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Study design Retrospective chart and radiographic review. Purpose To assess the incidence of and variables associated with spinal deformity progression after posterior segmental instrumentation and fusion at a single institution. Progression of the scoliotic deformity after posterior instrumented spinal fusion has been described. Recent studies have concluded that segmental pedicle screw constructs are better able to control deformity progression. Methods Retrospective review of a consecutive series of idiopathic scoliosis patients (n = 89) with major thoracic curves (Lenke types 1–4) treated with posterior segmental instrumentation and fusion. Deformity progression was defined as a 10° increase in Cobb angle between the first-erect and 2-year post-operative radiographs. Clinical and radiographic data between the two cohorts (deformity progression versus stable) were analyzed to determine the variables associated with deformity progression. Results Patients in the deformity progression group (n = 13) tended to be younger (median 13.7 vs. 14.7 years) and experienced a significant change in height (p = 0.01) during the post-operative period compared to the stable group (n = 76). At 2-years post-op, the patients in the deformity progression group had experienced a significantly greater change in upper instrumented vertebra (UIV) angulation, lower instrumented vertebra (LIV) angulation, and apical vertebral translation (AVT). Two-year post-op Scoliosis Research Society questionnaire (SRS-22) scores in the appearance domain were also significantly worse in the deformity progression group. Patients in the deformity progression group had a significantly greater difference between the lowest instrumented vertebra and stable vertebra compared to patients in the stable group (p = 0.001). Conclusions Deformity progression after posterior spinal fusion does occur after modern segmental instrumentation. Segmental pedicle screw constructs do not prevent deformity progression. Skeletally immature patients with a significant growth potential are at the highest risk for deformity progression. In immature patients, extending the fusion distally to the stable vertebra may minimize deformity progression. Level of evidence Level III.

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.

Recipients of autologous haemopoietic stem-cell transplants (auto-HSCT) have an increased risk of herpes zoster and herpes zoster-related complications. The aim of this study was to establish the efficacy and safety of an inactivated varicella zoster vaccine for the prevention of herpes zoster after auto-HSCT. In this randomised, double-blind, placebo-controlled phase 3 trial, participants were recruited from 135 medical centres (ie, stem-cell transplant centres and hospitals) in North America, South America, Europe, and Asia. Patients were eligible if they were aged 18 years or older, scheduled to receive an auto-HSCT within 60 days of enrolment, and had a history of varicella infection or were seropositive for antibodies to varicella zoster virus, or both. Exclusion criteria included a history of herpes zoster within the previous year of enrolment, and intended antiviral prophylaxis for longer than 6 months after transplantation. Participants were randomly assigned according to a central randomisation schedule generated by the trial statistician, to receive either the inactivated-virus vaccine from one of three consistency lots, a high-antigen lot, or placebo, stratified by age (<50 vs ≥50 years) and intended duration of antiviral prophylaxis after transplantation (≤3 months vs >3 to ≤6 months). Participants, investigators, trial staff, and the funder's clinical and laboratory personnel were masked to group assignment. Participants were given four doses of inactivated vaccine or placebo, with the first dose 5–60 days before auto-HSCT, and the second, third, and fourth doses at about 30, 60, and 90 days after transplantation. The primary efficacy endpoint was the incidence of herpes zoster, confirmed by PCR or adjudication by a masked clinical committee, or both, assessed in all participants randomly assigned to the vaccine consistency lot group or placebo group who received at least one dose of vaccine and had auto-HSCT. Safety was assessed in all randomised participants who received at least one dose of vaccine and had follow-up data. A prespecified vaccine efficacy success criterion required the lower bound of the 95% CI be higher than 25% for the relative reduction of the hazard ratio of herpes zoster infection in participants given the vaccine from one of the consistency lots compared with those given placebo. This trial is registered on ClinicalTrials.gov (NCT01229267) and EudraCT (2010–020150–34). Between Dec 7, 2010, and April 25, 2013, 560 participants were randomly assigned to the vaccine consistency lot group, 106 to the high-antigen lot group, and 564 to the placebo group. 249 (44%) of patients in the vaccine consistency lot group, 35 (33%) in the high-antigen lot group, and 220 (39%) in the placebo group discontinued before study end, mostly because of death or withdrawal. 51 participants were excluded from the primary efficacy endpoint analyses because they did not undergo auto-HSCT or were not vaccinated, or both (22 [4%] in the vaccine consistency lot group, and 29 [5%] in the placebo group). Mean follow-up for efficacy was 2·4 years (SD 1·3) in the vaccine consistency lot group and 2·3 years (SD 1·3) in the placebo group. 42 (8%) of 538 participants in the vaccine consistency lot group (32·9 per 1000 person-years) and 113 (21%) of 535 in the placebo group (91·9 per 1000 person-years) had a confirmed case of herpes zoster. The estimated vaccine efficacy was 63·8% (95% CI 48·4–74·6), meeting the pre-specified success criterion. For the combined vaccine groups versus the placebo group, the proportion of patients with serious adverse events (216 [33%] of 657 vs 181 [33%] of 554; risk difference 0·2%, 95% CI −5·1 to 5·5) and serious vaccine-related adverse events (five [1%] vs five [1%]; risk difference 0·1%, −1·4 to 1·1) were similar. Vaccine-related injection-site adverse events occurred more frequently in participants given vaccine than those given placebo (191 [29%] vs 36 [7%]; risk difference 22·6%, 95% CI 18·5–26·6; p<0·0001). This study shows for the first time in a large phase 3 trial that early vaccination of auto-HSCT recipients during the peri-transplant period can be effective for the prevention of an opportunistic infection like herpes zoster and that the vaccine is well tolerated. Merck & Co., Inc.

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.

Purpose Prior research has demonstrated the influence of preoperative shoulder elevation (SE), proximal thoracic curve magnitude, and upper instrumented vertebra (UIV) on shoulder balance after PSF for AIS. Our purpose was to evaluate the impact of these factors on shoulder balance in early onset idiopathic scoliosis (EOIS) patients treated with growth-friendly instrumentation. Methods This was a multicenter retrospective review. Children with EOIS treated with dual TGR, MCGR, or VEPTR and minimum 2-year follow-up were identified. Demographics and radiographic/surgical data were collected. Results 145 patients met inclusion criteria: 74 had right SE (RSE), 49 left SE (LSE), and 22 even shoulders (EVEN) preoperatively. Mean follow-up was 5.3 years (range, 2.0–13.1 years). The LSE group had a larger pre-index mean main thoracic curve (p = 0.021) but no difference was observed between groups at the post-index or most recent timepoints. RSE patients with UIV of T2 were more likely to have balanced shoulders post-index than patients with UIV of T3 or T4 (p = 0.011). Pre-index radiographic shoulder height (RSH) was predictive of post-index shoulder imbalance ≥ 2 cm in the LSE group (p = 0.007). A ROC curve showed a cut-off of 1.0 cm for RSH. 0/16 LSE patients with pre-index RSH < 1.0 cm had post-index shoulder imbalance ≥ 2 cm compared to 8/28 (29%) patients with pre-index RSH > 1.0 cm (p = 0.006). Conclusion Preoperative LSE > 1.0 cm is predictive of shoulder imbalance ≥ 2 cm after insertion of TGR, MCGR, or VEPTR in children with EOIS. In patients with preoperative RSE, UIV of T2 resulted in a higher likelihood of balanced shoulders postoperatively.

Background Clinically significant proximal junctional kyphosis (PJK) occurs in 20% of children with scoliosis treated with posterior distraction-based growth-friendly surgery. In an effort to identify modifiable risk factors, it has been theorized biomechanically that low radius of curvature (ROC) implants (i.e., more curved rods) may increase post-operative thoracic kyphosis, and thus may pose a higher risk of developing PJK. We sought to test the hypothesis that early onset scoliosis (EOS) patients treated with low ROC distraction-based implants will have a greater risk of developing clinically significant PJK as compared to those treated with high ROC (straighter) implants. Methods We conducted a retrospective review of prospectively collected data obtained from a multi-centre EOS database on children treated with rib-based distraction with a minimum 2-year follow-up. Variables of interest included: implant ROC at index (220 mm or 500 mm), participant age, pre-operative scoliosis, pre-operative kyphosis, and scoliosis etiology. PJK was defined as clinically significant if revision surgery with a superior extension of the upper instrumented vertebrae was performed. Results In 148 participants with scoliosis, there was a higher risk of clinically significant PJK with low ROC (more curved) rods (OR: 2.6 (95% CI 1.09–5.99), χ 2 (1, n  = 148) = 4.8, p  = 0.03). Participants had a mean pre-operative age of 5.3 years (4.6y 220 mm vs 6.2y 500 mm, p  = 0.002). A logistic regression model was created with age as a confounding variable, but it was determined to be not significant ( p  = 0.6). Scoliosis etiologies included 52 neuromuscular, 52 congenital, 27 idiopathic, 17 syndromic with no significant differences in PJK risk between etiologies ( p  = 0.07). Overall, participants had pre-op scoliosis of 69° (67° 220 mm vs 72° 500 mm, p  = 0.2), and kyphosis of 48° (45° 220 mm vs 51° 500 mm, p  = 0.1). The change in thoracic kyphosis pre-operatively to final follow-up (mean 4.0 ± 0.2 years) was higher in participants treated with 220 mm implants compared to 500 mm implants (220 mm: 7.5 ± 2.6° vs 500 mm: − 4.0 ± 3.0°, p  = 0.004). Conclusions Use of low ROC (more curved) posterior distraction implants is associated with a significantly greater increase in thoracic kyphosis which likely led to a higher risk of developing clinically significant PJK in participants with EOS. Level of evidence Level III – retrospective comparative study.