Orbital Vascular Anomalies: A Nomenclatorial, Etiological, and Nosologic Conundrum

Abstract

Purpose: Vascular anomalies are a heterogeneous group of disorders that frequently present in the periorbital region. They encompass 2 broad entities: vascular tumors, which possess a proliferative endothelium, and vascular malformations, which are basically localized defects of vascular morphogenesis. The primary goal of this review was to address inaccurate or controversial terminology in the oculoplastic literature concerning orbital and periorbital vascular anomalies and to categorize these lesions in an abridged and simplified hierarchical list that adheres as much as possible to the most recent (2018) iteration for the classification of vascular lesions proposed by the International Society for the Study of Vascular Anomalies (ISSVA). The secondary goal of this review was to review and update information regarding the genetic underpinnings of vascular anomalies and the downstream signaling pathways that are subsequently affected as a result of these genetic errors. Methods: A literature review was conducted in PubMed, MEDLINE, PubMed Central, National Center for Biotechnology Information Bookshelf, and Embase for several related keywords including “vascular anomalies, vascular malformations, vascular tumors, and cavernous venous malformation,” both with and without adding the keywords “eyelid,” “orbital,” and “periorbital.” In addition, a detailed search was conducted for controversial or obsolete keywords like “cavernous hemangioma,” “lymphangioma,” and “varices,” again in their systemic and orbital/periorbital context. Results: Crucial issues in the 2018 ISSVA classification regarding the proper categorization of orbital vascular anomalies, particularly venous lesions, were critically evaluated and revised, and a regional, simplified, and abridged modification of the ISSVA 2018 classification was proposed. Conclusions: Interdisciplinary and intradisciplinary dialogue concerning orbital vascular anomalies is seriously compromised due to the lack of a unanimous agreement on terminology and the absence of a unified classification concept system. The authors recommend that oculoplastic surgeons adopt ISSVA terminology whenever technically possible and scientifically sound. However, they also propose modifying the ISSVA 2018 classification specifically to adapt to the peculiarities of vascular anomalies in the periorbital region. At present, the simplified classification proposed here is a preliminary first step towards managing patients with orbital vascular anomalies with greater diagnostic and therapeutic precision, until such time in the future when the entire genetic makeup of orbital vascular anomalies is more completely elucidated. Optimistically, this could pave the way for a more robust classification and the ultimate therapeutic cure.

Two-Year Outcomes of Transcutaneous Non-Image Guided Bleomycin Sclerotherapy in Conjunctival Lymphatic Malformations: A Protocol-Based Management in 16 Eyes

“…Orbital and adnexal vascular anomalies are a heterogeneous group of disorders that encompass 2 broad entities; vascular tumors that possess a proliferative endothelium and vascular malformations, which are localized defects of vascular morphogenesis. 1 Orbital and adnexal LMs fall in the category of vascular malformations and consist of dilated lymphatic vessels containing lymph which clinically appears as a multiloculated cystic lesion. Conjunctival LMs rarely occur in an isolated form and rather typically coexist with deeper orbital LMs.…”

“…2 Although present since birth they may not always be evident until later in life and tend to grow with time without spontaneous regression. 1 These can be classified as superficial, deep, or combined LMs depending on the location. 3 Conjunctival LM represents the superficial component of these LMs and frequently occurs with the orbital and adnexal components.…”

Disorders of the Angular Vein

“…The reported diagnoses for angular vein malformations found in the literature include varix, venous malformation, and cavernous venous malformation. Tawfik et al 19 have noted inconsistencies in naming conventions for vascular anomalies, with diverse terminology used across different specialties. While the angular vein is primarily an extra orbital structure, using Tawfik’s classification scheme, angular vein malformations would be categorized within orbital venous malformations (OVM).…”

Vascular lesions of the pediatric orbit: A radiological walkthrough

“…Historically, the inaccurate and controversial terms used in the ophthalmologic literature limited interdisciplinary communication, leading to poor or mismanagement of vascular lesions ( 5 ).…”

“…However, despite the many efforts, it still has some drawbacks to consider, such as its lack of anatomical specificity. Indeed, it still does not account for some lesions specific to peculiar anatomical sites such as the orbit, while other lesions listed in the classification cannot technically exist in the same location ( 5 ).…”