Summary of background data: The skeletal system is affected in up to 60% of patients with neurofibromatosis type 1. The most commonly observed entities are spinal deformities and tibial dysplasia. Early recognition of radiologic osseous dystrophy signs is of utmost importance because worsening of the deformities without treatment is commonly observed and surgical intervention is often necessary. Due to the relative rarity and the heterogenic presentation of the disease, evidence regarding the best surgical strategy is still lacking.Purpose: To report our experience with the treatment of skeletal manifestations in pediatric patients with (neurofibromatosis type 1) NF-1 and to present the results with our treatment protocols.Materials and methods: This is a retrospective, single expert center study on children with spinal deformities and tibial dysplasia associated with NF-1 treated between 2006 and 2020 in a tertiary referral institution.Results: Spinal deformity:Thirty-three patients (n = 33) were included. Mean age at index surgery was 9.8 years. In 30 patients (91%), the deformity was localized in the thoracic and/or lumbar spine, and in 3 patients (9%), there was isolated involvement of the cervical spine. Eleven patients (33%) received definitive spinal fusion as an index procedure and 22 (67%) were treated by means of “growth-preserving” spinal surgery. Halo-gravity traction before index surgery was applied in 11 patients (33%). Progression of deformity was stopped in all patients and a mean curve correction of 60% (range 23–98%) was achieved. Mechanical problems with instrumentation requiring revision surgery were observed in 55% of the patients treated by growth-preserving techniques and in none of the patients treated by definitive fusion. One patient (3%) developed a late incomplete paraplegia due to a progressive kyphotic deformity.Tibial dysplasia:The study group comprised of 14 patients. In 5 of them (36%) pathological fractures were present on initial presentation. In the remaining 9 patients (64%), anterior tibial bowing without fracture was observed initially. Four of them (n = 4, 28%) subsequently developed a pathologic fracture despite brace treatment. Surgical treatment was indicated in 89% of the children with pathological fractures. This involved resection of the pseudarthrosis, autologous bone grafting, and intramedullary nailing combined with external fixation in some of the cases. In 50% of the patients, bone morphogenic protein was used “off-label” in order to promote union. Healing of the pseudarthrosis was achieved in all of the cases and occurred between 5 to 13 months after the index surgical intervention. Four of the patients treated surgically needed more than one surgical intervention in order to achieve union; one patient had a re-fracture. All patients had a good functional result at last follow-up.Conclusion: Early surgical intervention is recommended for the treatment dystrophic spinal deformity in children with NF-1. Good and sustainable curve correction without relevant thoracic growth inhibition can be achieved with growth-preserving techniques alone or in combination with short spinal fusion at the apex of the curve. Preoperative halo-gravity traction is a safe and very effective tool for the correction of severe and rigid deformity in order to avoid neurologic injury. Fracture union in tibial dysplasia with satisfactory functional results can be obtained in over 80% of the children by means of surgical resection of the pseudarthrosis, intramedullary nailing, and bone grafting. Wearing a brace until skeletal maturity is achieved is mandatory in order to minimize the risk of re-fracture.

Using a structured approach and expert consensus, we developed an evidence-based guideline on the diagnosis of back pain and the treatment of non-specific back pain in children and adolescents. The first part comprises etiology, risk factors, and diagnosis. The second part, published in the same issue, includes treatment and prevention. A comprehensive and systematic literature search was conducted to identify relevant guidelines and studies. Based on the findings of this literature search, recommendations on risk factors and diagnosis were formulated and voted on by experts in a structured consensus-building process. Notable red flags for specific back pain and evidence-based risk factors for non-specific back pain in children and adolescents were identified. Only three evidence-based recommendations could be formulated for causes, red flags, and risk factors for back pain, while two recommendations are based on expert consensus. Regarding diagnostics, eight expert consensus recommendations and one evidence-based recommendation could be provided. Despite the importance of adequate diagnosis for the treatment of back pain in children and adolescents, results of this work confirm the deficit in research investment in this area.

Using a structured approach and expert consensus, we developed an evidence-based guideline on the treatment and prevention of non-specific back pain in children and adolescents. A comprehensive and systematic literature search identified relevant guidelines and studies. Based on the findings of this literature search, recommendations on treatment and prevention were formulated and voted on by experts in a structured consensus-building process. Physical therapy (particularly physical activity) and psychotherapy (particularly cognitive behavioral therapy) are recommended for treating pediatric non-specific back pain. Intensive interdisciplinary treatment programs should be provided for chronic and severe pain. Drug therapy should not be applied in children and adolescents. Further research on non-specific back pain in childhood and adolescence is strongly needed to reduce the imbalance between the high burden of non-specific back pain in childhood and adolescence and the low research activity in this field.

Background: Serial casting is a treatment for early onset scoliosis (EOS) in young children to achieve curve correction before bracing or to postpone initial surgical treatment until the patient is older. Good results have been reported for patients with idiopathic early onset scoliosis (IS). However, there are few reports of results in non-idiopathic cases, and the benefits of non-surgical methods in the syndromic-associated early onset scoliosis subgroup are unknown. Methods: Retrospective single-institution study of patient charts and X-rays of all cases of sustained serial casting for EOS. Staged correction was obtained by applying three consecutive casts under general anaesthesia. These were changed every 4 weeks, followed by the implementation of a custom-made full-time Chêneau brace. Correction was measured by Cobb angle (CA) and rib-vertebra angle difference (RVAD) on whole spine anterior-posterior radiographs. Statistical analysis was performed via ANOVA. Results: The study group consisted of 6 patiens with IS and 10 with non-idiopathic scoliosis (NIS) – exclusively syndromic-associated. The mean age at onset of treatment was 35 months (±15). The mean follow up was 21 months (±15). In IS patients average CA/RVAD before treatment was 46°(±8)/20°(±12). In NIS patients average CA/RVAD before treatment was 55°(±15)/24°(±14). After application of the third cast, the CA/RVAD was reduced to 20°(±11)/11°(±10) in IS patients. Whereas in NIS patients average CA/RVAD after the thrid cast was 28°(±12)/18°(±13). At latest follow-up the CA/RVAD was 16°(±7)/9°(±8) in IS patients and 31°(±11)/17° (±15) in NIS patients. Conclusion: Syndromic etiology is not a contraindication for serial casting in EOS. Our results show a curve correction, measured in CA, of 65% in IS patients and 44% in NIS patients. Significant reduction in the morphologic deformity, measured in RVAD, was achieved in the IS cohort, but not in the NIS cohort. In all cases surgical treatment could be delayed.

Background/Objectives: This study aims to analyze the efficacy and safety of the two pelvic fixation systems, S-hooks (SH) and lumbar-sacral-alar-iliac (SAI) screws, when used in association with magnetically controlled growing rods (MCGRs) in non-ambulatory children with severe neuromuscular scoliosis (NMS). Methods: Forty-one patients were retrospectively examined and subdivided corresponding to ilium hook fixation or SAI screws. The major curve correction (%) and pelvic obliquity (PO) correction (%) were assessed utilizing scoliosis plain film radiographs over time. Complications and unplanned return to the operating room (UPROR) were recorded. Patient-specific pre- and postoperative values were included in a backward stepwise regression model to assess UPROR. Results: Mean age at index intervention was 9.4 years. Preoperative main curve was 81° and PO was 22°. 21 and 20 patients were categorized into the SH and SAI subgroups, respectively. Initial curve correction was significantly better in the SAI subgroup (63 vs. 42% in the SH, p = 0.045), while PO correction was equally good. Curve and PO correction were maintained throughout the follow-up period of 55 months. UPROR rate was 38% in the SH subgroup, and 5% in the SAI subgroup (p = 0.010). Regression analysis identified postoperative curve correction as predictive value for UPROR (p = 0.006). Conclusions: SAI screw fixation has a low UPROR rate and achieves superior curve correction. S-hooks are a viable option to correct PO and NMS in children with high operative risk to reduce operative time, but revision surgery is not uncommon.