2弥漫骨硬化的影像鉴别诊断陈恩明20160908-0923.ppt

*;*;病因Victimn法放之四海而皆准;Vascular—血管相关的病因;Sickle-cell disease: central endplate depression, secondary to infarction,resulting in ‘H’-shaped vertebral bodies.;镰状细胞病;Evaluation only. Created with Aspose.Slides for .NET 3.5 Client Profile 5.2.0.0. Copyright 2004-2011 Aspose Pty Ltd.;Evaluation only. Created with Aspose.Slides for .NET 3.5 Client Profile 5.2.0.0. Copyright 2004-2011 Aspose Pty Ltd.;Medullary infarct within the proximal tibial metaphysis: plain radiograph andT1 W MRI. Well-defined lesion with sclerotic margins and patchy centralcalcification. On MRI this corresponds to a hyperintense lesion (marrow fat)with a well-defined peripheral low intensity (sclerotic) margin containingpunctate central low intensity (calcification).;Large well-defined sclerotic infarct within the proximal femoral metaphysis.;Venous stasis in a 22-year-old woman with swelling of the left lower leg. (a) Radiographshows mature periostitis involving the medial cortex of the tibial diaphysis (arrowhead). Also noted is a phlebolith (arrow). (b) Oblique venogram shows a large venous varix close to the affected area of tibial cortex;*;*;C：一、遗传性硬化性骨发育不良;一个丁香园的病例;;;Evaluation only. Created with Aspose.Slides for .NET 3.5 Client Profile 5.2.0.0. Copyright 2004-2011 Aspose Pty Ltd.;;;;;;;;;丁香园版主sb72778;本病有一个特点椎板和棘突硬化明显，髋臼周围和骶髂关节附近硬化明显。符合广泛性骨皮质增厚？？广泛性骨皮质增厚又称泛发性骨皮质肥厚症、内骨膜肥厚症，常染色体隐性遗传型、Van Buchem病。1983年国际命名学会仍命名其为内骨膜肥厚症。此型特点是发病较晚，骨改变较显性型更明显、更广泛，程度更为严重，并易累及脑神经。主要临床表现为发病年龄较晚，多在30—50岁之间，极少数可在儿童期发病．主要表现为面部不匀称性增大，特别是颌骨异常宽大。还可有继发性脑神经受累症状．如面神经瘫和耳聋，以及视神经萎缩或视力障碍等。 【X线表现】 1、头颅内外板硬化，板障消失，下颌骨广泛致密硬化，增宽变方；乳突气房和鼻旁窦闭塞，眼眶亦变小。枕骨和下颌骨明显硬化。 2、椎体中心硬化，椎板和棘突明显硬化，椎体终板和后半脊柱更致密、增厚。 3、长管骨皮质弥漫性、对称性增厚，病变仅限于骨干，骨皮质增厚主要是由于骨内膜增生硬化，髓腔皮质界限消失，骨外形基本正常。 4、骨盆的病变常呈弥漫性或斑片状硬化，以髋臼周围和骶髂关节附近最明显。;tzhj ;1.泛发性骨皮质增厚症　　 泛发性骨皮质增厚症(hyperosteosiscorticalisge-neralisata),又称骨内膜性骨增生症(endostealhype-rosteosis)或VanBuchem氏病,系一种具有家族遗传倾向的全身性骨骼广泛硬化的疾病,罕见[1～5]。 本症于1955年首先由VanBuchem报告[2]。颅骨、下颌骨、锁骨、肋骨和长管骨骨干对称性硬化为本病特点[3]。本症病因不明,多数学者认为是遗传性疾病。VanBuchem认为系常染色体隐性