Quantitative Sensory Changes Following Posterior Spinal Fusion to Treat Adolescent Idiopathic Scoliosis.

BACKGROUND: Sensory changes surrounding the incision frequently develop after posterior spinal fusion (PSF) to treat adolescent idiopathic scoliosis (AIS). Anecdotally, patients may experience sensory changes on the chest wall. Such postsurgical sensory changes are not well described quantitatively. This study aims to evaluate the presence, intensity, and duration of mechanical sensory changes in AIS patients postoperatively. METHODS: A prospective cohort of AIS patients, 10 to 21 years old, was followed. Quantitative sensory testing (QST) included touch detection threshold [mechanical detection threshold (MDT)] and pain detection threshold (MPT), using VonFrey monofilaments and pinprick stimulators. QST was performed at 3 sites at T6: the right and left chest at the nipple line and adjacent to the incision below the inferior angle of the scapula. QST at the thenar eminence was the control. QST was collected at baseline, 3 days, 1, and 6 months postoperative. RESULTS: Thirty-four patients (21% males; mean age: 14.9 years old; median preoperative curve: 58 degrees) completed all testing. Mean deformity correction was 64% (SD: 10.4). Adjacent to the incision site, MDT was significantly higher compared with baseline at 3 days and 1 month ( P < 0.001) but not at 6 months ( P = 0.19), whereas MPT was significantly higher at 3 days, ( P < 0.001), 1 month ( P < 0.001), and 6 months ( P = 0.001). For the chest wall in all patients, MPT was higher on the left chest at 3 days ( P = 0.04) and on the right chest at 3 days ( P = 0.022) and 1 month ( P = 0.05). For patients with right-sided curves, MDT ( P = 0.01) and MPT ( P = 0.015) overall were significantly higher on the concave side (left) chest postoperatively. CONCLUSIONS: PSF is associated with sensory disturbances that are detectable within days, persist at 1 month, and improve at 6 months postoperatively adjacent to the incision and on the chest wall. We suspect that these sensory changes are transient. Describing postoperative sensory changes will help us better set postoperative expectations for patients undergoing PSF. LEVEL OF EVIDENCE: Level I.

Prevertebral Soft Tissue Thickness of the Cervical Spine in Children: An Insensitive but Specific Aid in the Diagnosis of Occult Trauma.

BACKGROUND: The purpose of this study was to investigate the sensitivity and specificity of current cervical prevertebral soft tissue swelling (PVST) values in a cohort of children with known cervical fractures or dislocations. METHODS: Forty two children (average age 11.9, range 1.4 to 17.0 y) with documented cervical spine injury and 61 children (average age 11.9, range 0.5 to 17.9 y) with cervical pain but no injury were reviewed (January 2004 to December 2015). PVST was measured on lateral cervical radiographs at C2, C3, and C6. Patients were stratified by age (0 to 2 y, 3 to 6 y, 7 to 10 y, 11 to 15 y, and 16 y and above). The Wilcoxon rank sum test was used to compare PVST measurements at each spine level across injury and noninjury cohorts. Sensitivity and specificity were estimated to assess the ability of abnormal reference values to detect when a true injury was present. In addition, positive predictive value and negative predictive value were also estimated. RESULTS: The majority of c-spine injuries (31/42; 76%) involved bony fracture and 57% (24/42) were treated with a collar or brace. Comparison of PVST measurement found no difference at C2 ( P =0.07), C3 ( P =0.07), or at C6 ( P =0.99) across injury and non-injury cohorts. Sensitivity was poor at single-level measures for C2 (26%), C3 (31%), and C6 (24%), while specificity was relatively high (92%, 87%, and 79%, respectively). When an increased value at either C2 or C3 indicated injury, sensitivity increased to 36%, and when an increased measurement at just one of the 3 measured levels indicated injury, the sensitivity increased to 48%, while the specificity decreased to 72%. While retropharyngeal measures were more likely to detect injury than retrotracheal, C6 alone was increased in 5 of the 20 injury patients. CONCLUSIONS: PVST measurements exhibit poor sensitivity but good specificity as indicators for the diagnosis of occult cervical trauma in children. Negative values do not exclude injury; positive values suggest further evaluation. LEVELS OF EVIDENCE: Level III.

Spinal Fusion in Pediatric Patients With Low Bone Density: Defining the Value of DXA.

BACKGROUND: Children with medical complexity are at increased risk of low bone mineral density (BMD) and complications after spinal fusion compared with idiopathic scoliosis patients. Our aim was to compare treatments and outcomes of children with medical complexity undergoing spinal fusion in those who had dual-energy x-ray absorptiometry (DXA) scans versus those who did not in an effort to standardize the workup of these patients before undergoing spinal surgery. METHODS: We conducted a retrospective review of patients with low BMD who underwent spinal fusion at a tertiary care pediatric hospital between 2004 and 2016. We consulted with a pediatric endocrinologist to create standard definitions for low BMD to classify each subject. Regardless of DXA status, all patients were given a clinical diagnosis of osteoporosis [at least 2 long bone or 1 vertebral pathologic fracture(s)], osteopenia (stated on radiograph or by the physician), or clinically low bone density belonging to neither category. The last classification was used for patients whose clinicians had documented low bone density not meeting the criteria for osteoporosis or osteopenia. Fifty-nine patients met the criteria, and 314 were excluded for insufficient follow-up and/or not meeting a diagnosis definition. BMD Z -scores compare bone density ascertained by DXA to an age-matched and sex-matched average. Patients who had a DXA scan were also given a DXA diagnosis of low bone density (≤-2 SD), slightly low bone density (-1.0 to -1.9 SD), or neither (>-1.0 SD) based on the lowest BMD Z -score recorded. RESULTS: Fifty-nine patients were analyzed. Fifty-four percent had at least 1 DXA scan preoperatively. Eighty-one percent of DXA patients received some form of treatment compared with 52% of non-DXA patients ( P =0.03). CONCLUSIONS: Patients referred for DXA scans were more likely to be treated for low BMD, although there is no standardized system in place to determine which patients should get scans. Our research highlights the need to implement clinical protocols to optimize bone health preoperatively. LEVEL OF EVIDENCE: Level II-retrospective prognostic study.

Variability in Antibiotic Treatment of Pediatric Surgical Site Infection After Spinal Fusion at A Single Institution.

BACKGROUND: Recent focus on surgical site infections (SSIs) after posterior spine fusion (PSF) has lowered infection rates by standardizing perioperative antibiotic prophylaxis. However, efforts have neglected to detail antibiotic treatment of SSIs. Our aim was to document variability in antibiotic regimens prescribed for acute and latent SSIs following PSF in children with idiopathic, neuromuscular, and syndromic scoliosis. METHODS: This study included patients who developed a SSI after PSF for scoliosis at a pediatric tertiary care hospital between 2004 and 2019. Patients had to be 21 years or younger at surgery. Exclusion criteria included growing rods, staged surgery, and revision or removal before SSI diagnosis. Infection was classified as acute (within 90 d) or latent. Clinical resolution of SSI was measured by return to normal lab values. Each antibiotic was categorized as empiric or tailored. RESULTS: Eighty subjects were identified. The average age at fusion was 14.7 years and 40% of the cohort was male. Most diagnoses were neuromuscular (53%) or idiopathic (41%).Sixty-three percent of patients had an acute infection and 88% had a deep infection. The majority (54%) of subjects began on tailored antibiotic therapy versus empiric (46%). Patients with a neuromuscular diagnosis had 4.0 times the odds of receiving initial empiric treatment compared with patients with an idiopathic diagnosis, controlling for infection type and time (P=0.01). Ninety-two percent of patients with acute SSI retained implants at the time of infection and 76% retained them as of August 2020. In the latent cohort, 27% retained implants at infection and 17% retained them as of August 2020. CONCLUSIONS: Patients with acute infections were on antibiotics longer than patients with latent infections. Those with retained implants were on antibiotics longer than those who underwent removal. By providing averages of antibiotic duration and lab normalization, we hope to standardize regimens moving forward and develop SSI-reducing pathways encompassing low-risk patients. LEVEL OF EVIDENCE: Level III.

Outcomes and Complications in Management of Congenital Myopathy Early-Onset Scoliosis.

BACKGROUND: Congenital myopathies (CMs) are complex conditions often associated with early-onset scoliosis (EOS). The purpose of this study was to investigate radiographic outcomes in CM patients undergoing EOS instrumentation as well as complications. Secondarily, we sought to compare these patients to a population with higher prevalence, cerebral palsy (CP) EOS patients. METHODS: This is a retrospective study of a prospectively collected multicenter registry. The registry was queried for EOS patients with growth-sparing instrumentation (vertical expandable prosthetic titanium ribs, magnetically controlled growing rods, traditional growing rod, or Shilla) and a CM or CP diagnosis with minimum 2 years follow-up. Outcomes included major curve magnitude, T1-S1 height, kyphosis, and complications. RESULTS: Sixteen patients with CM were included. Six (37.5%) children with CM experienced 11 complications by 2 years. Mean major curve magnitude for CM patients was improved postoperatively and maintained at 2 years (P<0.01), with no significant increase in T1-S1 height or maximum kyphosis(P>0.05). Ninety-seven patients with CP EOS were included as a comparative cohort. Fewer CP patients required baseline respiratory support compared with CM patients (20.0% vs. 92.9%, P<0.01). Fifty-four (55.7%) CP patients experienced a total of 105 complications at 2 years. There was no evidence that the risk of complication or radiographic outcomes differs between cohorts at 2 years, though CP EOS patients experienced significant improvement in all measurements at 2 years. CONCLUSIONS: EOS CM children face a high risk of complication after growing instrumentation, with similar curve correction and risk of complication to CP patients. LEVEL OF EVIDENCE: Level III.

Occipital Plate Fixation in the Pediatric Population.

BACKGROUND: Occipital plate fixation has been shown to improve outcomes in cervical spine fusion. There is a paucity of literature describing occipital plate fixation, especially in the pediatric population. The authors reviewed a case series of 34 patients at a pediatric hospital who underwent cervical spine fusion with occipital plate fixation between 2003 and 2016. This study describes how occipital plates aid the cervical spine union in a case series of diverse, complex pediatric patients. METHODS: Our orthopaedic database at our institution was queried for patients undergoing an instrumented cervical spine procedure between 2003 and 2016. Medical records were used to collect diagnoses, fusion levels, surgical technique, and length of hospitalization, neurophysiological monitoring, complications, and revision procedures. RESULTS: Thirty-four patients met the inclusion criteria. The mean age was 10.9 years (range, 3-21 y). Indications for surgery included cervical instability, basilar invagination, and os odontoideum. These indications were often secondary to a variety of diagnoses, including trisomy 21, Klippel-Feil syndrome, and rheumatoid arthritis. The mean length of hospitalization was 10 days (range, 2 to 80 d). There were no cases of intraoperative dural leak, venous sinus bleeding from occipital screw placement, or implant-related complications. Postoperative complications included 2 cases of nonunion. Eight patients (24%) had follow-up surgery, only 3 (9%) of which were instrumentation revisions. Both patients with nonunion had repeat occipitocervical fixation procedures and achieved union with revision. CONCLUSIONS: Occipital plate fixation was successful for pediatric cervical spine fusion in this diverse cohort. The only procedure-related complication demonstrated was delayed union or nonunion and implant loosening (4/34, 12%) and there were no plate-related complications. This novel case series shows that occipital plate fixation is safe and effective for pediatric patients with complex diagnoses. LEVEL OF EVIDENCE: Level IV-case series.

Intraoperative Use of O-arm in Pediatric Cervical Spine Surgery.

INTRODUCTION: Traditionally, fluoroscopy and postoperative computed tomographic (CT) scans are used to evaluate screw position after pediatric cervical spine fusion. However, noncontained screws detected postoperatively can require revision surgery. Intraoperative O-arm is a 3-dimensional CT imaging technique, which allows intraoperative evaluation of screw position and potentially avoids reoperations because of implant malposition. This study's objective was to evaluate the use of intraoperative O-arm in determining the accuracy of cervical implants placed by a free-hand technique using anatomic landmarks or fluoroscopic guidance in pediatric cervical spine instrumentation. METHODS: A single-center retrospective study of consecutive examinations of children treated with cervical spine instrumentation and intraoperative O-arm from 2014 to 2018 was performed. In total, 44 cases (41 children, 44% men) with a mean age of 11.9 years (range, 2.1 to 23.5 y) were identified. Instability (n=16, 36%) and deformity (n=10, 23%) were the most frequent indications. Primary outcomes were screw revision rate, neurovascular complications caused by noncontained screws, and radiation exposure. RESULTS: A total of 272 screws were inserted (60 occipital and 212 cervical screws). All screws were evaluated on fluoroscopy as appropriately placed. Four screws (1.5%) in 4 cases (9%) were noncontained on O-arm imaging and required intraoperative revision. A mean of 7.7 levels (range, 5 to 13) were scanned. The mean CT dose index and dose-length product were 15.2±6.87 mGy and 212.3±120.48 mGy×cm. Mean effective dose was 1.57±0.818 mSv. There was no association between screw location and noncontainment (P=0.129). No vertebral artery injuries, dural injuries, or neurologic deficits were related to the 4 revised screws. CONCLUSIONS: Intraoperative non-navigated O-arm is a safe and efficient method to evaluate screw position in pediatric patients undergoing cervical spine instrumentation. Noncontained screws were detected in 9% of cases (n=4). O-arm delivers low radiation doses, allows for intraoperative screw revision, and negates the need for postoperative CT scans after confirmation of optimal implant position. LEVEL OF EVIDENCE: Level IV.

Acute Implant-related Complications in Pediatric Cervical Spine Fusion.

BACKGROUND: The use of modern rigid instrumentation in pediatric cervical fusions decreases the risk of implant-related complications, both acute and long term. However, previous studies have indicated that acute implant-related issues still occur in the adult population. Reports of pediatric acute implant complications, occurring within 3 months of surgery, are under-represented in the literature. The purpose of this study is to document the prevalence of acute implant-related complications in a pediatric cervical fusion population. METHODS: A retrospective review of instrumented cervical fusions from August 2002 to December 2018 was conducted. Acute implant-related complications were defined as malposition, fracture, or disengagement of cervical instrumentation, including screws, rods, and plates, within 90 days of surgery. RESULTS: A total of 166 cases were included (55% male individuals) with an average age at surgery of 12.5 years (SD, ±5.28). Acute implant-related complications occurred in 5 patients (3%). All 5 patients had a syndromic diagnosis: Loeys-Dietz (n=1), osteopetrosis (n=1), neurofibromatosis (n=1), trisomy 20 (n=1), and achondroplasia (n=1). One case involved asymptomatic screw protrusion, 1 case lateral mass screw pull-out, 2 more had screw-rod disengagement, and the last experienced dislodgement of the anterior plate. The median time until the presentation was 25 days (range, 1 to 79 d). All patients (n=5) required surgical revision. CONCLUSIONS: This case series suggests that the overall incidence of acute cervical implant failure is low. However, failure is more likely to occur in patients with underlying syndromes compared with patients with different etiologies. Intraoperative use of 3-dimensional computed tomography imaging is recommended to evaluate the screw position and potentially avoid later surgery.

Why Irrigate for the Same Contamination Rate: Wound Contamination in Pediatric Spinal Surgery Using Betadine Versus Saline.

BACKGROUND: The risk of surgical site infection in pediatric posterior spine fusion (PSF) is up to 4.3% in idiopathic populations and 24% in patients with neuromuscular disease. Twenty-three percent of pediatric PSF tissue cultures are positive before closure, with a higher rate in neuromuscular patients. Our primary aim was to evaluate the feasibility of a complete randomized controlled trial to study the efficacy of surgical site irrigation with povidone-iodine (PVP-I) compared with sterile saline (SS) to reduce the bacterial contamination rate before closure in children undergoing PSF. METHODS: One hundred seventy-five subjects undergoing PSF were enrolled in a multicenter, single-blind, pilot randomized controlled trial. We recruited patients at low-risk (LR) and high-risk (HR) for infection 3:1, respectively. Before closure, a wound culture was collected. Nonviable tissues were debrided and the wound was soaked with 0.35% PVP-I or SS for 3 minutes. The wound was then irrigated with 2 L of saline and a second sample was collected. RESULTS: One hundred fifty-three subjects completed the protocol. Seventy-seven subjects were allocated to PVP-I (18 HR, 59 LR) and 76 to SS (19 HR, 57 LR). Cultures were positive in 18% (14/77) of PVP-I samples (2 HR, 12 LR) and in 17% (13/76) of SS samples (3 HR, 10 LR) preirrigation and in 16% (12/77) of PVP-I samples (5 HR, 7 LR) and in 18% (14/76) of SS samples (4 HR, 10 LR) postirrigation. Eight percent (3/37) HR subjects (1 PVP-I, 2 SS) experienced infection at 30 days postoperative. No LR subjects experienced infection. CONCLUSIONS: Positive cultures were similar across treatment and risk groups. The bacterial contamination of wounds before closure remains high regardless of irrigation type. A complete randomized controlled trial would be challenging to adequately power given the similarity of tissue positivity across groups. LEVEL OF EVIDENCE: Level II-pilot randomized controlled trial.

Growth-Friendly Spine Surgery in Escobar Syndrome.

BACKGROUND: The aims of this study were to characterize the spinal deformity of patients with Escobar syndrome, describe results of growth-friendly treatments, and compare these results with those of an idiopathic early-onset scoliosis (EOS) cohort to determine whether the axial stiffness in Escobar syndrome limited correction. METHODS: We used 2 multicenter databases to review the records of 8 patients with EOS associated with Escobar syndrome who had at least 2-year follow-up after initiation of growth-friendly treatment from 1990 to 2016. An idiopathic EOS cohort of 16 patients matched for age at surgery (±1 y), postoperative follow-up (±1 y), and initial curve magnitude (±10 degrees) was identified. A randomized 1:2 matching algorithm was applied (α=0.05). RESULTS: In the Escobar group, spinal deformity involved 7 to 13 vertebrae and ranged from no vertebral anomalies in 3 patients to multiple segmentation defects in 6 patients. Mean age at first surgery was 5 years (range, 1.4 to 7.8 y) with a mean follow-up of 7.5 years (range, 4.0 to 10 y). Mean major curve improved from 76 degrees at initial presentation, to 43 degrees at first instrumentation, to 37 degrees at final follow-up (both P<0.001). Mean pelvic obliquity improved from 16 degrees (range, 5 to 31 degrees) preoperatively to 4 degrees (range, 0 to 8 degrees) at final follow-up (P=0.005). There were no differences in the mean percentage of major curve correction between the idiopathic EOS and Escobar groups at the immediate postoperative visit (P=0.743) or final follow-up (P=0.511). There were no differences between the cohorts in T1-S1 height at initial presentation (P=0.129) or in growth per month (P=0.211). CONCLUSIONS: Multiple congenital fusions and spinal curve deformity are common in Escobar syndrome. Despite large areas of congenital fusion, growth-friendly constructs facilitate spinal growth and improve curve correction. These results are comparable to those in idiopathic EOS. LEVEL OF EVIDENCE: Level III-case-control study.

Staged Growing Rods With Preimplantation of Spinal Anchors for Complex Early Onset Scoliosis.

BACKGROUND: Treatment of early onset scoliosis (EOS) with growing rods (GR) can be challenging in patients with significant deformity, hyperkyphosis, or poor bone quality, due to risks of neurological deficit and hardware pull-out. The objective of this study is to report a series of EOS patients managed with a 2-stage GR technique used to minimize these complications. METHODS: Two-stage GR technique was performed in 8 patients at mean age of 5.4 (range, 3.4 to 7.9) years. At stage 1, proximal and distal anchors were implanted with local fusion. At stage 2, the distraction rods were inserted. There were at least 3 months between stages 1 and 2, and halo-gravity traction was used before stage 2 unless contraindicated. Demographic, clinical, and surgical data were retrospectively reviewed with mean 4.9 (range, 2.0 to 9.4) years of follow-up. Radiographic measurements including Cobb and kyphosis angles were evaluated before stage 1, after halo-gravity traction, after stage 2, and at last follow-up. Indications for staging, anchor healing time, and complications were collected and analyzed. RESULTS: Indications for 2-stage surgery were poor bone quality in 5 patients and neurological changes during initial attempt at GR placement in 3 patients. The mean time between stage 1 and 2 was 23 (15 to 45) weeks. Patients have undergone mean 7 (3 to 16) lengthenings. Three patients have been converted to magnetically controlled GR. The major coronal Cobb angle improved from mean 81 degrees (range, 61 to 97) preoperatively to 40 degrees (24 to 50) after stage 2 and remained at 40 degrees (27 to 53) at last follow-up. Kyphosis remained controlled from 45 degrees (10 to 76) preoperatively to 38 degrees (9 to 61) after stage 2 to 41 degrees (17 to 65) at last follow-up. Complications included superficial wound problems (4 patients), broken rods (2 patients), proximal migration (2 patients), and implant prominence (1 patient). At minimum 2-year follow-up, no patients had lingering neurological complications or instrumentation-bone failure of the GR construct. CONCLUSIONS: Two-stage GR technique can effectively be utilized in EOS patients with poor bone quality or in the event of intraoperative neurological changes. Satisfactory deformity correction can be obtained and routine serial lengthenings can take place with minimal complications. LEVEL OF EVIDENCE: Level IV- case series.

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.

Efficacy of nonfusion treatment for subacute and chronic atlanto-axial rotatory fixation in children.

BACKGROUND: Historical guidelines suggest limited efficacy for nonfusion treatment of chronic atlanto-axial rotatory subluxation/fixation (AARF). Surgical fusion has potential side effects; therefore, it is important to understand the role for nonfusion treatment of chronic AARF. This case series examines the success rate of nonfusion treatment. METHODS: A key word search was used to identify all patients with AARF with delayed presentation of ≥ 4 weeks. Fifteen patients met study inclusion criteria and had adequate clinical information for review. Data collected included age, sex, delay in presentation, mechanism of injury, severity of subluxation (Fielding classification), treatment, follow-up, and clinical information at latest follow-up. RESULTS: There were 11 girls and 3 boys with an average age of 7.2 years (range, 1.5 to 12 years). Initial treatment of 3 patients included fusion. Eleven patients were initially treated without fusion. Eight of these 11 patients were successfully reduced with either halter traction, halo gravity traction, and noninvasive halo or halo vest, and reduction was maintained without fusion (73%) at 10.3 years follow-up. Three patients (27%) experienced recurrence of AARF at an average time of 2 weeks (range, 0 to 4 wk) and were treated with fusion. Average delay in presentation for all patients was 18 weeks (range, 4 to 92 wk), whereas it was 6 weeks and 9.3 weeks in those successfully treated without fusion and in those who failed nonfusion treatment, respectively. Age, delay in presentation, and underlying diagnosis were similar for fusion and nonfusion groups. CONCLUSIONS: Although AARF may recur after a trial of reduction and nonfusion treatment, pediatric patients with delayed presentation (>1 month) of AARF may be treated with an initial trial of nonfusion treatment. Parents should be counseled about the risk of recurrence and possible need for ultimate fusion. LEVEL OF EVIDENCE: Level III, therapeutic retrospective comparative study.

Late-presenting dural leak following spine fusion in the pediatric population.

PURPOSE: The purpose is to describe how patients with a late-presenting dural leak (LPDL) after posterior spinal fusion (PSF) was diagnosed and treated at a single institution. METHODS: Of the 1991 patients who underwent a PSF between 2010 and 2018, 6 patients were identified with a clinical course consistent with a potential LPDL. RESULTS: Six patients with median age 16.9 years had onset of headache ranging 1-12 weeks postoperatively (median 6.5 weeks). All six patients presented with positional headache, and half (3/6) presented with emesis. 5/6 patients underwent contrast brain MRI, which demonstrated pachymeningeal enhancement. 4/5 patients with dural enhancement went on to have CT myelogram. Five patients had a CT myelogram, which identified a dural leak in all patients and localized the leak in four of five patients. All patients underwent an epidural blood patch, which resolved the pain in five patients. One patient without relief underwent revision surgery with removal of a medially placed screw and fibrin glue placement resolving symptoms. CONCLUSIONS: Postoperative dural leaks associated with PSF may present in a delayed fashion. The majority of leaks were not associated with screw malposition. In diagnosing patients with suspected LPDL, we suggest brain MRI with contrast as a first step. Most patients with pachymeningeal enhancement shown on contrast brain MRI had dural leaks that were identified through CT myelograms. For patients with a dural leak, if there is no disruption from screws, a blood patch appears to be an effective treatment. LEVEL OF EVIDENCE: IV.

Early Adherence to Prescribed Brace Wear for Adolescent Idiopathic Scoliosis Is Associated With Future Brace Wear.

STUDY DESIGN: Retrospective cohort study. OBJECTIVE: To assess if initial adherence to prescribed brace wear for adolescent idiopathic scoliosis (AIS) predicts future adherence and curve improvement. SUMMARY OF BACKGROUND DATA: AIS bracing can be effective if patients adhere to prescribed brace wear. Previous research has associated age, sex, and brace prescription length with future adherence. We hypothesize that a patient's initial adherence to brace wear may be associated with future adherence and outcomes. MATERIALS AND METHODS: Consecutive AIS patients who met the Scoliosis Research Society (SRS) criteria for bracing from 2015 to 2019 at a single center were reviewed. Patients were stratified into groups based on their adherence during the initial one-month brace wean-in period-adherent patients were defined as wearing the brace >80% of the daily prescribed amount. Brace wear was recorded by a thermosensor and assessed during the wean-in period, six-, 12-, and 24-month postbracing appointments. Statistical testing was conducted to analyze if initial adherence was associated with future adherence, curve change, and bracing success -defined as reaching Risser stage 4 with a Cobb angle <40°. RESULTS: Sixty patients (mean age=12.5 yr) were included, of which the majority were females (83%) with thoracic curves (70%). Thirty-two patients were considered adherent, and this cohort demonstrated improved adherence relative to the nonadherent group at the six-, 12-, and 24-month appointments ( P <0.001). Adherent patients also showed a significant reduction in their scoliosis at the 12-month appointment, unlike nonadherent patients ( P <0.001). Ninety-seven percent of adherent patients achieved bracing success compared with 71% of nonadherent ( P =0.016). Females were more likely to be adherent than males. CONCLUSIONS: Initial adherence to prescribed AIS brace wear was associated with future adherence, bracing success, and curve improvement. Early recognition of nonadherence may offer an opportunity for supportive intervention to improve brace wear behavior.

Outcome assessment in neuromuscular spinal deformity.

Patient-based outcome measures are important tools quantifying the disease-specific and/or global quality of life (QOL) effects of spinal deformity treatment. In patients with neuromuscular disorders such as cerebral palsy, muscular dystrophy, and myelomeningocele, treatment effects must be differentiated from underlying disease functional impairments. In general, the goals of spinal surgery in these patients are to improve QOL by enhancing sitting balance and posture, improving lung and gastrointestinal function, and reducing pain and deformity. In selected patients, improving ambulation and hand function may also be realistic surgical goals. QOL measures specific to both the neuromuscular diagnosis and spinal deformity provide higher quality information on treatment outcomes for a particular patient than standard radiographic measures. This article reviews patient-based outcome measures in spinal deformity patients with neuromuscular disorders, including their development and use in comparative outcome studies in the recent literature.

Early onset scoliosis: modern treatment and results.

BACKGROUND: Early onset scoliosis (EOS) is a potentially fatal, challenging group of diseases the management of which has markedly changed in the last decade. The purpose of this review is to provide the reader with a brief description of each of these new therapeutic modalities, their indications for use, and early clinical results. METHODS: A systematic review of peer-reviewed publications and abstracts related to the treatment of EOS in the last decade was carried out and synthesized into a review of modern treatment methods. RESULTS: Recent advances in techniques and understanding of preserving the thoracic space have improved the morbidity and mortality of children with progressive EOS. Derotational casting may be used in younger patients with curves between 25 and 60 degrees. The vertical expandable prosthetic titanium rib is best suited for patients with thoracic insufficiency syndrome. Single or dual growing rods may be used alone or in combination with vertical expandable prosthetic titanium rib to treat patients with progressive EOS who are not candidates for casting. Shilla technique is an alternative to growing rods that avoids the morbidity of repeated lengthenings but is not as well proven as the techniques described above. Other methods such as automatic growing rods and growth modulation techniques are still investigational, and their role needs to be defined after further study. CONCLUSIONS: Recent advances have improved the treatment of children with EOS. Treatment continues to be challenging with complication rates higher than treatment of idiopathic scoliosis. LEVEL OF EVIDENCE: Level V.

Evaluating the Efficacy of Rib-to-pelvis Growth-friendly Surgery for the Treatment of Non-ambulatory Early-Onset Scoliosis Myelomeningocele Patients.

INTRODUCTION: Early-onset scoliosis (EOS) is a well-known orthopaedic manifestation in patients with myelomeningocele. The rib-based growing system (RBGS) has been proposed as an alternative for these individuals because of the poor outcomes with traditional surgical techniques. We aimed to describe the effect of RBGS in patients with nonambulatory EOS myelomeningocele. METHODS: We retrospectively reviewed the Pediatric Spine Study Group Multicenter Database for all patients with nonambulatory EOS myelomeningocele treated with RBGS from 2004 to 2019. Demographics, surgical data, radiographic findings, and postoperative complications were obtained. The quality-of-life parameters were assessed postoperatively using the Early-onset Scoliosis Questionnaire-24. RESULTS: Thirty patients (18 women; 60%) were patients with nonambulatory EOS myelomeningocele treated with RBGS. The mean age at the initial surgery was 5.3 years. The thoracic (T1-T12) spine height showed a significant increase from initial surgery to the most recent follow-up (P < 0.001). Spine (T1-S1) height was also significantly increased (P < 0.001). The postoperative complication rate was 87%. The Early-onset Scoliosis Questionnaire-24 demonstrates significant improvements in the quality-of-life scores (P = 0.037). CONCLUSION: This study demonstrated that RBGS could improve the reported quality-of-life scores in patients with nonambulatory EOS myelomeningocele when assessed with an EOS-oriented tool. Moreover, we confirmed the ability of RBGS to hold or even correct spinal deformity.

Bracing for juvenile idiopathic scoliosis: retrospective review from bracing to skeletal maturity.

BACKGROUND: Juvenile idiopathic scoliosis (JIS) outcomes with brace treatment are limited with poorly described bracing protocols. Between 49 and 100% of children with JIS will progress to surgery, however, young age, long follow-up, and varying treatment methods make studying this population difficult. The purpose of this study is to report the outcomes of bracing in JIS treated with a Boston brace™ and identify risk factors for progression and surgical intervention. METHODS: This is a single-center retrospective review of 175 patients with JIS who initiated brace treatment between the age of 4 and 9 years. A cohort of 140 children reached skeletal maturity; 91 children had surgery or at least 2 year follow-up after brace completion. Standard in-brace protocol for scoliosis 320° was a Boston brace for 18-20 h/day after MRI (n = 82). Family history, MRI abnormalities, comorbidities, curve type, curve magnitude, bracing duration, number of braces, compliance by report, and surgical interventions were recorded. RESULTS: Children were average 7.9 years old (range 4.1-9.8) at the initiation of bracing. The Boston brace™ was prescribed in 82 patients and nine used night bending brace. Mid-thoracic curves (53%) was the most frequent deformity. Maximum curve at presentation was on average 30 ± 9 degrees, in-brace curve angle was 16 ± 8 degrees, and in-brace correction was 58 ± 24 percent. Patients were braced an average of 4.6 ± 1.9 years. 61/91 (67%) went on to posterior spinal fusion at 13.3 ± 2.1 (range 9.3-20.9) years and curve magnitude of 61 ± 12 degrees. Of those that underwent surgery, 49/55 (86%) progressed > 10°, 6/55 (11%) stabilized within 10°, and 0/55 (0%) improved > 10° with brace wear. No children underwent growth-friendly posterior instrumentation. Of the 28 who did not have surgical correction, 3 (11%) progressed > 10°, 13/28 (46%) stabilized within 10°, and 12/28 (43%) improved > 10° with brace wear. CONCLUSIONS: This large series of JIS patients with bracing followed to skeletal maturity with long-term follow-up. Surgery was avoided in 33% of children with minimal to no progression, and no child underwent posterior growth-friendly constructs. Risk factors of needing surgery were noncompliance and larger curves at presentation.

Postoperative adrenal crisis in an adolescent with Loeys-Dietz syndrome and undiagnosed adrenoleukodystrophy.

PURPOSE: We present an unusual case involving an adolescent who experienced cardiovascular collapse postoperatively. He had more than one rare life-threatening genetic disorder, and herein we review the anesthetic management of a patient with Loeys-Dietz syndrome (LDS) and adrenoleukodystrophy. CLINICAL FEATURES: A 12-yr-old male (41.5 kg, American Society of Anesthesiologists' physical status III) with LDS, a connective tissue disorder, underwent posterior spinal fusion for spondylolisthesis. This patient demonstrated many signs of LDS: bifid uvula, retrognathia, dilated aortic root, aortic arch aneurysm, inguinal hernias and vertebral subluxations, and multiple areas of tortuous intracranial vessels. A fibreoptic intubation was performed and a balanced anesthetic was administered, consisting of opioids and a low-dose volatile agent. The patient was stable throughout surgery, and at the end of the procedure, his trachea was extubated and he was admitted to the intensive care unit. During the postoperative period, the patient became acutely hemodynamically unstable. Initially, the intensive care team considered the differential diagnoses of postoperative hemorrhage, postoperative sepsis, and cardiac failure secondary to aortic dissection. Supportive care was instituted, and these diagnoses were systematically ruled out. The differential diagnosis was expanded, and the patient was treated with corticosteroids after baseline cortisol levels were drawn. Later he was found to have primary adrenal failure, and it was determined by biochemical and genetic blood analysis that he also had adrenoleukodystrophy. CONCLUSIONS: Although Occam's razor states that physicians should exercise diagnostic parsimony when treating patients, it is possible for a patient to have two or more life-threatening unrelated genetic disorders. Consequently, diagnosticians must always develop and test new hypotheses when treating patients.