Department of Biosurgery, Imperial College London, St Mary's Hospital Campus, Paddington, London, UK.
Eur J Vasc Endovasc Surg. 2011 Nov;42(5):696-8. doi: 10.1016/j.ejvs.2011.06.044. Epub 2011 Jul 22.
Ehlers-Danlos syndrome type IV (EDS type IV), the vascular type, results from mutations in the gene for type III procollagen (COL3A1). Affected patients are at risk for arterial, bowel, and uterine rupture. The timing, frequency and course of these events are unpredictable. We report a 50-year-old patient with previous complications of EDS type IV who presented with recurrent varicose veins that subsequent imaging identified as an arteriovenous fistula (AVF) at the site of previous phlebectomy. Patients with EDS type IV present vascular surgeons manifold management problems. A pre-existing diagnosis of EDS type IV should alert the clinician to the risk of unusual presentations, both acutely and as complications subsequent to intervention. Once identified, appropriate investigation and follow-up of these patients by a vascular surgeon is mandated.
埃勒斯-当洛斯综合征四型（EDS 四型），即血管型，是由 III 型前胶原（COL3A1）基因突变引起的。受影响的患者有发生动脉、肠道和子宫破裂的风险。这些事件的时间、频率和过程是不可预测的。我们报告了一位 50 岁的患者，曾患有 EDS 四型的并发症，表现为反复发作的静脉曲张，随后影像学检查发现该静脉位于先前静脉切除术的部位，存在动静脉瘘（AVF）。EDS 四型患者为血管外科医生带来了多种管理问题。预先存在的 EDS 四型诊断应提醒临床医生注意不寻常表现的风险，包括急性表现和干预后的并发症。一旦发现，血管外科医生应进行适当的检查和对这些患者的随访。
