Timely Surgical Intervention Leads to Better Sustained Coverage after Reconstructive Hip Surgery in Patients with Cerebral Palsy.

BACKGROUND: In up to 45-90% of non-ambulatory patients with cerebral palsy (CP), progressive hip migration can be observed. The goal of this study was to determine whether the implementation of a national hip surveillance guideline affected the outcome of hip reconstructions. METHODS: We reviewed 48 primary hip reconstructions at a median follow-up of 4.4 years. Surgical outcome was evaluated based on complication rates and radiographic evaluation postoperatively and at follow-up. Radiographic measurements included the migration percentage (MP), head-shaft angle and acetabular index. The impact of preoperative MP, postoperative MP, tone management, Gross Motor Function Classification System (GMFCS) classification and age on MP at follow-up were examined using a mixed model analysis. RESULTS: A decrease in preoperative MP was noted, from a median of 75.0% (2014) to 39.0% (2020). Lower preoperative MP showed a significant correlation to lower MP postoperatively (p = 0.012). Postoperative MP was a significant independent predictor of a lower MP at follow-up (p = 0.002). CONCLUSIONS: This study shows an improvement in the timing of hip reconstruction in patients with CP after implementation of the hip surveillance guideline. A reduction in preoperative MP resulted in improved postoperative outcomes. A lower postoperative MP was the most important predictor for sustained containment of the hip.

Current practices in the management of closed femoral shaft fractures in children: A nationwide survey among Dutch orthopaedic surgeons.

Background: There remains a lack of high-quality evidence on the treatment of pediatric femur shaft fractures. Therefore, treatment choices may still be based on personal preference of treating surgeons. To gain insight in considerations regarding treatment options, we conducted a survey among Dutch trauma and orthopedic surgeons. Methods: This survey was conducted in 2020, regarding treatment considerations for closed femoral shaft fractures in children in different age and weight groups. Results: One hundred forty-two surgeons were included in the analysis. 31% of participating surgeons considers surgical fixation in children of 2-4 years old, compared to 83% in children of 4-6 years old. In terms of weight, 30% considers surgery in children of 10-15 kg, compared to 77% considering surgery in children weighing 15-20 kg. While most surgeons find traction and spica cast suitable options for children younger than 4 years, a minority also considers these treatment modalities for children older than 4 (traction: 81% versus 19%, spica cast 63% versus 29% respectively). 33% of surgeons considers ESIN under 4 years of age, compared to 88% in children older than 4. Conclusion: An age of 4 years and a weight of 15 kg seem to be cut off points regarding preference of non-surgical versus surgical treatment of closed femoral shaft fractures. There is a wide range of ages and sizes for which treatment options are still being considered, sometimes differing from the national guideline. This questions guideline adherence, which may be due to a lack of available high-quality evidence.

Effect of tranexamic acid on intraoperative blood loss in pediatric osteotomies around the hip: Study protocol for a double-blind randomized placebo-controlled trial.

Background: Proximal femoral and/or pelvic osteotomies (PFPOs) can be indicated for a multitude of hip pathologies in (often asymptomatic) children, to prevent future hip problems. These procedures can result in significant blood loss. Tranexamic acid (TXA) is an antifibrinolytic agent routinely administered in adult trauma and joint reconstruction surgery to reduce blood loss. TXA is also registered for use in children and reported safe and beneficial for pediatric trauma, cardiac, and spinal surgery. However, for pediatric orthopedics, particularly for PFPOs, the available evidence is limited. Therefore, the current trial will investigate the potential reducing effect of preoperative TXA on intraoperative blood loss in pediatric PFPOs. Methods: In this single-center, double-blind, randomized placebo-controlled trial, we aim to include 180 participants aged from 1 to 18 years undergoing PFPOs for any indication at our institution. Participants will be randomized to receive either TXA or placebo (saline) during anesthetic induction. The primary outcome is intraoperative estimated blood loss (mL/kg), which is determined gravimetrically. Secondary outcomes include the percentage of patients with excessive blood loss (>20 mL/kg), procedure time and hospital stay, and postoperative hemoglobin level changes. Discussion: This will be the first prospective study investigating the effect of preoperative TXA on intraoperative blood loss in pediatric PFPOs. Its results will help to determine whether it would be advisable to adopt preoperative TXA as a standard medication to minimize blood loss and prevent complications in this vulnerable population. Trial registration: EudraCT: 2022-002384-30. Prospectively registered on September 26, 2022.

The Predictive Value of Radiographs and the Pirani Score for Later Additional Surgery in Ponseti-Treated Idiopathic Clubfeet, an Observational Cohort Study.

There are few validated predictors of the need for additional surgery in idiopathic clubfeet treated according to the Ponseti method. Our aim was to examine if physical examination (Pirani score) and radiographs at the age of three months (after initial correction of the clubfeet) can predict the future need for additional surgery. In this retrospective cohort study, radiographs of idiopathic clubfeet were made at the age of three months. The Pirani score was determined at the first cast, before tenotomy, and at the age of three months. Follow-up was at least five years. The correlation between the radiograph, Pirani score, and the need for additional surgery was explored with logistic regression analysis. Parent satisfaction was measured with a disease-specific instrument. The study included 72 clubfeet (50 children) treated according to the Ponseti method. Additional surgery was needed on 27 feet (38%). A larger lateral tibiocalcaneal angle (i.e., equinus) and a smaller lateral talocalcaneal angle (i.e., hindfoot varus) at the age of three months were correlated with the need for additional surgery. Higher Pirani scores before tenotomy and at the age of three months also correlated with additional surgery. Parent satisfaction was lower in patients who needed additional surgery. Both the Pirani scores and the lateral radiographs are predictive for future additional surgery.

Three-dimensional printing in paediatric orthopaedic surgery.

Three-dimensional (3D) printing is a rapidly evolving and promising field to improve outcomes of orthopaedic surgery. The use of patient-specific 3D-printed models is specifically interesting in paediatric orthopaedic surgery, as limb deformity corrections often require an individual 3D treatment. In this editorial, various operative applications of 3D printing in paediatric orthopaedic surgery are discussed. The technical aspects and the imaging acquisition with computed tomography and magnetic resonance imaging are outlined. Next, there is a focus on the intraoperative applications of 3D printing during paediatric orthopaedic surgical procedures. An overview of various upper and lower limb deformities in paediatrics is given, in which 3D printing is already implemented, including post-traumatic forearm corrections and proximal femoral osteotomies. The use of patient-specific instrumentation (PSI) or guiding templates during the surgical procedure shows to be promising in reducing operation time, intraoperative haemorrhage and radiation exposure. Moreover, 3D-printed models for the use of PSI or patient-specific navigation templates are promising in improving the accuracy of complex limb deformity surgery in children. Lastly, the future of 3D printing in paediatric orthopaedics extends beyond the intraoperative applications; various other medical applications include 3D casting and prosthetic limb replacement. In conclusion, 3D printing opportunities are numerous, and the fast developments are exciting, but more evidence is required to prove its superiority over conventional paediatric orthopaedic surgery.

Treatment of closed femoral shaft fractures in children aged 2-10 years: a systematic review and meta-analysis.

OBJECTIVE: To review current literature on treatment of closed femoral shaft fractures in children of 2-10 years old, with subgroup analysis of children aged 2-6 years, comparing intramedullary nailing (IMN) to conservative treatment modalities. METHODS: We included clinical trials and observational studies that compared traction and subsequent casting (TSC), spica casting and IMN for treatment of femur shaft fractures in children of 2-10 years of age. Subgroup analysis of children aged 2-6 years was performed. RESULTS: Compared to treatment with immediate spica casting, IMN led to significantly less coronal angulation (mean difference (MD): 2.03 degrees, confidence interval (CI) 1.15-2.90), less sagittal angulation (MD: 1.59 degrees, CI 0.82-2.35) and lower rates of LLD (Risk difference (RD): 0.07, CI 0.03-0.11). In terms of rehabilitation, IMN leaded to shorter time until walking with aids (MD: 31.53 days, CI 16.02-47.03), shorter time until independent ambulation (MD: 26.59 days, CI 22.07, 31.11) and shorter time until full weight bearing (MD: 27.05 days, CI 6.11, 47,99). Compared to TSC, IMN led to a lower rate of malunion (RD: 0.31, CI 0.05-0.56), shorter hospital stays (MD: 12.48 days, CI 11.57, 13.39), time until walking with aids (MD: 54.55, CI 40.05-69.04) and full weight bearing (MD: 27.05 days [6.11, 47,99]). CONCLUSION: Although a lack of quality evidence, this systematic review showed a clear tendency to treatment with elastic intramedullary nails of femoral shaft fractures in children of 2-10 years of age.

Tranexamic acid reduces blood loss in paediatric proximal femoral and/or pelvic osteotomies.

PURPOSE: Proximal femoral and/or pelvic osteotomies (PFPO) are associated with significant blood loss, which can be harmful, especially in paediatric patients. Therefore, considering methods to reduce blood loss is important. The purpose of this study was to examine the efficacy of tranexamic acid (TXA) in reducing intraoperative estimated blood loss (EBL) in paediatric patients undergoing a PFPO. METHODS: Paediatric patients who had a PFPO between 2014 and 2019 were retrospectively reviewed. Outcome measures included patient demographics, TXA use (none, preoperative and/or intraoperative bolus, pump), EBL, transfusion rate and thromboembolic complications. Univariate and multivariate analyses were performed to assess associations between investigated outcome measures and EBL. RESULTS: A total of 340 PFPO (263 patients) were included. Mean age at surgery was 8.0 years (sd 4.3). In all, 269 patients received no TXA, 20 had a preoperative bolus, 43 had an intraoperative bolus and eight patients had other TXA regimes (preoperative and intraoperative bolus or pump). Overall, mean blood loss was 211 ml (sd 163). Multivariate analysis showed significant associations between higher EBL and higher age at surgery, male sex, higher body mass index and longer procedure time. There was a significant association between lower EBL and a preoperative TXA bolus: 66 ml (33%) less EBL compared with patients without TXA (95% confidence interval -129 to -4; p = 0.04). No thromboembolic complications were reported in any of the studied patients. CONCLUSION: Preoperative TXA administration is associated with a decreased EBL in PFPO. No thromboembolic events were reported. Administering TXA preoperatively appears to be effective in paediatric patients undergoing a PFPO. LEVEL OF EVIDENCE: Level III - retrospective comparative study.

Congenital Forearm Pseudarthrosis, a Systematic Review for a Treatment Algorithm on a Rare Condition.

BACKGROUND: A congenital forearm pseudarthrosis is a rare condition and is strongly associated with neurofibromatosis type 1. Several surgical techniques are described in the literature, but the most optimal treatment strategy remains unclear. This systematic review aims to develop a treatment algorithm that may aid in clinical decision making. METHODS: The PROSPERO registration number for this study was CRD42018099602 and adheres to the PRISMA guidelines for systematic reviews. Embase, MEDLINE, Cochrane Central, Web of Science, and Google Scholar databases were searched for published studies reporting on congenital forearm pseudarthrosis not related to other underlying pathologies like bacterial infection or fibrous dysplasia. Results were not restricted by date or study type, only English literature was allowed. Studies were assessed for quality using the critical appraisal checklist for case reports from the Joanna Briggs Institute. Patient characteristics, underlying disease, type of surgery, union rate, and functional outcome were extracted from included studies. RESULTS: Of 829 studies identified, 47 were included in this review (17 case series and 30 case reports, a total of 84 cases). A one-bone forearm procedure showed highest union rates (92%), however, it results in loss of forearm rotation. Free vascularized fibula grafting showed high union rates (87%) and was related to good functional outcome of elbow flexion and forearm rotations. Other procedures showed disappointing outcomes. CONCLUSIONS: Congenital forearm pseudarthrosis is best treated with a free vascularized fibula grafting, a one-bone forearm procedure should be used as a salvage procedure. Evidence extracted from the case reports was sufficient to generate a treatment algorithm to be used in clinical pediatric practice. LEVEL OF EVIDENCE: Level IV-therapeutic.

Diagnosis and management in Pitt-Hopkins syndrome: First international consensus statement.

Pitt-Hopkins syndrome (PTHS) is a neurodevelopmental disorder characterized by intellectual disability, specific facial features, and marked autonomic nervous system dysfunction, especially with disturbances of regulating respiration and intestinal mobility. It is caused by variants in the transcription factor TCF4. Heterogeneity in the clinical and molecular diagnostic criteria and care practices has prompted a group of international experts to establish guidelines for diagnostics and care. For issues, for which there was limited information available in international literature, we collaborated with national support groups and the participants of a syndrome specific international conference to obtain further information. Here, we discuss the resultant consensus, including the clinical definition of PTHS and a molecular diagnostic pathway. Recommendations for managing particular health problems such as dysregulated respiration are provided. We emphasize the need for integration of care for physical and behavioral issues. The recommendations as presented here will need to be evaluated for improvements to allow for continued optimization of diagnostics and care.

Screening program for neonates at risk for developmental dysplasia of the hip: comparing first radiographic evaluation at five months with the standard twelve week ultrasound. A prospective cross-sectional cohort study.

PURPOSE: To assess whether delayed radiological hip screening at five months (versus ultrasound at 3 months) results in a higher incidence of persistent developmental dysplasia of the hip (DDH) at 18 months. METHODS: We analyzed 3536 screened neonates (2009-2013) at age two to three weeks. In the case of risk factors for DDH, 460 infants were assigned to a pelvic radiograph at five months between 2009 and 2010 and 651 infants were assigned to an ultrasound at three months (2011-2013). In the case of DDH, appropriate treatment was started and radiological follow-up occurred at eight, ten, 12, and 18 months. We compared incidence and severity of persistent DDH at 18 months. Analysis was performed using linear regression. RESULTS: Both groups were comparable for risk factors (breech, gender, twins, family history). Eighty-nine patients (2.5%) showed DDH (n = 43 (group 1), n = 46 (group 2)). At 18 months, ten patients showed persistent DDH (n = 8 (group 1), n = 2 (group 2) (7.7% vs. 0.3% respectively)). The mean acetabular index (AI) at 18 months in group 1 (left hip) is 22.4° (95% CI 20.6-24.3°) vs. group 2 at 22.3° (95% CI 21.2-23.4°) (p = 0.098). The mean AI in group 1 (right hip) is 21.9° (95% CI 18.9-24.9°) vs. 21.2° (95% CI 20.5-22.0°) in group 2 (p = 0.293). Adjusted for risk factors, there is no difference in incidence of persistent DDH between both groups after 18 months (OR 0.519; 0.07, 3.845). DISCUSSION: This study revealed no significant difference in incidence or severity of persistent DDH at 18 months between the two screening groups. These results suggest justification for delayed screening to prevent overtreatment of immature hips. CONCLUSION: In clinically stable hips, delayed ultrasound between three and five months is regarded as safe and could prevent for overtreatment of mild dysplastic hips.

[Müller-Weiss disease: idiopathic avascular necrosis of the navicular bone]. / Ziekte van Müller-Weiss: idiopathische avasculaire necrose van os naviculare.

BACKGROUND: Müller-Weiss disease is a rare and complex foot disorder. The underlying aetiology of this condition involves abnormal loading of the navicular bone combined with delayed ossification. Müller-Weiss disease is progressive and ultimately results in deformation and fragmentation of the navicular bone with plantar-flexed hindfoot varus. In patients with a severe form of this foot deformity, treatment involves arthrodesis of the midfoot with bridging of the navicular bone. CASE DESCRIPTION: A 43-year-old female patient was seen in our outpatient clinic with pain in her right foot. Her symptoms had been present for seven months and had developed spontaneously without prior trauma. Imaging investigations resulted in the diagnosis of Müller-Weiss disease in both her right and her left foot. CONCLUSION: It is important to recognize this condition at an early stage so that patients can receive conservative treatment initially.

[A boy with a painful upper leg]. / Een jongen met een pijnlijk bovenbeen.

A 13-year-old boy came to the Emergency Department with a Salter Harris type 1 epiphysiolysis of the distal femur. These fractures can be treated with a closed reduction and possible fixation. They cause a growth disturbance in 65 % of dislocated cases and therefore should be clinically evaluated until skeletal maturity.

[A man with a dell over the symphysis pubica]. / Man met pijnlijke delle over symphysis pubica.

A 46-year-old man was referred to our emergency room after minimal trauma. He was diagnosed with symphysiolysis, which was based on a congenital variant instead of a traumatic injury.