Rootman Daniel B, Diniz Stefania B, Cohen Liza M
Division of Orbital and Ophthalmic Plastic Surgery, Doheny and Stein Eye Institutes, University of California, Los Angeles, United States.
J Neurol Surg B Skull Base. 2021 Feb;82(1):116-128. doi: 10.1055/s-0040-1722702. Epub 2021 Mar 23.
The systematic classification of vascular disease as proposed and refined by the International Society for the Study of Vascular Anomalies (ISSVA) divides vascular pathology first into tumors and malformations. Malformations are described as simple and complex, where simple malformations contain a single vascular system and complex malformations comprised of multiple vascular systems. Arteriovenous malformations are considered in terms of inflow characteristics which are primarily responsible for the key management challenges. Management utilizing endovascular embolization and/or surgical resection is often employed; however, recurrence can occur, particularly in diffuse cases. There may be an increasing role for systemic antiangiogenic therapy in such cases. Lymphaticovenous malformations are divided into the principle components on the lymphatic and venous sides for clarity of discussion. Lymphatic malformations are described morphologically as macrocystic and microcystic, and physiologically in terms of the processes responsible for growth. In both cases, surgical options are challenging and local therapeutics intended to close large luminal spaces in the case of macrocystic and to slow biological signaling for growth in microcystic. Venous malformations are described physiologically in terms of flow and distensibility, as volume plays a critical role in the limited space of the orbital cavity. Combined embolic-surgical approaches can be effective for management. More complicated, combined lesions can be managed by dividing the lesion into principal components and treating each appropriately.
国际血管异常研究学会（ISSVA）提出并完善的血管疾病系统分类法，首先将血管病理学分为肿瘤和畸形。畸形被描述为简单型和复杂型，其中简单畸形包含单一血管系统，复杂畸形由多个血管系统组成。动静脉畸形根据流入特征来考虑，这些特征是关键管理挑战的主要原因。通常采用血管内栓塞和/或手术切除进行治疗；然而，复发可能会发生，尤其是在弥漫性病例中。在这种情况下，全身抗血管生成治疗的作用可能会越来越大。为了便于讨论，淋巴管静脉畸形分为淋巴和静脉两方面的主要成分。淋巴畸形在形态上被描述为大囊型和微囊型，在生理上根据生长相关过程来描述。在这两种情况下，手术选择都具有挑战性，对于大囊型，局部治疗旨在封闭大的腔隙，对于微囊型，旨在减缓生长的生物信号传导。静脉畸形在生理上根据血流和扩张性来描述，因为体积在眼眶有限的空间中起着关键作用。联合栓塞 - 手术方法对治疗可能有效。更复杂的联合病变可以通过将病变分为主要成分并分别进行适当治疗来处理。
