Midterm Outcomes of Multimodal Approach to Treating Severe Scoliosis in Patients With Osteogenesis Imperfecta.

INTRODUCTION: The surgical management of severe scoliosis in patients with osteogenesis imperfecta (OI) is challenging because of curve rigidity, small stature, and inherent bone fragility. This study evaluated the midterm outcomes of our multimodal approach to address these issues, integrating perioperative bisphosphonate therapy, preoperative/intraoperative traction, various osteotomies, segmental pedicle screw instrumentation with cement augmentation, and bone morphogenetic protein-2 application. METHODS: A single-center retrospective review of 30 patients (average age 14.1 ± 2.2 years; 18 were female) diagnosed with OI and scoliosis was conducted. These patients underwent posterior spinal fusion between 2008 and 2020 and completed a minimum follow-up of 2 years. We measured radiographic parameters at each visit and reviewed the incidence of complications. A mixed-effects model was used to evaluate changes in radiographic parameters from preoperative measurements to the first and latest follow-ups. RESULTS: The patient cohort consisted of 2 individuals with type I OI, 20 with type III, 6 with type IV, and 2 with other types (types V and VIII). Surgical intervention led to a notable improvement in the major curve magnitude from 76° to 36°, with no notable correction loss. In addition, the minor curve, apical vertical translation, lowest instrumented vertebra tilt, and pelvic obliquity were also improved. In the sagittal plane, thoracic kyphosis and lumbar lordosis remained unchanged while thoracolumbar kyphosis markedly improved. Two patients experienced proximal junctional kyphosis with screw pullout, one of whom required revision surgery. One patient developed a superficial infection that was successfully treated with oral antibiotics. No instances of neurologic deficits or cement extravasation were observed. DISCUSSION: This study demonstrated the effectiveness and safety of our multimodal approach to treating scoliosis in patients with OI, achieving a 53% major curve correction with minimal complications over 2-year follow-up. These findings provide notable insights into managing scoliosis in this population. LEVEL OF EVIDENCE: Level IV (case series).

Subclassification of Sanders Maturation Stage 3 Demonstrates Differences in Spine and Total Height Velocity Between 3A and 3B in Patients with Idiopathic Scoliosis.

STUDY DESIGN: A single-center retrospective case-control study. OBJECTIVE: To compare the spine and total height velocity between Sanders maturation stage (SMS) 3A and 3B. SUMMARY OF BACKGROUND DATA: Identifying SMS 3 is critical for treating growing children because it represents the early phase of rapid adolescent growth. However, there is limited literature available that clearly describes the growth differences between 3A and 3B. METHODS: The current study included consecutive patients with idiopathic scoliosis staged SMS 3 from January 2012 to December 2021. T1-S1 spine height, total body height, and curve magnitude were measured at the initial and follow-up visits. In addition to the spine and total height velocity calculated per month, corrected height velocity was estimated for curve magnitude using a validated formula. Mann-Whitney U test was used to compare SMS 3A and 3B outcomes, followed by a multiple linear regression model to evaluate the association of the SMS subclassifications to growth velocity adjusted for confounding factors. RESULTS: A total of 204 patients (66% girls, mean age: 12.3±1.3 y) met the inclusion criteria. Patients staged SMS 3A had higher spine height velocity (mm/month) in both girls (2.3 vs. 1.5, P<0.001) and boys (2.6 vs. 1.7, P<0.001), as well as total height velocity (mm/month; (5.8 vs. 4.3, P<0.001 for girls; 6.6 vs. 4.5, P<0.001 for boys). Corrected velocity showed similar results with greater spine and total height velocity in SMS 3A. Multivariate analysis indicated a significant association of the SMS subclassification to the spine and total height velocity. The scoliosis curve progression was comparable between SMS 3A and 3B. CONCLUSION: SMS 3A and 3B had differential growth velocity in the spine and total body height. These results advocated the significance of SMS 3 subclassification for managing scoliosis treatment, including observation, bracing, and surgical interventions with fusion and growth modulation. LEVEL OF EVIDENCE: Level III (Case-control study).

Bone alterations of pamidronate therapy in children with cerebral palsy complicating orthopedic management.

Cerebral palsy (CP) is a heterogeneous group of disorders with different clinical types and underlying genetic variants. Children with CP are at risk for fragility fractures secondary to low bone mineral density, and although bisphosphonates are prescribed for the treatment of children with bone fragility, there is limited information on long-term bone impact and safety. Children with CP usually present overtubulated bones, and the thickening of cortical bone by pamidronate treatment can potentially further narrow the medullary canal. Our purpose was to report bone alterations attributable to pamidronate therapy that impact orthopedic care in children with CP. The study consisted of 41 children with CP treated with pamidronate for low bone mineral density from 2006 to 2020. Six children presented unique bone deformities and unusual radiologic features attributed to pamidronate treatment, which affected their orthopedic care. The cases included narrowing of the medullary canal and sclerotic bone, atypical femoral fracture, and heterotopic ossification. Treatment with bisphosphonate reduced the number of fractures from 101 in the pretreatment period to seven in the post-treatment period (P < 0.001). In conclusion, children with CP treated with bisphosphonate have a reduction in low-energy fractures; however, some fractures still happen, and pamidronate treatment can lead to bone alterations including medullary canal narrowing with sclerotic bone and atypical femoral fractures. In very young children, failure to remodel may lead to thin, large femoral shafts with cystic medullary canals. More widespread use of bisphosphonates in children with CP may make these bone alterations more frequent. Level of evidence: Level IV: Case series with post-test outcomes.