Prognostic factors for failed Pavlik harness treatment in infants with developmental dysplasia of the hip: a retrospective cohort study.

PURPOSE: Pavlik harness treatment is the most common treatment in newborns diagnosed with developmental dysplasia of the hip (DDH). The success rates and predictors for failure have been debated over the last decade. In this study, we explored our treatment failure rate and potential prognostic factors that could predict the failure of Pavlik harness (PH) treatment in patients with DDH. METHODS: Two hundred and sixty-five patients were treated with PH based on the Graf hip types of classification. Age, gender, first born status, family history, foot deformity, plagiocephaly, breech presentation, hip abduction, hip stability, Graf hip type, Galeazzi sign, bilateralism, and femoral nerve palsy were tested as predictors for failure in multivariate logistic regression mode. Success and failure were determined by the normalization of the hip based on the Graf hip classification. RESULTS: The failure rate of patients treated with Pavlik harness was 16.6% which is within the reported range of failure rate. The mean age of patients who were successfully treated was 6.73 weeks in comparison to 8.84 weeks for those who failed. Age, plagiocephaly, hip instability, Graf classification, and the development of femoral nerve palsy were found to be predictors for failure of PH treatment upon univariate analysis only. However, only the presence of Galeazzi sign, hip instability, high grades of Graf hip classification, and the development of femoral nerve palsy proved to be independent predictors for failed PH treatment upon multivariate logistic regression analysis. CONCLUSIONS: Pavlik harness treatment is a successful treatment with an average success of 83.4%. Several independent predictors for failure of PH treatment have been identified. These include a positive Galeazzi sign, a frankly dislocated hip, Graf types III and IV, and the development of femoral nerve palsy.

The genomic landscape of rare disorders in the Middle East.

BACKGROUND: Rare diseases collectively impose a significant burden on healthcare systems, especially in underserved regions, like the Middle East, which lack access to genomic diagnostic services and the associated personalized management plans. METHODS: We established a clinical genomics and genetic counseling facility, within a multidisciplinary tertiary pediatric center, in the United Arab Emirates to locally diagnose and manage patients with rare diseases. Clinical genomic investigations included exome-based sequencing, chromosomal microarrays, and/or targeted testing. We assessed the diagnostic yield and implications for clinical management among this population. Variables were compared using the Fisher exact test. Tests were 2-tailed, and P < .05 was considered statistically significant. RESULTS: We present data on 1000 patients with rare diseases (46.2% females; average age, 4.6 years) representing 47 countries primarily from the Arabian Peninsula, the Levant, Africa, and Asia. The cumulative diagnostic yield was 32.5% (95% CI, 29.7-35.5%) and was higher for genomic sequencing-based testing than chromosomal microarrays (37.9% versus 17.2%, P = 0.0001) across all indications, consistent with the higher burden of single gene disorders. Of the 221 Mendelian disorders identified in this cohort, the majority (N = 184) were encountered only once, and those with recessive inheritance accounted for ~ 62% of sequencing diagnoses. Of patients with positive genetic findings (N = 325), 67.7% were less than 5 years of age, and 60% were offered modified management and/or intervention plans. Interestingly, 24% of patients with positive genetic findings received delayed diagnoses (average age, 12.4 years; range 7-37 years), most likely due to a lack of access to genomic investigations in this region. One such genetic finding ended a 15-year-long diagnostic odyssey, leading to a life-threatening diagnosis in one patient, who was then successfully treated using an experimental allogenic bone marrow transplant. Finally, we present cases with candidate genes within regions of homozygosity, likely underlying novel recessive disorders. CONCLUSIONS: Early access to genomic diagnostics for patients with suspected rare disorders in the Middle East is likely to improve clinical outcomes while driving gene discovery in this genetically underrepresented population.

The Y-Pelvic Osteotomy in Treating Bladder Exstrophy: A Surgical Technique.

Bladder exstrophy (BE) is a rare congenital anomaly caused by an embryological defect in the closure of the abdominal wall. It comprises a spectrum of defects about severity, including epispadias in the mildest form and cloacal exstrophy in the worst. Surgical correction is required to achieve urinary continence, maintain normal renal function, achieve secured abdominal wall closure, and create cosmetically and functionally satisfactory genitalia. Iliac bone osteotomy is considered essential to achieve the above goals in most patients by reducing the tension of the closed abdominal wall layers, particularly when present late in infancy. Several types of pelvic iliac bone osteotomy have been described to aid bladder and cloacal exstrophy closure. They can be grouped into posterior iliac osteotomy, anterior iliac osteotomy, oblique (also called diagonal) iliac osteotomy, and a combination of posterior and anterior iliac osteotomy. We described here the Y-pelvic osteotomy, which was developed by the Manchester Orthopaedic Group in the United Kingdom. It has the advantage of anterior and posterior osteotomies but also has less risk to the neurovascular structures, less blood loss, and ease of surgical technique. The osteotomy was named the Y-pelvic osteotomy due to the morphological shape it resembles.

Comparison of Three Circular Frames in Lower Limb Deformity Correction: A Biomechanical Study.

Background The use of circular frames in correcting lower limb deformity is well-established and has evolved dramatically over the years. Three new frames have been introduced recently, and this study is set to compare them in terms of accuracy and efficiency in correcting a similar long bone deformity. These frames are the Taylor Spatial Frame (TSF; Smith & Nephew, London, United Kingdom), the Truelok Hexapod System (TL-HEX; Orthofix, Lewisville, Texas), and Orthex (OrthoPediatrics, Warsaw, Indiana). Methods This is a biomechanical study comparing the above three types of circular frames to correct similar deformities in Sawbones models. The deformities that are compared were: (1) 30° valgus deformity of the distal femur; (2) 30° varus deformity of the proximal tibia. Each frame was applied to the deformed bone in the standard way that we apply to normal bone. X-rays were taken before and after the deformity correction. The frames' software was used to estimate the deformities. The variations between the software's estimations and the known bone deformities were compared. Residual deformity after initial correction and the number of re-programmings was compared among these three frames. The least residual deformity and re-programming is the favorable outcome. Results All the Sawbones models had a 30° actual coronal angulation. The Orthex software estimated the deformity at around 25.35° (SD 4.6), TSF 25.6° (SD 2), and TL-HEX 29.87° (SD 2.1). One-way analysis of variance (ANOVA) showed a significant difference in the findings (P-value 0.014). Accuracy was measured by comparing the residual deformity in angulation in the coronal plane after the first and second correction. The Orthex median residual deformity was 1°, TSF was 2.5°, and TL-HEX was 3° with a range of less than 5° for all of them. The independent samples Kruskal-Wallis test shows that there is no significant difference between the three groups (P=0.549). The frequency of strut changes required throughout the correction was not significant among the three frames using the Fisher exact test (P=0.336). TSF struts are not designed to be readjusted. Conclusion The three frames were comparable in terms of accurate correction of the two deformities, strut changes, and strut adjustments. The TL-HEX frame software was superior to other frames in terms of analyzing the deformity but the difference, although statistically significant clinically, was not.

Paediatric orthopaedic surgery during the SARS-CoV-2 pandemic. A safe and pragmatic approach to service provision.

The SARS-COV-2 pandemic has brought unparalleled challenges to healthcare provision. Being a newly discovered virus, there is a lack of previous experience and published evidence to guide healthcare providers on how to deliver services. We would like to share our approach to service delivery in a newly open children's hospital in the United Arab Emirates with a particular focus on paediatric orthopaedic services.

Minimally invasive hip arthroplasty: a quantitative review of the literature.

PURPOSE: To perform a comprehensive quantitative review of the published literature and to assess the methodology of studies comparing the surgical outcomes in minimally invasive hip arthroplasty (MIHA). METHODS: We conducted a comprehensive literature search using Medline, Embase, Cochrane, CINAHL and Google Scholar. The bibliographies of papers were also examined. All relevant articles in peer-reviewed journals were retrieved except those not mentioning outcomes, case reports, review of literature and letters to editors. Two authors independently scored the quality of the studies using a modified Coleman Methodology Score with 10 criteria which allow critical analysis of the design and implementation of a particular study. The results are recorded as a final score between 0 and 100. We collected data for year of publication, type of study, patient numbers, surgical method, follow-up, complications and patient satisfaction. RESULTS: Thirty-six studies met our inclusion criteria giving details of 6434 HAs, 78.5% (4031) of which were implanted using MIHA techniques. The only statistically significant outcome was a reduction in length of hospital stay (P = 0.02). With no significant difference noted between the two groups with respect to operating time, blood loss, dislocation and revision rates, neurological injury and incidence of peri-operative fracture, patient selection and surgeons' experience may have had a significant effect on outcome. For instance, studies reporting outcomes on an average patient age of 48 years had significantly different results to one reporting on patients with a mean age of over 70 years. Scores were predominantly low for quality of the studies, with patient number, follow-up time and validated outcome measures being the weakest areas. CONCLUSION: At present, there is still a lack of quality evidence to advocate the expansion of MIHA. The better designed studies suggest that it should even be limited further to recognized expert centres. The complication rates and learning curve may be altered by changes in training and adapting surgical techniques. We emphasize the need for meticulous design in future studies comparing the outcomes of these two procedures.