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冯雷克林霍增氏神经纤维瘤病中肋间动脉动脉瘤破裂所致的自发性血胸。

Spontaneous hemothorax caused by a ruptured intercostal artery aneurysm in von Recklinghausen's neurofibromatosis.

作者信息

Chang Wei-Chou, Hsu Hsian-He, Chang Hung, Chen Cheng-Yu

机构信息

Department of Radiology, Tri-Service General Hospital and National Defense Medical Center, Taipei, Taiwan.

出版信息

J Formos Med Assoc. 2005 Apr;104(4):286-9.

Abstract

Aneurysms arising from an intercostal artery are very rare vascular malformations in von Recklinghausen's neurofibromatosis, which often have a silent clinical presentation and are difficult to diagnose before rupture. We report a case of von Recklinghausen's neurofibromatosis with massive hemothroax caused by spontaneous rupture of an intercostal artery aneurysm in a 29-year-old man. The diagnosis was eventually confirmed by percutaneous angiography and treated with endovascular embolization. During a 10-month follow-up period, the patient had a satisfactory recovery. This case illustrates that angiography and possible endovascular embolization should be the first strategy in managing hemothorax in patients with von Recklinghausen's disease.

摘要

起源于肋间动脉的动脉瘤是冯·雷克林霍增氏神经纤维瘤病中非常罕见的血管畸形,通常临床表现隐匿,在破裂前难以诊断。我们报告一例29岁男性,因肋间动脉瘤自发破裂导致大量血胸的冯·雷克林霍增氏神经纤维瘤病病例。最终经皮血管造影确诊,并采用血管内栓塞治疗。在10个月的随访期内,患者恢复良好。该病例表明,血管造影及可能的血管内栓塞应作为冯·雷克林霍增氏病患者血胸治疗的首选策略。

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