相继发生的双侧胫骨硬化性骨髓炎医案报告课件

相继发生的双侧胫骨硬化性骨髓炎1例报告,徐宏宇 ,王刚祥, 周亮, 谢建新，周海东,病史,患者，女，41岁，因“右小腿酸痛1年，伴左小腿酸痛4月”收治入本院。既往有慢性乙型肝炎和失眠史，有右足开放性跖骨骨折手术史（23年前）。,病史,1年前无明显诱因下出现右小腿酸痛不适，酸痛呈间隙性，无畏寒发热，无盗汗，久站、久行及夜间酸痛加重，休息后无明显缓解，无右下肢麻木，右小腿酸痛无明显进行性加重，无明显肿胀。患者去某中医院就诊，考虑“慢性肌肉劳损”，给予中药汤剂口服（具体药物不详），汤剂口服长达2个月，疗效不明显。患者又去某私立医院骨伤科门诊就诊，予血常规、血沉、C反应蛋白及类风湿因子检测，均无明显异常，仍考虑“慢性肌肉劳损”， 给予“尼美舒利、妙纳”口服2周，用大黄粉和玄明粉外敷右小腿，疗效仍然不明显。4月前出现左小腿酸痛不适，无红肿，无左下肢麻木，较右小腿重，伴左踝关节酸胀，有腰部酸痛。患者又去某省级医院神经科就诊，考虑“抑郁症”，予“百忧解”口服，双侧小腿酸痛仍然无法缓解。患者遂来本院骨科门诊就诊，MRI检查提示：腰4腰5椎间盘突出，压迫硬膜囊。肌电图提示:右侧腓总神经轻度损伤。考虑“腰4/腰5椎间盘突出症”，给予“迪克乐克、消脱止、弥可保”口服10天，双小腿酸痛无法缓解，左小腿酸痛加重。患者遂再次来本院骨科门诊复诊，,查体,右小腿肌肉轻度萎缩，皮肤有色素沉者（大黄粉和玄明粉外敷所致），无静脉曲张，皮温未升高，右小腿无明显局部压痛，右足背有一长约7.0cm疤痕，无压痛，第2、3趾挛缩，活动受限，其余足趾活动正常，左小腿肌肉无明显萎缩，肢体无肿胀，皮肤有色素沉者（大黄粉和玄明粉外敷所致），无静脉曲张，皮肤无发红，左小腿无明显局部压痛，左踝关节无压痛，左踝及足趾活动正常。腰椎无畸形，腰4腰5椎间隙有压痛及叩击痛，双下肢感觉运动正常，直腿抬高试验阴性。,影像学资料,X线检查提示：右胫骨中段皮质增粗，骨髓腔闭塞，左胫骨中段皮质增粗，骨髓腔部分闭塞，可见层状骨膜反应。 CT检查提示：右胫骨中段皮质增粗，骨髓腔闭塞，左胫骨中段皮质增粗，骨髓腔部分闭塞，均无骨质破坏，无软组织肿块。,实验室检查,血常规、生化全套、血沉、C反应蛋白及类风湿因子检测均无异常。,入院诊断,双侧胫骨硬化性骨髓炎 慢性乙型肝炎 失眠症,手术经过,入院后在硬膜外麻醉下行双侧胫骨硬化性骨髓炎开窗减压万古霉素硫酸钙植入术。取右小腿前外侧切口，在C臂机引导下定位，用磨钻在胫骨上开窗，面积约为1.0cmX12.0cm，发现髓腔闭塞，磨钻打通，清除硬化骨，象石灰样粉末出来，克氏针打通髓腔，骨皮质呈象牙状，无渗血，右侧骨膜无增生；左侧胫骨骨膜增生，髓腔闭塞大部分闭塞，髓腔内有少量肉芽组织，无脓液，用以上方法开窗，清除硬化骨，打通髓腔。双氧水、生理盐水、碘伏反复冲洗髓腔。万古霉素硫酸钙颗粒填充髓腔，创腔放置引流管。术后静脉常规使用抗生素1周，口服抗生素4周，使用丹参针静滴2周改善局部微循环。术后患者左小腿酸痛明显减轻，右小腿酸痛轻度减轻。术后2周创口拆线，愈合良好。,讨论,硬化性骨髓炎 （sclerosing osteomyelitis ），又名Garre骨髓炎，以病程缓慢，临床症状轻微，病变处骨质不形成脓肿及窦道，主要表现为骨的进行性、广泛性、硬化性的低毒性感染性疾病。病因尚未完全确定，以胫骨和下颌骨为好发部位，虽然名为骨髓炎，却不易找到致病菌，另外无明显的感染症状和阳性的实验室监测指标。此病较少见，相继发生的双侧胫骨硬化性骨髓炎的报告更是罕见，机理不明，可能与23年前的右足开放性跖骨骨折感染引起的菌血症有关，细菌在双小腿血液循环淤滞，细菌的代谢产物刺激特异性机体发生变态反应，致使胫骨骨髓腔内发生广泛纤维化，循环发生障碍，骨内的氧张力下降，促使骨内膜下骨样组织增生，沉积和钙化，Haver管阻塞出现反应性骨内膜增厚，骨皮质呈梭形增生一系列病理变化。关于硬化性骨髓炎国内文章有多部位、家族性、先后发病等的病例报告12。,影像学,硬化性骨髓炎和一般化脓性骨髓炎不同，它不会产生脓肿、死骨和形成瘘管。X线片上可见骨皮质弥漫性增厚，致密，呈硬化状，与正常骨无明显分界，骨髓腔较正常狭窄或闭塞。CT检查可以探查出普通X线片难以辨出的小透亮区。,药物治疗,硬化性骨髓炎起病时为慢性病程，患肢呈间隙性弥漫性部位不确定的酸痛，能忍受，可逐渐缓解，但易反复发作，皮肤温度升高不明显，关节活动不受影响，使用抗生素和NSAID类药物无效。该病外观无明显异常表现（患者不能确定某一局限部位疼痛），局部无确定性压痛，无明显的阳性体征和阳性的实验室监测指标，对该病认识不足均是引起本病误诊的原因，往往被诊断为慢性肌肉劳损、腰椎间盘突出症或抑郁症。,鉴别诊断,本病常需于骨样骨瘤、梅毒性骨炎相鉴别诊断。骨样骨瘤在骨干上端一边骨皮质增生，中间有小透亮区，为窝巢状。梅毒血清试验阳性，多发性骨质增厚，两侧对称。,令人困惑的治疗,硬化性骨髓炎多累及骨干，血运极差，而髓腔狭窄或闭塞，抗生素极难达到局部饱和及渗透，多用手术治疗。采用病灶清除和髓腔再通是一有效和防止复发的治疗方法。有学者用全长骨干开窗病灶清除灌注负压吸引治疗硬化性骨髓炎3，并认为只有灌洗冲洗引流的基础上改变骨的血运才能取得良好的疗效。本病例开窗减压，去除硬化骨及髓腔内炎性肉芽组织，打通髓腔后，放置含万古霉素的硫酸钙，双小腿酸痛减轻，但病程较长的左小腿酸痛缓解较病程较短的右小腿差，考虑左侧病程长，胫骨髓腔内发生广泛纤维化，骨髓血运极差。关于病程较长（大于6个月）的硬化性骨髓炎是否需要多部位减压（通过MRI检查定位）或通过其他的辅助治疗，文献无明确报道。患者小腿长期酸痛，除了与慢性感染相关，可能与下肢动脉缺血有关，可惜没有进行血管造影来验证。推测病程较长（大于6个月）的患者单纯通过骨干开窗病灶清除及放置含万古霉素的硫酸钙的治疗可能疗效欠佳，需要通过其他的辅助治疗来降低骨内高压及改善骨骼血液循环，例如高压氧和介入溶栓治疗，这是笔者之见，未经临床证实。,新的治疗,有效的治疗？ Unsatisfactory。 术后大多仍然是不宁腿综合症。 国外文献中药物治疗中缓解的个例。 药物为利塞膦酸钠和氯屈膦酸二钠,Diffuse sclerosing osteomyelitis of the mandible下颌骨,S Jacobsson Int J Oral Surg 13(5):363-85 (1984) PMID 6437998 The medical care of patients with diffuse sclerosing osteomyelitis (DSO) of the mandible下颌骨 has been unsatisfactory. The main reasons for this have been insufficient knowledge of the disease and its natural history, difficulties in establishing the correct diagnosis, and the unknown etiology. The clinical features and natural history of the disease were studied by means of repeated radiographic and scintigraphic recordings. The biopsy technique was improved by using a slowly rotating coarse trepan bur. Histological and enzyme histochemical investigations were performed for determination of the histopathological diagnosis. Orthopantomograms in combination with intraoral views and 99mTc-scintigraphy were used for the radiographic diagnosis and follow-up studies. Bacteriological and serological investigations were performed in attempt to clarify the obscure etiology. The occurrence in the patients sera of antibodies to antigens prepared from cultured bacteria was studied. ASTA, IgM, IgG, and IgA were determined and lymphocyte stimulation tests were performed. The investigations made it possible to distinguish DSO as a separate entity with rather characteristic clinical, histological and radiographic features. The histological diagnosis was facilitated by an improved biopsy technique and enzyme histochemical recordings. Different rather unspecific tissue reactions were together found to form a pattern which was strongly indicative of DSO. The radiographic and scintigraphic investigations gave valuable findings which increased the diagnostic accuracy and improved the prognostic and therapeutic judgements. The bacteriological and serological investigations indicated that propionibacterium acnes and peptostreptococcus intermedius were of etiological importance but could not explain the chronicity of the disease. The results of the different investigations gave a better understanding of DSO and made it possible to provide more appropriate care for patients in different stages of the disease. Long-term antibiotic therapy was found to have a positive influence on the course of the disease in its early stages, while cortisone therapy, and sometimes decortication, were found to be more effective in chronic stages.,Diffuse chronic sclerosing osteomyelitis and the synovitis, acne, pustolosis, hyperostosis, osteitis (SAPHO) syndrome in two sisters.,Two sisters with diffuse chronic sclerosing osteomyelitis of the mandible下颌骨 and the humerus and the synovitis, acne, pustolosis, hyperostosis and osteitis syndrome (SAPHO syndromeSAPHO综合征（synoVitis,acne,pustulosis,hvoerostosis,osteitis syndrome即滑膜炎、痤疮、脓疱疮、骨肥厚、骨炎综合征，是一种非常罕见的临床综合征. ) are presented. The diagnoses of diffuse chronic sclerosing osteomyelitis at the age of 12 years and 27 years, respectively, were based on typical medical history, clinical symptoms and radiographic, histologic and scintigraphic findings. Because skin lesions and scintigraphic enhancement of the sternoclavicular joints with hyperostosis were present, a SAPHO syndrome was diagnosed in both sisters. Microbiological cultures of biopsy specimens revealed coagulase-negative Staphylococcus aureus at the humerus and Haemophilus parainfluenzae, Streptococcus, Actinomyces and Veilonella species at the mandible. Repeated operative procedures, including decortications, resection and reconstruction, and multiple histologic and microbiologic studies were performed over a period of up to 20 years. Since HLA typing yielded identical gene loci, we suggest that hereditary and autoimmune factors may play a role in the pathogenesis of these cases.,Chronic diffuse sclerosing osteomyelitis treated with risedronate,We describe a 21-year-old woman with chronic diffuse sclerosing osteomyelitis (CDSO) of the left femur. The patient presented with shortening of the left leg and intractable pain that was unrelieved with conventional analgesia. Radiological imaging and open bone biopsy confirmed the diagnosis of chronic diffuse sclerosing osteomyelitis. Treatment with risedronate利塞膦酸钠 was commenced and a dramatic response in the patients symptoms and biochemical markers of bone turnover was observed. To our knowledge this is the first case of CDSO treated successfully with risedronate. 作 者Wright SA (Department of Rheumatology, Musgrave Park Hospital, Belfast, Northern Ireland.stephenwright866.fsnet.co.uk) ； Millar AM Coward SM Finch MB 刊 名The Journal of rheumatology 2005 年32卷7期,Disodium clodronate in the treatment of diffuse sclerosing osteomyelitis (DSO) of the mandible.,Diffuse sclerosing osteomyelitis (DSO) of the mandible is a chronic condition, the cause of which is not known. Jaw pain, occurring irregularly, is a typical symptom. The aim of the study was to assess the effectiveness of disodium clodronate for relieving pain in patients with DSO. Disodium clodronate氯屈膦酸二钠 is a bisphosphonate used to treat diseases of bone an