Department of Radiology, College of Medicine, Chungbuk National University, Cheongju, Republic of Korea.
Department of Radiology, 58928Chungbuk National University Hospital, Chungbuk National University Hospital, Cheongju, Republic of Korea.
J Int Med Res. 2021 Jun;49(6):3000605211025344. doi: 10.1177/03000605211025344.
Neurofibromatosis type 1 (NF1) is an autosomal dominant disease characterized by neuorocutaneous lesions and multisystem involvement. Other notable features of NF1 include vasculopathy in the form of stenosis, occlusion, aneurysm, pseudoaneurysm, arteriovenous deformity, and rupture, which are difficult to manage and can have fatal outcomes. We describe two cases of extensive and progressive brachial artery hemorrhage following blunt trauma in patients with NF1. Management of these patients included combined endovascular and surgical treatment based on the patients' condition. The patients had a poor prognosis because of uncontrolled bleeding. While one patient died, the other survived, but the involved arm was amputated. Endovascular treatment is a widely used, popular, minimally invasive, and safe method to control the bleeding associated with NF1. However, this treatment can be challenging at times. Close collaboration between an interventional radiologist and surgeon is necessary for optimal treatment and careful follow-up for this condition.
神经纤维瘤病 1 型(NF1)是一种常染色体显性疾病,其特征为神经皮肤病变和多系统受累。NF1 的其他显著特征包括血管病变,其形式为狭窄、闭塞、动脉瘤、假性动脉瘤、动静脉畸形和破裂,这些病变难以处理,可能导致致命后果。我们描述了两例 NF1 患者在钝性创伤后出现广泛且进行性的肱动脉出血的病例。根据患者的病情,对这些患者的管理包括联合血管内和手术治疗。由于出血无法控制,这些患者预后不良。其中一名患者死亡,另一名患者存活,但受累的手臂被截肢。血管内治疗是一种广泛应用、受欢迎、微创且安全的方法,可控制与 NF1 相关的出血。然而,这种治疗有时具有挑战性。对于这种情况,介入放射学家和外科医生之间需要密切协作,以进行最佳治疗和仔细的随访。