologies included 54 congenital, 10 neuromuscular, 37 syndromic, 32 idiopathic, and 2 unknown. A total of 89 rib-based and 46 spine-based surgeries were performed at a mean age of 5.3±2.83 years. On sagittal radiographs, spinopelvic parameters were measured preoperatively and at last follow-up: scoliosis angle (Cobb method, CA), thoracic kyphosis (TK), lumbar lordosis (LL), pelvic incidence (PI), pelvic tilt (PT), sacral slope and PJA. Radiographic PJK was defined as PJA≥10 degrees and PJA≥10 degrees greater than preoperative measurement. The requirement for the proximal extension of the upper instrumented vertebrae was considered a proximal junctional failure (PJF). Analysis of risk factors for the development of PJK and PJF was performed. RESULTS: At final follow-up (mean: 4.5±2.6 y), CA decreased (P<0.005), LL (P=0.029), and PI (P<0.005) increased, whereas PI-LL (pelvic incidence minus lumbar lordosis) did not change (P=0.706). Overall, 38% of children developed radiographic PJK and 18% developed PJF. Preoperative TK>50 degrees was a risk factor for the development of radiographic PJK (relative risk: 1.67, P=0.04). Children with high postoperative CA [hazard ratio (HR): 1.03, P=0.015], postoperative PT≥30 degrees (HR: 2.77, P=0.043), PI-LL>20 degrees (HR: 2.92, P=0.034), as well as greater preoperative to postoperative changes in PT (HR: 1.05, P=0.004), PI (HR: 1.06, P=0.0004) and PI-LL (HR: 1.03, P=0.013) were more likely to develop PJF. Children with rib-based constructs were less likely to develop radiographic PJK compared with children with spine-based distraction constructs (31% vs. 54%, respectively, P=0.038). CONCLUSIONS: In EOS patients undergoing growth-friendly surgery for EOS, preoperative TK>50 degrees was associated with increased risk for radiographic PJK. Postoperative PI-LL>20 degrees, PT≥30 degrees, and overcorrection of PT and PI-LL increased risk for PJF. Rib-based distraction construct decreased the risk for radiographic PJK in contrast with the spine-based constructs. LEVEL OF EVIDENCE: Level III.

Growth Friendly Surgery and Serial Cast Correction in the Treatment of Early-onset Scoliosis for Patients With Prader-Willi Syndrome.

BACKGROUND: Prader-Willi syndrome (PWS) patients can present with scoliosis which can be treated with serial cast correction (SCC) or with growth friendly surgery (GFS). This study's purpose was to describe the results of SCC as well as GFS for PWS patients with early-onset scoliosis (EOS). METHODS: PWS patients were identified from 2 international multicenter EOS databases. Scoliosis, kyphosis, spine height (T1-S1), right/left hemithoracic heights/widths (RHTH, LHTH, RHTW, LHTW) were measured pretreatment, postoperation, and at 2-year follow-up. Complications were recorded. RESULTS: Overall, 23 patients with 2-year follow-up were identified. Pretreatment; patients treated with SCC (n=10) had mean age of 1.8±0.6 years; body mass index (BMI), 16±1.5 kg/m; scoliosis, 45±18 degrees; kyphosis, 56±9 degrees; T1-S1, 22.4±2.4 cm; RHTH, 8.0±2.0 cm; LHTH, 8.5±1.7 cm; RHTW, 6.6±1.3 cm; and LHTW, 8.0±1.0 cm. Patients treated with GFS (n=13) had mean age of 5.8±2.6 years; BMI, 21±5.4 kg/m; scoliosis, 76±14 degrees; kyphosis, 59±25 degrees; T1-S1, 24.1±3.6 cm; RHTH, 10.0±1.6 cm; LHTH, 10.6±1.6 cm; RHTW, 9.4±2.5 cm; and LHTW, 8.1±2.8 cm. At 2-year follow-up, patients treated with SCC had mean scoliosis 37±11 degrees (18% correction, P=0.06); kyphosis, 42±6 degrees (NS); T1-S1, 26.4±2.1 cm (P<0.01); RHTH, 9.0±1.1 cm (13%; P=0.30); LHTH, 10.0±1.5 cm (18%, P<0.01); RHTW, 7.4±1.1 cm (12%, P<0.01); and LHTW, 8.0±1.0 cm (0%, P=0.34). At 2-year follow-up, patients treated with GFS had mean scoliosis 42±13 degrees (45% correction, P<0.000001); kyphosis, 53±13 degrees (10%, P=0.19); T1-S1, 31.5±5.4 cm (P<0.00001); RHTH, 12.0±2.4 cm (20%; P<0.01); LHTH, 12.0±1.7 cm (13%; P<0.01); RHTW, 9.8±1.3 cm (4%; P=0.27); and LHTW, 7.9±2.3 cm (3%;P=0.11). As an entire group, patients with a BMI>17 kg/m² had more device-related than disease-related complications (P=0.09). Patients treated with SCC had 0.9 complications per patient. Patients treated with GFS had 2.2 complications per patient [≤5 y more often had ≥2 complications (P=0.05)]. CONCLUSIONS: At 2-year follow-up, SCC and GFS were both effective in treating EOS in PWS patients. Patients treated with SCC had significant improvements in spine height and LHTH. Patients treated with GFS had significant improvements in scoliosis magnitude, spine height, RHTH, and LHTH. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Patients Without Intraoperative Neuromonitoring (IONM) Alerts During VEPTR Implantation Did Not Sustain Neurological Injury During Subsequent Routine Expansions: A Retrospective Multicenter Cohort Study.

BACKGROUND: The purpose of this study was to determine the rate of intraoperative neurological monitoring (IONM) alerts and neurological injury during vertical expandable prosthetic titanium rib (VEPTR) treatment and evaluate the utility of IONM during VEPTR expansion procedures in patients who have not previously had neurological injury or IONM alerts. METHODS: After institutional review board approval, VEPTR procedures and IONM records were reviewed at 17 institutions for patients treated with VEPTR from 2005 to 2011. All consecutive cases in patients with minimum 2-year follow-up were included. Patients with prior history of growing rods or other invasive spine-based surgical treatment were excluded. Surgeries were categorized into implant, revision, expansion, and removal procedures. Cases with IONM alerts or neurological injury had additional detailed review. Descriptive statistics were used for data analysis. RESULTS: In total, 2355 consecutive VEPTR procedures (352 patients) consisting of 299 implant, 377 revision, 1587 expansion, and 92 removal procedures were included. In total, 620 VEPTR procedures had IONM, and 539 of those had IONM records available for review. IONM alerts occurred in 9/539 procedures (1.7%): 3/192 implants (1.6%), 3/58 revisions (5.2%), and 3/258 expansions (1.2%). New neurological injury occurred in 3/2355 procedures (0.1%), 3/352 patients (0.9%). All 3 injuries were in implant procedures, only 1 had an IONM alert. All 3 had upper extremity motor deficits (1 had sensory deficit also). All had full recovery at 17, 30, and 124 days postinjury. One patient without prior neurological injury or IONM alert had an IONM alert during expansion that resolved after an increase in blood pressure. The remaining IONM alerts during expansions were all in children with prior IONM alerts during implant, revision, or exchange procedures. CONCLUSIONS: The highest rate of neurological injury in VEPTR surgery was found for implant procedures. There were no instances of neurological injury during VEPTR expansion, revision, or removal procedures. IONM did not identify new neurological injuries in patients undergoing VEPTR expansion who did not previously have a history of IONM signal change or neurologic injury. LEVEL OF EVIDENCE: Level IV-diagnostic study.

Stakeholder-engaged research is necessary across the criminal-legal spectrum.

People with lived experience of incarceration have higher rates of morbidity and mortality compared to people without history of incarceration. Research conducted unethically in prisons and jails led to increased scrutiny of research to ensure the needs of those studied are protected. One consequence of increased restrictions on research with criminal-legal involved populations is reluctance to engage in research evaluations of healthcare for people who are incarcerated and people who have lived experience of incarceration. Ethical research can be done in partnership with people with lived experience of incarceration and other key stakeholders and should be encouraged. In this article, we describe how stakeholder engagement can be accomplished in this setting, and further, how such engagement leads to impactful research that can be disseminated and implemented across disciplines and communities. The goal is to build trust across the spectrum of people who work, live in, or are impacted by the criminal-legal system, with the purpose of moving toward health equity.

Can distraction-based surgeries achieve minimum 18 cm thoracic height for patients with early onset scoliosis?

PURPOSE: Karol et al. introduced the concept that 18 cm thoracic height is the critical point where a patient with early onset scoliosis (EOS) can maintain adequate pulmonary function. Our purpose was to determine if distraction-based surgeries will increase thoracic spine height to at least 18 cm in patients with EOS. METHODS: Patients with EOS treated with distraction-based systems (minimum 5 years follow up, minimum five lengthenings). Radiographic analysis of thoracic spine height (T1-T12) at the last lengthening procedure. RESULTS: One hundred and fifty-three patients (67 congenital, 21 neuromuscular, 38 syndromic, 27 idiopathic) with pre-operative mean age 4.6 years, scoliosis 75°, kyphosis 47° were evaluated. Their mean age at final lengthening procedure was 11 years (6-16), average number of lengthening procedures was 10.5 (4-21), mean final scoliosis was 53°, and mean final kyphosis was 58°. Final thoracic height was > 18 cm in 65% and was > 22 cm in 31% of patients. Based on etiology, only 48% of the congenital patients reached 18 cm compared to 81% neuromuscular, 84% syndromic and 67% idiopathic. This height gain was closely related to the percentage of scoliosis correction achieved for each etiology. Comparing congenital etiology to other etiologies, there was a lower percentage of patients in the congenital group that passed the 18 cm threshold (48% vs. 78%) (p < 0.05). CONCLUSION: At minimum 5 years follow up, distraction-based surgeries increased thoracic height for patients with EOS to greater than 18 cm in 65% of patients; however, only 48% of congenital patients reached this thoracic height threshold. DESIGN: Retrospective review of prospectively collected registry data. LOI III.

Superior Extension of Upper Instrumented Vertebrae in Distraction-based Surgery: A Surrogate for Clinically Significant Proximal Junctional Kyphosis.

BACKGROUND: Proximal junctional kyphosis (PJK) is a reported complication of distraction-based growth-friendly surgery for early-onset scoliosis (EOS). A potential consequence of PJK is revision surgery with superior extension of the upper instrument vertebrae (UIV). The purpose of this study was to determine the risk of radiographic and clinically significant PJK during growth-friendly surgery. METHODS: This is a retrospective review of children treated with distraction-based growth-friendly surgeries from two EOS registries with minimum two-year follow-up. PJK is defined as clinically significant in this study if surgery with superior extension of the UIV was performed. RESULTS: Of 419 total patients, there was a 20% risk of developing clinically significant PJK (24% rib vs. 15% spine-based anchors, p = .03). These patients had a mean preoperative age of 5.6 years (5.2-year rib vs. 6.0-year spine, p < .001), scoliosis of 73° (69° rib vs. 77° spine, p < .001), and kyphosis of 51° (47° rib vs. 56° spine, p < .01). Regression analysis demonstrated that these differences in age, scoliosis, and kyphosis between anchor type did not account for a significant proportion of the measured variance. CONCLUSIONS: There was a 20% risk of developing clinically significant PJK, with a slightly higher risk for patients treated with rib-based proximal anchors (24%) than for those patients treated with spine-based proximal anchors (15%). LEVEL OF EVIDENCE: Level III.

Growth-Friendly Surgery Is Effective at Treating Early-Onset Scoliosis Associated With Goldenhar Syndrome.

OBJECTIVE: To evaluate the radiographic results and complications of growth-friendly (GF) surgery in the treatment of early-onset scoliosis (EOS) associated with Goldenhar syndrome. BACKGROUND: Goldenhar syndrome has been associated with spinal deformity, which may be progressive. Efficacy and complication rate of GF treatment has not been reported for this population of patients with EOS. METHODS: Patients with Goldenhar syndrome and EOS with two years' follow-up were identified from two international multicenter EOS databases. Scoliosis, kyphosis, spine height, and hemithoracic height/width were determined preimplant, immediately postoperative, and at the two-year follow-up. Severity of complications (SV) was recorded (Smith et al. JPO 2015). RESULTS: Ten patients met inclusion criteria and had a mean age of 4.6 ± 2.5 years at GF implantation (one spine and nine rib-based). Mean preoperative scoliosis was 64°, postimplant 52°, and at mean follow up of 2.4 ± 0.5 years was 50° (p = .09). Preoperative kyphosis was 36°, postimplant 38°, and final 42° (p = .08). Preoperative T1-S1 height was 23.5 cm, postimplant 23.6 cm, and final 27.3 cm (p = .06). Preoperative convex hemithoracic height was 10.4 cm, postimplant 7.9 cm, and final 12.8 cm (p < .05). Preoperative concave hemithoracic height was 8.4 cm, postimplant 8.8 cm, and final 9.9 cm (p = .30). Preoperative right hemithoracic width was 8.02 cm, postimplant 7.22 cm, and final 7.86 cm (p = .07). Preoperative left hemithoracic width was 7.18 cm, postimplant 7.86 cm, and final 8.60 cm (p = .43). Eight patients had ≥1 complication with SV I (n = 7), SV II (n = 2), and SV IIA (n = 7). These included infection (n = 4), migration (n = 3), pneumonia (n = 2), and instrumentation failure (n = 2). CONCLUSION: At minimum two-year follow-up, GF surgical intervention for the treatment of EOS associated with Goldenhar syndrome trended toward improvements in scoliosis and spine height, but had a significant improvement in convex hemithoracic height; however, the majority of patients experienced severity grade I or II complications. LEVEL OF EVIDENCE: Level IV.

Paper #42: Spinopelvic alignment affects Health-related Quality of Life (HRQoL) for Patients with Early Onset Scoliosis.

Recent studies in the adult scoliosis population have shown effects of spinopelvic parameters on HRQoL outcomes. In this retrospective cohort study of 75 EOS patients treated with growth-friendly surgery, spinopelvic parameters affected HRQoL (EOSQ-24). Pre-operative PI>60° increased the risk for poor pain outcomes, pre- and post-operative LL>60° increased the risk for poor satisfaction, post-operative PI-LL mismatch of O >20° and post-operative PJK increased the risk of Fatigue.

Paper #29: Does the Type of Proximal Anchor Used During Distraction-Based Surgeries for Patients With Non-Idiopathic EOS Affect Spine Length?

At minimum 5 yr f/u, distraction-based surgeries (Spine-based (SB)&Rib-based (RB)) are an effective way to increase spine length for non-idiopathic EOS. Spine length is greater for spine based implants pre-operatively and this length is maintained to the 15th lengthening; however, if normalized to pre-op spine length, rib-based implants achieved greater percentage of increase in spine length beyond the 10th lengthening surgeries.

Complications and Radiographic Outcomes of Posterior Spinal Fusion and Observation in Patients Who Have Undergone Distraction-Based Treatment for Early Onset Scoliosis.

STUDY DESIGN: Retrospective, multicenter. OBJECTIVES: To compare surgical and radiographic outcomes of early-onset scoliosis (EOS) patients who had stopped lengthening for ≥2 years without additional surgery to those who had posterior spinal fusion (PSF) at the end of lengthening. SUMMARY OF BACKGROUND DATA: Because of the risk of significant complications with PSF in patients with EOS, "watchful waiting" at the end of lengthening has been suggested as a viable alternative. METHODS: Retrospective review of the Children's Spine Study Group (CSSG) database identified all patients with the diagnosis of EOS who had distraction-based treatment, who were ≥2 years from their last distraction, and who had complete records. Radiographic measures were obtained by a single unbiased trained observer. Treatment outcomes including curve correction, height and length gain, as well as complications were recorded. RESULTS: The 37 patients (21 females and 16 males) had a mean age of 7.2 years; 12 were in the observation (OBS) and 25 in the PSF group. The PSF group had a slightly greater coronal Cobb angle and maximal kyphosis at the end of distraction. Although there was some correction of the coronal Cobb angle and maximal kyphosis following PSF, the differences between the two groups were not statistically significant at final follow-up. At final follow-up, the OBS group obtained 88% of T1-T12 height and 90% of T1-L1 length of that obtained by the PSF group. Twenty-six complications occurred in 15 patients, all in the PSF group. CONCLUSIONS: Observation may be a viable alternative to PSF after distraction-based treatment in a subset of patients with EOS. PSF was found to provide no significant curve correction or gains in spine height and length compared to observation and carries a significant risk of complications. LEVEL OF EVIDENCE: Level III, therapeutic.

Variability of Surgical Site Infection With VEPTR at Eight Centers: A Retrospective Cohort Analysis.

STUDY DESIGN: Retrospective review. OBJECTIVES: To describe clinical characteristics and infection rates in modern vertical expandable prosthetic titanium rib (VEPTR) surgery. SUMMARY OF BACKGROUND DATA: Prior studies have demonstrated infection rates from 10% to 30% with VEPTR surgery. METHODS: A retrospective query was done on an institutional review board-approved, multicenter prospectively collected database for patients implanted with VEPTR from 2007 to 2013 at eight sites. This identified 213 patients with appropriate data for analysis. Average follow-up was 4.1 years (range 1.7-6.3). Data collected included a Classification of Early-Onset Scoliosis (C-EOS) diagnosis, American Society of Anesthesiologists Physical Status (ASA-PS), major Cobb angle, construct type, clinical symptoms, and microbiology. The distribution of infection rates across all the study sites was compared. The exact p value was estimated by Monte Carlo simulation. RESULTS: Overall, 18% (38/213) of patients implanted with VEPTR developed infection requiring operative debridement. There were significantly different infection rates among the sites, ranging from 2.9% to 42.9% (p = .029). The average time to infection was 70 days (range 8-236) after the infecting procedure. The majority of infections were due to gram-positive bacteria (80%, 44/55), the most prevalent being methicillin-sensitive Staphylococcus aureus (45%, 25/55). There were 20 patients (53%, 20/38) with either partial or complete implant removal to resolve infection; however, only 3 of 38 (8%) of these resulted in abandonment of VEPTR treatment. There was no difference in infection rate across the primary C-EOS diagnosis categories (p = .21) or based on ASA score (p = .53). After controlling for study site, the odds ratio of an infection following an implant procedure versus an expansion was 2.8 (p = .002). There was no difference in the odds ratio of an infection between the other procedure types (implant, expansion, exchange/revision). CONCLUSIONS: There were significant differences in infection rates between sites. The variability in infection rate indicates a need for guided efforts to standardize best practices for infection control in VEPTR surgery. LEVEL OF EVIDENCE: III, therapeutic study.