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作 者:郑翰林 王保仓[2] 李勇[2] 闫明[2] 王辉[2]
机构地区:[1]华北理工大学研究生学院,河北063000 [2]唐山市第二医院骨病科,河北063000
出 处:《中国骨与关节杂志》2017年第2期155-157,共3页Chinese Journal of Bone and Joint
摘 要:蜡泪样骨病( Melorheostosis )是临床上一种罕见的原因不明的骨骼发育障碍性疾病。常常侵犯一侧肢体,且下肢多见,增生的骨质从上而下沿骨干一侧向下流注,酷似蜡烛表面的烛泪,故名蜡泪样骨病,又称单肢型骨硬化、流动性骨质硬化症、蜡油样骨病、肢骨纹状肥大症等[1-2]。本病可根据 X 线特有的改变及病理学检查确诊,国内外均罕见,唐山市第二医院骨病科于2016年收治1例,现报道如下。Obejective To investigate the clinical and X-ray features of melorheostosis, and to provide reference for clinical diagnosis and treatment. Methods A 55-year-old male paitent underwent operative treatment for melorheostosis of the right clavicle in 2016. The patient occasionally complained because he found a right shoulder mass. The physical examination results showed the swelling skin surface and the doctor could touch the non removable hard mass. The activities of the right shoulder joint was normal. The definite diagnosis was determined before the operation through the clinical and imaging examinations and pathological examination. Results The skin surface of the patient’s right shoulder was flat and the lump had flattened out after operation. The postoperative X-ray showed the lesion has been removed. The texture was hard like ivory and the surface was irregular, which looked like a melted candle. The staining was performed mainly in resected hyperplasia bone tissues, in which twisted and deformed Haversian canals were involved, as well as dense disorder of bone plate layer. Conclusions Melorheostosis is a kind of skeletal developmental disorder, which is rare and agnogenic in clinic, with deformity of the extremity, pain, limb stiffness and limitation of motion. The disease can be confirmed by its peculiar X-ray changes and pathological examination. The characteristic X-ray appearance consists of irregular hyperostotic changes of the cortex resembling melted wax dripping down the side of a candle. At present, there is no special treatment for this disease, so usually symptomatic conservative treatment or surgical curettage. Physical therapy and symptomatic treatment can be given to those patients who have pain symptoms, and the satisfactory therapeutic effects can be achieved.
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