A New Look at Vertebral Body Tethering (VBT): Through the Modified Clavien-Dindo-Sink (mCDS) Classification.

INTRODUCTION: Anterior vertebral body tethering (AVBT) is increasingly popular as an option for surgical treatment of idiopathic scoliosis (IS). While the technology remains new, it is important for families and patients to be able to compare it to the current standard of care, posterior spinal fusion (PSF). The purpose of this study is to describe the complication rate of AVBT in IS using the mCDS and to compare it to the recently reported complication rate of PSF in IS. METHODS: A multicenter pediatric spine deformity database was queried for all idiopathic scoliosis patients who underwent vertebral body tethering. There were 171 patients with a minimum 9-month follow-up included in this study. Complications were retrospectively graded by 2 attending pediatric spine surgeons using the mCDS classification system. RESULTS: Data from 171 patients with idiopathic scoliosis was available for analysis, with 156/171 (91%) of patients being female and an average age of 12.2 years old at surgery. There were 156 thoracic tethers (1 with an LIV below L2), 5 lumbar tethers, 9 staged double tethers, and only 1 patient with same-day double tether. Fifty-five (55) (32%) patients experienced a total of 69 complications. The most common complication type for VBT by mCDS was Grade IIIb, encompassing 29/69 (42%) of complications. The second most frequent complication grade was Grade I at 23/69 (33%). Thirty-four (34) out of 69 (49%) of the VBT complications reported required either procedural/surgical intervention or admission to the ICU. CONCLUSIONS: This is the first study to directly compare the complication profile of VBT to PSF using the mCDS. Forty-nine percent (49%) of the VBT complications reported were at least Grade III, while only 7% of complications in the control PSF cohort from the literature were Grade III or higher. The mCDS complication classification brings light to the early learning experience of a new technique compared to the widely accepted standard of PSF for IS. LEVEL OF EVIDENCE: III - Retrospective comparative study.

Comparison of Distal Spine Anchors and Distal Pelvic Anchors in Children With Hypotonic Neuromuscular Scoliosis Treated With Growth-friendly Instrumentation.

BACKGROUND: Lower preoperative pelvic obliquity (PO) and L5 tilt have been associated with good radiographic outcomes when the fusion ended short of the pelvis in children with neuromuscular scoliosis (NMS). Our purpose was to identify indications to exclude the pelvis in children with hypotonic NMS treated with growth-friendly instrumentation. METHODS: This was a multicenter retrospective review. Children with spinal muscular atrophy and muscular dystrophy treated with dual traditional growing rod, magnetically controlled growing rod, or vertical expandable prosthetic titanium rib with minimum 2-year follow-up after the index surgery were identified. RESULTS: A total of 125 patients met the inclusion criteria. Thirty-eight patients had distal spine anchors (DSAs) and 87 patients had distal pelvic anchors (DPAs) placed at the index surgery. Demographics and length of follow-up were similar between the groups but there was a greater percentage of DPA patients who were nonambulatory [79 patients (91%) vs. 18 patients (47%), P <0.0001]. Preindex radiographic measures were similar except the DSA patients had a lower PO (11 vs. 19 degrees, P =0.0001) and L5 tilt (8 vs. 12 degrees, P =0.001). Postindex and most recent radiographic data were comparable between the groups. There was no difference in the complication and unplanned returns to the operating room rates.Subanalysis of the DSA group based on ambulatory status showed similar radiographic measures except the ambulatory patients had a lower PO at all time points (preindex: 5 vs. 16 degrees, P =0.011; postindex: 6 vs. 10 degrees, P =0.045; most recent follow-up: 5 vs. 14 degrees, P =0.028). Only 1 ambulatory DSA patient had a PO ≥10 degrees at most recent follow-up compared with 6 nonambulatory DSA patients. Three (8%) DSA patients, all nonambulatory, underwent extension of their instrumentation to the pelvis. CONCLUSIONS: Pelvic fixation should be strongly considered in nonambulatory children with hypotonic NMS treated with growth-friendly instrumentation. At intermediate-term follow-up, revision surgery to include the pelvis was rare but DSAs do not seem effective at maintaining control of PO in nonambulatory patients. DSA and DPA were equally effective at maintaining major curve control, and complication and unplanned returns to the operating room rates were similar. LEVEL OF EVIDENCE: Level III-therapeutic.

When Will You Succeed Casting Patients With Early-onset Scoliosis? Prospective Evaluation of Predictive Radiographic Parameters.

BACKGROUND: In a recent retrospective study, in cast correction of the major curve correlated with final curve size in patients with early-onset scoliosis treated with casting. We therefore sought to perform a prospective study with controlled methodology to determine if there are parameters associated with reduction of coronal deformity. METHODS: A prospective, observational study was conducted between 2014 and 2019 at selected sites willing to comply with a standard radiographic and follow-up protocol. Radiographic data was collected at time points of precast, in traction, initial in-cast, and at minimum 1 year follow-up. Multivariate linear regression models were utilized to control for potential confounders using a stepwise procedure. Twenty-nine patients met inclusion criteria. RESULTS: On multivariate analysis, traction major curve (P=0.043) and initial in-cast (P=0.011) major curve Cobb angles were independently associated with final out of cast major curve Cobb angle. The only factor that was independently associated with failure to cure (<15-degree major curve) was traction major curve Cobb angle (P=0.046). A threshold traction major curve Cobb angle of 20 degrees was found to have good accuracy with 81% sensitivity and 73% specificity (receiver operator curve area: 0.869, P<0.001). A traction major curve Cobb angle over 20 degrees would accurately predict failure of casting treatment to cure scoliosis in 79% of cases. A threshold in-cast major curve Cobb angle of 21 degrees was found to have slightly less accuracy than traction with 69% sensitivity, 82% specificity, and 74% accuracy (receiver operator curve area: 0.830, P=0.004). CONCLUSIONS: Radiographic measurements in traction and initially in the cast are predictive of curve size at follow-up for children with early-onset scoliosis treated with casting. The standardization and utility of traction films should be further explored. LEVEL OF EVIDENCE: Level II.

The Effect of Surgeon Experience on Outcomes Following Growth Friendly Instrumentation for Early Onset Scoliosis.

BACKGROUND: The purpose of this study was to utilize a multicenter, multisurgeon cohort to assess the effect of surgeon experience on outcomes of growth friendly instrumentation (GFI) in early onset scoliosis (EOS). We hypothesized that unplanned return to the operating room (UPROR), estimated blood loss (EBL), and surgical time would be greater amongst early career surgeons (ECSs) when compared with advanced career surgeons (ACSs). METHODS: An international pediatric spine database was queried for patients ages 2 to 10 years treated by posterior distraction-based GFI with at least of 2-year follow up. Two groups were created for analysis based on surgeon experience: ECSs (with ≤10 y of experience) and ACSs (with >10 y of experience). The primary outcome was UPROR. Additional outcomes included: operating room time, EBL, neurological deficits, infection rate, hardware failure, and the Early Onset Scoliosis Questionnaire (EOSQ-24). Subgroup analysis was performed for further assessment based on procedure type, superior anchor type, etiology, and curve severity. RESULTS: A total of 960 patients met inclusion criteria including 243 (25.3%) treated by ECS. Etiology, sex, superior anchor, and EOSQ-24 scores were similar between groups (P>0.05). There were no clinically significant differences in patient age or preoperative major coronal curve. UPROR (35.8% vs. 32.7%, P=0.532), infection (17.0% vs. 15.6%, P=0.698), operating room time (235 vs. 231 min, P=0.755), and EBL (151 vs. 155 mL, P=0.833) were comparable between ECS and ACS groups. The frequency of having at least 1 complication was relatively high but comparable among groups (60.7% vs. 62.6%, P=0.709). EOSQ-24 subdomain scores were similar between groups at 2-year follow-up (P>0.05). Subgroup analysis revealed that ECS had increased surgical time compared with ACS in severe curves >90 degrees (270 vs. 229 min, P=0.05). CONCLUSIONS: This study represents the first multicenter assessment of surgeon experience on outcomes in EOS. Overall, surgeon experience did not significantly influence UPROR, complication rates, EBL, or surgical time associated with GFI in this cohort of EOS patients. LEVEL OF EVIDENCE: Level III.

Mortality in Early-Onset Scoliosis During the Growth-friendly Surgery Era.

INTRODUCTION: Early-onset scoliosis (EOS) is a spinal deformity that occurs in patients 9 years of age or younger. Severe deformity may result in thoracic insufficiency, respiratory failure, and premature death. The purpose of this study is to describe the modern-day natural history of mortality in patients with EOS. METHODS: The multicenter Pediatric Spine Study Group database was queried for all patients with EOS who are deceased, without exclusion. Demographics, underlying diagnoses, EOS etiology, operative and nonoperative treatments or observation, complications, and date of death were retrieved. Descriptive statistics and survival analysis with Kaplan-Meier curves were performed. RESULTS: There were 130/8009 patients identified as deceased for a registry mortality rate of 16 per 1000 patients. The mean age at death was 10.6 years (range: 1.0 to 30.2 y) and the most common EOS etiology was neuromuscular (73/130, 56.2%; P<0.001). Deceased patients were more likely be treated operatively than nonoperatively or observed (P<0.001). The mean age of death for patients treated operatively (12.3 y) was older than those treated nonoperatively (7.0 y) or observed (6.3 y) (P<0.001) despite a larger deformity and similar index visit body mass index and ventilation requirements. Kaplan-Meier analysis confirmed an increased survival time in patients with a history of any spine operation compared with patients without a history of spine operation (P<0.0001). Operatively treated patients experienced a median of 3.0 complications from diagnosis to death. Overall, cardiopulmonary related complications were the most common (129/271, 47.6%; P<0.001), followed by implant-related (57/271, 21.0%) and wound-related (26/271, 9.6%). The primary cause of death was identified for 78/130 (60.0%) patients, of which 57/78 (73.1%) were cardiopulmonary related. CONCLUSIONS: This study represents the largest collection of EOS mortality to date, providing surgeons with a modern-day examination of the effects of surgical intervention to better council patients and families. Both fatal and nonfatal complications in children with EOS are most likely to involve the cardiopulmonary system. LEVEL OF EVIDENCE: Level IV-therapeutic.

Best Practice Guidelines for Surgical Site Infection in High-risk Pediatric Spine Surgery: Definition, Prevention, Diagnosis, and Treatment.

BACKGROUND: Prior "best practice guidelines" (BPG) have identified strategies to reduce the risk of acute deep surgical site infection (SSI), but there still exists large variability in practice. Further, there is still no consensus on which patients are "high risk" for SSI and how SSI should be diagnosed or treated in pediatric spine surgery. We sought to develop an updated, consensus-based BPG informed by available literature and expert opinion on defining high-SSI risk in pediatric spine surgery and on prevention, diagnosis, and treatment of SSI in this high-risk population. MATERIALS AND METHODS: After a systematic review of the literature, an expert panel of 21 pediatric spine surgeons was selected from the Harms Study Group based on extensive experience in the field of pediatric spine surgery. Using the Delphi process and iterative survey rounds, the expert panel was surveyed for current practices, presented with the systematic review, given the opportunity to voice opinions through a live discussion session and asked to vote regarding preferences privately. Two survey rounds were conducted electronically, after which a live conference was held to present and discuss results. A final electronic survey was then conducted for final voting. Agreement ≥70% was considered consensus. Items near consensus were revised if feasible to achieve consensus in subsequent surveys. RESULTS: Consensus was reached for 17 items for defining high-SSI risk, 17 items for preventing, 6 for diagnosing, and 9 for treating SSI in this high-risk population. After final voting, all 21 experts agreed to the publication and implementation of these items in their practice. CONCLUSIONS: We present a set of updated consensus-based BPGs for defining high-risk and preventing, diagnosing, and treating SSI in high-risk pediatric spine surgery. We believe that this BPG can limit variability in practice and decrease the incidence of SSI in pediatric spine surgery. LEVEL OF EVIDENCE: Not applicable.

Contouring the magnetically controlled growing rods: impact on expansion capacity and proximal junctional kyphosis.

OBJECTIVE: To quantitatively determine the relationship between the contouring of the magnetically controlled growing rods (MCGR), their expansion capacity and the risk of developing proximal junctional kyphosis in early onset scoliosis (EOS). MCGRs allow gradual expansion and correction of the spinal deformity in EOS while reducing the need for repeated surgeries. As the expansion of the MCGRs is controlled externally, several factors can impact the discrepancy between the intended and actual expansions of the rods. The expansion capacity of the growing rods as a function of the expanded length has been tested in experimental setups; however, no study has evaluated the role of contouring of the MCGRs on its function and long-term surgical outcome. METHODS: A total of 25 EOS patients, a total of 48 MCGRs, with right thoracic curves, were studied retrospectively. All patients had two view spinal radiographs at pre-operative, after MCGR implantation, and after 6 lengthening visits. The first post-operative radiographs were used to calculate the 3D contour of the MCGR at the proximal end. 2D ultrasound images before and after lengthening visits were used to measure the rod lengthening at each visit. The relationship between the increase in the rod length and rod curvature was determined. Finally, the rod curvature was correlated to the changes in proximal junctional kyphosis (PJK) angle between the pre-operative and the most recent follow-up, i.e., after 6 visits. RESULTS: The average rod 3D angle at the proximal end was 13.5° ± 9.7° [0°-37.2°]. The overall increased length after six lengthening visits for the rod at the concave side was 18.8 mm and at the convex side was 16.9 mm. 62% of the patients with a contoured rod at the proximal end developed a PJK exceeding 10° whereas in patients with a straight rod PJK occurred in 9.1%. CONCLUSIONS: Contouring the MCGR impacts both the mechanics of the rod expansion and the prevalence of PJK in EOS patient population.

Characterizing Use of Growth-friendly Implants for Early-onset Scoliosis: A 10-Year Update.

BACKGROUND: Growth-friendly treatment of early-onset scoliosis (EOS) has changed with the development and evolution of multiple devices. This study was designed to characterize changes in the use of growth-friendly implants for EOS from 2007 to 2017. METHODS: We queried the Pediatric Spine Study Group database for patients who underwent index surgery with growth-friendly implants from July 2007 to June 2017. In 1298 patients, we assessed causes of EOS; preoperative curve magnitude; age at first surgery; patient sex; construct type; lengthening interval; incidence of "final" fusion for definitive treatment; and age at definitive treatment. α=0.05. RESULTS: From 2007 to 2017, the annual proportion of patients with idiopathic EOS increased from 12% to 33% (R=0.58, P=0.006). Neuromuscular EOS was the most common type at all time points (range, 33% to 44%). By year, mean preoperative curve magnitude ranged from 67 to 77 degrees, with no significant temporal changes. Mean (±SD) age at first surgery increased from 6.1±2.9 years in 2007 to 7.8±2.5 years in 2017 (R=0.78, P<0.001). As a proportion of new implants, magnetically controlled growing rods increased from <5% during the first 2 years to 83% in the last 2 years of the study. Vertically expandable prosthetic titanium ribs decreased from a peak of 48% to 6%; growth-guidance devices decreased from 10% to 3%. No change was seen in mean surgical lengthening intervals (range, 6 to 9 mo) for the 614 patients with recorded lengthenings. Final fusion was performed in 88% of patients who had undergone definitive treatment, occurring at a mean age of 13.4±2.4 years. CONCLUSIONS: From 2007 to 2017, neuromuscular EOS was the most common diagnosis for patients treated with growth-friendly implants. Patient age at first surgery and the use of magnetically controlled growing rods increased during this time. Preoperative curve magnitude, traditional growing rod lengthening intervals, and rates of final fusion did not change. LEVEL OF EVIDENCE: Level II.

Establishing Consensus on the Best Practice Guidelines for Use of Halo Gravity Traction for Pediatric Spinal Deformity.

BACKGROUND: Although halo gravity traction (HGT) has been used to treat children with severe spinal deformity for decades, there is a distinct lack of high-quality evidence to speak to its merits or to dictate ideal manner of implementation. In addition, no guidelines exist to drive research or assist surgeons in their practice. The aim of this study was to establish best practice guidelines (BPG) using formal techniques of consensus building among a group of experienced pediatric spinal deformity surgeons to determine ideal indications and implementation of HGT for pediatric spinal deformity. METHODS: The Delphi process and nominal group technique were used to formally derive consensus among leaders in pediatric spine surgery. Initial work identified significant areas of variability in practice for which we sought to garner consensus. After review of the literature, 3 iterative surveys were administered from February through April 2018 to nationwide experts in pediatric spinal deformity. Surveys assessed anonymous opinions on ideal practices for indications, preoperative evaluation, protocols, and complications, with agreement of 80% or higher considered consensus. Final determination of consensus items and equipoise were established using the Nominal group technique in a facilitated meeting. RESULTS: Of the 42 surgeons invited, responses were received from 32, 40, and 31 surgeons for each survey, respectively. The final meeting included 14 experts with an average 10.5 years in practice and average 88 annual spinal deformity cases. Experts reached consensus on 67 items [indications (17), goals (1), preoperative evaluations (5), protocols (36), complications (8)]; these were consolidated to create final BPG in all categories, including statements to help dictate practice such as using at least 6 to 8 pins under 4 to 8 lbs of torque, with a small, tolerable starting weight and reaching goal weight of 50% TBW in ∼2 weeks. Nine items remained items of equipoise for the purposes of guiding future research. CONCLUSIONS: We developed consensus-based BPG for the use and implementation of HGT for pediatric spinal deformity. This can serve as a measure to help drive future research as well as give new surgeons a place to begin their practice of HGT. LEVEL OF EVIDENCE: Level V-expert opinion.

Early Onset Scoliosis: Is there an Improvement in Quality of Life With Conversion From Traditional Growing Rods to Magnetically Controlled Growing Rods?

BACKGROUND: Modern treatment of early onset scoliosis (EOS) includes implantation of traditional growing rods (TGR) or magnetically controlled growing rods (MCGR) for posterior-based distraction if nonoperative treatment plans failed. The recent MCGR innovation has largely been expected to improve patient and family burden by avoiding frequent return to the operating room. At least one study has shown no HRQoL difference between TGR and MCGR, but none have specifically investigated a cohort of conversion patients whose families have experienced both. METHODS: An EOS database was queried for all patients treated with TGR, MCGR, and those converted from TGR to MCGR who had at least 1 pretreatment and 1 posttreatment HRQoL assessment (EOSQ-24). Data on demographics, diagnoses, and major curve size, as well as EOSQ-24 domain scores were studied. RESULTS: There were 156 TGR, 114 MCGR, and 32 conversion patients, with an average of 2.0 years between first and final EOSQ-24 surveys. There was no significant difference in outcome in any EOSQ-24 domain between the converts and the other 2 treatment groups. There was a significant post-op improvement in MCGR versus TGR in transfers and energy level (P=0.01; P=0.01). CONCLUSIONS: Although patient families and their surgeons may subjectively report improved HRQoL after conversion from TGR lengthenings to in-office MCGR lengthenings, these improvements were seen in transfer and fatigue domains, not burden or satisfaction. Although the EOSQ-24 is a well-validated EOS metric, it may not be sensitive enough to overcome the QoL floor-effect associated with the diagnosis itself, or perhaps the improvement in QoL must be assessed over a longer interval to assess meaningful change. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Observed Length Increases of Magnetically Controlled Growing Rods are Lower Than Programmed.

BACKGROUND: Magnetically controlled growing rods (MCGRs) are increasingly used in the treatment of early onset scoliosis (EOS). Few studies have reported whether desired lengthening can reliably be achieved, or if prior spine instrumentation and large tissue depths affect lengthening. In this clinical study of EOS patients, it was hypothesized that increases in rod length would equal programmed increases, patients with prior spine instrumentation would lengthen less than patients without prior surgery, and larger tissue depths would decrease lengthening success. METHODS: A retrospective chart review was conducted on EOS patients with single and dual MCGRs placed between April 2014 to September 2015 and distracted at a single institution. Rod distraction was measured at each visit using ultrasound. Differences between programmed and actual distraction for each patient, and between groups with and without prior spine instrumentation, were determined by 2-tailed t tests. Regression and correlation were used to determine the relationship between tissue depth and length increases. RESULTS: Thirty-one patients were included, 18 males, 13 females, age 8.1 (±2.5) years, with major curves measuring 60 (±14.6) degrees at time of MCGR insertion. In the 12 patients with prior instrumentation, time from initial growing rod placement to MCGR insertion was 23.1 (±10.6) months. The number of surgical procedures before MCGR insertion was 2.8 (±2.0). Total length increase relative to the programmed distraction was 86% (±21) (P<0.001). Length increases for patients with and without prior surgery were 87% (±23) and 86% (±19), respectively (P>0.9). Total lengthening was inversely proportional to tissue depth (r=0.38, P<0.01); the decrease in lengthening achieved was 2.1%/mm of tissue depth. CONCLUSIONS: Increases in rod length were 14% lower than the programmed distraction. Prior instrumentation did not impact the amount of rod distraction. Greater distance between the rod and the skin surface negatively affected the magnitude of distraction.

Initial Cast Correction as a Predictor of Treatment Outcome Success for Infantile Idiopathic Scoliosis.

BACKGROUND: Cast treatment for infantile idiopathic scoliosis patients ultimately corrects deformity in varying amounts. As the reasons for these differential outcomes are not fully elucidated, the aim of this study was to identify clinical and radiographic variables correlated with better cast correction. METHODS: Patients in the Children's Spine Study Group and Growing Spine Study Group registries who underwent cast treatment for idiopathic scoliosis between 2005 and 2013 with 1-year minimum follow-up were included. Data including major curve and rib-vertebra angle difference before cast, initial in-cast application, after cast treatment, and at most recent follow-up were collected. Univariable and multivariable regression analyses were used to identify factors associated with lower major curves at most recent follow-up. RESULTS: A total of 68 patients were identified and followed for a mean of 2.5 (range, 1.1 to 5.4) years after cast treatment. Cast treatment lasted an average of 16.7 months, with a median of 6 cast applications (range, 2 to 19). Twenty-five subjects (37%) had a most recent major curve <15 degrees (success), whereas 43 had a curve that was >15 degrees (unresolved). Multivariable linear regression determined that younger age (P=0.02), smaller precast major curve (P<0.001), and greater percent major curve correction in initial cast (P=0.006) were associated with smaller major curve at most recent follow-up. Multivariable logistic regression determined that success patients were younger than unresolved patients (average age, 1.4 vs. 2.1 y; P=0.003), and had smaller in-cast major curves after initial cast application (average, 18 vs. 27 degrees; P=0.002). CONCLUSIONS: Infantile idiopathic scoliosis patients casted at an earlier age, with smaller major curves, and greater percent major curve correction in initial cast have the best prognosis. Patients' percent major curve correction, which may represent curve flexibility and/or cast quality, is a predictor of treatment success when age and precast major curve are also taken into account. LEVEL OF EVIDENCE: Level III-retrospective study.

Utilizing the "Stable-to-be Vertebra" Saves Motion Segments in Growing Rods Treatment for Early-Onset Scoliosis.

BACKGROUND: There is no consensus on the selection of distal instrumentation levels in growing rod surgery. Many surgeons utilize the stable zone of Harrington, but there is not overwhelming evidence to support this preference. The aim of this study was to determine the value of bending/traction radiographs in selection of distal instrumentation levels of a growing rod construct in children with idiopathic or idiopathic-like early-onset scoliosis (EOS). METHODS: Twenty-three consecutive patients with idiopathic or idiopathic-like EOS who underwent growing rod surgery at 2 separate institutions between 2006 and 2011 were included. Lengthening procedures were performed periodically at 6-month intervals. Analyses were performed retrospectively for age at index surgery, follow-up period, and radiographic measurements. Lower instrumented levels, neutral vertebra, stable vertebrae (SV), and stable-to-be vertebrae (StbV) were identified on the preoperative radiographs. Coronal Cobb angles, tilt of lower instrumented vertebra (LIV) and LIV+1, and disk wedging under the LIV and LIV+1 were measured on the early postoperative and latest follow-up radiographs. RESULTS: Average age at index surgery was 83.6 months. Mean follow-up period was 68.1 months. Initial analysis showed that the relationship of LIV to the StbV was a better predictive of LIV+1 tilt than the SV at the final follow-up. Therefore, the patients were grouped according to the relationship of the LIV to the StbV. LIV was the StbV in 9 patients, proximal to the StbV in 8 patients, and distal to the StbV in 6 patients. At the latest follow-up, tilt of LIV+1 exceeded 10 degrees in 7 of the 8 patients where LIV was proximal to the StbV, whereas only in 1 of 9 patients where LIV was StbV, and in none of the 6 patients where LIV was distal to the StbV. The data indicate that selection of the StbV as the LIV could spare an average of 1.8 vertebral segments when compared with the SV, as StbV is never distal but almost always proximal to the SV. CONCLUSIONS: Choosing the StbV as the LIV saves motion segments and prevents distal adding on, while providing satisfactory deformity correction in idiopathic and idiopathic-like EOS. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

Surgical aspects of spinal growth modulation in scoliosis correction.

Spine growth modulation for scoliosis correction is a technique for slowing growth on the convex side of the curve and enhancing growth on the concave side by using the Heuter-Volkmann principle; this results in gradual deformity correction. The theoretic advantages include speedier recovery because of the minimally invasive approach used, as well as motion preservation. Several devices have been used in humans, including vertebral body stapling, with either a flexible titanium clip or a nitinol staple, and anterior spinal tethering. Prerequisites for the use of these devices are a relatively flexible curve and sufficient remaining growth in the patient. Although vertebral body stapling is effective for moderate curves of less than 40°, anterior spinal tethering can be used for curves greater than 40°. The titanium clip and spinal tethers are used exclusively for thoracic scoliosis, whereas nitinol staples can be used for the thoracic spine or the lumbar spine. The thoracoscopic technique is used for thoracic instrumentation, and the mini-open retroperitoneal technique is used for lumbar staple insertion. The insertion of a titanium clip and an anterior spinal tether requires sacrifice and mobilization of the segmental vessels, whereas nitinol staples can be inserted without such sacrifice. Single lung ventilation and CO2 insufflation are used to improve visualization with the thoracoscope. The curve should be instrumented from an end vertebra to an end vertebra. Postoperative immobilization depends on the type of device used. Most complications are approach related, such as atelectasis caused by a mucus plug, pain at the chest tube site, and pneumothorax. Device-related complications are rare. Overcorrection is a concern. In patients with early onset scoliosis, a hybrid construct with vertebral stapling and growing rods or a vertical expandable prosthetic titanium rib has been suggested. A failure of the spinal growth modulation procedure does not preclude spinal fusion. None of the devices for spine growth modulation have been approved by the FDA for human use and are still investigational. Early results are promising, and continued clinical studies are necessary.

Dural ectasia in a child with Larsen syndrome.

INTRODUCTION: We present a case of an incidental finding of dural ectasia in a child diagnosed with Larsen syndrome. Larsen syndrome is a rare inherited disorder of connective tissue characterized by facial dysmorphism, congenital joint dislocations of the hips, knees and elbows, and deformities of the hands and feet. Dural ectasia is as an abnormal expansion of the dural sac surrounding the spinal cord and may result in spinal morphologic changes, instability, and spontaneous dislocation. To the best of our knowledge, the presence of dural ectasia in Larsen syndrome has not previously been reported. CASE STUDY: A 6-year-old boy diagnosed with Larsen syndrome presented with an upper thoracic curve measuring 74 degrees, a right thoracic curve measuring 65 degrees, and significant cervicothoracic kyphosis with 50% anterior subluxation of C6 on C7 and C7 on T1. Advanced imaging studies showed dural ectasia (evidenced by spinal canal and dural sac expansion), thinning of pedicles and lamina, and C4 and C6 pars defects with cervical foramen enlargement. The patient received growing rod instrumentation (attached to cervical spine fixation) by a combined anterior/posterior surgical approach using intraoperative halo. Complications included intraoperative medial breach (fully resolved), wound dehiscence, 2 instances of bilateral broken rods, and a broken cervical rod. Following 7 lengthening procedures, the patient underwent definitive fusion. DISCUSSION: Surgeons should be aware of the potential for dural ectasia in patients with Larsen syndrome. Its presence will cause difficulties in the surgical intervention for spinal deformity. Multiple factors must be considered, and surgical approach and technique will require modification to avoid complications. Although dural ectasia confounds surgical intervention in these patients, surgery still appears to outweigh the risks associated with delayed intervention. The presence of dural ectasia should not preclude surgical decompression and stabilization. This report adds to the body of knowledge on the treatment of Larsen syndrome by demonstrating the potential existence of dural ectasia and highlights the importance of careful and thorough preoperative evaluation and diagnostic imaging.

Growing Rod versus Posterior Spinal Fusion Treatment of Juvenile Idiopathic Scoliosis: Unique Characteristics and Surgical Outcomes.

Progressive spinal curvature in juvenile idiopathic scoliosis (JIS) is challenging to treat. When conservative management fails, treatments include growing rods (GRs) or posterior spinal fusion (PSF). The purpose of this study is to compare the patient characteristics and outcomes of GR and PSF treatment of JIS. We performed a retrospective review of demographic, radiographic, and surgical data for all JIS patients requiring surgical treatment between 2012 and 2020. Patients who underwent any GR treatment were compared to PSF patients. A total of 36 patients (13 GR, 23 PSF) were reviewed. PSF patients had a larger pre-operative spinal height (p = 0.002), but similar pre-operative major curve magnitudes (p = 0.558). PSF treatment resulted in similar change in the T1-S1 length (p = 0.002), but a greater correction of the curve magnitude (p < 0.055) compared to GR patients. Eight patients initially treated with GRs later underwent definitive PSF treatment. This subset of patients had a greater spinal height before PSF (p = 0.006), but similar immediate post-PSF T1-S1 lengths (p = 0.437) and smaller changes in spinal height from PSF (p = 0.020) than primary PSF patients. At final follow-up, patients who underwent primary PSF versus PSF after GR had similar spinal heights (p = 0.842). The surgical intervention chosen to manage progressive JIS often differs based on patient characteristics. While this choice may impact immediate outcomes, the outcomes at final follow up are similar.

Lowest instrumented vertebrae in early onset scoliosis: is there a role for a more selective approach?

PURPOSE: This purpose of this study was to assess the impact of patient and implant characteristics on LIV selection in ambulatory children with EOS and to assess the relationship between the touched vertebrae (TV), the last substantially touched vertebrae (LSTV), the stable vertebrae (SV), the sagittal stable vertebrae (SSV), and the LIV. METHODS: A multicenter pediatric spine database was queried for patients ages 2-10 years treated by growth friendly instrumentation with at least 2-year follow up. The relationship between the LIV and preoperative spinal height, curve magnitude, and implant type were assessed. The relationships between the TV, LSTV, SV, SSV, and the LIV were also evaluated. RESULTS: Overall, 281 patients met inclusion criteria. The LIV was at L3 or below in most patients with a lumbar LIV: L1 (9.2%), L2 (20.2%), L3 (40.9%), L4 (29.5%). Smaller T1 - T12 length was associated with more caudal LIV selection (p = 0.001). Larger curve magnitudes were similarly associated with more caudal LIV selection (p = < 0.0001). Implant type was not associated with LIV selection (p = 0.32) including MCGR actuator length (p = 0.829). The LIV was caudal to the TV in 78% of patients with a TV at L2 or above compared to only 17% of patients with a TV at L3 or below (p < 0.0001). CONCLUSIONS: Most EOS patients have an LIV of L3 or below and display TV-LIV and LSTV-LIV incongruence. These findings suggest that at the end of treatment, EOS patients rarely have the potential for selective thoracic fusion. Further work is necessary to assess the potential for a more selective approach to LIV selection in EOS. LEVEL OF EVIDENCE: III.

Development of Consensus-Based Best Practice Guidelines for the Peri- and Post-operative Care of Pediatric Patients with Spinal Deformity and Programmable Implanted Devices.

STUDY DESIGN: Modified Delphi consensus study. OBJECTIVE: To develop consensus-based best practices for the care of pediatric patients who have implanted programmable devices (IPDs) and require spinal deformity surgery. SUMMARY OF BACKGROUND DATA: Implanted programmable devices (IPDs) are often present in patients with neuromuscular or syndromic scoliosis who require spine surgery. Guidelines for monitoring and interrogating these devices during the peri-operative period are not available. METHODS: A panel was assembled consisting of 25 experts (i.e., spinal deformity surgeons, neurosurgeons, neuro-electrophysiologists, cardiologists, and otolaryngologists). Initial postulates were based on literature review and results from a prior survey. Postulates addressed the following IPDs: vagal nerve stimulators (VNS), programmable ventriculo-peritoneal shunts (VPS), intrathecal baclofen pumps (ITBP), cardiac pacemakers and implantable cardioverter-defibrillators (ICD), deep brain stimulators (DBS), and cochlear implants. Cardiologist and otolaryngologists participants responded only to postulates on cardiac pacemakers or cochlear implants, respectively. Consensus was defined as ≥80% agreement, items that did not reach consensus were revised and included in subsequent rounds. A total of three survey rounds and one virtual meeting were conducted. RESULTS: Consensus was reached on 39 total postulates across six IPD types. Postulates addressed general spine surgery considerations, use of intraoperative monitoring and cautery, use of magnetically-controlled growing rods (MCGRs), and use of an external remote controller to lengthen MCGRs. Across IPD types, consensus for the final postulates ranged from 94.4-100%. Overall, experts agreed that MCGRs can be surgically inserted and lengthened in patients with a variety of IPDs and provided guidance for the use of intraoperative monitoring and cautery, which varied between IPD types. CONCLUSION: Spinal deformity correction surgery often benefits from the use of intraoperative monitoring, monopolar and bipolar cautery, and MCGRs. Final postulates from this study can inform the peri- and post-operative practices of spinal deformity surgeons who treat patients with both scoliosis and IPDs. LEVEL OF EVIDENCE: V- Expert opinion.

Predictors of emotional functioning in youth after surgical correction of idiopathic scoliosis.

BACKGROUND: Patients with idiopathic scoliosis, although otherwise healthy, often have significant concerns about their self-image and appearance. In a group of juveniles and adolescents, this can impact adjustment in school, functioning in peer groups, and general sense of well-being. There are limited data to help physicians reliably and precisely identify those who are at higher risk of poor emotional adjustment even after spine deformity correction. The purpose of this study was to determine the predictors of emotional maladjustment in juvenile and adolescent patients after surgical correction of idiopathic scoliosis. METHODS: A total of 233 juveniles, mean age 11.26 ± 1.02 (range, 8 to 12) years and 909 adolescents, mean age 14.91 ± 1.61 (range, 13 to 21) years, who underwent surgical correction for idiopathic scoliosis and who were participating in a prospective longitudinal multicenter database, were enrolled in the study. Participants completed the Scoliosis Research Society-22 (SRS-22) questionnaire before surgery and 2 years postoperatively. Radiographs were used to measure Cobb angle and surface measurements were used to determine decompensation and trunk shift. RESULTS: Adolescents reported poorer mental health preoperatively (P<0.05) and 2 years postoperatively (P<0.001) than juveniles; however, both groups reported improved mental health (P<0.001) and self-image (P<0.01) postoperatively. Mental health 2 years postoperatively was predicted by preoperative self-image (P<0.05), mental health (P<0.001), and main thoracic Cobb angle (P<0.05) in the juvenile group. Within the adolescent group, mental health 2 years postoperatively was predicted by preoperative mental health (P<0.001); self-image 2 years postoperatively was predicted by preoperative mental health (P<0.01) and self-image (P<0.001). CONCLUSIONS: Self-image and mental health are significantly improved after spine deformity correction in juveniles and adolescents with idiopathic scoliosis. However, consistent with normative development, adolescents are at higher risk for emotional maladjustment than juveniles. Surgical decision making in scoliosis correction should take the emotional status of the patient into consideration.

Challenges of spine surgery in patients with chondrodysplasia punctata.

BACKGROUND: Chondrodysplasia punctata (CDP) is a common manifestation of an etiologically heterogenous group of disorders. There is very little data regarding the development and management of spinal deformity in patients with CDP. The purpose of this study was to present a multicenter series of CDP, to describe the surgical outcomes of spinal deformities in CDP patients and to emphasize important considerations that may influence choice of surgical treatment of spinal deformity in this patient population. METHODS: The medical records and spinal radiographs of patients with the diagnosis of CDP followed in 2 centers between 1975 and 2011 were retrospectively reviewed. Epiphyseal stippling was present on radiographs in all patients who fulfilled the clinical criteria. RESULTS: Among the 17 patients who were diagnosed with CDP, 13 had spinal deformities. The mean age at diagnosis of spinal deformity was 14.6 months (range, 1 wk to 9 y). Males and females were close to equally represented (10 males and 7 females). Twelve patients (92%) required surgery to correct spinal deformity. Patients were followed for a median of 8.4 years (range, 2.8 to 19.5 y). The total number of surgical procedures performed was 17 averaging 1.5 per patient. Four patients required >1 procedure. Eighty percent of the patients who required >1 surgical procedure were females with probable diagnosis of X-linked dominant CDP. Revision surgery was indicated in 50% of the patients treated with combined anterior and posterior fusion and 20% of the patients treated with posterior fusion alone. CONCLUSIONS: Spinal deformity in CPD patients may range from significant kyphoscoliosis to minimal deformity that does not require any treatment. For those patients in whom spine surgery was indicated, a high incidence of revision surgery and curve progression after fusion was recorded. Female patients with probable diagnosis of X-linked dominant CDP were more likely to require a second surgical procedure. Isolated posterior fusion showed less favorable results compared with combined anteroposterior fusion in terms of revision surgery. LEVEL OF EVIDENCE: Level IV-therapeutic study.