Urbano Jose, Viteri-Noël Adrian, Cobos-Alonso Jorge, Del Olmo Vicente Gómez
Vascular and Interventional Radiology, Ramón y Cajal University Hospital, IRYCIS, 28034 Madrid, Spain.
Internal Medicine, Hospital Universitario Ramón y Cajal, IRYCIS, 28034 Madrid, Spain.
Radiol Case Rep. 2025 May 12;20(8):3684-3688. doi: 10.1016/j.radcr.2025.04.057. eCollection 2025 Aug.
Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is an autosomal dominant genetic disorder that interferes with angiogenesis and leads to abnormal vascular development. It presents as mucocutaneous telangiectasia when small or arteriovenous malformations (AVMs) when large. AVMs involve different organs, such as the lungs, brain, liver, spinal cord, and gastrointestinal tract, and can cause various life-threatening complications depending on their location and size. Splenic vascular malformations are rare in patients with HHT and require treatment owing to the risk of rupture and spontaneous hemoperitoneum. This case report details the management of an 8-cm atypical AVM located in the spleen, which was treated with selective transarterial embolization using a liquid embolic agent.
遗传性出血性毛细血管扩张症(HHT),也称为伦杜-奥斯勒-韦伯综合征,是一种常染色体显性遗传病,会干扰血管生成并导致异常的血管发育。小的表现为黏膜皮肤毛细血管扩张,大的则为动静脉畸形(AVM)。动静脉畸形累及不同器官,如肺、脑、肝、脊髓和胃肠道,并根据其位置和大小可导致各种危及生命的并发症。脾脏血管畸形在HHT患者中较为罕见,由于有破裂和自发性血腹的风险,需要进行治疗。本病例报告详细介绍了一例位于脾脏的8厘米非典型动静脉畸形的治疗情况,该病例采用液体栓塞剂进行选择性经动脉栓塞治疗。