Treatment of infantile idiopathic scoliosis using a novel thoracolumbosacral orthosis: a case report

Background The recommended treatment for mild to moderate infantile idiopathic scoliosis curves involves serial casting. There are concerns, however, regarding the safety of repeated casting in very young children owing to the requirement for anesthetization during the casting process. Very little research has been conducted on the influence of bracing as an initial treatment for scoliosis in this age group. This report details the successful treatment of a large thoracic curve using a thoracolumbosacral orthosis in an infant diagnosed with infantile idiopathic scoliosis. Case presentation The Dutch-Australian patient presented at 11 weeks of age with a 44° thoracic scoliosis and a rib vertebral angle difference of 14°. The history and physical examination failed to reveal a cause of the curvature, and a diagnosis of infantile idiopathic scoliosis was made. The patient was prescribed a thoracolumbosacral orthosis (ScoliBrace) to be worn on a part-time basis for a period of 8 months. At the end of the bracing program, the patient’s curve had been reduced to 7° and a rib–vertebral angle difference of 0°. A final follow-up of the patient at 2 years after the cessation of treatment revealed no evidence of scoliosis. The parents were compliant with the bracing protocol and reported that the treatment was tolerated by the infant. Conclusion The use of an orthosis as a standalone treatment in this patient resulted in significant reduction in a large thoracic scoliosis. Based on the results witnessed in this patient, further investigation into bracing as an alternative to casting is warranted.

Casting in infantile idiopathic scoliosis as a temporising measure: A systematic review and meta-analysis

Objective: Treatment of infantile idiopathic scoliosis remains vague. Because implantation of temporary telescopic devices carries a high risk of complications, interest in the older technique of serial casting is growing as a temporising measure before invasive procedures. The goal of this review was to meta-analyse studies examining the effect and safety of casting in infantile idiopathic scoliosis. Methods: Two reviewers independently searched for relevant studies in PubMed and Embase databases through November 2018. The studies included were limited to infantile idiopathic scoliosis patients who underwent casting, had a mean Cobb angle of 20 or more and written in English. The methodological quality of the chosen studies was assessed. The primary outcome was the difference in Cobb angle means from before and after casting. The secondary outcome was adverse events of casting. Heterogeneity was explored and a funnel plot was drawn. Results: Of the 366 studies screened, 10 studies were included in the meta-analysis (243 subjects) and all were non-randomised. The casting was consistently associated with a reduction in the mean Cobb angle. The pooled mean difference was 24.85° (95% confidence interval: 19.25 to 30.46, p < 0.001). A number of reversible adverse events were reported, most commonly skin irritation and transient pulmonary symptoms. Heterogeneity between studies was high. In the meta-regression analysis, the starting Cobb angle did not influence Cobb angle change, but there was an inverse correlation between the mean difference in Cobb angle and mean age. Conclusion: Casting seems to be effective and safe in decreasing Cobb angle even in high curve magnitudes. In older patients, casting showed less Cobb angle correction.

Regulation of terminal hypertrophic chondrocyte differentiation in Prmt5 mutant mice modeling infantile idiopathic scoliosis

ABSTRACTIdiopathic scoliosis (IS) is the most common type of musculoskeletal defect effecting children and is classified by age of onset, location, and degree of spine curvature. Although rare, the onset of IS during infancy is the more severe and rapidly progressive form of the disease, leading to increased mortality due to significant respiratory compromise. The pathophysiology of IS, in particular for infantile IS, remain elusive. Here, we show that PRMT5 is critical for the regulation of terminal hypertrophic chondrocyte differentiation in the spine and models infantile IS in mouse. Conditional ablation of PRMT5 in osteochondral progenitors led to impaired terminal hypertrophic chondrocyte differentiation and asymmetric defects of endochondral bone formation in the perinatal spine. Analysis of several markers of endochondral ossification revealed increased COLX and Ihh expression and a dramatic reduction of Mmp13 and RUNX2 expression in the intervertebral disc and vertebral growth plate. Furthermore, we demonstrate that PRMT5 function in committed chondrogenic lineages is required for regulation of COLX expression in the adult spine. Together, our results establish PRMT5 as a critical regulator of hypertrophic chondrocyte differentiation and endochondral bone formation in spine development and maintenance.

Pedicle stress shielding following growing rod implantation: case report

Growing rod surgery for skeletally immature patients helps correct severe scoliosis while allowing continued spinal column growth. Previous reports have studied vertebral body changes following growing rod surgery, but there are currently no published reports on alterations in pedicle morphology. Given the potential need for definitive spinal fusion with pedicle screw instrumentation, an awareness of changes in pedicle morphology is critical. A morphometric analysis of pedicles was performed using 3D reconstructions of 3 CT scans (preoperative and at 3 and 6 years) obtained in a young girl with infantile idiopathic scoliosis (T7 apex) who underwent unilateral rib-to-spine growing rod (2nd–4th ribs to L1) implantation with lengthening every 6 months for 6 years. The pedicle widths on the growing rod side from T5 to T9 (apex ± 2) were all smaller at 6 years postoperatively than preoperatively, while the same-level pedicles opposite the device significantly increased in width. These findings support anecdotal intraoperative reports by surgeons and provide evidence of pedicle stress shielding due to growing rod distraction and force deprivation.