Outcomes of Simultaneous Lengthening and ACL Reconstruction in Fibular Hemimelia: A Retrospective Case Series.

Background: Fibular hemimelia is the most common congenital long bone deficiency. It is often associated with femoral and tibial deficiencies which result in a clinically evident leg length discrepancy. The primary soft tissue concern is ACL/PCL deficiency. If treatment includes bony lengthening, joint stability is imperative to avoid complications. In this study, we detail a novel technique for long bone lengthening and ACL reconstruction in a single, cohesive surgery. This consolidates the need for multiple procedures and offers improved limb length symmetry and knee stability for this patient population. Clinical outcomes of pediatric patients with hemimelia who underwent either femoral or tibial lengthening with PRECICE® nail and concomitant ACL reconstruction are presented. Methods: After IRB approval, we identified five patients with complex fibular hemimelia who underwent ACL reconstruction and concomitant lengthening with at least two years of follow-up. Two patients (40%) presented with congenital short femur, and three (60%) with congenital short tibia. In each case, ACL reconstruction and either femoral or tibial guided growth via PRECICE® nail were performed. Operative techniques involving both soft tissue and bony methodology are described in detail. Results: All patients had objective improvement in knee stability as assessed both intra and post operatively, as well as successful intermedullary lengthening without complications related to joint stability. Three patients had minor complications unrelated to joint stability that did not interfere with overall result. Conclusion: Fibular hemimelia associated with hypoplasia of bony and soft tissue structures can be successfully addressed with concomitant ligamentous reconstruction at the time of implantation of lengthening devices. This addresses knee instability and reduces both number of operative procedures and potential complications related to joint instability while pursuing bony lengthening. Level of Evidence: V.

Prediction of Late Dysplasia Based on Ultrasound and Plain X-Ray at 6 Months.

BACKGROUND: Developmental dysplasia of the hip represents a spectrum of deformity. Residual dysplasia at 2 years of age is associated with an increased risk for osteoarthritis and functional limitations. We compared the prognostic value of 6-month imaging modalities and aimed to identify optimal diagnostic metrics for the prediction of residual dysplasia. METHODS: After IRB approval, patients who underwent Pavlik treatment between 2009 and 2018 with 2-year follow-up were identified. Sonographs [ultrasound (US)] and radiographs (x-ray) were obtained at 6-month and 2-year-old visits. Dysplasia at 2 years was defined as an acetabular index (AI) >24 degrees. Receiver operating characteristic curves were constructed to quantitatively compare the prognostic ability of US and x-ray-based measures at 6 months. Youden's index [(YI) (values range from 0 (poor test) to 1 (perfect test)] was used to evaluate existing cutoffs at 6 months of age (normal measurements: alpha angle (AA) ≥60 degrees, femoral head coverage (FHC) ≥50%, and AI <30 degrees) relative to newly proposed limits. RESULTS: Fifty-nine patients were included, of which 28.8% of patients (95% CI: 17.3 to 40.4%) had acetabular dysplasia at 2 years. After adjusting for sex, AA [Area under the Curve (AUC): 80] and AI (AUC: 79) at 6 months of age were better tests than FHC (AUC: 0.77). Current diagnostic cutoffs for AA (YI: 0.08), AI (YI: 0.0), and FHC (YI: 0.06) at 6 months had poor ability to predict dysplasia at 2 years. A composite test of all measures based on proposed cutoffs (AA ≥73 degrees, FHC > 62% and AI ≤24 degrees) was a better predictor of dysplasia at 2 years (Youden's index (YI): 0.63) than any single metric. CONCLUSIONS: The rate of residual dysplasia remains concerning. The 6-month x-ray and US both play a role in the ongoing management of the developmental dysplasia of the hip. The prediction of dysplasia is maximized when all metrics are considered collectively. Existing parameters were not accurate; We recommend the following cutoffs: AA ≥73 degrees, FHC > 62%, and AI ≤24 degrees. These cutoffs must be validated. LEVEL OF EVIDENCE: Prognostic Level II.

Application of Accepted Use Criteria for the Treatment of Developmental Dysplasia of the Hip Decreases the Number of Infants Treated With a Pavlik Harness.

BACKGROUND: This analysis examined how the application of the American Academy of Orthopedic Surgeons appropriate use criteria (AUC) for developmental dysplasia of the hip in infants would change treatment patterns and outcomes for Graf IIA hips at a single quaternary pediatric hospital. METHODS: After Institutional Review Board approval, patient medical records were reviewed and data were collected. Graf IIa hips were defined as alpha angle (AA) 50 to 59 degrees. AA and femoral head coverage (FHC) were measured from initial and 6-month ultrasounds and acetabular index (AI) was measured from radiographs at 6 months of age. Instability (positive Ortolani and Barlow tests) was noted. On the basis of the American Academy of Orthopedic Surgeons AUC for managing developmental dysplasia of the hip, hips were further categorized as normal (FHC ≥45%), borderline (FHC 35% to 44%), or dysplastic (FHC <35%). RESULTS: Overall, 13% (49/371) of Graf IIa hips (AA 50 to 59 degrees) were dysplastic (FHC <35%). Total 24% (89/371) were clinically unstable. Total 42% (37/89) of unstable Graf IIa hips were dysplastic. Only 4% of stable Graf IIa hips were dysplastic (12/282). Out of 371 Graf IIa hips, 256 were treated with Pavlik harness (n=250) or Rhino brace (n=6). Among stable, nondysplastic (SND) hips (those with normal and borderline FHC≥35%), 33% (52/158) were treated because of a more severe contralateral side. If the AUC had been applied, 67% (106/158) of SND Graf IIa hips would not have been treated. Among the n=162 hips that returned for a 6-month radiograph, there was no difference in AI in the 115 treated and 47 untreated SND hips (mean difference treatment vs. no treatment: -1.5, 95% CI, -3.1 to 0.2, P =0.0808). CONCLUSIONS: Using AUC recommendations, our center could reduce the number of SND Graf IIa hips we treat by 67%. Although 24% of Graf IIa hips were clinically unstable and 13% were dysplastic based on FHC, most Graf IIa hips had normal or borderline FHC per the AUC and may do well with observation and follow-up ultrasound at 12 weeks old. LEVEL OF EVIDENCE: Level III-diagnostic study.

Genetic variants associated with the occurrence and progression of adolescent idiopathic scoliosis: a systematic review protocol.

BACKGROUND: Adolescent idiopathic scoliosis (AIS) is a structural lateral spinal curvature of ≥ 10° with rotation. Approximately 2-3% of children in most populations are affected with AIS, and this condition is responsible for approximately $1.1 billion in surgical costs to the US healthcare system. Although a genetic factor for AIS has been demonstrated for decades, with multiple potentially contributory loci identified across populations, treatment options have remained limited to bracing and surgery. METHODS: The databases MEDLINE (via PubMed), Embase, Google Scholar, and Ovid MEDLINE will be searched and limited to articles in English. We will conduct title and abstract, full-text, and data extraction screening through Covidence, followed by data transfer to a custom REDCap database. Quality assessment will be confirmed by multiple reviewers. Studies containing variant-level data (i.e., GWAS, exome sequencing) for AIS subjects and controls will be considered. Outcomes of interest will include presence/absence of AIS, scoliosis curve severity, scoliosis curve progression, and presence/absence of nucleotide-level variants. Analyses will include odds ratios and relative risk assessments, and subgroup analysis (i.e., males vs. females, age groups) may be applied. Quality assessment tools will include GRADE and Q-Genie for genetic studies. DISCUSSION: In this systematic review, we seek to evaluate the quality of genetic evidence for AIS to better inform research efforts, to ultimately improve the quality of patient care and diagnosis. SYSTEMATIC REVIEW REGISTRATION: PROSPERO registration #CRD42021243253.

Genetic animal modeling for idiopathic scoliosis research: history and considerations.

PURPOSE: Idiopathic scoliosis (IS) is defined as a structural lateral spinal curvature ≥ 10° in otherwise healthy children and is the most common pediatric spinal deformity. IS is known to have a strong genetic component; however, the underlying etiology is still largely unknown. Animal models have been used historically to both understand and develop treatments for human disease, including within the context of IS. This intended audience for this review is clinicians in the fields of musculoskeletal surgery and research. METHODS: In this review article, we synthesize current literature of genetic animal models of IS and introduce considerations for researchers. RESULTS: Due to complex genetic and unique biomechanical factors (i.e., bipedalism) hypothesized to contribute to IS in humans, scoliosis is a difficult condition to replicate in model organisms. CONCLUSION: We advocate careful selection of animal models based on the scientific question and introduce gaps and limitations in the current literature. We advocate future research efforts to include animal models with multiple characterized genetic or environmental perturbations to reflect current understanding of the human condition.

Anti-VEGF antibody delivered locally reduces bony bar formation following physeal injury in rats.

Physeal injuries can result in the formation of a "bony bar" which can lead to bone growth arrest and deformities in children. Vascular endothelial growth factor (VEGF) has been shown to play a role in bony bar formation, making it a potential target to inhibit bony repair tissue after physeal injury. The goal of this study was to investigate whether the local delivery of anti-VEGF antibody (α-VEGF; 7.5 µg) from alginate:chitosan hydrogels to the tibial physeal injury site in rats prevents bony bar formation. We tested the effects of quick or delayed delivery of α-VEGF using both 90:10 and 50:50 ratio alginate:chitosan hydrogels, respectively. Male and female 6-week-old Sprague-Dawley rats received a tibial physeal injury and the injured site injected with alginate-chitosan hydrogels: (1) 90:10 (Quick Release); (2) 90:10 + α-VEGF (Quick Release + α-VEGF); (3) 50:50 (Slow Release); (4) 50:50 + α-VEGF (Slow Release + α-VEGF); or (5) Untreated. At 2, 4, and 24 weeks postinjury, animals were euthanized and tibiae assessed for bony bar and vessel formation, repair tissue type, and limb lengthening. Our results indicate that Quick Release + α-VEGF reduced bony bar and vessel formation, while also increasing cartilage repair tissue. Further, the quick release of α-VEGF neither affected limb lengthening nor caused deleterious side-effects in the adjacent, uninjured physis. This α-VEGF treatment, which inhibits bony bar formation without interfering with normal bone elongation, could have positive implications for children suffering from physeal injuries.

Regenerative Medicine Approaches for the Treatment of Pediatric Physeal Injuries.

The physis, or growth plate, is a cartilaginous region at the end of children's long bones that serves as the primary center for longitudinal growth and characterizes the immature skeleton. Musculoskeletal injury, including fracture, infection, malignancy, or iatrogenic damage, has risk of physeal damage. Physeal injuries account for 30% of pediatric fractures and may result in impaired bone growth. Once damaged, cartilage tissue within the physis is often replaced by unwanted bony tissue, forming a "bony bar" that can lead to complications such as complete growth arrest, angular or rotational deformities, and altered joint mechanics. Children with a bony bar occupying <50% of the physis usually undergo bony bar resection and insertion of an interpositional material, such as a fat graft, to prevent recurrence and allow the surrounding uninjured physeal tissue to restore longitudinal bone growth. Clinical success for this procedure is <35% and often the bony bar and associated growth impairments return. Children who are not candidates for bony bar resection due to a physeal bar occupying >50% of their physis undergo corrective osteotomy or bone lengthening procedures. These approaches are complex and have variable success rates. As such, there is a critical need for regenerative approaches to not only prevent initial bony bar formation but also regenerate healthy physeal cartilage following injury. This review describes physeal anatomy, mechanisms of physeal injury, and current treatment options with associated limitations. Furthermore, we provide an overview of the current research using cell-based therapies, growth factors, and biomaterials in the different animal models of injury along with strategic directions for modulating intrinsic injury pathways to inhibit bony bar formation and/or promote physeal tissue formation. Pediatric physeal injuries constitute a unique niche within regenerative medicine for which there is a critical need for research to decrease child morbidity related to this injurious process.

Rotation and Displacement Predict Adverse Events in Pediatric Supracondylar Fractures.

The goal of this study was to identify supracondylar fracture patterns that were predictive of adverse events and poor outcomes. The study consisted of a retrospective review of patients admitted for surgical treatment of a supracondylar humerus fracture between June 2008 and August 2010. Preoperative radiographs were assessed based on appearance (simple vs oblique vs comminuted), coronal plane displacement (angulated, posterior, posteromedial vs posterolateral), and rotation (rotation vs no rotation). Logistic regression models were used to examine the relationship between fracture pattern and clinical outcome parameters in 373 patients who were followed for 4 weeks or more postoperatively. Outcome parameters included postoperative complications (infection, delayed healing, pin migration, revision surgery), need for physical or occupational therapy, need for postoperative intravenous narcotics, and preoperative nerve injury. Rotation and coronal displacement patterns of the fracture segments were significantly associated with postoperative complications, postoperative need for physical or occupational therapy as a result of residual stiffness, and nerve injury (P<.05). Compared with posteriorly displaced fractures, posterolaterally displaced fractures were associated with significantly greater odds of complications (P=.045), need for physical or occupational therapy (P<.001), and nerve injury (P<.001). Additionally, fractures with rotation were associated with significantly greater odds of complications (P<.001), need for physical or occupational therapy (P<.001), and nerve injury (P<.001) compared with fractures without rotation. Rotation and coronal plane displacement were predictive of complications, need for physical or occupational therapy, and nerve injury, and thus should be considered as potential prognostic variables when evaluating the initial injury pattern.

Intra-articular Radial Head Fractures In the Skeletally Immature Patient: Complications and Management.

BACKGROUND: Intra-articular (IARH) and extra-articular (EARH) radial head fractures in skeletally immature patients are rare injuries that have not been well studied. The objective of this study was to investigate the rate of complications associated with IARH fractures relative to EARH fractures in pediatric patients treated at a tertiary referral children's hospital. METHODS: With IRB approval, Current-Procedural Terminology codes were used to identify all patients who underwent management of radial head and/or neck fractures between 2005 and 2012. A retrospective chart review was used to collect variables related to: demographics, fracture type, treatment method(s), complications, need for physical/occupational therapy, and the need for subsequent surgery. Mid-P exact tests and logistic regression analyses were used to compare differences in the incidence of complications, need for physical therapy (PT), and need for revision surgery between the IARH and EARH fracture groups. RESULTS: Among the 311 patients included in the cohort, 12 (3.86%) were affected by IARH fractures and 299 (96.14%) were affected by EARH fractures. The mean age at the time of injury was 11.46 (±3.09) years and 8.32 (±3.31) years in the IARH and EARH group, respectively. The estimated incidence of complications was significantly (P<0.0001) higher in the IARH group (50 per 100) compared with the EARH group (1.34 per 100). A significantly (P<0.0001) greater proportion of the subjects with IARH fractures also required revision surgery (25% IARH vs. 0% EARH) and PT (50% IARH vs. 19.59% EARH). CONCLUSIONS: Compared with EARH fractures, IARH fractures were associated with a significantly higher rate of complications, greater need for PT, and greater need for surgical intervention. The significant complication rate associated with pediatric IARH fractures necessitates an increased awareness of this fracture pattern and prompt, aggressive diagnostic and treatment modalities. LEVEL OF EVIDENCE: Therapeutic studies: Level III.

Posterior sternoclavicular joint injuries in skeletally immature patients.

The management of sternoclavicular injuries in skeletally immature patients has not been well described. The purpose of this study was to describe the authors' experience treating this rare and potentially life-threatening injury. All skeletally immature patients who underwent treatment for a medial clavicular physeal fracture or sternoclavicular dislocation between 2003 and 2011 were identified using ICD-9 diagnostic codes. Sternoclavicular injuries with posterior displacement were isolated from this cohort for a thorough chart review. Patients were contacted to complete brief phone surveys and shoulder-specific outcome instruments. A total of 12 boys (mean age, 14.8±2.74 years), followed for an average of 10.3 months (range, 0-54 months), were identified. The incidence of significant associated symptoms was 8.3% (1 of 12). Eight patients were initially treated with closed reduction, 2 (25%) successfully and 6 (75%) requiring subsequent open reduction. Four of the 12 patients underwent an immediate open reduction. Braided composite sutures were used to treat all injuries that underwent open reduction (10 of 12). Complete data were obtained from 6 patients, all of whose injuries had been treated with open reduction. All 6 had returned to their full activity level, and all self-reported perfect Quick Disabilities of the Arm, Shoulder and Hand (QuickDASH) and Simple Shoulder Test scores (0 and 12, respectively). Among skeletally immature patients, medial clavicular physeal fractures and sternoclavicular dislocations can be effectively managed with closed or open reduction. When closed reduction is unsuccessful or is contraindicated, open reduction with braided composite sutures is associated with excellent results.

Paediatric spinal fusion surgery and the transition to home-based care: provider expectations and carer experiences.

There are more than 12 million children with special healthcare needs (CSHCNs) in the United States, many of whom require specialised health-care to treat chronic physical and developmental conditions. This study is a qualitative investigation of programme, surgical and at-home recovery experiences among CSHCNs and their family carers who participated in a spine surgical care programme at a paediatric hospital in the Western United States. The programme is designed to manage increased surgical risk and the transition of care from hospital to home for children with severe scoliosis undergoing spinal fusion surgery. We conducted 30 semi-structured in-depth interviews with 14 surgical team members and 16 family carers of children who had programme evaluations and spinal surgeries in 2006. Data were collected in 2008 and 2009 in hospital or at home locations to gather programme participation feedback from families and inform the adequacy of programme support to families during at-home recovery. Data were analysed by reflexive team and content analysis methodologies. Results showed the programme was effective at improving preoperative surgical evaluation and helping families to anticipate some aspects of the surgical experience and hospital discharge. However, the impact of spinal fusion surgery and the subsequent transition to home-based care was profoundly emotional for patients and their carers. Our data indicate that programme providers underestimated the extent of emotional trauma experienced by patients and families, particularly during the at-home recovery process. The data also suggest meaningful differences in providers' and carers' expectations for surgery. Carers' disappointment with their recovery experiences and the perceived lack of post-discharge support impacted their interpretations of and perspectives on their surgical experience. Implications of this research for surgical care programmes include the need for assessment and provision of support for physical, social, and emotional burdens experienced by patients and carers at pre-surgical, surgical and at-home recovery phases.