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肋骨病(多发性骨干硬化症):影像学表现及鉴别诊断

Ribbing disease (multiple diaphyseal sclerosis): imaging and differential diagnosis.

作者信息

Seeger L L, Hewel K C, Yao L, Gold R H, Mirra J M, Chandnani V P, Eckardt J J

机构信息

UCLA School of Medicine, Department of Radiological Sciences 90095-6952, USA.

出版信息

AJR Am J Roentgenol. 1996 Sep;167(3):689-94. doi: 10.2214/ajr.167.3.8751682.

DOI:10.2214/ajr.167.3.8751682
PMID:8751682
Abstract

OBJECTIVE

This study describes the clinical presentation and the course of Ribbing disease in six patients and illustrates imaging features on plain radiography, conventional and computed tomography, and 99mTc-methylene diphosphonate bone scans.

MATERIALS AND METHODS

Between 1982 and 1990, six female patients presented with painful bony lesions that were believed to be Ribbing disease. Ten bones were affected: both tibiae in three patients, a unilateral tibia in one, both femora in one, and a unilateral femur in one. Plain radiographs and either conventional or computed tomography were available for all patients and 99mTc-methylene diphosphonate bone scans, for five patients. All patients underwent open biopsy and/or surgical decompression.

RESULTS

The diagnosis was reached in all patients through a combination of clinical findings (lack of systemic signs of infection or laboratory values suggesting metabolic bone disease), imaging, histologic evaluation, and specimen cultures. Radiographs and tomographic studies showed benign-appearing endosteal and periosteal cortical thickening. Intense uptake of radionuclide tracer was confined to the shaft of all involved bones. All pathologic specimens revealed nonspecific changes that included a slow increase in the mass of cortical and endosteal bone. These specimens also assisted in excluding neoplastic or infectious causes for the new bone formation.

CONCLUSION

Ribbing disease is a rare disorder that, on imaging studies, may simulate stress fracture, chronic infection, bone-forming neoplasia, or a systemic metabolic or endocrine disorder. Clinical and imaging features may suggest the correct diagnosis.

摘要

目的

本研究描述了6例里宾病患者的临床表现及病程,并阐述了X线平片、传统及计算机断层扫描以及99m锝-亚甲基二膦酸盐骨扫描的影像学特征。

材料与方法

1982年至1990年间,6例女性患者出现疼痛性骨病变,被认为是里宾病。10处骨骼受累:3例患者双侧胫骨受累,1例单侧胫骨受累,1例双侧股骨受累,1例单侧股骨受累。所有患者均有X线平片及传统或计算机断层扫描资料,5例患者有99m锝-亚甲基二膦酸盐骨扫描资料。所有患者均接受了开放活检和/或手术减压。

结果

通过综合临床发现(无感染的全身症状或提示代谢性骨病的实验室检查结果)、影像学检查、组织学评估及标本培养,所有患者均得以确诊。X线平片和断层扫描显示骨内膜和骨膜皮质增厚,表现为良性。放射性核素示踪剂的浓聚局限于所有受累骨骼的骨干。所有病理标本均显示非特异性改变,包括皮质骨和骨内膜骨量缓慢增加。这些标本也有助于排除新骨形成的肿瘤性或感染性原因。

结论

里宾病是一种罕见疾病,在影像学检查中可能类似应力性骨折、慢性感染、成骨性肿瘤或全身性代谢或内分泌疾病。临床和影像学特征可能提示正确诊断。

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