Cardiopulmonary Status in Adults with Osteogenesis Imperfecta: Intrinsic Lung Disease May Contribute More Than Scoliosis.
Cardiopulmonary Status in Adults with Osteogenesis Imperfecta: Intrinsic Lung Disease May Contribute More Than Scoliosis. Clin Orthop Relat Res. 2020 Jul 07;: Authors: Khan SI, Yonko EA, Carter EM, Dyer D, Sandhaus RA, Raggio CL Abstract BACKGROUND: Osteogenesis imperfecta (OI) is a heterogeneous group of collagen-related disorders characterized by osteopenia, bone fractures, spine deformities, and nonskeletal complications. Cardiopulmonary complications are the major cause of morbidity and mortality in adults with OI. The cause of such problems was often attributed solely to the presence of large scoliosis curves affecting pulmonary function and, indirectly, cardiovascular health. However, recent studies suggest this may not be the case. Therefore, determining the relationships and causative agents of cardiopulmonary problems in patients with OI, specifically pulmonary impairment, is important to improving the overall wellbeing, quality of life, and survival of these patients. QUESTIONS/PURPOSES: (1) Is cardiopulmonary fitness in OI solely related to the presence of scoliosis? (2) What is the prevalence of heart and lung complications in this adult population? (3) Does the presence of pulmonary impairment impact quality of life in adults with OI? METHODS: This is a prospective observational cross-sectional study. Within 1 year, each participant (n = 30) completed pulmonary function testing, echocardiogram, ECG, chest CT, AP spine radiography, ...
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Publication date: Available online 10 October 2020Source: IJC Heart &VasculatureAuthor(s): Dominik Linz, Jeroen Hendriks
Background: Controversy exists regarding the need for proximal fibular epiphysiodesis in conjunction with proximal tibial epiphysiodesis to prevent relative overgrowth of the fibula. The purpose of this study was to determine the incidence of relative fibular overgrowth in patients who had undergone proximal tibial epiphysiodesis with or without proximal fibular epiphysiodesis to manage leg-length discrepancy. Methods: We identified patients who had undergone proximal tibial epiphysiodesis, with or without concomitant fibular epiphysiodesis, followed to skeletal maturity, and with adequate scanograms to measure tibial...
Conclusions: In this dual-center retrospective series, the single-incision triple innominate osteotomy was extremely effective for improving acetabular coverage and stabilizing unstable hips in a variety of underlying diagnoses with an acceptably low rate of complications. Level of Evidence: Level IV—case series.
Conclusions: The authors rejected their null hypothesis. Children do have the capacity to remodel radiographically measurable sagittal plane malunion of SCHFs. Children younger than 5 years of age can remodel 100% displacement of the center of the capitellum, whereas those over 8 years of age have minimal remodeling capacity.
Conclusions: Distal radius fractures have a large capacity to remodel in the pediatric population. This remodeling occurs in a predictable and reliable manner, even in the coronal plane. On the basis of this study, coronal angulation was shown to remodel at a rate of at least 2 degrees per month for the first 6 months following the injury, which should likely continue at a similar rate for the first year after the injury. Repeat manipulation is not indicated in skeletally immature patients where the maximum coronal angulation is
Conclusions: Intra-articular distal radius fractures in skeletally immature patients have a considerably higher rate of physeal growth arrest than extra-articular physeal fractures. Following acute management aimed at restoring and preserving anatomic physeal and articular alignment, follow-up radiographs should be obtained to evaluate for physeal arrest in skeletally immature children. Patients and families should be counseled regarding the high rate of growth disturbance and the potential need for deformity correction in the future, particularly in younger children. Level of Evidence: IV—case series.
Conclusion: For OI children, symptoms suggestive of obstructive sleep disorders should be searched for systematically, especially when autonomy is compromised, body mass index is high, trunk deformations are identified, and in case of severe type of OI.
ConclusionFor OI children, symptoms suggesting obstructive sleep disorders should be searched for systematically, especially in children with compromised autonomy, high body mass index, trunk deformations, and severe OI type.