ORIGINAL RESEARCH Intracranial Vascular Anomalies in Patients with Periorbital Lymphatic and Lymphaticovenous Malformations

RESULTS: The malformation was left-sided in 70% of patients. Twenty-two patients (70%) had intracranial vascular anomalies: DVA (n 20; 61%), CCM (n 2; 6%), DAVM (n 4; 12%), pial AVM (n 1; 3%), and SP (n 1; 3%). Arterial shunts were present in the soft tissues in 2 patients (6%). Three patients had jugular venous anomalies. Three patients (9%) had cerebral hemiatrophy, 2 (6%) had focal cerebral atrophy, and 2 had Chiari I malformation. CONCLUSIONS: Intracranial vascular anomalies, some of which are potentially symptomatic and re- quire treatment, are present in more than two thirds of patients with periorbital LM. Initial imaging of patients with orbital LM should include the brain as well as the orbit. L ymphatic malformations (LMs), like other structural vas- cular anomalies, usually present in predictable anatomic locations and patterns. These slow-flow lesions are most commonly located, in order of frequency, in the head and neck, axilla, chest, and proximal upper extremity. These sites of predilection may be related to maldevelopment of primitive jugular, subclavian, and axillary lymphatic sacs. LM in the orbit is uncommon. It typically involves the sub- conjunctival and periocular tissues, and often extends into the adjacent frontotemporal region and cheek. The exten- sive lesions typically cause swelling, intraorbital hemor- rhage, ocular proptosis, blepharoptosis, and cellulitis. Although abnormal lymphatic channels and spaces pre- dominate, they may be combined with anomalous venous channels, in which case they are termed lymphaticovenous malformations (LVMs). Although lymphatic endothelium can now be identified histologically by applying special stains,thesetechniqueswerenotavailableinthepast;often, histologic examination of resected tissue could not distin- guish between LM and LVM. It is known that patients with extensive facial venous mal- formations have a high incidence of associated intracranial developmental venous anomalies (DVAs).1 Likewise, intra- cranial vascular anomalies (ICVAs) have been described in some patients with periorbital LVMs. We undertook this study to confirm the association of orbital LM (or LVM) and ICVA and to document the frequency of this association, as well as its clinical significance.