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Background: Arthrogryposis multiplex congenita (AMC) is a condition that describes neonates born with ≥2 distinct congenital contractures. Despite spinal deformity in 3% to 69% of patients, inadequate data exist on growth-friendly instrumentation (GFI) in AMC. Our study objectives were to describe current GFI trends in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched idiopathic EOS (IEOS) cohort to determine whether spinal rigidity or extremity contractures influenced outcomes.Methods: Children with AMC and spinal deformity of ≥30° who were treated with GFI for ≥24 months were identified from a multicenter EOS database (1993 to 2017). Propensity scoring matched 35 patients with AMC to 112 patients with IEOS with regard to age, sex, construct, and curve. Multivariable linear mixed modeling compared changes in spinal deformity and the 24-item Early Onset Scoliosis Questionnaire (EOSQ-24) across cohorts. Cohort complications and reoperations were analyzed using multivariable Poisson regression.Results: Preoperatively, groups did not differ with regard to age (p = 0.87), sex (p = 0.96), construct (p = 0.62), rate of nonoperative treatment (p = 0.54), and major coronal curve magnitude (p = 0.96). After the index GFI, patients with AMC had reduced percentage of coronal correction (35% compared with 44%; p = 0.01), larger residual coronal curves (49° compared with 42°; p = 0.03), and comparable percentage of kyphosis correction (17% compared with 21%; p = 0.52). In GFI graduates (n = 81), final coronal curve magnitude (55° compared with 43°; p = 0.22) and final sagittal curve magnitude (47° compared with 47°; p = 0.45) were not significantly different at the latest follow-up after definitive surgery. The patients with AMC had reduced T1-S1 length (p < 0.001), comparable T1-S1 growth velocity (0.66 compared with 0.85 mm/month; p = 0.05), and poorer EOSQ-24 scores at the time of the latest follow-up (64 compared with 83 points; p < 0.001). After adjusting for ambulatory status and GFI duration, patients with AMC developed 51% more complications (incidence rate ratio, 1.51 [95% confidence interval (CI), 1.11 to 2.04]; p = 0.009) and 0.2 more complications/year (95% CI, 0.02 to 0.33 more; p = 0.03) compared with patients with IEOS.Conclusions: Patients with AMC and EOS experienced less initial deformity correction after the index surgical procedure, but final GFI curve magnitudes and total T1-S1 growth during active treatment were statistically and clinically comparable with IEOS. Nonambulatory patients with AMC with longer GFI treatment durations developed the most complications. Multidisciplinary perioperative management is necessary to optimize GFI and to improve quality of life in this complex population.Level Of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Background: Although there is a high rate of reoperation after final fusion following the treatment of early-onset scoliosis with use of traditional growing rods, the risk factors for reoperation are unknown. The purpose of the present study was to identify risk factors associated with the need for reoperation after final fusion for the treatment of early-onset scoliosis.Methods: A multicenter database for patients with early-onset scoliosis was retrospectively analyzed. Patients managed with traditional growing rods and final fusion were identified (n = 248). The inclusion criteria were ≥1 lengthening procedure with traditional growing rods and ≥2 years of follow-up after final fusion or revision surgery within 2 years after final fusion (167 patients; 67%). Patients requiring reoperation following final fusion were compared with patients who did not require reoperation. The data that were analyzed included demographic characteristics, comorbidities, spinal deformity characteristics, radiographic measurements, perioperative details, and complications during all stages of treatment. A multivariate regression model was used to identify independent risk factors.Results: The mean duration of follow-up from the initial visit to the latest visit was 10.7 ± 4.1 years, and the mean duration of follow-up after final fusion was 4.9 ± 3.1 years. Thirty-two (19%) of the 167 patients required reoperation following final fusion. Curve progression requiring revision surgery during lengthening with traditional growing rods (adjusted odds ratio [aOR], 21.137 per event; p = 0.028), the number of levels spanned with traditional growing rods (aOR, 1.378 per level; p = 0.007), and the duration of treatment with traditional growing rods (aOR, 1.220 per year; p = 0.035) were independently associated with revision surgery after final fusion.Conclusions: Independent risk factors for curve progression requiring reoperation during lengthening with traditional growing rods that require operative intervention include increasing number of levels spanned with traditional growing rods and longer duration of treatment with traditional growing rods. These findings may help with patient counseling and potentially guide surgeon decision-making.Level Of Evidence: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Background: Nonambulatory children with myelodysplasia are most likely to develop spinal deformity. As the deformity progresses, the overall health of the patient deteriorates. Traditional management of the deformity with fusion results in a short trunk, crankshaft deformity, and spine and lung growth inhibition. One alternative that potentially minimizes these problems is the vertebral expandable prosthetic titanium rib (VEPTR). Questions/purposes: We therefore asked whether the use of the VEPTR in immature nonambulating children with myelodysplasia with spinal deformity would (1) correct deformity; (2) allow growth; and (3) allow adequate respiratory function. Patients and Methods: We identified 20 nonambulatory patients with myelodysplasia who were part of a multicenter Investigational Device Exemption study of 214 patients treated with the VEPTR system. Demographics, standard radiographic measurements, pulmonary function parameters, and complications in 16 patients were analyzed. Average age at first surgery was 48.6 months. The minimum followup was 25 months (mean, 59 months; range, 25-164 months). Results: The Cobb angle decreased postoperatively in nine patients, increased less than 10° in five patients, and increased less than 20° in two patients. The mean increase in thoracic spinal length (growth) by year after the initial procedure with lengthening was 0.48 cm. Ventilatory function improved in 11 patients and deteriorated in five patients. Intraoperative complications occurred in two patients. Complications directly related to the implant were seven infections and five implant migrations. Conclusions: Our observations suggest VEPTR is a reasonable treatment option for spinal deformity in the immature, nonambulatory myelodysplasia population correcting the spinal deformity, allowing spinal growth, and maintaining adequate respiratory function. The rate of complications is within the range reported for spinal fusion using standard approaches. Level of Evidence: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

The article discusses the complications that arise from surgical treatment of early onset scoliosis (EOS). EOS can be a symptom of thoracic insufficiency syndrome and EOS hampers the normal growth of the spine, thorax and lungs. Treatment of EOS may be through surgery to enable body growth and delay the onset of deformity of the spine. Surgical techniques include the spinal growing rods (GR) or vertical expandable prosthetic titanium rib (VEPTR).

The vertical expandable prosthetic titanium rib (VEPTR) was originally designed to treat chest and spine deformities in young children. However, older children with complex spinal deformities may also benefit from placement of a VEPTR when vertebral column resections are deemed too risky neurologically. We report: (1) the changes in Cobb angle, T1 angle, and head tilt; and (2) the occurrence of complications in children older than 10 years of age treated with VEPTR. From a database of 214 patients treated in a Food and Drug Administration Investigational Device Exemption study of VEPTR, we identified 10 patients with assorted diagnoses who underwent surgery after age 10 and had a minimum of 24-month followup (mean, 39.6 months; range, 24-75 months). No patient sustained neurologic injury. Patients underwent an average of five lengthenings. The mean preoperative Cobb angle was 64.7° and improved to 48.4°. Head shift improved an average of 3.8 cm. Two device-related complications occurred (both in the same patient). Four patients have since undergone definitive spinal fusion. For a select group of patients 10 years of age or older, the VEPTR offers a reasonable alternative to potentially risky vertebral column resections for correcting deformities in selected patients. Level of Evidence: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Background: The vertical expandable prosthetic titanium rib (VEPTR) device is used in the treatment of thoracic in- sufficiency syndrome and certain types of early-onset spinal deformity. The purpose of this study was to evaluate the risk of neurologic injury during surgical procedures involving use of the VEPTR and to determine the efficacy of intraoperative spinal cord neuromonitoring. Methods: Data were collected prospectively during a multicenter study. Surgical procedures were divided into three categories: primary device implantation, device exchange, and device lengthening. Further retrospective evaluation was undertaken in cases of neurologic injury or changes detected with neuromonitoring. Results: There were 1736 consecutive VEPTR procedures at six centers: 327 (in 299 patients) consisted of a primary device implantation, 224 were a device exchange, and 1185 were a device lengthening. Perioperative clinical neurologic injury was noted in eight (0.5%) of the 1736 cases: these injuries were identified after five (1.5%) of the 327 procedures for primary device implantation, three (1.3%) of the 224 device exchanges, and none of the 1185 device-lengthening procedures. Of the eight cases of neurologic injury, six involved the upper extremity and two involved the lower extremity. The neurologic deficit was temporary in seven patients and permanent in one patient, who had persistent neurogenic arm and hand pain. lntraoperative neuromonitoring demonstrated changes during six (0.3%) of the 1736 procedures: five (1.5%) of the 327 procedures for primary device implantation and one (0.08%) of the 1185 device-lengthening procedures. The surgery was altered in all six cases, with resolution of the monitoring changes in five cases and persistent signal changes and a neurologic deficit (upper-extremity brachial plexopathy) in one. Two patients had false-negative results of monitoring of somatosensory evoked potentials, and one had false-negative results of monitoring of somatosensory evoked potentials and motor evoked potentials during implant surgery; two had a brachial plexopathy and one had monoplegia postoperatively, with all three recovering. Conclusions: Neurologic injury during VEPTR surgery occurs much more frequently in the upper extremities than in the lower extremities. The rates of potential neurologic injuries (neurologic injuries plus instances of changes detected by monitoring) during primary implantation of the VEPTR (2.8%) and during exchange of the VEPTR (1.3%) justify the use of intraoperative neuromonitoring of the upper and lower extremities during those procedures. As neuromonitoring did not demonstrate any changes in children without a previous VEPTR-related monitoring change and there were no neurologic injuries during more than 1000 VEPTR-lengthening procedures, intraoperative neuromonitoring may not be necessary during those procedures in children without a history of a neurologic deficit during VEPTR surgery. [ABSTRACT FROM AUTHOR]

The article presents a research determining the definition of the vertebral column resection (VCR) procedure, indication, results and complications in patients with severe pediatric spinal deformity. Patients were followed-up for 17 months after the surgery. The results revealed that VCRs showed excellent radiographic correction with 57 percent complication rate and indications were divided into 5 diagnostics categories including kyphoscoliosis, severe scoliosis and global kyphosis.

Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is an autosomal-recessive disorder characterized by abnormal vertebral segmentation and defects affecting spine formation, with complete bilateral fusion of the ribs at the costovertebral junction producing a "crab-like" configuration of the thorax. The shortened spine and trunk can severely affect respiratory function during early childhood. The condition is prevalent in the Puerto Rican population, although it is a panethnic disorder. By sequencing a set of candidate genes involved in mouse segmentation, we identified a recessive E103X nonsense mutation in the mesoderm posterior 2 homolog (MESP2) gene in a patient, of Puerto Rican origin and from the Boston area, who had been diagnosed with STD/JLS. We then analyzed 12 Puerto Rican families with STD probands for the MESP2 E103X mutation. Ten patients were homozygous for the E103X mutation, three patients were compound heterozygous for a second nonsense mutation, E230X, or a missense mutation, L125V, which affects a conserved leucine residue within the bHLH region. Thus, all affected probands harbored the E103X mutation. Our findings suggest a founder-effect mutation in the MESP2 gene as a major cause of the classical Puerto Rican form of STD/JLS. [ABSTRACT FROM AUTHOR]

The article presents a study on the development and testing of the Spinal Appearance Questionnaire (SAQ) for reliability, validity, and responsiveness in patients with idiopathic scoliosis. The SAQ was designed to measure patients' and their parents' perception of spinal deformity's appearance. It shows that the SAQ is reliable, responsive to curve improvement, and shows strong evidence of validity. It also provides more detail than the Scoliosis Research Society (SRS) in the appearance domain.