Early symptoms and long-term clinical outcomes of distal limb's cutaneous arterio-venous malformations: a retrospective multicentre study of 19 adult patients
Early symptoms and long-term clinical outcomes of distal limb's cutaneous arterio-venous malformations: a retrospective multicentre study of 19 adult patients
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DOI：
10.1111/jdv.12961
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发表时间：
2016-01-01
影响因子：
9.2
通讯作者：
Barbarot, S.
中科院分区：
文献类型：
--
作者：
Guillet, A.;Connault, J.;Barbarot, S.
皮肤动静脉畸形（AVM）是由动脉和静脉之间没有中间毛细血管间隙直接连接而成的高流量血管畸形。“远端肢体AVM”，即累及手或脚，是罕见的，其功能预后通常很差。人们对其早期临床症状知之甚少。本研究的目的是确定远端肢体皮肤动静脉畸形的早期临床症状，并确定其长期临床结果。方法回顾性分析2000年1月至2013年11月在2个地区三级医疗中心随访的成年远端肢体动静脉畸形患者。这些信息是从患者的临床记录中收集的，并通过结构化的电话问卷完成。结果19例患者纳入研究：4例（21%）为足部AVM， 15例（79%）为手部AVM。首发临床症状如下：肿胀（47%），疼痛（47%），一个或多个静脉扩张（37%），罕见的皮肤颜色异常，高热和搏动感。中位诊断延迟为首次表现后9年。在接受治疗的17例患者中，53%接受了栓塞治疗，12%接受了手术，35%两者都接受了。平均随访57.6个月后，接受治疗的13名患者中有31%完全缓解；31%部分缓解；15%的患者在最初改善后复发，23%的患者治疗失败。总体而言，74%的患者有严重的AVM发展：37%的患者有手指或手部截肢，42%的患者仍有症状和/或不稳定。结论本研究提示远端肢体动静脉畸形的初始表现不明显且非特异性，导致诊断延迟约10年，预后较差。当出现以下症状：疼痛和/或肿胀，有时伴有大静脉时，医生应尽早做出诊断。
Introduction Cutaneous arterio-venous malformations (AVM) are high-flow vascular malformations made up of a direct link between arteries and veins without intermediary capillary space. 'Distal limb's AVM', which mean involving hands or feet, are rare and their functional prognosis is often poor. Little is known about their early clinical symptoms. The objectives of this study were to identify early clinical symptoms of distal limb's cutaneous AVMs and to determine their long-term clinical outcome.Methods A retrospective study was carried out including adult patients who had distal limb's AVM, who were followed up between January 2000 and November 2013 in two regional tertiary care centres. The information was collected from patients' clinical records and completed by a structured telephone questionnaire.Results Nineteen patients were included in the study: four (21%) with foot AVM and 15 (79%) with hand AVM. The first clinical symptoms were as follows: swelling (47%), pain (47%), one or several venous dilatations (37%) and rarely abnormal skin colour, hyperthermia and pulsating sensation. The median diagnosis delay was 9 years after the onset of first manifestations. Amongst the 17 patients who underwent a treatment, 53% had embolotherapy session(s), 12% surgery and 35% had both. After an average follow-up of 57.6 months, 31% of the 13 patients contacted who were receiving treatment were in complete remission; 31% had partial remission; 15% had relapse after initial improvement and 23% had treatment failure. Overall, 74% of patients had a serious development of the AVM: 37% had digital or hand amputation, and 42% remained symptomatic and/or unstable.Conclusion This study suggests that initial manifestations of distal limb's AVMs are discreet and non-specific, leading to a diagnosis delay of about 10 years, with poor prognosis. Doctors should evoke the diagnosis earlier, when these symptoms are shown: pain and/or swelling, sometimes with a large vein.