Distal Femur Anterior Hemiepiphysiodesis for Fixed Knee Flexion Deformity in Neuromuscular Patients: A Systematic Review.

BACKGROUND: In the past decade, distal femur anterior hemiepiphysiodesis for fixed knee flexion deformity has gained popularity. In this study, we aim to systematically review the literature on this technique as a treatment for fixed knee flexion deformity in patients with neuromuscular disorders. METHODS: A systematic review was performed in the following databases: PubMed, Embase, and Medline. The inclusion criteria included anterior hemiepiphysiodesis of the distal femur for patients with neuromuscular disorders and fixed knee flexion deformities. RESULTS: Our search yielded 419 titles. A total of 12 articles were qualified for final review based on the inclusion and exclusion criteria. The total number of patients was 279, with 483 knees corrected. The male to female ratio was 2.1:1, and the mean age of the patients was 11.3 ± 1.4 years (range 4-17 years). The mean preoperative flexion deformity was 23.1° ± 10.0° (range 10°-90°). The mean residual flexion deformity at the final follow-up was 8.6° ± 9.0° (range 0°-32.5°), which corresponds to a statistically significant decrease compared with the preoperative deformity (p < 0.05). CONCLUSION: Anterior hemiepiphysiodesis for fixed knee flexion deformity provides favourable outcomes with low complication rates in patients with neuromuscular disorders. Future studies should focus on comparing the long-term outcomes of the different metal implants used for these operations. LEVEL OF EVIDENCE: Level III. See Instructions for Authors for a complete description of levels of evidence.

Talectomy for arthrogrypotic foot deformities: A systematic review.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is one of the most common congenital joint contracture syndromes. Talectomy has been proposed for severe foot deformities in AMC, but the literature is limited. The purpose of this systematic review is to evaluate the indications, outcomes and complications of talectomy in arthrogrypotic foot deformities. METHODS: The articles were found through Embase and Medline. Screening was conducted by two independent investigators with disagreements resolved by a third reviewer. Relevant data regarding demographics, outcomes and complications were collected. RESULTS: Of 232 feet, 71.98 % and 62.22 % had clinical and radiographic improvements respectively. Amongst 122 patients, 92.62 % could ambulate following surgery. Recurrent deformities and revision surgery were seen in 16.81 % and 13.36 % of cases respectively. CONCLUSIONS: Talectomy is a valid surgical option for severe arthrogrypotic foot deformities with favorable post-operative outcomes and low complication rate.

Neglected Monteggia fracture: a review.

Monteggia fracture is characterized by radial head dislocation combined with proximal ulnar fracture. If not diagnosed at an early stage, these lesions can gradually lead to forearm deformities and dysfunction, finally resulting in neglected Monteggia fracture. When the radial head is not reduced, several deformities develop at the humeroradial joint, including cubitus valgus and osteoarthritis. Adequate radiographs are crucial when the surgeons deal with forearm injuries. At present, proximal ulnar osteotomy and open reduction of chronic radial head dislocation provides satisfactory functional outcomes because of anatomic alignment reconstruction. Supplementary procedures, including transcapitellar pinning and repair or reconstruction of the annular ligament, which are performed in order to enhance stability of the humeroradial joint, should be thoroughly assessed based on joint rotational stability after reduction and on potential complications.

Retrograde Application of Humerus Fassier-Duval Rod in Osteogenesis Imperfecta: A New Surgical Technique.

BACKGROUND: The aim of this study is to describe the technique of retrograde application of Fassier-Duval (FD) rod for the humerus in patients with osteogenesis imperfecta (OI). This technique was developed to overcome the downsides of the previously used techniques of humerus rodding. METHODS: The study was done at a tertiary care pediatric orthopaedic hospital from April 2014 to August 2021. Skeletally immature patients with OI who underwent retrograde FD rodding were included. This surgery was performed for humeral shaft fractures/bowing limited to the distal half of the bone to ensure appropriate stability of the fixation. Surgical technique of the procedure is described in detail. RESULTS: Six patients with OI, of which 2 (33.3%) had FD rodding bilaterally, were included. The mean age at rodding was 7.6±3.5 (range: 3 to 14) years. The mean duration of postoperative follow-up was 45.5±18.0 (range: 24 to 75) months. All patients had full healing of the fracture/osteotomy, with functional alignment of their humeri. No surgical complications were observed; however, 1 (12.5%) segment only had a traumatic humerus fracture following a fall that was associated with rod migration, occurring 60 months postoperatively. This was treated with a retrograde FD rodding again, with fracture augmentation with plate and screws. CONCLUSIONS: The retrograde FD rodding technique of the humerus in OI patients is relatively simple and preserves the soft tissue surrounding the shoulder joint, with favorable outcomes. Studies with larger sample size and long-term follow-up duration are needed. LEVEL OF EVIDENCE: Level IV-case series.

Metatarsal Gradual Lengthening for Brachymetatarsia Using a Mini-rail External Fixator System.

Aim: To describe the surgical technique for gradual lengthening of the metatarsal using the Orthofix MiniRail System (Orthofix Medical Inc., Lewisville, TX, USA). Background: Brachymetatarsia refers to the shortening of the metatarsal bone. When indicated, metatarsal lengthening is performed to treat this deformity. Technique: A dorsal approach to the short metatarsal is performed, protecting the neurovascular structures and the extensor tendons. The most proximal wire or screw is inserted first, followed by the most distal wire or screw. The distal wire or screw should not be inserted in the metatarsal head. The middle wires or screws are inserted thereafter. All wires or screws should be placed perpendicular to the anatomic axis of the bone. Once the MiniRail lengthener is assembled, a 1.6 mm K-wire is inserted from the tip of the toe into the metatarsal head, blocking the motion of the metatarsophalangeal joint and avoiding joint subluxation during lengthening. The osteotomy is then performed perpendicular to the metatarsal shaft, in between the middle two wires and screws. Conclusion: The surgical technique for gradual lengthening of the metatarsal using the Orthofix MiniRail System was described in detail with accompanying step-by-step intraoperative clinical and fluoroscopic images. Clinical significance: This surgical technique of metatarsal lengthening is simple and effective. An understanding of the surgical technique of gradual lengthening of the metatarsal bone is important when treating shorting deformities of more than 1 cm. How to cite this article: Marwan Y, Abu Dalu K, Bernstein M, et al. Metatarsal Gradual Lengthening for Brachymetatarsia Using a Mini-rail External Fixator System. Strategies Trauma Limb Reconstr 2022;17(3):184-188.

Treatment of Femur Neck Fracture in Children with Osteogenesis Imperfecta: Two Case Reports.

CASE: We present 2 cases of femoral neck fracture (FNF) in patients with osteogenesis imperfecta (OI) with a previously placed intramedullary nail (IMN). Case 1: A 12-year-old girl who sustained a right FNF after spine surgery. Case 2: An 18-year-old boy who sustained a right FNF after IMN of the left side. Both patients underwent our modified surgical technique as described below. Both patients recovered well overall. CONCLUSIONS: FNF in patients with OI poses a significant challenge especially in the presence of an IMN. Here, we describe a surgical technique that provides stable fixation with a less stress riser effect by bypassing the screws and a previously placed nail.

Treatment and outcomes of arthrogryposis in the lower extremity.

In this multiauthored article, the management of lower limb deformities in children with arthrogryposis (specifically Amyoplasia) is discussed. Separate sections address various hip, knee, foot, and ankle issues as well as orthotic treatment and functional outcomes. The importance of very early and aggressive management of these deformities in the form of intensive physiotherapy (with its various modalities) and bracing is emphasized. Surgical techniques commonly used in the management of these conditions are outlined. The central role of a multidisciplinary approach involving all stakeholders, especially the families, is also discussed. Furthermore, the key role of functional outcome tools, specifically patient reported outcomes, in the continuous monitoring and evaluation of these deformities is addressed. Children with arthrogryposis present multiple problems that necessitate a multidisciplinary approach. Specific guidelines are necessary in order to inform patients, families, and health care givers on the best approach to address these complex conditions.

Research platform for children with arthrogryposis multiplex congenita: Findings from the pilot registry.

A pediatric registry for arthrogryposis multiplex congenita (AMC) proposes to advance research by providing the platform to inform the distribution, etiology, and natural history of AMC. The registry was piloted on 40 families of children (mean = 8.25 years, 48% males) presenting with AMC across two hospitals in North America. Data on the child's demographic and newborn variables, mothers' and fathers' demographic variables, lifestyle habits, and medical history were collected using a telephone interview with the primary caregiver and review of medical charts. Mean gestational age was 38 weeks, 97% of children presented with lower extremity deformities, and 74% of neonatal interventions targeted the lower extremity. Newborns spent an average of 14 days in the hospital (range 2-56 days) mostly for diagnostic workup and feeding difficulties. Half (49%) of the sample had internal organ involvement. Genetic testing was done on 48% of the children, including chromosome studies, single gene, whole-exome/genome sequencing, and/or microarray studies. Genetic findings were inconclusive in most. Two-thirds of mothers (67%) reported inconsistently feeling fetal movements. This pilot study contributed to the refinement of participant selection, identification of data source, expansion of data sets, and areas for future exploration prior to the implementation of a multisite AMC pediatric registry.

Development of a research platform for children with arthrogryposis multiplex congenita: study protocol for a pilot registry.

INTRODUCTION: Arthrogryposis multiplex congenita (AMC) describes a heterogeneous group of conditions with multiple congenital contractures. These conditions may be attributed to genetic or other factors inducing decreased fetal movements, including maternal and paternal factors. Discovering the underlying genetic pathways has important repercussions for prevention, gene therapy and genetic counselling. The current literature mainly consists of small-scale, single-site studies, limiting comparability and pooling of findings across individual studies. A pilot registry for children presenting with AMC is proposed to provide the framework for a large-scale AMC registry. This registry will provide the platform to support high-quality studies to inform the distribution, clinical practice and genetics contributing to this group of conditions. METHODS AND ANALYSIS: The registry will be piloted on 40 families of children from birth to 21 years of age presenting with AMC. Data will be collected on the child (demographic and newborn variables), mother and father (demographic, lifestyle habits and medical history). To promote standardised data collection, a manual of operations will be developed. Descriptive statistics will be used to summarise relevant data, regression analyses will be used to explore associations to generate hypotheses regarding factors contributing to AMC. Qualitative analysis will also be used to better describe the various phenotypes. ETHICS AND DISSEMINATION: Ethics approval was obtained at the participating sites. The pilot registry will provide the platform for multisite AMC registry that will generate multiple research avenues to enhance current care and establish new therapies. Following this pilot study, the participant selection criteria will be refined and datasets will be expanded to include rehabilitation and surgical interventions, and genetic sequencing. The best timing for the questionnaire administration and frequency of follow-up prior to the implementation of a multisite AMC registry will be determined.

Pauwels Osteotomy for Femoral Neck Nonunion in Two Adult Siblings with Osteogenesis Imperfecta.

This is a retrospective review of two adult siblings with osteogenesis imperfecta (OI) type III (according to Sillence classification), who sustained a spontaneous femoral neck fracture and subsequent nonunion. The diagnosis of OI in these two patients was made based on clinical, radiological and genetic findings. The fracture was most likely caused by femoroacetabular impingement secondary to OI induced acetabular protrusio. A valgus osteotomy according to Pauwels'principles and fixation of the osteotomy and nonunion with a locking plate resulted in healing despite compromised bone quality and limited bone stock. Long-term follow up (4.5 years and 6.5 years, respectively) is provided. When treating this difficult problem, improving the mechanobiological environment and decreasing the femoroacetabular impingement by a Pauwels type osteotomy should be considered.

Incidence of knee height asymmetry in a paediatric population of corrected leg length discrepancy: a retrospective chart review study.

PURPOSE: The purpose of the study is to provide a methodology to quantify knee height asymmetry (KHA) and to establish the incidence of knee height asymmetry in a patient population visiting the limb length discrepancy clinic in a paediatric-orthopaedic hospital centre. METHOD: A retrospective chart review was performed on all patients who attended the limb length discrepancy clinic and underwent corrective surgery at the Shriners Hospital for Children-Canada from December 2009 to December 2015. Full-standing anteroposterior radiographs were used to measure pre- and post-surgery limb length discrepancy and knee height asymmetry for 52 individuals included in the study. RESULTS: Sixty-seven percent of the studied population had a KHA of 20 mm or less, 25% had a KHA between 20 and 40 mm, and 8% had a KHA of over 40 mm. The average KHA preoperatively for all 52 individuals was 17 ± 14 mm (range 0-59 mm), which represents roughly 2.5% of total limb length. There was a 3-mm non-significant reduction in KHA size between pre-and post-operative states (p = 0.22). CONCLUSION: The current study provides a method to quantify knee height asymmetry. Using this method, it was shown that knee height asymmetry is frequent in youth with limb length discrepancy in both pre- and post-corrective surgery states. The relatively high incidence of knee height asymmetry highlights the importance to investigate the impact of knee height asymmetry in youth living with a limb length discrepancy.

Utility outcome assessment of pes planus deformity.

BACKGROUND: Despite being a common condition, there are no objective measures in the literature to reflect the burden of pes planus on affected individuals. Our primary objective was to evaluate this burden by recruiting a sample from the general population using validated utility outcome measures. METHODS: Participants were recruited online and filled a questionnaire to help measure the health burden of pes planus. Three recognized utility outcome scores were used to compare the health burden of monocular blindness, binocular blindness, and pes planus. These included the standard gamble (SG), time trade-off (TTO), and visual analogue score (VAS). Paired t test, independent t test, and linear regression were used for statistical analysis. RESULTS: Ninety-two participants were included in the final analysis. The utility outcome scores (VAS, TTO, SG) for pes planus were 73±17, 0.90±0.08, and 0.88±0.12, respectively. The linear regression analysis showed that age was inversely proportional to the time trade-off. However, race, educational level, and income were not significant predictors of utility outcome score for pes planus. CONCLUSIONS: This study shows that the perceived burden of living with pes planus is comparable to living with some debilitating conditions. Our participants were willing to sacrifice 3.6 years of life, and have a procedure with a theoretical 12% mortality risk to attain perfect health.

Assessment of the effect of systemic delivery of sclerostin antibodies on Wnt signaling in distraction osteogenesis.

Sclerostin is a known inhibitor of the Wnt signaling pathway which is involved in osteogenesis and, when inactivated, stimulates bone formation. To our knowledge, this effect has not been studied in the context of distraction osteogenesis (DO). Tibial DO was conducted on a total of 24 wild-type mice, which were then divided into 2 groups-a saline injection group (control) and an anti-sclerostin (Scl-Ab) injection group (treatment). The mice in the treatment group received 100 mg/kg intravenous injections of the antibody weekly until killing. The 12 mice in each group were subdivided into four time points according to post-osteotomy time of killing-11 days (mid-distraction), 17 days (late distraction), 34 days (mid-consolidation) and 51 days (late consolidation), with 3 mice per subgroup. After killing, the tibia specimens were collected for immunohistochemical analysis. Our results show that the group injected with anti-sclerostin had an earlier peak (day 11) in the distraction phase of the osteogenic molecules involved in the Wnt signaling pathway in comparison to the placebo group. In addition, downregulation of the inhibitors of this pathway was noted in the treatment group when compared with the placebo group. Furthermore, LRP-5 showed a significant increase in expression in the treatment group. Sclerostin inhibition has a significant effect on the DO process through its effect on the Wnt pathway. This effect was evident through the decreased effect of sclerostin on LRP-5 and earlier upregulation of the osteogenic molecules involved in this pathway.

Strategies of enhancing bone regenerate formation in distraction osteogenesis.

Distraction osteogenesis (DO) is a commonly used technique in multiple orthopedic sub-specialties, including trauma, oncology and pediatrics. This technique aims to produce new bone formation in the distraction gap in a controlled manner. The issue with this technique has been the high risk of complications, one of which is poor regenerate formation during the distraction process. Although several factors (including patient and operative factors) and techniques (including surgical, mechanical and pharmacological) have been described to ensure successful regenerate formation during the process of DO, these factors are sometimes difficult to control clinically. Our aim from this review is to highlight the different factors that affect DO, modalities to assess the regenerate and review treatment options for poor regenerate in the distraction gap. In addition, we propose a management protocol derived from the available literature that can be used to facilitate the management of inadequate regenerate formation.

Functional Outcome of Forearm Rodding in Children With Osteogenesis Imperfecta.

BACKGROUND: The impact of corrective forearm surgery on functional ability in children with osteogenesis imperfecta (OI) has not previously been reported. This study addresses this issue. METHODS: A retrospective chart review was conducted on 19 children with OI who underwent 22 corrective forearm procedures between 1996 and 2013. Functional ability was assessed preoperatively and every year postoperatively using the Pediatric Evaluation of Disability Inventory (PEDI). RESULTS: The mean PEDI self-care score increased by 6.8 (P=0.017) and the mean PEDI mobility score increased by 7.2 (P=0.020) at 1-year postsurgery. Functional gains were greater in moderate OI (types IV, V, and VI) than in severe OI (type III). Improved function was maintained in the majority of cases at a mean of 8.9 years postcorrection. CONCLUSIONS: Corrective forearm surgery in children with OI leads to improved functional ability. LEVEL OF EVIDENCE: Level IV.

Functional Outcome of Humeral Rodding in Children With Osteogenesis Imperfecta.

BACKGROUND: The impact of humeral rodding on functional ability in children with osteogenesis imperfecta (OI) has not previously been reported. This article investigates this issue. METHODS: A retrospective chart review was conducted on 35 children with OI who underwent humeral rodding at our institution between 1995 and 2013. Fassier-Duval rods were inserted in 19 cases, K-wires in 13 cases, and Rush rods in 3 cases. Functional ability was assessed preoperatively and every year postoperatively using the self-care and mobility domains of the Pediatric Evaluation of Disability Inventory (PEDI). RESULTS: The mean PEDI self-care score increased by 5.7 (P=0.028) and the mean PEDI mobility score increased by 3.6 (P=0.008) at 1-year postsurgery. Improved function was maintained in the majority of cases at a mean of 7.0 years postcorrection. CONCLUSIONS: Humeral rodding in children with OI leads to significant improvement in functional ability. LEVEL OF EVIDENCE: Level IV.