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Background: Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is a rare autosomal recessive disorder affecting the formation of the spine, characterized by a complete bilateral fusion of the ribs at the costovertebral junction, producing a "crablike" appearance of the thorax. Despite being declared a core indication for a V-osteotomy vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty of the posterior thorax, the natural history of STD in untreated subjects remains poorly documented. In this study, we report radiographic and pulmonary function findings and Patient-Reported Outcomes Measurement Information System (PROMIS) and 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) scores for untreated adult subjects with STD to gain insights into the natural history., Methods: We identified 11 skeletally mature, untreated subjects with STD. Findings on medical evaluation, demographics, radiographic parameters, pulmonary function, genetic testing results, PROMIS measures, and EOSQ-24 scores were assessed., Results: Five male and 6 female subjects (mean age, 32.3 years [range, 15 to 70 years]) with a confirmed STD diagnosis based on radiographs and genetic testing were evaluated. Mean body mass index (BMI) was 24.4 kg/m 2 (range, 18 to 38.9 kg/m 2 ), and mean thoracic height was 16 cm (range, 12 to 17 cm). Pulmonary function tests (PFTs) showed a mean forced vital capacity (FVC) of 22% of predicted, mean forced expiratory volume in 1 second (FEV1) of 24% of predicted, and FEV1/FVC ratio of 107% of predicted. The mean PROMIS dyspnea score was 40 ± 8 points (range, 27.7 to 52.1 points). The mean total EOSQ-24 score was 77.3 ± 18 points (range, 43.9 to 93.2 points)., Conclusions: Our study characterizes the natural history of STD in untreated subjects. We confirmed the expected restrictive pattern in pulmonary function, but interestingly, our subjects exhibited better EOSQ scores compared with those reported in neuromuscular populations. PFT results and thoracic height did not correspond to PROMIS and EOSQ scores, questioning the use of those parameters as a surgical indication. We therefore suggest that the STD diagnosis as an absolute indication for VEPTR expansion thoracoplasty surgery be reconsidered., Level of Evidence: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence., Competing Interests: Disclosure: No external funding was received for this work. The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article ( http://links.lww.com/JBJS/H817 )., (Copyright © 2023 by The Journal of Bone and Joint Surgery, Incorporated.)

Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest form is provided with the online version of the article (http://links.lww.com/JBJS/H782).

Background: The present study compares prosthetic treatment options for proximal femoral focal deficiency in terms of gait analysis, oxygen consumption, and patient-reported outcomes., Methods: Twenty-three patients who had been managed with a prosthesis for unilateral proximal femoral focal deficiency underwent gait analysis; this group included 7 patients who had received an equinus prosthesis, 6 who had received a rotationplasty prosthesis, and 10 who had undergone Syme amputation and had received an above-the-knee prosthesis. Cadence parameters, kinematic and kinetic data, and oxygen consumption were measured, and the Gait Deviation Index (GDI) was calculated. Medical records and radiographs were reviewed. The Pediatric Outcomes Data Collection Instrument (PODCI) was completed by the child's parent., Results: Patients underwent gait analysis at a mean age of 11.6 years (range, 4 to 19 years). Proximal femoral focal deficiency classification was not predictive of the chosen treatment. Patients in the rotationplasty group had undergone more procedures than those in the Syme amputation and equinus groups (mean, 3.3, 1.8, and 0.7 procedures, respectively) (p = 0.001). Oxygen cost did not differ between groups; however, all required greater energy expenditure than normal (170%, 144%, and 159%, in the equinus, rotationplasty, and Syme amputation groups, respectively) (p = 0.427). Likewise, hip power, abductor impulse, and GDI did not differ, but all groups had GDI scores >3 standard deviations below normative values. Patients in the equinus group walked faster (97% of normal for age) than those in the rotationplasty (84%) and Syme amputation groups (83%) (p = 0.018), whereas those in the Syme amputation group had superior knee range of motion (55° from the prosthetic knee) than those in the equinus (20°) and rotationplasty groups (15° generated from the ankle) (p = 0.003). There were no differences in terms of the PODCI subscales for pain, sport/physical function, happiness, or global function. Transfer/basic mobility improved with age (r = 0.516, p = 0.017), but no other associations were found between gait variables and PODCI scores., Conclusions: Rotationplasty provided no patient-reported benefit and no functional benefit in terms of gait parameters or oxygen consumption, despite requiring more surgical procedures compared with other prosthetic options. Patients with an equinus prosthesis walked the fastest, whereas treatment with a Syme amputation and prosthetic knee yielded equivalent gait parameters and oxygen consumption as compared with those for patients using an equinus prosthesis. These findings contradict those of previous reports that rotationplasty provides superior function over other proximal femoral focal deficiency prosthetic treatment options., Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJS/G613)., (Copyright © 2021 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: In this study, we sought to evaluate radiographic, functional, and quality-of-life outcomes of patients who have completed growth-sparing management of early-onset scoliosis., Methods: This prospective study involved patients with early-onset scoliosis who underwent growth-sparing treatment and either "final" fusion or observation for ≥2 years since the last lengthening procedure. Demographics, radiographic parameters, pulmonary function test (PFT) values, and scores of patient-reported assessments (Early-Onset Scoliosis Questionnaire [EOSQ] and Scoliosis Research Society [SRS]-30) were obtained. At the most recent follow-up, patients performed 2 additional functional outcome tests: step-activity monitoring and a treadmill exercise-tolerance test., Results: Twelve patients were evaluated as "graduates" of growth-sparing management of early-onset scoliosis (mean of 37 months since the most recent surgery). The major scoliosis curve measurement averaged 88° before treatment and 47° at the most recent follow-up. T1-S1 height increased from a mean of 22.3 cm to 34.7 cm and T1-T12 height, from 13.3 to 22.3 cm. At the most recent follow-up, the mean forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) as a percentage of the predicted volume were 52.1% and 55.3%, respectively, and were essentially unchanged from the earliest PFT that patients could perform (FEV1 = 53.8% of predicted and FVC = 53.5% of predicted). There was no difference between graduates and controls with respect to activity time or total steps in step-activity monitoring, and in the exercise-tolerance test, graduates walked at the same speed but at a higher heart rate and at a significantly higher (p <0.001) VO2 cost (rate of oxygen consumed per distance traveled). The EOSQ mean score was 102.2 of a possible 120 points, and the SRS mean score was 4.1 of a possible 5 points., Conclusions: A realistic long-term goal for the management of early-onset scoliosis appears to be spine elongation and maintenance of pulmonary function at a level that is no less than the percentage of normal at initial presentation. Functional testing and patient-reported outcomes at a mean of 3 years from the last surgery suggest that activity levels were generally equal to those of controls but required greater physiologic demand. General health and physical function outcomes revealed continued impairment in these domains., Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Background: Questions remain as to the effect of pedicle screws on spinal canal development in young children. The purpose of this study was to determine the effects of unilateral placement of dual screws across the neurocentral synchondrosis on spinal canal development as assessed with histological analysis and measurement of the canal dimensions in an immature pig model., Method: Twenty-seven one-month-old pigs were assigned to two groups on the basis of the surgical approach used to place unilateral double screws that did or did not cross the neurocentral synchondrosis. In one group, sixteen pigs underwent a posterior approach from T7 to T14 and were divided into four subgroups: no screws (without screw fixation), short screws (dual pedicle screws that did not cross the neurocentral synchondrosis), long screws (dual pedicle screws that crossed the neurocentral synchondrosis), and screw removal (long dual pedicle screws that were removed at six weeks postoperatively). In the other group, eleven pigs underwent an anterior approach, with double vertebral body screws placed via thoracotomy. These animals were divided into two subgroups: short screws (dual vertebral body screws that did not cross the neurocentral synchondrosis) and long screws (dual vertebral screws that crossed the neurocentral synchondrosis). All animals were killed at seventeen weeks. The total area, width, and depth of the spinal canal were measured on axial computed tomography (CT) images. Quantitative histological analysis was performed to measure the rate of neurocentral synchondrosis closure., Results: Use of unilateral double pedicle screws across the neurocentral synchondrosis through a posterior approach resulted in 97% neurocentral synchondrosis closure with a 20% decrease in the canal area and a 15% decrease in the canal depth. Use of unilateral double vertebral body screws across the neurocentral synchondrosis through an anterior approach resulted in 71% neurocentral synchondrosis closure with a 15% decrease in the canal area and an 8% decrease in the canal width., Conclusions: Unilateral double pedicle screws crossing the neurocentral synchondrosis adversely affected spinal canal growth in immature pigs., Clinical Relevance: Pedicle screws should be used with caution in very young children, and a delay in surgical treatment until they are older should be considered., (Copyright © 2014 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: Early-onset scoliosis is a heterogeneous condition, with highly variable manifestations and natural history. No standardized classification system exists to describe and group patients, to guide optimal care, or to prognosticate outcomes within this population. A classification system for early-onset scoliosis is thus a necessary prerequisite to the timely evolution of care of these patients., Methods: Fifteen experienced surgeons participated in a nominal group technique designed to achieve a consensus-based classification system for early-onset scoliosis. A comprehensive list of factors important in managing early-onset scoliosis was generated using a standardized literature review, semi-structured interviews, and open forum discussion. Three group meetings and two rounds of surveying guided the selection of classification components, subgroupings, and cut-points. Initial validation of the system was conducted using an interobserver reliability assessment based on the classification of a series of thirty cases., Results: Nominal group technique was used to identify three core variables (major curve angle, etiology, and kyphosis) with high group content validity scores. Age and curve progression ranked slightly lower. Participants evaluated the cases of thirty patients with early-onset scoliosis for reliability testing. The mean kappa value for etiology (0.64) was substantial, while the mean kappa values for major curve angle (0.95) and kyphosis (0.93) indicated almost perfect agreement. The final classification consisted of a continuous age prefix, etiology (congenital or structural, neuromuscular, syndromic, and idiopathic), major curve angle (1, 2, 3, or 4), and kyphosis (-, N, or +) variables, and an optional progression modifier (P0, P1, or P2)., Conclusions: Utilizing formal consensus-building methods in a large group of surgeons experienced in treating early-onset scoliosis, a novel classification system for early-onset scoliosis was developed with all core components demonstrating substantial to excellent interobserver reliability. This classification system will serve as a foundation to guide ongoing research efforts and standardize communication in the clinical setting., (Copyright © 2014 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: Literature guiding the management of early-onset scoliosis consists primarily of studies with a low level of evidence. Evaluation of clinical equipoise (i.e., when there is no known superiority among treatment modalities) allows for prioritization of research efforts. The objective of this study was to evaluate areas of clinical uncertainty among pediatric spine surgeons regarding the treatment of early-onset scoliosis., Methods: Fourteen experienced pediatric spine surgeons participated in semistructured interviews to identify clinical variables that influence decision making in the treatment of early-onset scoliosis. A series of case scenarios of 315 patients with idiopathic and neuromuscular early-onset scoliosis was then developed to be representative of those encountered in clinical practice. Using an online survey, eleven surgeons selected their choice of eight treatment options for each case scenario. Associations between case characteristics and treatment choices were assessed with chi-square and logistic regression analysis. Participants then reviewed the areas of treatment uncertainty identified in the survey, nominated additional research questions of interest, and ranked their interest to further explore the identified research questions., Results: Collective equipoise was identified in numerous scenarios in the survey spanning a range of ages and magnitudes of scoliosis, and additional questions were identified during the nominal group technique. Areas that had the greatest clinical uncertainty included the management of patients who have finished treatment with a growing-rod, timing of rod-lengthening intervals, and indications for spine-based and rib-based proximal instrumentation anchors. The use of rib anchors compared with spine-based anchors was ranked highly for consideration in future clinical trials., Conclusions: Variability in decision making with regard to the optimum treatment of certain subsets of patients with early-onset scoliosis reflects gaps in the available evidence. Structured consensus methods identified priorities for higher levels of research in this area of scoliosis. Higher-level studies, including randomized trials, should focus on answering the questions highlighted in this report.

Background: In the treatment of idiopathic clubfeet, the Ponseti method and the French functional method have been successful in reducing the need for surgery. The purpose of this prospective study was to compare the results of these two methods at one institution., Methods: Patients under three months of age with previously untreated idiopathic clubfeet were enrolled. All feet were rated for severity prior to treatment. After both techniques had been described to them, the parents selected the treatment method. Outcomes at a minimum of two years were classified as good (a plantigrade foot with, or without, a heel-cord tenotomy), fair (a plantigrade foot that had or needed to have limited posterior release or tibialis anterior transfer), or poor (a need for a complete posteromedial surgical release). Two hundred and sixty-seven feet in 176 patients treated with the Ponseti method and 119 feet in eighty patients treated with the French functional method met the inclusion criteria., Results: The patients were followed for an average of 4.3 years. Both groups had similar severity scores before treatment. The initial correction rates were 94.4% for the Ponseti method and 95% for the French functional method. Relapses occurred in 37% of the feet that had initially been successfully treated with the Ponseti method. One-third of the relapsed feet were salvaged with further nonoperative treatment, but the remainder required operative intervention. Relapses occurred in 29% of the feet that had been successfully treated with the French functional method, and all required operative intervention. At the time of the latest follow-up, the outcomes for the feet treated with the Ponseti method were good for 72%, fair for 12%, and poor for 16%. The outcomes for the feet treated with the French functional method were good for 67%, fair for 17%, and poor for 16%., Conclusions: Nonoperative correction of an idiopathic clubfoot deformity can be maintained over time in most patients. Although there was a trend showing improved results with use of the Ponseti method, the difference was not significant. In our experience, parents select the Ponseti method twice as often as they select the French functional method.

Background: Results of the Charnley-Williams method of intramedullary fixation for treatment of congenital pseudarthrosis of the tibia have varied, in part because of variations in surgical technique. The outcomes of three variations of this procedure were compared to determine which technique was the most likely to result in union., Methods: The results in twenty-three consecutive patients with congenital pseudarthrosis of the tibia were reviewed at four to fourteen years following initial surgical treatment with an intramedullary rod. Three types of procedures were performed: type A, which consisted of resection of the tibial pseudarthrosis with shortening, insertion of an intramedullary rod into the tibia, and tibial bone-grafting combined with fibular resection or osteotomy and insertion of an intramedullary rod into the fibula; type B, which was identical to type A except that it did not include fibular fixation; and type C, which consisted of insertion of a tibial rod and bone-grafting but no fibular surgery. The outcome was classified as grade 1 when there was unequivocal union with full weight-bearing function and maintenance of alignment requiring no additional surgical treatment; grade 2 when there was equivocal union with useful function, with the limb protected by a brace, and/or valgus or sagittal bowing for which additional surgery was required or anticipated; and grade 3 when there was persistent nonunion or refracture, requiring full-time external support for pain and/or instability., Results: Eleven patients (48%) ultimately had a grade-1 outcome; nine, a grade-2 outcome; and three, a grade-3 outcome. The final outcome was not associated with either the initial radiographic appearance of the lesion or the age of the patient at the time of the initial surgery. The results following type-A and B operations were better than those after type-C procedures. Surgery on an intact fibula resulted in a lower prevalence of grade-3 outcomes than was found when an intact fibula was not operated on (p = 0.05). Transfixation of the ankle joint by the intramedullary rod did not decrease the prevalence of grade-3 outcomes., Conclusions: There is little justification for a type-C operation, as it either resulted in a persistent nonunion or failed to improve an equivocal outcome in every case. Leaving an intact fibula undisturbed to maintain stability or length also was not successful in this series. In addition, the presence of fibular insufficiency (fracture or a pre-pseudarthrotic lesion) was highly prognostic for subsequent valgus deformity (occurring in ten of twelve cases), whether or not the fibula eventually healed.

A retrospective study of 2442 patients who had idiopathic scoliosis was performed to determine the prevalence of back pain and its association with an underlying pathological condition. Five hundred and sixty (23 per cent) of the 2442 patients had back pain at the time of presentation, and an additional 210 (9 per cent) had back pain during the period of observation. There was a significant association between back pain and an age of more than fifteen years, skeletal maturity (a Risser sign of 2 or more), postmenarchal status, and a history of injury. There was no association with gender, family history of scoliosis, limb-length discrepancy, magnitude or type of curve, or spinal alignment. At the latest follow-up evaluation, 324 (58 per cent) of the 560 patients who had had back pain at presentation had no additional symptoms. Forty-eight (9 per cent) of the 560 patients who had back pain had an underlying pathological condition: twenty-nine patients had spondylolysis or spondylolisthesis, nine had Scheurmann kyphosis, five had a syrinx, two had a herniated disc, one had hydromyelia, one had a tethered cord, and one had an intraspinal tumor. A painful left thoracic curve or an abnormal neurological finding was most predictive of an underlying pathological condition, although only eight of the thirty-three patients who had such findings were found to have such a condition. When a patient with scoliosis has back pain, a careful history should be recorded, a thorough physical examination should be performed, and good-quality plain radiographs should be made. If this initial evaluation reveals normal findings, a diagnosis of idiopathic scoliosis can be made, the scoliosis can be treated appropriately, and non-operative treatment can be initiated for the back pain. It is not necessary to perform extensive diagnostic studies to evaluate every patient who has scoliosis and back pain.

Four patients who had Larsen syndrome and cervical kyphosis were managed operatively and followed for an average of seventy months (range, forty to ninety-two months). The preoperative cervical kyphosis ranged from 35 to 65 degrees. The patients had had a posterior cervical arthrodesis alone when they were infants, at an average age fo fourteen months (range, ten to sixteen months). In three infants, the kyphosis either stabilized (one patient) or reversed into lordosis (two patients). Thus, the kyphosis corrected gradually by continued anterior growth in the presence of a solid posterior fusion. In the fourth infant, the kyphosis progressed to 110 degrees because of pseudarthrosis. This child had anterior decompression and arthrodesis for an acute neurological deficit. We believe that cervical kyphosis is sometimes present but not diagnosed in patients who have Larsen syndrome. Early diagnosis followed by operative stabilization should help such patients avoid neurological deficits. Posterior cervical arthrodesis alone, performed in infancy, provided stability and the opportunity for the gradual correction of the deformity by continued anterior growth in three of our four patients.

The prevalence of curve progression was evaluated in 210 boys who had idiopathic scoliosis. A minimum age of eight years, a deformity of at least 10 degrees, and radiographic follow-up of one year or progression of the curve within the first year of follow-up were the criteria for inclusion in the study. Of the 210 patients, sixty-eight (32 per cent) had progression of 10 degrees or more. Four of the five patients who had had an initial curve of 50 degrees or more subsequently had a spinal arthrodesis. The risk of progression was significantly greater for patients who were at an earlier Risser stage (p < 0.002) and for those who were younger (p < 0.005). The risk of progression was also greater for patients who had had a larger curve at the time of presentation; of the sixty-three boys for whom the Risser grade was 1, 2, 3, 4, or 5 when they were first seen and who had a curve of 25 degrees or more, twenty (32 per cent) had progression, compared with only two (5 per cent) of the thirty-eight who had a Risser grade of 1, 2, 3, 4, or 5 and a curve of 24 degrees or less. Of the thirty-four patients for whom the Risser grade was 4 when they were first seen, five (15 per cent) had progression.(ABSTRACT TRUNCATED AT 250 WORDS)

We investigated the effects of increased intraosseous pressure on new-bone formation in the proximal metaphysis of the caprine tibia. Intraosseous hypertension was produced by obstruction of venous outflow by ligation of the popliteal vein draining the proximal aspect of the tibia and occlusion of the medullary space with bone cement (groups 1 and 2). After the obstruction of venous outflow (day 0), the intraosseous pressure measured at the proximal tibial metaphysis increased significantly from a mean of 15.5 millimeters of mercury before the obstruction to a mean of 28.7 millimeters of mercury in groups 1 and 2. In group 1, obstruction of venous outflow was combined with intraosseous infusion of autogenous whole blood under pressure to maintain the intraosseous pressure between thirty and forty-five millimeters of mercury during days 0 through 5. The time for venous drainage was still prolonged and intraosseous pressures were still increased on days 5 and 10 (means, 26.8 and 26.2 millimeters of mercury, respectively) in groups 1 and 2. The intraosseous hypertension produced in group 1 was associated with a significant increase in periosteal (138 per cent), endocortical (369 per cent), and cancellous new-bone formation (889 per cent) at the tibial metaphysis compared with control values. Osseous necrosis within the metaphysis was not observed.

In twenty-five patients, the Pavlik harness failed to obtain or maintain reduction in thirty of thirty-five congenital dislocations of the hip. All of the patients had met the clinical criteria for use of the harness in our institution: they were less than seven months old, the femoral head pointed to the triradiate cartilage on anteroposterior radiographs that were made with the child wearing the harness, and they had no evidence of neuromuscular disease or teratological dislocation. These patients were compared with seventy-one patients (eighty-one dislocations) who had also been treated with the Pavlik harness and in whom a stable reduction was obtained and maintained. Statistically significant risk factors for failure of the harness included an absent Ortolani sign at the initial evaluation, bilateral dislocation, and an age of more than seven weeks before treatment with the harness was begun. All thirty hips in which the harness failed to obtain or maintain reduction had a subsequent attempt at closed reduction after preliminary Bryant traction. Fifteen of these hips were successfully reduced closed, but two later redislocated and needed an open reduction. The remaining fifteen hips needed an open reduction, and two redislocated and needed a second open reduction.

We studied the radiographs of thirty-two patients with Down syndrome for evidence of atlanto-axial instability. One of the patients had instability in 1970 and seven had it in 1983. The interval between the atlas and the odontoid process in the patients who demonstrated motion at that interval radiographically averaged 2.78 millimeters in 1970 and 6.93 millimeters in 1983 (p less than 0.005). Four patients whose radiographs showed atlanto-axial motion in 1970 lost that motion by 1983, and in seven patients who did not show atlanto-axial instability in 1970 it developed by 1983. Atlanto-axial instability was more likely to develop in boys who were more than ten years old. Accessory upper-cervical ossicles became evident in three patients, none of whom had atlanto-axial motion. However, one of these three patients had an abnormally wide atlanto-axial interval.

The cases of two patients with delayed paraplegia after segmental spinal instrumentation with sublaminar wiring are reported. Both patients had complex spinal deformities and had transient neural deficits after the first-stage procedure of anterior release and spine fusion. They had uneventful spinal-cord monitoring during the second-stage procedure of posterior instrumentation and fusion, and function of the lower extremities was present immediately after that operation. Paraplegia then ensued, and was recognized thirty hours later in one patient and six days later in the other. Considering our reproducible and reliable experience (no false-negative results) with spinal cord monitoring in 307 operations, we propose that the delayed onset of paraplegia resulted from a progression of ischemic and edema-producing events that had not developed sufficiently intraoperatively to be reflected by the monitoring. The paraplegia became evident only when the subarachnoid space was obstructed because of progressive postoperative neural edema. The presence of sublaminar implants in narrow, kyphotic segments of the spinal canal probably exacerbated the neural irritation by dural impingement, which was seen myelographically.