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多发性外生骨疣综合征与基底动脉动脉瘤:一例报告

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

作者信息

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

机构信息

Department of Physical Medicine and Rehabilitation, University of Ioannina, Ioannina, Greece.

Department of Radiology, University of Ioannina, Ioannina, Greece.

出版信息

J Vasc Interv Neurol. 2018 Nov;10(2):28-32.

Abstract

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.

摘要

背景

遗传性多发性骨软骨瘤(HME)是一种遗传性疾病,其特征是在儿童期及青春期会形成多个骨软骨瘤。与HME相关的血管并发症并不常见。

方法

本文将介绍一例HME患者,该患者因基底动脉尖动脉瘤(BTA)急性破裂导致蛛网膜下腔出血(SAH)而入院。将对该主题的相关文献进行系统回顾。

结果

我们描述了一例罕见的48岁男性患者,其上下肢均出现多个骨软骨瘤,且无此类病变的家族史。患者在经历了短暂(五天)的头痛病史后出现意识丧失,随后出现强直性发作,最终证实是由BTA破裂继发SAH所致。无前驱外伤、颈部推拿或既往感染史。动脉瘤通过血管内介入治疗(用弹簧圈闭塞动脉瘤)成功治疗。经过适当的治疗和康复计划,患者逐渐从吞咽困难和四肢轻瘫中几乎完全康复。在系统回顾中,发现了8例(包括本文介绍的这例)继发于孤立性脊柱骨软骨瘤或多发性骨软骨瘤的椎基底血管系统中风病例,但只有本病例与基底动脉动脉瘤相关。

结论

尽管骨软骨瘤患者出现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情况,因为SAH可能是一种严重并发症。

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