C1-2 rotatory subluxation as a presenting sign in juvenile rheumatoid arthritis.

STUDY DESIGN: Case report. INTRODUCTION: Juvenile rheumatoid arthritis (JRA) typically presents with fever, rash, anterior uveitis, and/or joint pain. We present three cases with initial torticollis due to rotatory subluxation of C1-C2 as an initial sign of JRA. CASE REPORTS: Three girls, ages 5-9, presented with C1-2 rotatory subluxation. Traction was able to reduce the atlanto-axial joint in all cases. Based on imaging, history, exam, and laboratory results, they were diagnosed with JRA. After reduction of the atlantoaxial joint, they were transitioned to a halo vest and disease-modifying antirheumatic drugs (DMARDs). The older 2 children underwent C1-2 fusion. The younger child has minimal symptoms and has not undergone surgical intervention 4 years from initial presentation. CONCLUSION: Rotatory subluxation can be the first presenting sign of JRA. Younger children may be able to be treated conservatively with traction and medication, while older children may require occiput to C2 fusion due to bony destruction and basilar invagination. LEVEL OF EVIDENCE: IV.

A comparison of screw versus drill and curettage epiphysiodesis to correct leg-length discrepancy.

PURPOSE: To compare two common surgical techniques of epiphysiodesis: drill/curettage epiphysiodesis (PDED) versus cross screw epiphysiodesis (PETS). The hypothesis is that the two techniques have similar efficacy but demonstrate differences in length of hospital stay (LOS), time to return to activity and complication rates. METHODS: A retrospective review of growing children and adolescents less than 18 years old who required an epiphysiodesis with leg-length discrepancy (LLD) of 2 cm to 6 cm with minimum two years of follow-up was conducted. Characteristics including age at surgery, gender, epiphysiodesis location, side, operative time, LOS and hardware removal were compared across treatment groups. LLD, expected growth remaining (EGR) and bone age were determined preoperatively and at most-recent visit. The correction ratio (change in EGR) was calculated along with a 95% confidence interval (CI) to assess if correction in leg length was achieved. RESULTS: A total of 115 patients underwent epiphysiodesis in the femur (53%), tibia (24%) or a combination (24%). The cohort was 47% male, with a mean age of 12.6 years (7.7 to 17.7) at surgery. Median follow-up was 3.7 years (2.0 to 12.7). In all, 23 patients underwent PETS and 92 patients had PDED. Both treatment groups achieved expected LLD correction. There was no significant difference in median operative time, complication rates or LOS. PETS patients returned to activity at a mean 1.4 months (interquartile range (IQR) 0.7 to 2.1) while PDED patients returned at a mean 2.4 months (IQR 1.7 to 3) (p < 0.001). CONCLUSION: Effectiveness in achieving expected correction, LOS and operative time are similar between screw and drill/curettage epiphysiodesis. Patients undergoing PETS demonstrated a faster return to baseline activity than patients with PDED. LEVEL OF EVIDENCE: III.

Survey to describe variability in early onset scoliosis cast practices.

PURPOSE: To investigate paediatric orthopaedists' cast practices for early onset scoliosis regarding patient selection, cast application, radiographic evaluation, treatment cessation and adjunctive bracing. METHODS: A casting survey was distributed to all paediatric orthopaedists in Children's Spine and Growing Spine Study Groups (n = 92). Questions included physician and patient characteristics, technique, treatment, outcomes, radiographic measurements and comparison to other treatments. A total of 55 orthopaedists (60%) responded, and descriptive statistics were calculated on the subset who cast (n = 45). RESULTS: A majority of respondents use cast treatment for idiopathic and syndromic scoliosis patients, but not for neuromuscular or congenital scoliosis patients. Major curve angle ranked most important in orthopaedists' decision to commence cast treatment, in comparison with rib-vertebra angle difference or clinical observations. The major curve angle threshold to initiate casting was a median of 30° (20° to 70°), and the minimum patient age was median ten months (3 to 24). First in-cast and out-of-cast radiographs are taken standing, supine, awake, under anesthesia and/or in traction. In all, 58% consistently cast over or under the arm, while 44% vary position by patient. Respondents were divided about the use of a brace after cast treatment: 22% do not prescribe a brace, 31% always do and 36% do in some patients. CONCLUSIONS: Future multicentre research studies must standardize radiographic practices and consider age and major curve angle at cast initiation and termination, scoliosis aetiology, shoulder position and treatment duration. Practices need to be aligned or compared in these areas in order to distinguish what makes for the best cast treatment possible.Level of Evidence: V, Expert opinion.

Clinically apparent adverse reactions to intra-wound vancomycin powder in early onset scoliosis are rare.

PURPOSE: Spine surgeons have increasingly used intraoperative application of topical vancomycin powder (TVP) to prevent surgical site infections (SSIs). The goals of this study were to define the rate of pharmacological adverse reaction to TVP in young patients undergoing posterior spinal surgery and to summarise institutional variation in TVP dosing. METHODS: This retrospective observational study included ten spine centres in the United States and one in Europe. Patients with early onset scoliosis who underwent posterior spine surgery were eligible for inclusion. Age, weight, TVP dose and surgery type were recorded. Surgeries where patient age was > 12 years were excluded. Pharmacological adverse reactions were defined as clinical instances of Red Man Syndrome, rash, nephrotoxicity, proteinuria, hepatotoxicity or ototoxicity. The rate of pharmacological adverse reaction to TVP was calculated. Dosing practices were summarised. RESULTS: Patient age was in the range of seven months to 12 years (median ten years). Of 1398 observations, there was one possible pharmacological adverse reaction. This was in a ten-year-old, 20.4-kg female patient with neuromuscular sco-liosis undergoing growing rod implantation. She was dosed with 1500 mg of TVP and immediately developed a transient rash without systemic symptoms. This abated over minutes without any medical intervention. There were no other adverse reactions in the sample. The population rate of pharmacological adverse reaction was 0.072% (95% confidence interval 0 to 0.4). Significant variability in dosing practices existed between centres. CONCLUSION: Pharmacological adverse reactions to TVP are rare. Future work may establish evidence-based guidelines for TVP dosing based on patient weight and other variables.