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Between January 2003 and January 2006, 23 patients were operated on using the less invasive plate osteosynthesis technique. The minimum follow-up period of 12 months was completed in 20 patients. The mean healing time was 14.6 weeks, defined as three of four cortices having stable bridging callus. In one patient with delayed union, healing was observed after 28 weeks. Functional outcomes were evaluated using the Constant Score and the Hospital for Special Surgery (HSS) Score. 19 patients had good to excellent elbow function with a mean HSS Score of 93.5 points. All patients achieved satisfactory shoulder function with a mean Constant Score of 85.8 points compared to 90.6 on the healthy side. Complications observed were one paresthesia of lateral cutaneous nerve of forearm (no radial nerve injury) and one loosening of the LCP (Locking Compression Plate) screws due to technical error.

We report a Thai father (patient 1) and his daughter (patient 2) affected with osteogenesis imperfecta type IV and dentinogenesis imperfecta. Both were heterozygous for the c.1451G>A (p.Gly484Glu) mutation in COL1A2. The father, a Thai boxer, had very mild osteogenesis imperfecta with no history of low-trauma bone fractures. Scanning electron micrography of the primary teeth with DI of the patient 2, and the primary teeth with DI of another OI patient with OI showed newly recognized dental manifestations of teeth with DI. Normal dentin and cementum might have small areas of ectopic mineralizations. Teeth affected with DI have well-organized ectopic mineralizations in dentin and cementum. The "French-fries-appearance" of the crystals at the cemento-dentinal junction and abnormal cementum have never been reported to be associated with dentinogenesis imperfecta, either isolated or osteogenesis imperfecta-associated. Our study shows for the first time that abnormal collagen fibers can lead to ectopic mineralization in dentin and cementum and abnormal cementum can be a part of osteogenesis imperfecta.

SummaryDysplasia epiphysealis hemimelica or Trevor's disease is a rare disorder of localised osteochondral overgrowth affecting the epiphysis of extremities. This paper reports a 12-year-old boy presenting with a large bony mass at the left ankle diagnosed as dysplasia epiphysealis hemimelica. The articular surface of the ankle joint of the patient was evaluated with preoperative and postoperative MRIs. The 2-year postoperative MRI showed early osteoarthritis of the ankle, therefore demonstrating the importance of early excision avoiding more complex resections of intra-articular lesions. BACKGROUND

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