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肋骨单发性骨纤维发育不良

Monostotic fibrous dysplasia of the ribs.

作者信息

Traibi Akram, El Oueriachi Faycal, El Hammoumi Massine, Al Bouzidi Abderahman, Kabiri El Hassane

机构信息

Department of Thoracic Surgery, Mohamed V Military Teaching Hospital, Rabat, Morocco.

出版信息

Interact Cardiovasc Thorac Surg. 2012 Jan;14(1):41-3. doi: 10.1093/icvts/ivr048. Epub 2011 Nov 16.

Abstract

Fibrous dysplasia (FD) is a sporadic benign skeletal disorder that can affect one bone (monostotic form) or multiple bones (polyostotic bone). Around 6-20% of monostotic FD occurs in the ribs. The objective of this study was to report our experience in the management of the monostotic FD of the ribs. Between January 2004 and December 2009, seven cases of FD of the rib (six men and one woman, mean age 30.4 years, range 17-40 years) were operated on. The patients were evaluated with plain radiographs and computer tomography (CT). All our patients were symptomatic; two patients presented chest pain and swelling and other patients presented only chest pain. One rib was involved in all our patients (monostotic form): the site was fifth rib (four cases), sixth rib (two cases) or second rib (one case). Radiologically, plain films and CT showed an expansible lesion with a ground-glass centre and thinning of the cortex. Rib resection was performed in all patients; there were no postoperative complications and no recurrence in all cases at mean 43 month follow-up. In symptomatic monostotic FD of ribs, the involved segment of bone may be excised to rule out malignancy and for painful lesions.

摘要

骨纤维异常增殖症(FD)是一种散发性良性骨骼疾病,可累及单骨(单骨型)或多骨(多骨型)。约6% - 20%的单骨型FD发生于肋骨。本研究的目的是报告我们在肋骨单骨型FD治疗方面的经验。2004年1月至2009年12月期间,对7例肋骨FD患者(6例男性,1例女性,平均年龄30.4岁,范围17 - 40岁)进行了手术。患者接受了X线平片和计算机断层扫描(CT)评估。所有患者均有症状;2例患者表现为胸痛和肿胀,其他患者仅表现为胸痛。所有患者均累及一根肋骨(单骨型):部位为第5肋骨(4例)、第6肋骨(2例)或第2肋骨(1例)。影像学上,X线平片和CT显示为一个膨胀性病变,中心呈磨玻璃样,皮质变薄。所有患者均行肋骨切除术;平均随访43个月,所有病例均无术后并发症及复发。对于有症状的肋骨单骨型FD,可切除受累骨段以排除恶性病变并治疗疼痛性病变。

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