关键词: Osteopoikilosis bone metastases melorheostosis

来  源:   DOI:10.13107/jocr.2021.v11.i03.2106   PDF(Pubmed)

Abstract:
UNASSIGNED: Osteopoikilosis (OPK) is an extremely rare benign condition with autosomal dominant inheritance characterized by sclerosing bony dysplasia with multiple benign enostoses. It is characterized by symmetrically distributed numerous, small, well-defined, homogenous circular or ovoid radiodensities clustered in epiphysis and metaphysis of long bones in periarticular region, and in some cases diffusely present throughout axial and appendicular skeleton. There is no age and sex predilection; age at the time of diagnosis ranges from 15 to 60 years. It is usually asymptomatic but rarely in 15-20% patients slight juxta-articular pain and joint effusions can be seen. These are incidental radiological findings in most of the cases, also sometimes confused as bony metastasis. There are no specific clinical features; histological features are similar to bony island and it may be associated with connective tissue disorders, synovial osteochondromatosis, and a rare bone condition melorheostosis.
UNASSIGNED: We present a case of OPK in a 32-year-old male with bilateral hip and shoulder pain, based on the available literature and focus on clinical significance, due to its mimicking capability of other more severe conditions such as bone metastases and an extremely uncommon cause of bone pain.
UNASSIGNED: OPK is an uncommon hereditary condition involving juxta-articular region of long bones with intricate etiopathogenesis, often discovered incidentally on radiographs. It is characterized by multiple, symmetrical ovoid radiodensities, and in most instances confused with osteoblastic metastasis. This concludes that OPK is a condition that should be kept in mind to avoid misdiagnosis, in particular osteoblastic metastasis and undue distress to both the patients and doctors.
摘要:
骨质疏松症(OPK)是一种极为罕见的良性疾病,具有常染色体显性遗传,其特征是硬化性骨发育不良伴多发良性骨结球。它的特点是对称分布众多,小,定义明确,均匀的圆形或卵圆形的放射性密度聚集在关节周围区域的长骨的骨phy和干is端。在某些情况下,弥漫性存在于整个轴向和附肢骨骼中。没有年龄和性别偏好;诊断时的年龄范围为15至60岁。它通常是无症状的,但很少在15-20%的患者中可以看到轻微的并关节疼痛和关节积液。这些是大多数病例的附带放射学发现,有时也混淆为骨转移。没有特定的临床特征;组织学特征与骨岛相似,可能与结缔组织疾病有关。滑膜骨软骨瘤病,和一种罕见的骨骼畸形。
我们介绍了一名32岁男性双侧髋部和肩部疼痛的OPK病例,根据现有文献并关注临床意义,由于其模仿其他更严重的疾病如骨转移和骨痛的极其罕见的原因的能力。
OPK是一种罕见的遗传性疾病,涉及长骨的近接区域,具有复杂的病因,经常在射线照片上偶然发现。它的特点是多重,对称卵形放射密度,在大多数情况下与成骨细胞转移混淆。这得出结论,OPK是一个应该记住的条件,以避免误诊,特别是成骨细胞转移和过度困扰患者和医生。
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