PDF(Pubmed)
Abstract：
Melorheostosis is a rare benign bone pathology involving bone dysplasia and hyperostosis. The disease can be recognized with a characteristic radiographic feature of radiopaque lesions dripping along a long bone\'s diaphysis. The aberrant bone formation and development manifests mainly as pain, edema, and paresthesia of the affected limb. Severe cases may report limb deformity as well as limited range of motion. Until now, there have been approximately 300 cases reported about melorheostosis worldwide and its diverse clinical picture and age distribution. In Vietnam, there is only one known case of melorheostosis discovered incidentally via radiography. The scarcity of cases presents a challenge within the medical community in recognizing and diagnosing the condition, and a delayed diagnosis can lead to severe contracture and compromised limb motility. In this article, we reported an 82-year-old case of polyostotic melorheostosis with late onset and predominant edema, affecting the sternum, the ribs, and multiple bones of the right extremities and presented our clinical approach for a geriatric patient with chronic limb edema. Our case is distinctive in terms of anatomical location as well as the predominant 20-year non-pitting edema. A prompt diagnosis was made upon the classic dripping candle wax radiographic features emphasizing the role of plain X-ray in establishing the diagnosis without extraneous utilization of other modalities and invasive procedures. Exclusion of other causes of chronic edema such as lymphadenopathy, malignancy as well as parasitic infection is of clinical importance.
摘要：
Melorheostosis是一种罕见的良性骨病理，涉及骨发育不良和骨增生。该疾病可以通过沿长骨骨干滴落的不透射线病变的特征性影像学特征来识别。异常的骨形成和发育主要表现为疼痛，水肿,和患肢的感觉异常。严重病例可能报告肢体畸形以及活动范围有限。直到现在,全球约有300例报告了有关melorheostosis及其不同的临床表现和年龄分布。在越南,只有一例已知的通过射线照相偶然发现的melorheostosis病例。病例的稀缺在医学界认识和诊断疾病方面提出了挑战，延迟诊断会导致严重挛缩和肢体运动受损。在这篇文章中,我们报道了一例82岁的多发性骨性恶性骨病，迟发性和以水肿为主，影响胸骨,肋骨,和右肢的多个骨骼，并介绍了我们对患有慢性四肢水肿的老年患者的临床方法。我们的病例在解剖位置以及主要的20年非凹陷性水肿方面是独特的。根据经典的滴落蜡烛蜡射线照相特征进行了迅速诊断，强调了普通X射线在建立诊断中的作用，而无需额外利用其他方式和侵入性程序。排除慢性水肿的其他原因，如淋巴结病，恶性肿瘤以及寄生虫感染具有临床意义。
