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91-8-第八节 骨髓水肿综合征
骨髓水肿综合征(BMES )是一个独立的、具有自限性的、一过性临床过程。它的特征是髓部疼痛、X线显示骨质疏松、MRI显示骨髓水肿,通常只发生在怀孕3个月的妇女和中年男性。临床表现有髓部进行性疼痛、疼痛步态以及受累肢体的功能障碍。后者与疼痛不成正比,受累舰关节活动不受限,但肢体活动时常出现疼痛。症状和影像学改变平均持续6个月。通常每次只有一侧靛受累,但有40%的患者会出现症状复发或累及另一髓或其他关节。
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典型的临床过程可分为三期,第一期持续4周,疼痛急性发作及出现进行性功能障碍;X线表现正常;骨扫描显示整个股骨头、颈直至转子间线出现示踪剂摄取增加;MRI信号改变的区域与骨扫描相同,T1加权相为低信号,T2加权相为高信号。另外可看到关节有渗出,MRI和骨扫描的改变可早在髓关节出现疼痛后 48小时观察到。第二期,也即平台期,持续1-2个月,症状稳定,X线显示股骨头和颈广泛的骨质疏松,偶尔也累及骸臼。没有软骨下骨折、致密性硬化或者退行性改变等骨坏死的表现。第三期,也即恢复期,持续4一6个月,X线显示骨密度恢复正常,症状全部消退。
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BMES的病因尚不清楚,有关的推测假说包括非创伤性交感神经反射功能紊乱;病毒介导的破骨细胞活动增加;一过性缺血导致选择性骨髓坏死而不是骨坏死。后一种学说的支持者认为,BMES可能是一个短时间缺血引起的可逆性骨坏死的先兆过程,病人骨功能检查显示股骨头骨内压显著升高,造影剂清除异常。症状出现后 3周股骨头活检标本显示,骨髓成分弥漫性间质水肿、脂肪细胞碎裂以及大量的新骨形成。在正常的骨小梁上有连续的类骨层沉积,其中含有活骨细胞。骨量没有丢失,但骨的矿化相对减低,类骨与矿化骨的比例增加,X线显示股骨头有成骨表现。
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BMES的患者可采用保护性负重和止痛剂等对症治疗。尽管髓芯减压术可缓解急性疼痛,但对这个自限性疾病不必冒手术的风险。很少见到出现 BMES的孕妇因跌倒而致股骨颈骨折需要进行复位和内固定的报告。由于X线、骨扫描以及MR1的改变不是独特的病理表现,因此,应当排除浸润性肿瘤、骨外伤和感染等疾病。 6 W: R$ P4 {9 u
+ W/ v3 K$ @; \( s* o 骨坏死可以定义为骨和骨髓成分的缺血性坏死并可能导致软骨下骨折、关节变形和关节病的病理过程。它可有多种原因引起,包括创伤、使用激素及嗜酒等,也可以没有明确的发病因素。骨坏死的自然病程有多种情况,但通常呈进行性而且可以致残,尤其当发生于大的负重关节时。血管和代谢损害的不完全愈合使髓关节易于出现软骨下骨折和塌陷。骨坏死的治疗仍然是尚未解决的临床难题。多种手术方法已被尝试用于防止疾病进展,但没有一种能取得肯定成功的疗效,尤其在病变出现软骨下塌陷后。未来研究应致力于更准确的确定疾病的自然病程,并根据病灶的大小和位置判定治疗效果。探索早期病变的非手术疗法,进行随机、对照、前瞻性研究确定最佳的手术方式。
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