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Background: Sotos syndrome (SS), or cerebral gigantism, describes children with macrocephaly, craniofacial abnormalities, general overgrowth, ligamentous laxity, developmental delay, and neurological disabilities. Fewer than 500 cases have been reported since Sotos and colleagues described the condition in 1964 and no literature exists on the management of spinal deformity in children under 10 years old.The aims of this study were: (1) to characterize the presentation of spinal deformities in patients with SS; and (2) to provide preliminary results of growth-friendly instrumentation (GFI) in these children., Methods: Thirteen children (9 boys) with SS and minimum of 2-year follow-up were identified from 2 multicenter early-onset scoliosis (EOS) databases (1997-2017). Mean age at index surgery and follow-up duration were 5.0 years (range, 1.8 to 10 y) and 7.2 years (range, 2.1 to 14.9 y), respectively. Patients underwent GFI for a mean of 5.7 years (range, 2 to 10.2 y), with an average of 9 lengthenings (range, 2 to 18). Definitive spinal fusion was performed in 4 patients (31%). Major curve magnitude, T1-T12 and T1-S1 lengths, thoracic kyphosis, and lumbar lordosis were evaluated preindex, postindex, latest GFI, and postfusion, when possible., Results: Five thoracolumbar (38%), 4 double major (31%), 2 main thoracic (15%), and 2 double thoracic curves (15%) were seen that spanned a mean of 6.8 levels (5 to 9). Major curves improved 36% (range, 5% to 71%), from a mean of 71 degrees (range, 48 to 90 degrees) to 46 degrees (range, 20 to 73 degrees) postindex surgery (P<0.001). Major curves remained stable at a mean of 52 degrees (range, 20 to 87 degrees) at latest GFI (P=0.36). True T1-T12 and T1-S1 growth velocities during GFI were 0.5 mm/mo (range, 0.4 to 0.8 mm/mo) and 0.8 mm/mo (range, 0.1 to 2.1 mm/mo), respectively. Twenty-six complications occurred in 9 patients (69%) averaging 2 complications per patient (range, 0 to 7)., Conclusions: This is the first study to evaluate the outcomes of GFI in children with SS and EOS. Compared with published data for outcomes of GFI in EOS, children with SS may have less major curve correction. Growth-friendly surgery remains an effective treatment method for EOS in patients with SS., Levels of Evidence: Level IV-retrospective case-series.

Objective: to describe acute brachial diplegia as the initial manifestation of Lyme disease., Background: bilateral, predominantly motor, cervical radiculoplexus neuropathy, the "dangling arm syndrome," has not been reported as a complication of acute Lyme infection., Methods: retrospective series of 5 patients from 2 tertiary neuromuscular centers., Results: there were 4 men and 1 woman with an average age of 69 years. One recalled a tick bite, and preceding constitutional symptoms included headache (2) and fever, arthralgias, and fatigue in 1 patient each. Proximal arm weakness and acute pain developed within 3 weeks from onset; pain was bilateral in 3 patients and unilateral in 2 patients, and was described as severe throbbing. Arm weakness was bilateral at onset in 3 patients, and right sided in 2 patients followed by spread to the left arm within days. All the patients had weakness in the deltoid and biceps that was 3/5 or less (Medical Research Council scale), with variable weakness of the triceps and wrist extensors; 1 patient had a flail right arm and moderate (4/5) weakness of the proximal left arm muscles. Light touch was normal in the regions of weakness, and 1 patient had mildly reduced pin sensation over the forearm. Serum IgM Lyme titers were elevated in all the patients and were detected in the cerebrospinal fluid in 4 tested patients. The cerebrospinal fluid protein ranged between 135 and 176 mg/dL with lymphocytic pleocytosis (range, 42 to 270 cells). Electrodiagnostic studies showed normal median and ulnar motor potentials with asymmetrically reduced sensory amplitudes in the median (4), ulnar (3), and radial, and lateral antebrachial cutaneous potentials in 1 patient each. Two patients had acute denervation in the cervical or proximal arm muscles. There was full recovery after antibiotic therapy in 4 patients and considerable improvement in 1 patient after 2 months., Conclusion: acute brachial diplegia is a rare manifestation of acute Lyme infection and responds promptly to antibiotic therapy.

Study Design: Retrospective case series from a multicenter database., Objective: To evaluate the risk of neurologic injury during growing rod surgeries and to determine whether intraoperative neuromonitoring is necessary for all growing rod procedures., Summary of Background Data: Although the use of growing rod constructs for early-onset spinal deformity has become a commonly accepted treatment, the incidence of neurologic events during growing rod surgeries remains unknown., Methods: We reviewed data from a multicenter database on 782 growing rod surgeries performed in 252 patients. VEPTR devices and any constructs with rib attachments were excluded. A questionnaire was sent to all surgeons contributing cases requesting detailed information about all neurologic events associated with any growing rod surgery., Results: There were 782 growing rod surgeries performed on 252 patients including 252 primary growing rod implantations, 168 implant exchanges, and 362 lengthenings. Five hundred sixty-nine of 782 (73%) cases were performed with neuromonitoring. Only one clinical injury occurred in the series, resulting in an injury rate of 0.1% (1/782). This deficit occurred during an implant exchange while attempting pedicle screw placement, and resolved within 3 months. There were 2 cases with neuromonitoring changes during primary implant surgeries (0.9%, 2/231), 1 change during implant exchanges (0.9%, 1/116), and 1 neuromonitoring change during lengthenings (0.5%, 1/222). The single monitoring change that occurred during a lengthening was in a child with an intracanal tumor who also had a monitoring change during the primary surgery. There are anecdotal cases (outside this study group series) of neuromonitoring changes during simple lengthenings in children with uneventful primary implantations., Conclusion: Based on our study, the largest reported series of growing rod surgeries, the rate of neuromonitoring changes during primary growing rod implantation (0.9%) and exchange (0.9%) justifies the use of intraoperative neuromonitoring during these surgeries. As there were no neurologic events in 361 lengthenings in patients with no previous neurologic events, the question may be raised as to whether intraoperative neuromonitoring is necessary for simple lengthenings in these patients. However, caution should be maintained when interpreting our results as anecdotal cases of neurologic changes from simple lengthenings do exist outside of this series.

Study Design: Review article., Objective: To discuss natural history of congenital scoliosis and kyphosis., Summary of Background Data: Review of previously published literature on natural history of congenital spine deformities., Methods: Medline and google search for congenital scoliosis, kyphosis, and kyphoscoliosis, congenital spine anomalies, deformities, and pathologies, and congenital vertebral anomalies, deformities, and pathologies was performed., Results: Congenital vertebral anomalies have potential to progress and careful assessment and monitoring is essential and early intervention may be desirable., Conclusion: Congenital vertebral anomalies invariably result from disturbed asymmetric growth and can have serious consequences.

Background: We assessed the safety and feasibility of permanent implantation of a novel coronary sinus mitral repair device (PTMA, Viacor Inc)., Methods and Results: Symptomatic (New York Heart Association class 2 or 3) patients with primarily functional mitral regurgitation (MR) were included. A diagnostic PTMA procedure was performed in the coronary sinus venous continuity. MR was assessed and the PTMA device adjusted to optimize efficacy. If MR reduction (> or =1 grade) was observed, placement of a PTMA implant was attempted. Implanted patients were evaluated with echocardiographic, quality of life, and exercise capacity metrics. Nineteen patients received a diagnostic PTMA study. Diagnostic PTMA was effective in 13 patients (MR grade 3.2+/-0.6 reduced to 2.0+/-1.0), and PTMA implants were placed in 9 patients. Four devices were removed uneventfully (7, 84, 197, and 216 days), 3 for annuloplasty surgery due to observed PTMA device migration and/or diminished efficacy. No procedure or device-related major adverse events with permanent sequela were observed in any of the diagnostic or implant patients. Sustained reductions of mitral annulus septal-lateral dimension from 3D echo reconstruction dimensions were observed (4.0+/-1.2 mm at 3 months)., Conclusions: Percutaneous implantation of the PTMA device is feasible and safe. Acute results demonstrate a possibly meaningful reduction of MR in responding patients. Sustained favorable geometric modification of the mitral annulus has been observed, though reduction of MR has been limited. The PTMA method warrants continued evaluation and development.

Study Design: Retrospective analysis of patients treated with single and dual growing rods who had completed their course of treatment, had definitive fusion, and had a minimum of 2 years follow-up., Objectives: To determine which technique was the most effective in the management of severe spinal deformity in young children: control of the spinal deformity, spinal growth, and the incidence of complications., Summary of Background Data: Growing rod techniques provide proximal and distal segmental "claw" foundations, but their overall results through definitive fusion have not been clearly determined., Methods: A total of 28 consecutive patients who had growing rod procedures followed through definitive spinal fusions were analyzed. There were three patient groups: Group 1 (N = 5), single submuscular rod and short apical fusion; Group 2 (N = 16), single growing rod alone; and Group 3 (N = 7), dual growing rods., Results: The interval between initial rod insertion and definitive spinal fusion was similar in all three groups. The best overall results occurred in Group 3, whereas the patients in Group 1 had the worse results. Both Groups 2 and 3 provided good initial correction of the spinal deformity and allowed spinal growth. Group 2 had better frontal and sagittal plane balance and the lowest complication rate., Conclusion: The use of growing rods is effective in controlling severe spinal deformities and allowing spinal growth. Dual rods are stronger than single rods and, therefore, provide better initial correction and maintenance of correction. The use of an apical fusion does not appear to be effective over the course of treatment.

Study Design: A retrospective case review of children treated with dual growing rod technique at our institutions. Patients included had no previous surgery and a minimum of 2 years follow-up from initial surgery., Objectives: To determine the safety and effectiveness of the previously described dual growing rod technique in achieving and maintaining scoliosis correction while allowing spinal growth., Summary of Background Data: Historically, the growing rod techniques have used a single rod and the reported results have been variable. There has been no published study exclusively on the results of dual growing rod technique for early-onset scoliosis., Methods: From 1993 to 2001, 23 patients underwent dual growing rod procedures using pediatric Isola instrumentation and tandem connectors. Diagnoses included infantile and juvenile idiopathic scoliosis, congenital, neuromuscular, and other etiologies. All had curve progression over 10 degrees following unsuccessful bracing or casting. Of 189 total procedures within the treatment period, 151 were lengthenings with an average of 6.6 lengthenings per patient. Analysis included age at initial surgery and final fusion (if applicable), number and frequency of lengthenings, and complications. Radiographic evaluation included measured changes in scoliosis Cobb angle, kyphosis, lordosis, frontal and sagittal balance, length of T1-S1 and instrumentation over the treatment period, and space available for lung ratio., Results: The mean scoliosis improved from 82 degrees (range, 50 degrees-130 degrees) to 38 degrees (range, 13 degrees-66 degrees) after initial surgery and was 36 degrees (range, 4 degrees-53 degrees) at the last follow-up or post-final fusion. T1-S1 length increased from 23.01 (range, 13.80-31.20) to 28.00 cm (range, 19.50-35.50) after initial surgery and to 32.65 cm (range, 25.60-41.00) at last follow-up or post-final fusion with an average T1-S1 length increase of 1.21 cm per year (range, 0.13-2.59). Seven patients reached final fusion. The space available for lung ratio in patients with thoracic curves improved from 0.87 (range, 0.7-1.1) to 1.0 (range, 0.79-1.23, P = 0.01). During the treatment period, complications occurred in 11 of the 23 patients (48%), and they had a total of 13 complications. Four of these patients (17%) had unplanned procedures. Following final fusion, 2 patients required extensions of their fusions because of curve progression and lumbosacral pain., Conclusion: The dual growing rod technique is safe and effective. It maintains correction obtained at initial surgery while allowing spinal growth to continue. It provides adequate stability, increases the duration of treatment period, and has an acceptable rate of complication compared with previous reports using the single rod technique.

Study Design: A prospective clinical study in which autologous rib graft, harvested during the thoracotomy in staged scoliosis correction, is stored within the patient for use during the second stage (posterior intrumentation and fusion)., Objective: To determine whether the bone stored by this technique is biologically viable and microbiologically safe., Summary of Background Data: To the authors' knowledge, this method of storage of bone has never been described previously., Methods: During the first operation, the excised rib was divided into 3-5 cm fragments and stored in a sub-muscular plane adjacent to the posterior elements of the spine before closure. The graft was then retrieved at the second stage. Samples were sent for histologic and microbiologic examination before implantation., Results: On histologic examination, more than 50% of the osteocytes retained their basophilic staining, indicating that they were viable. In addition, osteoclastic activity was notably absent. There was no significant bacterial contamination of the samples. Clinically, all patients achieved satisfactory bone fusion., Conclusion: Homeostatic equilibrium in humans provides the ideal environment in which bone graft can be stored. There is no increased risk of infection, and the osteogenic potential of the graft is retained.

Study Design: Retrospective review of patient records with current clinical and radiographic assessment., Objective: To evaluate the long-term result of anterior and posterior convex spinal growth arrest, with or without instrumentation, in managing infantile idiopathic scoliosis., Summary of Background Data: There were 12 male and 10 female patients studied, with a mean follow-up period of 10 years, 9 months. The mean Cobb angle before surgery was 65 degrees. All had a rib vertebral angle difference more than 20 degrees. The mean age at surgery was 6 years. Nine patients had epiphysiodesis alone; nine patients also underwent Harrington instrumentation simultaneously, and four underwent Harrington instrumentation 2-4 years later., Method: Clinical evaluation and sequential measurements of Cobb angle were done., Results: The epiphysiodesis-only group had a mean preoperative Cobb angle of 72 degrees, mean progression of curves of +12 degrees, and mean rate of progression of +2.5 degrees per year: the group's postoperative figures were 92 degrees, +15 degrees, and +3 degrees per year, respectively. The epiphysiodesis and late Harrington rod group had a mean preoperative Cobb angle of 56 degrees, mean progression of +12 degrees, and a mean rate of progression of +5 degrees per year; the group's postoperative Cobb angle averaged 62 degrees, progression +6 degrees, and rate of progression +1 degree per year. The epiphysiodesis with simultaneous Harrington rod group had a preoperative mean Cobb angle of 60 degrees, mean progression of +18 degrees, and mean rate of progression of +6 degrees per years. After surgery, these improved to 58 degrees, correction of 2 degrees, and rate of correction of 0.5 degree per year., Conclusion: Combined anterior and posterior convex spinal growth arrest alone does not prevent progression of deformity in infantile idiopathic scoliosis. The addition of posterior instrumentation can slow or arrest deformity progression but not reverse it.

Study Design: Retrospective review of patient records with recent clinical and radiologic assessment., Objective: To evaluate the long-term result of anterior and posterior convex ephiphysiodesis in the management of congenital scoliosis resulting from fully segmented nonincarcerated hemivertebra., Summary of Background Data: Thirty patients (16 male/14 female patients) were reviewed. Follow-up was a minimum of 3 years (average, 8 years 10 months; range, 3-22.5 years). Nineteen patients were skeletally mature, and the mean age of the remaining 11 was 11.75 years., Method: Clinical evaluation and sequential measurements of Cobb angle was made independently by two observers., Results: Compared with preoperative values, the rate of change in Cobb angle was reversed in 23 patients, arrested or slowed in five patients, and unchanged or progressed in two patients. The annual rate of change in Cobb angle was +1.9 degrees before surgery and -1.2 degrees after surgery, a difference of 3.1 degrees. This is highly statistically significant (P < 0.001). Total correction in Cobb angle correlates with age at time of surgery (P < 0.03). The rate of correction in Cobb angle after surgery correlates with the total correction achieved (P < 0.001) and with age at time of surgery (P < 0.05). The greater correction is achieved when surgery is performed at a young age. The preoperative rate of increase in Cobb angle does not correlate with correction (P < 0.76). The site of the hemivertebrae influenced final outcome with best results in the lumbar spine., Conclusion: Combined anterior and posterior convex epiphysiodesis is a reliable method for the correction of deformity resulting from hemivertebrae.

The diagnostic significance of visualizing the right ventricle on thallium-201 myocardial perfusion scans (T-scan) at rest was studied in 53 patients. In 33 patients the right ventricle was visualized clearly on the T-scan (group A). Hemodynamic evidence of right ventricular hypertension with systolic pressure greater than or equal to 30 mmHg was present in 28 of 33 (85%) of these patients. Right ventricular volume overload with left-to-right shunt greater than 2:1 was present in three patients. Other tests were diagnostic for right ventricular enlargement and or pulmonary hypertension as follows: chest x-ray (58%), echocardiogram (36%) and electrocardiogram (15%). In an unselected group of 20 patients (group B) where resting T-scan did not show visualization of the right ventricle, the right ventricular systolic pressure was less than 30 mm Hg in all. The other noninvasive tests did not reveal presence of right ventricular hypertrophy or enlargement. T-scan appears to be a useful and sensitive test in detecting right ventricular pressure or volume overload compared with other noninvasive tests. This may be useful in detection of patients with right ventricular hypertrophy or enlargement secondary to pulmonary hypertension or other causes.