Department of Neurosurgery, Shanghai Chang Zheng Hospital, Shanghai, PR China.
Department of Radiology, Shanghai Chang Zheng Hospital, Shanghai, PR China.
Brain Inj. 2022 May 12;36(6):810-816. doi: 10.1080/02699052.2022.2077986. Epub 2022 May 23.
As a subtype of neurofibromatosis, the plexiform neurofibroma is a benign, autosomally inherited disorder and predisposed to tumour formation. However, life-threatening haemorrhage into facial plexiform neurofibroma is extremely rare.
In the current study, we showed a facial plexiform neurofibroma case with massive haemorrhage in the cranio-maxillofacial region. An emergent selective angiography of the external carotid artery was performed to identify the offending artery, which was then selectively occluded by the combination of detachable coils and Onyx-34. Thus, the minimally invasive drainage surgery was successfully performed to evacuate the haematoma.
We believe the endovascular embolization achieved its purpose by providing an initial salvage strategy for stopping active haemorrhage in plexiform neurofibroma, allowing surgeons to perform open surgery with lower complications rate.
丛状神经纤维瘤是神经纤维瘤病的一种亚型，为良性、常染色体显性遗传性疾病，易发生肿瘤形成。然而，发生在面部的丛状神经纤维瘤导致危及生命的大出血极为罕见。
本研究报道了 1 例发生在头面部巨大丛状神经纤维瘤大出血的病例。紧急行颈外动脉选择性血管造影以明确责任血管，并用可脱性弹簧圈和 Onyx-34 联合栓塞责任血管，随后成功进行了微创血肿引流术。
我们认为血管内栓塞通过提供初始的抢救策略来停止丛状神经纤维瘤的活动性出血，为外科医生进行并发症发生率更低的开放性手术创造了条件，从而达到了目的。
