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.

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.

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.

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.

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.

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.

Safety and Efficacy of Botulinum Toxin A in Children Undergoing Lower Limb Lengthening and Deformity Correction: Results of a Double-blind, Multicenter, Randomized Controlled Trial.

BACKGROUND: Lengthening of the lower limb is a complex procedure in which pain management and complications such as pin-site infections and muscle contractures impact the family and affect the child's quality of life. As a result, the paralytic and antinociceptive actions of neurotoxins may be indicated in managing these complications; however, few studies have explored ways to improve outcomes after lengthenings. The objective of this study was to evaluate the safety and efficacy of botulinum toxin A (BTX-A) in children undergoing lower limb lengthenings and deformity correction. METHODS: Participants with a congenital or acquired deformity of the lower extremity requiring surgery to one limb were randomized to receiving either BTX-A as a single dose of 10 units per kilogram body weight, or an equivalent volume of saline solution. Pain, medication, quality of life, and physical function were assessed at different time-points. Adverse events were recorded in all participants. T test and χ tests were used to compare potential differences across both groups. RESULTS: Mean age of the 125 participants was 12.5 years (range, 5 to 21 y), and lengthenings averaged 4.2 cm. Maximum pain scores on day 1 postoperatively were lower in the BTX-A group (P=0.03) than in the placebo group, and remained significant favoring botox when stratifying by location of lengthening (femur vs. tibia). Clinical benefits for BTX-A were found for 3 quality of life domains at mid-distraction and end-distraction. When stratifying according to location of lengthening, there were significantly fewer pin-site infections in the tibia favoring botox (P=0.03). The amount of adverse events and bone healing indices were no different in both groups. CONCLUSIONS: The clinical differences in quality of life, the lower pain on the first postoperative day, and the lower number of pin-site infections in the tibia favoring BTX-A support its use as an adjunctive treatment to the lengthening process. The detailed analyses of pain patterns help inform families on the pain expectations during lower limb lengthenings. The amount of adverse events were no different in both groups, and bone healing rates were similar, indicating that the use of BTX-A in children undergoing limb lengthening and deformity correction is safe. LEVEL OF EVIDENCE: Level I.

Does Sclerostin Depletion Stimulate Fracture Healing in a Mouse Model?

BACKGROUND: Sclerostin is a secreted glycoprotein that inhibits the intracellular Wnt signaling pathway, which, when inactivated, stimulates bone formation. This has been seen in fracture studies, which have shown larger and stronger calluses with accelerated fracture healing in sclerostin knockout and sclerostin antibody injection models. However, the effects of these two mechanisms have not been compared in the context of fracture healing. QUESTIONS/PURPOSES: We sought to determine the degree to which sclerostin inhibition (Scl-Ab injection) and complete sclerostin depletion inhibit fracture healing in a mouse model as evaluated by (1) morphometric trabecular bone measures at the fracture site, and (2) fracture site structural strength. METHODS: Ten-week-old male sclerostin knockout (n = 20) and wild type (n = 40) mice underwent insertion of a tibial intramedullary pin after which a midshaft tibial osteotomy was performed. The mice were divided in three groups: sclerostin knockout (n = 20), wild type with sclerostin antibody injection (intravenous dose of 100 mg/kg weekly) (n = 20), and wild type with saline injection (n = 20). The mice for each group where subdivided and euthanized at 14, 21, 28, and 35 days after surgery, at which time the fractured tibias were assessed with microCT (to assess morphometric trabecular bone measures: bone volume to total volume (BV/TV), trabecular thickness, trabecular number, and structural model index at the fracture site. Biomechanical testing in the form of three-point bending also was done to assess fracture site structural strength. A difference greater than 3.7% in our primary outcome (BV/TV) would be required to detect a difference between groups with a power of 80%, as per our power analysis. RESULTS: The wild type with sclerostin antibody and the sclerostin knockout groups showed increased trabecular BV/TV at the fracture site compared with the wild type group with saline at all times, however no difference was seen between the treatment groups with the numbers available, except at 28 days postoperatively when the sclerostin knockout group showed greater BV/TV than the wild type sclerostin antibody group (47.0 ± 3.5 vs 40.1 ± 2.1; p < 0.05). On biomechanical testing the wild type sclerostin antibody showed increased stiffness at Days 14 and 28 compared with the wild type with saline group (70.9 ± 6.4 vs 14.8 ± 8.1; p = 0.001), (106.8 ± 24.3 vs 74.9 ± 16.0; p = 0.004); respectively. However, with the numbers available, no differences were detected between the wild type with sclerostin antibody and the sclerostin knockout groups in terms of whole-bone structural strength. CONCLUSIONS: Sclerostin antibody injections showed promising results, which were not different with the numbers available, from results achieved with complete depletion of sclerostin, especially at earlier stages of the healing process, and therefore completed the healing process at an earlier time. CLINICAL RELEVANCE: Sclerostin antibody injections appear to enhance fracture healing to a degree that is not different than complete sclerostin depletion, but larger animal studies are required to assess the accurate dosage and timing of administration in the fracture healing process to further evaluate its potential clinical utility to enhance fracture healing.

Hip joint osteochondroma: systematic review of the literature and report of three further cases.

The aim of this study is to systematically review the literature with regards to surgical treatment of patients with hip joint osteochondromas, and to report our surgical management of three paediatric patients who had femoral neck or acetabular osteochondromas in association with acetabular dysplasia. We performed a systematic review using PubMed and Embase databases for all studies that reported surgical treatments for patients with peritrochanteric or acetabular osteochondroma with or without acetabular dysplasia. We also retrospectively reviewed three patients who were diagnosed with a hip osteochondroma in association with actetabular dysplasia. These patients were known to have hereditary multiple exostoses (HME). The systematic review revealed 21 studies that met our inclusion criteria. All studies were case reports and retrospective in nature and failed to conclude a uniform treatment plan. The three reported cases illustrate successful excision of hip osteochondromas and treatment of acetabular dysplasia. Early excision of hip osteochondromas might prevent acetabular dysplasia in HME patients. Routine radiographic pelvic survey at the time of diagnosis of HME is recommended for early detection of hip osteochondromas and acetabular dysplasia in these children.

Dega osteotomy for the correction of acetabular dysplasia of the hip: a radiographic review of 21 cases.

BACKGROUND: The Dega osteotomy is a versatile procedure that is widely used to treat neuromuscular hip dysplasia. There is a paucity of the English language literature on its use in acetabular dysplasia seen in developmental dysplasia of the hip (DDH). METHODS: A retrospective radiographic and chart review was performed for all patients diagnosed with DDH who underwent a modified Dega osteotomy between March 1995 and December 2008 at the Shriners Hospital for Children or the Montreal Children's Hospital (Montréal, Canada) by 2 orthopaedic surgeons. Radiographic parameters were measured at the preoperative, immediate postoperative, and final follow-up time points. These parameters included the acetabular index, center edge angle, Reimer's extrusion index, Shenton line, and grading by the Severin classification. RESULTS: A total of 20 patients (21 hips), of which 18 were female, underwent a modified Dega osteotomy at an average age of 55.6 months (range, 20 to 100 mo). Of the 21 hips (1 bilateral and 19 single cases), 9 hips involved the right side and 12 hips involved the left side. Before surgery, 9 patients had a subluxated hip, 7 patients had a dislocated hip, and 5 patients had a dysplastic hip. Ten hips underwent concomitant procedures including 10 open reductions with capsulorraphy. The acetabular index improved from 37 degrees (SD 8) preoperatively to 19 degrees (SD 8) on the date of last visit. The center edge angle improved from 2 (SD 17) to 25 degrees (SD 12). CONCLUSIONS: The results of this study demonstrate that the modified Dega osteotomy produces near-normal lateral coverage parameters in children with DDH. LEVEL OF EVIDENCE: Therapeutic study, clinical case series: level IV.

Correction of proximal tibial recurvatum using the Ilizarov technique.

BACKGROUND: Genu recurvatum is a debilitating deformity of the knee that can progress during growth and lead to significant deformity if left untreated. Recurvatum can be osseous, ligamentous, or mixed. Osseous genu recurvatum is most often due to asymmetrical growth arrest of the proximal tibial physis affecting primarily the tibial tubercle. Several methods have been described in the literature regarding the correction of this deformity; nevertheless, there is a paucity of knowledge of the correction of proximal tibial recurvatum using the Ilizarov technique. METHODS: A retrospective chart and radiographic review of all patients treated by distraction osteogenesis with angular correction distal to the tibial tubercle using an Ilizarov external fixator was performed. A total of 9 patients were treated during the study period and the average age at the time of surgery was 14.3 years (range, 7-16 y). There were 3 female patients and 6 male patients. Four cases were due to deformity secondary to trauma and the treatment thereof, 2 patients had spondyloepiphyseal dysplasia, and 3 patients had unknown etiology. The angle of recurvatum (RG) and the angle of tilt of the tibial plateau (RT) preoperatively measured 28 degrees (range, 18 to 32 degrees) and 69 degrees (range, 60 to 82 degrees), respectively. At final follow-up, the RG and RT measured 7 degress (range, 4 to 12 degrees) and 93 degrees (range, 86 to 100 degress), respectively. Using the scoring system of Lecuire and colleagues, the overall results were good to excellent at a mean follow-up of 4.4 years. CONCLUSIONS: Correction of osseous proximal tibial recurvatum by the Ilizarov method, allows obtaining an improved sagittal mechanical axis in the lower extremity.

Ambulation gains after knee surgery in children with arthrogryposis.

BACKGROUND: Arthrogryposis multiplex congenita is a rare congenital disorder associated with multiple musculoskeletal contractures that causes substantial morbidity. Knee involvement is commonly seen among children with arthrogryposis, with flexion contracture of the knee being the most frequent knee deformity. Knee flexion contractures in the pediatric population are particularly debilitating as they affect ambulation. Treatment for knee flexion contractures requires numerous orthopaedic procedures and an extensive follow-up period. The purpose of this study was to assess the effectiveness of orthopaedic procedures, namely distal femoral supracondylar extension osteotomy and/or Ilizarov external fixator, on the ambulation status of children with knee flexion contracture and whether any functional gains are maintained at the latest follow-up. METHODS: All children with arthrogryposis followed at our institution who had surgical correction for knee flexion contractures were included in this study. Fourteen patients were identified and their medical records were reviewed. The etiology for all patients was amyoplasia. The mean age at first surgery was 7.0 years (range, 2 to 16 y). The mean length of follow-up was 59.3 months (range, 12 to 117 mo). Contractures were treated with femoral extension osteotomy (n=8), Ilizarov external fixator (n=1), or both (n=5). Three patients earlier had posterior soft tissue releases, including hamstrings lengthenings, proximal gastrocnemius release, and release of posterior capsule. RESULTS: Preoperatively, 11 patients were nonambulatory, 2 patients were household ambulators, and 1 patient walked with orthoses in the community. There was an average of 1.8 knee surgeries done per patient, namely distal femoral extension osteotomy and/or Ilizarov external fixator. At the latest follow-up, 8 patients were ambulatory with technical aids (orthosis, walker, braces, or rollator walker), 2 patients were household ambulators, 1 patient used a wheelchair but was independent for transfers, and 3 patients remained nonambulatory. The mean flexion contracture before the first surgery was 63.7 ± 26.8 degrees. Postoperatively, the mean flexion contracture was 13.2 ± 16.7 degrees. At the latest follow-up, the mean flexion contracture was 34.0 ± 24.1 degrees. There were complications in 2 patients, including infected hardware which resolved with antibiotic treatment, and neurologic compromise which resolved on its own. CONCLUSIONS: Surgical correction of knee flexion deformities by distal femoral extension osteotomy and/or Ilizarov external fixator was effective in improving the ambulation status of children with arthrogryposis. At latest follow-up, the gradual loss of total arc of motion and the recurrence of knee flexion contractures did not limit the ambulatory gains achieved. LEVEL OF EVIDENCE: IV, Case series.

Delivery of recombinant bone morphogenetic proteins for bone regeneration and repair. Part A: Current challenges in BMP delivery.

Recombinant human bone morphogenetic proteins (rhBMPs) have been extensively investigated for developing therapeutic strategies aimed at the restoration and treatment of orthopaedic as well as craniofacial conditions. In this first part of the review, we discuss the rationale for the necessary use of carrier systems to deliver rhBMP-2 and rhBMP-7 to sites of bone tissue regeneration and repair. General requirements for growth factor delivery systems emphasizing the distinction between localized and release-controlled delivery strategies are presented highlighting the current limitations in the development of an effective rhBMP delivery system applicable in clinical bone tissue engineering.