PDF(Pubmed)
Abstract：
Idiopathic avascular necrosis of the scaphoid bone, Preiser\'s disease, was originally described as a deteriorative pathology whereby the osseous structure necroses due to loss of blood supply. It may present with multifactorial etiology, which is still largely not well understood. We describe a case of Preiser\'s disease in a 70-year-old female, with worsening pain and loss of range of motion in her right wrist over a two-year period. Past medical history was significant for Sjogren\'s disease, fibromyalgia, and dystonia. Pain began several months following traumatic right dorsal wrist injury. Diagnosis of traumatic scaphoid fracture was originally suspected. Conservative treatment was unsuccessful. Radiographs did not demonstrate evidence of primary fracture. CT scan and MRI demonstrated osteonecrosis of the proximal pole of the scaphoid, but no evidence of fracture, either residual or healing, was found. Proximal row carpectomy was performed for avascular necrosis of the scaphoid. Histology confirmed diagnosis and verified absence of fracture. Postoperatively, the patient\'s pain and range of motion improved. This report combines histological findings of Preiser\'s disease with radiographic images which may ameliorate understanding of the clinical pathophysiology. We describe an unusual manifestation of Preiser\'s disease whereby a single traumatic event, in the absence of fracture, led to idiopathic scaphoid avascular necrosis, which may have been associated with Sjogren\'s syndrome and fibromyalgia. These conditions may have negatively impacted microvasculature and decreased bone mineral density, inversely correlated with the production of fatty marrow, facilitating the onset of osteonecrosis in the scaphoid.
摘要：
特发性舟骨缺血性坏死,Preiser\的疾病，最初被描述为恶化的病理学，由于血液供应的丧失，骨结构坏死。它可能存在多因素病因，这在很大程度上仍然没有得到很好的理解。我们描述了一个70岁女性的Preiser病病例，在两年的时间里，她的右手腕疼痛加剧，活动范围下降。既往病史对干燥病具有重要意义，纤维肌痛,和肌张力障碍.右腕背侧外伤后几个月开始疼痛。最初怀疑诊断为创伤性舟骨骨折。保守治疗不成功。X光片没有显示原发性骨折的证据。CT扫描和MRI显示舟骨近端骨坏死，但没有骨折的证据,无论是残留还是愈合，找到了。对于舟骨的缺血性坏死，进行了近端行腕骨切除术。组织学证实诊断并证实无骨折。术后,患者的疼痛和活动范围得到改善。该报告将Preiser病的组织学发现与放射学图像相结合，可以改善对临床病理生理学的理解。我们描述了Preiser疾病的一种不寻常表现，即单个创伤事件，在没有骨折的情况下，导致特发性舟骨骨缺血性坏死，这可能与干燥综合征和纤维肌痛有关。这些情况可能会对微血管系统产生负面影响，并降低骨矿物质密度，与脂肪骨髓的产生呈负相关，促进舟骨骨坏死的发作。
