多发性外骨骼增生综合征及基底动脉动脉瘤1例报告。

Avraam Ploumis, Andreas Liampas, Michail Angelidis, Areti Theodorou, Vasilios Xydis, Ioannis Gelalis, Peter Zampakis, Vasilios Panagiotopoulos
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摘要

背景:遗传性多发性外生骨瘤(HME)是一种遗传性遗传病,以形成多个骨软骨瘤为特征,发展贯穿整个儿童期直至青春期。与HME相关的血管并发症并不常见。方法:一个病例的HME谁住进医院蛛网膜下腔出血(SAH),由于急性破裂的基底动脉瘤(BTA)的结果,将提出。本文将系统地回顾相关文献。结果:我们描述了一个罕见的病例,48岁的男性患者在上肢和下肢出现多发性外生骨瘤,没有这种病变的家族史。在短暂(5天)的头痛史后,患者经历了意识丧失的发作,随后出现了痉挛性发作,最终证实是由于BTA破裂而继发于SAH。患者无外伤、颈部操作或既往感染史。动脉瘤成功治疗血管内手术(动脉瘤闭塞与线圈)。经过适当的治疗和康复计划,患者逐渐从吞咽困难和四肢瘫痪中恢复过来,几乎完全恢复。在系统综述中,共发现8例椎基底血管系统卒中继发于单发性脊柱骨软骨瘤或多发性骨软骨瘤的病例(包括本文报道的1例),但仅本病例伴有基底动脉动脉瘤。结论:尽管骨软骨瘤患者基底动脉动脉瘤的发病机制尚不清楚,但医学界需要意识到这种罕见的情况,因为SAH可能是一种严重的并发症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Multiple Exostoses Syndrome and Basilar Artery Aneurysm: A Case Report.

Background: Hereditary multiple exostoses (HME) is an inherited genetic condition, characterized by the formation of multiple osteochondromas, developing throughout childhood and into puberty. Vascular complications associated with HME are uncommon.

Methods: A case of a patient with HME who was admitted to hospital with subarachnoid hemorrhage (SAH), as a result of acute rupture of a basilar tip aneurysm (BTA), will be presented. Relevant literature on this topic will be systematically reviewed.

Results: We describe a rare case of a 48-year-old male patient presenting multiple exostoses in both upper and lower limbs, with no familial history of such lesions. The patient experienced an episode of loss of consciousness, followed by tonal seizures, after a short (five-day) history of headache, proved finally to be secondary to SAH due to rupture of a BTA. There was no antecedent of trauma, neck manipulation, or previous infection. Aneurysm was successfully treated with the intravascular procedure (aneurysm occlusion with coil). Progressively, the patient recovered from dysphasia and tetraparesis, almost completely, following the appropriate treatment and rehabilitation program.In the systematic review, eight cases (including the one presented) of vertebrobasilar vascular system stroke secondary to solitary spinal osteochondroma or multiple osteochondromas were found, but only the present case was associated with basilar artery aneurysm.

Conclusion: Despite the fact that the etiopathogenesis of basal artery aneurysm presentation in a patient with osteochondromas remains unknown, medical society needs to be aware of this rare condition, as SAH may be a severe complication.

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