Background: Total knee arthroplasty (TKA) in patients with skeletal dysplasias is particularly challenging as a result of the anatomic variances and substantial bony deformities. Little has been written regarding technical considerations that should be made when performing TKA in skeletal dysplasia.
Questions/purposes: We describe special operative considerations that must be made when performing TKA on patients with skeletal dysplasia, including implant selection and ligamentous balancing.
Patients And Methods: We retrospectively reviewed 12 TKAs in eight patients with varying degrees of deformity (ranging from 30° of varus to 45° of valgus) secondary to three types of skeletal dysplasias: multiple hereditary exostosis, achondroplasia, and osteogenesis imperfecta. Clinical notes, operative records, and radiographic data were reviewed. Minimum followup was 1 year (average, 4 years; range, 1-10 years).
Results: We used customized implants in three of the 12 knees. Constrained tibial inserts were used in five knees. All 12 knees underwent releases (soft tissue or epicondylar osteotomy) to address gap balancing or patellar tracking. Average Knee Society scores improved from 35.9 preoperatively to 82.9 postoperatively and average function scores improved from 47.9 preoperatively to 96.7 postoperatively. Complications included two transient peroneal nerve palsies.
Conclusions: Special considerations must be made with regard to implant selection and ligamentous balancing as a result of the unusual anatomy and deformities that accompany skeletal dysplasia, but the short-term clinical results reveal consistent improvements in pain and function.
Level Of Evidence: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.
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http://dx.doi.org/10.1007/s11999-010-1516-0 | DOI Listing |
Cranial fibrous dysplasia (FD) syndrome is a benign, rare, and idiopathic skeletal disorder characterized by the replacement and expansion of medullary bone by disorganized fibro-osseous tissue. Trigeminal neuralgia (TN) is most commonly caused by vascular compression at the trigeminal nerve root entry zone. Secondary TN caused by cranial FD syndrome is extremely rare.
View Article and Find Full Text PDFJ Orthop Surg Res
December 2024
Department of Pediatric Orthopedics, Shengjing Hospital of China Medical University, No. 36 Sanhao Street, Heping District, Shenyang City, Liaoning Province, 110004, China.
Background: The muscles that encase the hip serve a crucial role in both joint stability and functional efficacy, and as developmental dysplasia of the hip (DDH) progresses, the surrounding musculature may undergo specific adaptations that reduce joint stability, thereby exacerbating dislocation. Yet, the exact nature of changes in muscle morphology and quality remains inadequately investigated. This study aimed to compare magnetic resonance imaging (MRI) evaluations of the iliopsoas and other hip flexor and extensor muscles in children with unilateral DDH before and after treatment.
View Article and Find Full Text PDFJ Bone Miner Res
December 2024
Paris Cité University, Reference center for skeletal dysplasia, INSERM UMR 1163, Imagine Institute, Necker Enfants Malades Hospital (AP-HP), Paris, France.
Chondrodysplasias with multiple dislocations are rare skeletal disorders characterized by hyperlaxity, joint dislocations, and growth retardation. Chondrodysplasias with multiple dislocations have been linked to pathogenic variants in genes encoding proteins involved in the proteoglycan biosynthesis. In this study, by exome sequencing analysis, we identified a homozygous nonsense variant (NM_001297654.
View Article and Find Full Text PDFEur J Hum Genet
December 2024
Department of Medical Genetics, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India.
Skeletal dysplasias are a clinically and genetically heterogeneous group of rare disorders. Studies from large cohorts are essential to provide insights into the disease epidemiology, phenotypic spectrum, and mutational profiles. Here we enumerate additional 248 Indians from 197 families with a skeletal dysplasia, following a similar study earlier.
View Article and Find Full Text PDFMedicine (Baltimore)
December 2024
Xuzhou Clinical College of Xuzhou Medical University, Xuzhou Central Hospital, Xuzhou, China.
Rationale: The aim of this study is to investigate the de novo mutation and clinical features of latent transforming growth factor-beta-binding protein 3 (LTBP3) gene-associated geleophysic dysplasia 3, and possible mechanisms of action.
Patient Concerns: A nonconsanguineous couple was recruited for this study due to the presence of intrauterine growth restriction. The pregnant woman and her elder daughter presented with skeletal abnormalities with diabetes.
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