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Garré氏硬化性骨髓炎:病例报告  

2015-04-26 17:28:36|  分类: 骨科文献 |  标签: |举报 |字号 订阅

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Introduction

Garré’s osteomyelitis is a rare inflammatory disease of chronic nature, characterized by periosteal reactions, which induces bone neoformation. It mainly affects the region of the mandible and, in rare cases, may be located in the metaphyseal region of the long bones. This disease is also known as Garré’s sclerosing osteomyelitis (GSO), chronic osteomyelitis with proliferative periostitis, chronic sclerosing osteomyelitis, ossifying periostitis or non-suppurative chronic sclerosing osteomyelitis. The clinical condition is characterized by an insidious start, with local pain and reaction in the affected bone. The symptoms have an episodic non-progressive nature and may persist for several months. On the other hand, the duration of GSO is very variable among patients, who may present the disease for several years. The function of the affected bone is generally preserved and, during the interval between crises, most patients are seen to be healthy.

引言

Garré氏骨髓炎是一种罕见的慢性炎症性疾病,其特点为骨膜反应,可诱导新骨形成。主要侵及下颌骨区,在极少数情况下,可能位于长骨干骺端部位。该疾病又名Garré氏硬化性骨髓炎(GSO、慢性骨髓炎伴增殖性骨膜炎、慢性硬化性骨髓炎,骨化性骨膜炎或非化脓性慢性硬化性骨髓炎。临床症状具有发病隐渐的特性,以局部疼痛和强烈的成骨反应为主,其症状特征为发作性、非进行性,并可持续数月。另一方面,表现GSO多年的患者中,该疾病持续时间较多变。受累骨的功能一般被复原,在病情急转的间隔期间,大多数患者被认为是健康的。


The aim of this study was to report on a rare case of Garré’s sclerosing osteomyelitis, in which the proximal region of the tibias and distal region of the femurs were affected in a 54-year-old patient.

本研究旨在报道一例罕见的Garré氏硬化性骨髓炎患者,其为一名54岁女性,胫骨近端和股骨远端部位处均受累。


Case report

The patient was a 54-year-old woman with a history of treatment for lupus using corticoid, over the preceding 20 years. She presented severe osteoporosis and had been using alendronate for five years. The specific clinical condition had been evolving over the preceding year, with pain, edema and chronic limitation of movement in her left knee, which presented slight joint effusion and pain on palpation of the distal femoral metaphysis. She was not febrile, in a good general state and without reddening or discharge of secretions at the site.

病例报告

该患者为一名54岁女性,采用肾上腺皮质激素治疗红斑狼疮20年多年。患者表现严重的骨质疏松,已使用阿仑磷酸钠治疗五年。与上年相比,左膝特异性临床病变已蔓延并且出现疼痛、水肿以及运动长期受限,触诊股骨远端骨骺时呈现少量关节腔积液和轻微疼痛。患者一般状态良好,不发热,局部未发红且无分泌物排出。


Radiographs and tomographic scans were produced on the left knee. The images showed trabecular osteolysis of the distal metaphysis of the left femur and periosteal reaction in both proximal tibias and both distal femurs. This was compatible with chronic osteomyelitis, of low virulence and slow progression (Figs. 1A–C, 2A–C and 3A–B). The imaging diagnosis was complemented by means of magnetic resonance, which showed T2 hypersignal both in the femur and in the tibia, thus indicating GSO.

左膝进行了X线与CT扫描。图像显示左股骨远端骨骺端小梁骨溶解,以及两侧胫骨近端与两侧股骨远端均发生骨膜反应,这符合了慢性骨髓炎低致病性与进展缓慢性(图1A-C,图2A-C和图3A-B)。借助于MR成像辅助诊断,结果显示股骨和胫骨处均呈T2 高信号,因此该病可诊断为GSO

Garré氏硬化性骨髓炎:病例报告 - 小骨头 - 小骨头的博客

Fig. 1 – Radiographic appearance of the lesion. (A)Radiograph in anteroposterior view showing periosteal reaction in the femur.(B) Radiograph in lateral view showing periosteal reaction in the tibia. (C)Radiograph in lateral view showing distal femur after the operation, with a bone window for drainage, curettage, culturing and biopsy, and showing the various layers of the periosteal reaction.

1  病变X片表现:(A)正位X片显示股骨骨膜反应;(B)侧位X片显示胫骨骨膜反应;(C)侧位X片显示术后股骨远端开窗引流,病灶刮除,培养以及活检,并显示骨膜反应的各个阶段


Garré氏硬化性骨髓炎:病例报告 - 小骨头 - 小骨头的博客

Fig. 2 – Computed tomography of the left knee, with bone window, showing trabecular osteolysis and periosteal reaction. Coronal (A), sagittal (B) and axial slice (C).

2  左膝切口CT片显示骨小梁溶解以及骨膜反应;冠状位(A),矢状位(B)和轴向切片(C


Garré氏硬化性骨髓炎:病例报告 - 小骨头 - 小骨头的博客

Fig. 3 – Magnetic resonance of the left knee. Coronal (A) and axial (B) T2 slices showing hypersignal indicative of purulent secretion.

3  左膝MR片:冠状位(A)和轴向T2切片(B)显示高强度信号,表明有脓性分泌物


Surgical treatment consisting of curettage of the left distal femur was performed. Puncturing the knee joint did not show any purulent secretion. The bone window thus made subsequently discharged secretion, but this produced negative cultures. The material obtained from the surgery was sent for biopsy analysis, which showed infection and chronic inflammation marked by fibrosis, xanthogranulomatous reaction, foci of suppuration and absence of acid-alcohol resistant bacilli (AARB), fungi or signs of malignity (Fig. 4).

手术治疗,包括对左股骨远端进行刮除术。膝关节穿刺并未出现任何脓性分泌物。然而骨切口处排出分泌物,但血培养呈阴性。手术中获得的分泌物用于活检分析,结果显示骨感染、纤维化慢性炎症、黄色肉芽肿反应、化脓性病变、无抗酸-醇性杆菌(AARB)、真菌或毒性菌征(图4)。

Garré氏硬化性骨髓炎:病例报告 - 小骨头 - 小骨头的博客

Fig. 4 – Histological study on bone biopsy from the left femur, in hematoxylin-eosin (A) and (B), showing infection and chronic inflammation marked by fibrosis, xanthogranulomatous reaction and foci of suppuration indicative of GSO.

4  左侧股骨活检组织学研究显示:GSO表现骨感染,纤维化慢性炎症,黄色肉芽肿反应和化脓性病变症状(AB均为苏木精-伊红染色)


The patient underwent venous antibiotic therapy with oxacillin (4 g/day) and subsequently oral medication consisting of cephalexin (2 g/day for six months), with improvement of inflammation in the left knee.

患者静脉注射苯唑西林抗生素(4/天),随后口服头孢氨苄(2/天,服用6个月),左膝炎症改善。


Discussion

Carl Alois Philipp Garré was a Swiss surgeon and bacteriologist who in 1893 published an article dealing with the manifestations of osteomyelitis. His name became associated with the disease, which became known as Garré’s sclerosing osteomyelitis, although he was not responsible for describing it. He reported that there was peripheral formation of a bone reaction due to irritation or mild infection, which resulted in thickening of the periosteum of long bones. The etiology of this disease has still not been fully clarified. Bacterial infection is suspected, but the cultures are generally negative and the chronic process maybe maintained through low-virulence infection or even after the infection has been treated. If the germ cannot be detected through culturing, investigation by means of the polymerase chain reaction (PCR) should be requested.

讨论

卡尔·阿洛伊斯·菲利普Garré是一位瑞士外科医生及细菌学家,其在1893年发表了一篇关于骨髓炎临床表现的文章。虽然他不是负责全程报道,但还是将他的名字与疾病相关联,则称为Garré氏硬化性骨髓炎。他报道说,由于炎症或轻度感染,周围形成骨反应,引起长骨骨膜增厚。该病病因仍未完全确定,疑似为细菌感染,但细菌培养普遍呈阴性,慢性病程系低毒性感染,甚至治疗后长期保持。如果不能通过培养来检测细菌,必要时可借助聚合酶链式反应(PCR)研究。


A multifocal sclerosing bone reaction with clinical, radiological and histological characteristics similar to GSO maybe encountered in several diseases, such as fibrous dysplasia, syphilis, pustulosis palmoplantaris, ulcerative rectocolitis, Crohn’s disease, SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) and Paget’s disease. Additional differential diagnoses in cases of unifocal sclerosing bone reaction may include osteoid osteoma, Ewing’s disease, osteosarcoma and eosinophilic granuloma.

模拟与GSO相关的临床、影像学和组织学特征,一些疾病亦可表现为多灶性硬化骨反应,如骨纤维结构不良、梅毒、掌跖脓疱病、溃疡性直肠结肠炎、Crohn’s病、SAPHO综合征(滑膜炎、痤疮、脓疱病、骨质增生与骨炎症)及Paget’s病。其他单灶性骨硬化反应病例的鉴别诊断还包括骨样骨瘤、Ewing’s病、骨肉瘤以及嗜酸性肉芽肿。


The osteoporosis presented by our patient may have contributed toward the start of the infectious/inflammatory process that originated GSO, since the bone hypovascularization that occurs in osteoporosis facilitates inflammation, followed by necrosis of this tissue. Lupus, which is a chronic systemic disease, seems to facilitate the establishment of osteomyelitis. Another point of interest in the present case can be highlighted: use of corticoids is an important triggering factor for osteoporosis. Thus, it is reasonable to suppose that the treatment for lupus in the present case may have contributed toward the appearance of osteoporosis and, together, the two diseases may have favored development of GSO.

患者表现骨质疏松症的病因可能归咎于引起GSO感染/炎症的过程,因为出现在骨质疏松症的骨内血管形成减慢会促进炎症反应,随后该组织坏死。狼疮是一种慢性全身性疾病,疑似可帮助诊断骨髓炎。本病例中的另一个观点强调:使用肾上腺皮质激素是骨质疏松症的一个重要触发因素。因此,本病例中狼疮有助于治疗骨质疏松症的假设合理,然而同时这两种疾病也可能致使GSO恶化。


Chronic osteomyelitis presents low mortality but high morbidity. Clinically, the chronic phase is always preceded by an acute infectious process, with phlogistic signs (pain, heat, redness, tumor growth, deformity and limitation). However, in patients who have become debilitated through other diseases, as was the case in our patient, the initial condition may be masked, which has the consequence that the diagnosis will only become possible in the chronic phase.

慢性骨髓炎死亡率较低,但发病率较高。在临床上,慢性期前总有一个急性感染的过程,常伴有炎性症状(疼痛、灼热、发红、肿瘤增殖、畸形以及缺损)。然而,累及其他疾病的患者在本病例中的初始治疗可忽略,因为仅在其慢性期方可诊断。


In relation to the imaging diagnosis, Vasiliev et al. evaluated 121 patients between the ages of 4 and 74 years, with a diagnosis of osteomyelitis in limbs. The most precise examination was spiral computed tomography, which had an accuracy of 96.7%, sensitivity of 99.1% and specificity of 80%, while radiographs presented 81.8, 84.9 and 60%, respectively. In the beginning, a combination of sclerotic areas and cystic areas could be observed in the region of the lesions, and the latter is gradually replaced by sclerotic areas. During exacerbations, bone formation due to periosteal reaction could be seen, resembling an onion skin. Thus, the disease is marked by thickening and sclerosis of the affected bone.

关于影像学诊断,Vasiliev等人评估了4-74岁之间的121例骨髓炎患者的四肢。最精确的螺旋CT检查:精确度为96.7%,灵敏度为99.1%以及特异度为80%,而X光检查精确度、灵敏度、特异度分别为81.8%84.9%60%。在初期时,病灶部位可观察到硬化区与囊性区融合一起,而后逐渐被硬化区取代。在急性发作期间,由于骨膜反应可看到骨形成,颇似一层洋葱皮。因此,这种疾病的特征表现为骨膜增厚和硬化反应。


Surgical treatment in association with antibiotic therapy is the best option for chronic osteomyelitis. Antibiotic therapy alone is ineffective, independent of the administration route, because the “bone sequestration” found in the chronic disease comprises fragments of necrotic bone and thus does not present a blood supply that would allow antibiotic to arrive at the infected tissue.

手术治疗与抗生素治疗相结合是治疗慢性骨髓炎的最佳选择。采取独立的给药途径以及单独抗生素治疗是无效的,因为我们在慢性疾病包括骨坏死碎片中发现了“骨吸存”,因此血液供应不良,需送达抗生素至受染组织。


MediCool医库软件 余娟 陆晓玲 编译

原文来自:

Garré’s sclerosing osteomyelitis: case report

Revista Brasileira de Ortopedia, Volume 49, Issue 4, July–August 2014, Pages 401-404


原文转载地址:病例漫谈30:Garré氏硬化性骨髓炎:病例报告 - 病例漫谈 - 骨科论坛 骨科|ASP.net|论坛 - Powered by Discuz!NThttp://www.medibone.cn/bbs/showtopic-2067.aspx

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