Children's Healthcare of Atlanta, Vascular Anomalies Clinic, Atlanta, Georgia, USA.
Division of Pediatric Hematology-Oncology, Children's Healthcare of Atlanta, Aflac Cancer and Blood Disorders Center, Emory University Medical Center and Children's Pediatric Institute, Atlanta, Georgia, USA.
Pediatr Blood Cancer. 2022 May;69(5):e29610. doi: 10.1002/pbc.29610. Epub 2022 Mar 2.
Congenital venous malformations (VMs) are low-flow vascular anomalies that can cause coagulation abnormalities. This phenomenon, referred to as localized intravascular coagulopathy (LIC), is characterized by elevated D-dimer, hypofibrinogenemia, and/or thrombocytopenia. Increased risk for LIC includes patients with an extensive VM, multifocal VM, and Klippel-Trenaunay and CLOVES syndromes. Peri-procedural anticoagulation has been given to prevent complications from LIC in patients undergoing surgical/interventional procedures; however, the rate of clinically relevant complications from sclerotherapy is largely unknown. The purpose of this study is to describe a single-institution's incidence of LIC in patients with VMs and coagulopathy-related complications following sclerotherapy.
DESIGN/METHODS: Retrospective chart review of patients, 0-21 years of age, with VM who underwent sclerotherapy without peri-procedural anticoagulation and had coagulation profiles evaluated within 1 month prior to sclerotherapy.
diagnosis type (high vs. low risk for LIC), coagulation profile (including PT/PTT, D-dimer, fibrinogen, and platelet count), sclerosant used, and post-procedure outcomes. Coagulopathy-related complications included clinically relevant bleeding, deep vein thrombosis (DVT), and pulmonary embolism (PE).
N = 138 patients; 59.4% were female. The most common location of VM was the lower extremity (47.8%; 66/138). Of patients with high-risk VMs (29/138 [21%]), 11/29 (37.9%) had laboratory values consistent with LIC, whereas 5% (5/109) of low-risk VMs had LIC. In sum, 492 sclerotherapy procedures were performed with no complications of bleeding, DVT, or PE.
Patients undergoing sclerotherapy for VM with abnormal coagulation profiles may not require peri-procedural low molecular weight heparin (LMWH). Further studies are needed to precisely define which patients would benefit from anticoagulation.
先天性静脉畸形（VM）是一种低流量血管异常，可导致凝血异常。这种现象称为局部血管内凝血异常（LIC），其特征是 D-二聚体升高、纤维蛋白原降低和/或血小板减少。LIC 风险增加包括广泛 VM、多灶性 VM、Klippel-Trenaunay 和 CLOVES 综合征患者。为了预防手术/介入治疗过程中 LIC 引起的并发症，已经给予围手术期抗凝治疗；然而，硬化治疗后与凝血相关的并发症的临床相关发生率尚不清楚。本研究的目的是描述一家机构接受硬化治疗的 VM 患者的 LIC 发生率以及与凝血相关的并发症。
回顾性分析年龄在 0-21 岁之间的 VM 患者的病历，这些患者在接受硬化治疗时未接受围手术期抗凝治疗，且在硬化治疗前 1 个月内进行了凝血分析。
诊断类型（LIC 高风险与低风险）、凝血分析（包括 PT/PTT、D-二聚体、纤维蛋白原和血小板计数）、所用硬化剂以及治疗后结果。与凝血相关的并发症包括有临床意义的出血、深静脉血栓形成（DVT）和肺栓塞（PE）。
共纳入 138 例患者，其中 59.4%为女性。VM 最常见的部位是下肢（47.8%，66/138）。在 29 例高风险 VM 患者中（29/138[21%]），11/29（37.9%）的患者实验室检查值符合 LIC，而 109 例低风险 VM 患者中有 5 例（5%）发生 LIC。总的来说，138 例患者共进行了 492 次硬化治疗，无出血、DVT 或 PE 等并发症发生。
对于 VM 患者，如果存在异常凝血谱，接受硬化治疗可能无需围手术期低分子肝素（LMWH）。需要进一步研究来精确确定哪些患者受益于抗凝治疗。
