Pediatric Clavicle Fractures and Congenital Pseudarthrosis Unraveled

Clavicle fractures are commonly seen in the pediatric and adolescent populations. In contrast, congenital pseudarthrosis of the clavicle is rare. Although both conditions may present with similar signs and symptoms, especially in the very young, clear differences exist. Clavicle fractures are often caused by trauma and are tender on palpation, while pseudarthrosis often presents with a painless protuberance on the clavicle, which becomes more prominent as the child grows. Its presence may only become apparent after trauma, as it is usually asymptomatic. The diagnosis is confirmed on plain radiography, which shows typical features to distinguish both entities. Both clavicle fractures and congenital pseudarthrosis are generally treated conservatively with a high success rate. Operative treatment for a fracture can be indicated in the case of an open fracture, severely displaced fracture, floating shoulder, neurovascular complications or polytrauma. Congenital pseudarthrosis requires operative treatment if the patient experiences progressive pain, functional limitation and late-onset thoracic outlet symptoms, but most operations are performed due to esthetic complaints.

Clinical Features of Congenital Pseudarthrosis of the Tibia in Children：a Study Focusing on 514 Cases in China

Background: Congenital pseudarthrosis of the tibia (CPT) is a refractory and rare disease. Because of its extremely low incidence, little is known about its clinical features. In this retrospective study, the aim of this study was to analyze the clinical characteristics of patients with CPT. Methods: This is a retrospective study of children with CPT identified by radiological review. Investigations of CPT included general condition, the characteristics of CPT, treatment methods and surgical complications. Results: We have collected 514 CPTs from 1999 to March 2020 in our hospital, 317 (61.67%) boys, 197 (38.33%) girls; 330 (62.86%) in Crawford IV; 510 (97.14%) in mid and distal 1/3 tibia; 481 (93.58%) in less than 3 years of first appearance of symptom; 297 (57.78%) in less than 3 years of the first visit of outpatient. The most common postoperative complication was ankle valgus (101, 39.60%), followed by limb length discrepancy（91, 35.69%), refracture (38, 14.90%), osteomyelitis (15, 5.88%) and removal of internal fixation (10,3.93%) . Conclusions: CPT with higher incidence of Crawford IV frequently occurs in boys and middle or distal part of the tibia; most patients have the first appearance of symptom and the first visit of outpatient before 3 years; the major surgical complications were ankle valgus and limb length discrepancy.

Efficacy Comparison of Three Kinds of Distal Tibial Hemiepiphyseal Implants in the Treatment of Postoperative Ankle Valgus of Congenital Pseudarthrosis of the Tibia

Background: Congenital pseudarthrosis of the tibia is a complex and serious disease in orthopedics which often requires multiple operations for treatment. Postoperative ankle valgus deformity is easily seen after the operation of congenital pseudarthrosis of the tibia. The aim of this study is to retrospectively evaluate the safety of three different implants for treating postoperative ankle valgus after congenital pseudarthrosis of the tibia.Methods: A total of 41 patients with postoperative ankle valgus after congenital pseudarthrosis of the tibia from December 2010 to July 2019 were selected. Out of these 41 patients, 23 patients were treated with “U”-shaped tension screw, 10 patients were treated with hollow screw and 8 patients were treated with cortical bone screw. The evaluation index was tibiotalar angle. The general data, preoperative, postoperative and final follow-up imaging data were recorded, and the deformity correction rate and complications were compared. Results: all the patients were performed with postoperative follow-up visit for at least 12 months (31 months on average). In the “U”-shaped tension screw group, the preoperative tibiotalar angle was (74.8±4.8°), the tibiotalar angle was (85.8±4.5°) when the internal fixation was removed; in the hollow screw group, the average preoperative tibiotalar angle was (72.2±6.1°), the average tibiotalar angle was (88.4±5.1°) when the internal fixation was removed; in the cortical bone screw group, the average preoperative tibiotalar angle was (75.1±4.2°), the average tibiotalar angle was (88.4±5.1°) when the internal fixation was removed. The correction effect of the “U”-shaped tension screw group was better than that of the other two groups, but the difference was not significant (the correction rate of the “U”-shaped tension screw group was 0.71°/month, with that of in the hollow screw group and cortical bone screw group being 0.64°/month and 0.61°/month respectively, P＞0.05). One case of internal fixation complication was reported in the hollow screw group; two cases of missing correction effect were reported, one in cortical bone screw group and one in hollow screw group; and two cases showing symptom of wound pain were reported in the “U”-shaped screw group. Conclusion: Ankle valgus is a common postoperative complication of congenital tibial pseudarthrosis. Temporary hemiepiphyseal is an effective treatment for postoperative ankle valgus deformity of congenital pseudarthrosis of the tibia in children. Through comparison, the "U"-shaped tension screw provides relatively better orthopedic results and has a lower rate of internal fixation complications.

Mathematical modeling of the osteosynthesis of the lower leg bones using a titanium mesh for their congenital pseudoarthrosis in the lower third

Background. Congenital pseudarthrosis of the lower leg bones belongs to the group of orphan diseases and manifests itself in non-union (pseudarthrosis) of the lower leg bones, which leads to the formation of deformity and shortening of the limb, and, as a result, is accompanied by a persistent violation of the limb’s supportability. To date, the question of choosing a surgical technique and optimal fixation in the surgical treatment of congenital pseudarthrosis of the lower leg bones remains unresolved. The purpose was to investigate the stress-strain state of the shin model with pseudarthrosis of its bones in the lower third during their osteosynthesis using a titanium mesh. Materials and methods. Osteosynthesis was simulated with an intramedullary nail, wires, and a block of bone grafts on both shin bones with the imposition of a titanium mesh over the grafts under the influence of two types of compression and torsion loads. Results. Studies have shown that additional fixation of the bone graft block with a titanium mesh allows reducing the stress level at almost all control points of the model of osteosynthesis of the lower leg bones in their congenital pseudarthrosis, both under compression and torsion. As the most positive changes, we can note a decrease in the stress values on the tibia around the nonunion line. This can be explained by the fact that the bone graft block reinforced with a titanium mesh takes on a great load. The same fact contributes to the increase in the level of stress at the lower border of the graft block and tibia. The negative factors include an increase in the level of stress along the line of non-union of the fibula. This is also explained by an increase in the modulus of elasticity of the bone graft block, which further loads the fibula. Conclusions. The use of a titanium mesh for additional fixation of the shin bones in the treatment of their congenital pseudarthrosis has a positive effect on the stress-strain state of the model, which is confirmed by a decrease in the stress level at almost all control points of the model, both under compression and torsion loads. The most positive changes are observed along the line of non-union of the tibia.

Application of the “telescopic rod” in a combined surgical technique for the treatment of congenital pseudarthrosis of the tibia in children

Background The current surgical treatment of choice is the combination surgical technique, involving tibial intramedullary fixation to maintain the mechanical axis and mechanical stability of tibial pseudarthrosis. In traditional combined surgery, the Williams rod is often used. Long-term intramedullary fixation of the foot and ankle will affect the ankle joint function of children. The intramedullary rod is relatively shorter due to the growth of the distal tibia. In addition, there are some complications such as epiphyseal bone bridge and high-arched foot. The use of a telescopic intramedullary rod may avoid these complications. Purposes To investigate the initial effect of the “telescopic rod” in a combined surgical technique for the treatment of congenital pseudarthrosis of the tibia in children. Methods A retrospective study including 15 patients with Crawford type IV CPT who were treated using a combined surgical technique and the telescopic rod from January 2017 to May 2018. The average age at the time of surgery was 43.3 months (16–126 months). Of the 15 patients, 7 had proximal tibia dysplasia and 12 exhibited neurofibromatosis type 1. The combined surgical technique using the telescopic rod included the excision of pseudarthrosis, intramedullary rod insertion, installation of Ilizarov’s fixator, tibia-fibular cross union, and wrapping autogenic iliac bone graft. The incidence of refracture, ankle valgus, tibial valgus, and limb length discrepancy (LLD) in patients were investigated. Results All patients achieved primary union with an average follow-up time of 37.3 months (26–42 months). The mean primary union time was 4.5 months (4.0–5.6 months). Nine cases showed LLD (60%), with an average limb length of 1.1 cm (0.5–2.0 cm). Ankle valgus, proximal tibial valgus, telescopic rod displacement, and epiphyseal plate tethering occurred in 1 case (6.6%) (18°), 3 cases (20%) (10°, 5°, and 6°, respectively), 6 cases (40%), and 2 cases (13%), respectively. There were no refractures during the follow-up periods. Conclusion Although there are complications such as intramedullary rod displacement while using the telescopic rod in a combined surgery, the primary healing rate of congenital pseudarthrosis of the tibia in children is high.

Free Vascularized Fibular Graft for Congenital Pseudarthrosis Tibia: A Case Series

Introduction: Congenital pseudarthrosis tibia (CPT) probably one of the most difficult to treat among all diseases in the children. There are several surgical approaches that have been used to treat CPT including on-lay graft, double on-lay grafts, pedicle grafts, osteotomy, bypass graft and intramedullary rods. Prognosis of CPT has changed considerably with the use of Free Vascularized Fibular Graft (FVFG). Despite these advances, several operations are often necessary to obtain union of CPT and the risk of amputation is never entirely eliminated Case presentation: We presented 2 case with CPT. The first case is a 4 year old girl with CPT-Associated Neurofibromatosis, patient was brought to orthopedic polyclinic complaining bend on her right leg since she was born, the parent also complained her child has abnormality when walking since she was 14 months old. The second case is a 3 year old girl with CPT complaining bend on her right leg, abnormalities and pain when walking since she was aged 1 years 11 months. We performed free vascularized fibular graft for both of the patient. Result: The results in our cases showed bone union in 14 weeks, 16 weeks and 18 weeks respectively. The optimal of technique options should be adapted to the type of pseudarthrosis and especially to the extent of the bone defects. good results can be found with intramedullary nailing with a bone graft or the Ilizarov technique Conclusion: The optimal of technique options should be adapted to the type of pseudarthrosis and especially to the extent of the bone defects Keywords: Congenital pseudarthrosis tibia, Free vascularized fibular graft, case series.

Are Children Suffering From Congenital Pseudarthrosis of the Tibia Associated With Bone Quality Decreased?

Background: Congenital pseudarthrosis of the tibia (CPT) is a rare and difficult to treat congenital disease in neonates. Our team's previous study found that exosomes derived from serum of children with CPT can reduce the effect of bone formation. In this study, we used ultrasound bone densitometry to detect the bone quality between children with CPT and those non-metabolic disease children, to determine the bone strength of children with CPT. Methods: A total of 37 CPT children and 40 children without the bone metabolic disease (control group) were recruited in our hospital. The ultrasonic bone densitometer was used to examine the bilateral calcaneus of the subjects. According to the measurement results, we collected the broadband ultrasonic attenuation (BUA), sound transmission velocity (SOS), quantitative ultrasound index (QUI), bone strength index(STI) and bone mineral density estimation(BMDe) values. For the intergroup analysis, t test was used to determine the differences of various quantitative measurements. Multivariable regression was used to examine the associations between quantitive ultrasound measurements differences and age, body mass index (BMI), neurofibromatosis type 1 (NF1) and CPT Crawford type. Intra-class correlation coefficient (ICC) was calculated to estimate intra- and inter-rater agreements. Results: Seventy-four calcaneus scans from CPT patients (23 boys and 14 girls) and 80 calcaneus scans (24 boys and 16 girls) from the control. The CPT patients exhibited significantly lower SOS (1368.75±136.78 m/s), STI (7.2319±38.6525), QUI (8.2532±56.1720) and BMDe (-0.0241±0.3552 g/cm3) than the control (SOS: 1416.02±66.15 m/s, STI: 7.96±16.884, QUI: 28.8299±25.461, BMDe: 0.0180±0.1610 g/cm3). Multiple linear regression revealed that SOS, STI, QUI was statistically significant and negatively correlated with CPT Crawford classification.Conclusions: We found the incidence of decreased bone quality in CPT group was higher than that in the non-bone metabolic disease group. This phenomenon was not related to NF1 while related to CPT Crawford classification, which suggested that the higher grade of the CPT Crawford classification, the lower the bone strength and the higher the risk of fracture.

Orthopaedic management of congenital pseudarthrosis of the tibia

Congenital pseudoarthrosis of the tibia is an uncommon but important condition, often associated with neurofibromatosis, in which the tibia has a region of abnormal bone prone to fracture and subsequent non-union with a fibrocartilaginous pseudoarthrosis forming at the fracture site. The limb is prone to malalignment and distal deformity. Management requires correction and stabilization of the deformity with excision of the affected tissue of the tibia and reconstruction either with bone grafting, transport, or transfer of vascularized fibula.