Pycnodysostosis is a rare autosomal recessive disease due to a mutation in the gene for the enzyme Cathepsin K. It is characterized by short stature, craniofacial dysmorphias, osteosclerosis, and brittle bones. There are only a few reports in the literature describing surgical interventions for long bone fractures in pycnodysostosis patients, most of which describe intramedullary nail treatment of isolated long bone fractures. We describe a case in which a pregnant female with pycnodysostosis presented with a shaft fracture of the left femur following minor trauma and a history of increasing thigh pain. Radiographs obtained in the emergency room also revealed an impending subtrochanteric fracture of the contralateral side. The acute left femoral shaft fracture was treated with an adolescent-sized intramedullary nail; it was decided to defer surgery on the contralateral side until after pregnancy. Three months later, the patient had the contralateral femur prophylactically fixated with a plate and screws. One year after the index surgery, both methods demonstrated satisfactory healing both clinically and radiographically. Although we recommend use of an intramedullary nail for long bone fractures in patients with pycnodysostosis, a plate can be utilized if health conditions or skeletal morphology precludes use of a nail.
Obesity is not a reliable predictor of complications following upper extremity surgery. Thus, requiring preoperative screening for obese patients may constitute an unnecessary burden on medical resources. Further study is needed to identify specific demographics that might serve as more accurate predictors of poor outcomes in obese patients undergoing surgery of the upper extremity.
Background:Several radiographic parameters have been associated with a discoid lateral meniscus. However, limited information is available regarding the radiographic findings of a discoid meniscus in the pediatric population.Purpose:To determine the effect of age and weightbearing (WB) on radiographic parameters associated with discoid lateral menisci in pediatric patients.Study Design:Cohort study (diagnosis); Level of evidence, 3.Methods:Radiographs of pediatric patients with arthroscopically confirmed lateral discoid menisci were compared with age-, side-, and sex-matched individuals with confirmed normal menisci. Radiographic parameters of lateral joint space width (LJSW) and fibular head height (FHH) were measured by 3 physicians.Results:Significant differences were found between the discoid and control groups when LJSW (P = .002) and FHH (P < .001) were compared. Interrater reliability was good for LJSW (intraclass correlation coefficient [ICC], 0.635) and excellent for FHH (ICC, 0.759). WB radiographs were noted to have better interrater reliability compared with non-WB radiographs for LJSW (ICC, 0.729 vs 0.514, respectively) but had reduced interrater reliability for FHH (ICC, 0.625 vs 0.868, respectively). Subgroup analysis with age stratification indicated that FHH was significantly decreased (indicative of a high fibular head) in the discoid group for all age groups (P < .001 for <10 years and 10-14 years; P = .030 for >14 years); however, LJSW was significantly different only in patients older than 14 years.Conclusion:Increased LJSW and FHH were associated with discoid lateral menisci and showed satisfactory interrater reliability. Radiographic evaluation for potential discoid meniscus in pediatric and adolescent patients may be improved by use of fibular height to indicate the presence of a discoid lateral meniscus across age groups, while lateral joint space may be more reliable for older patients.
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