Combined venous lymphatic malformations of the orbit (so-called lymphangiomas) association with noncontiguous intracranial vascular anomalies

The authors present seven cases of orbital combined venous lymphatic vascular malformations (CVLVM) (lymphangioma) with evidence of noncontiguous intracranial vascular anomalies. The study design was a review. Radiologic studies of 25 patients with combined venous lymphatic vascular malformations were evaluated for noncontiguous intracranial vascular anomalies. Features of the intracranial anomalies and orbital lesions, their clinical presentation, and prognosis are described. Seven patients (28%) had associated noncontiguous intracranial vascular anomalies. Intracranial hemorrhage occurred in one of these patients. The intracranial anomalies had radiologic characteristics of developmental venous anomalies (DVAs). Diffuse orbital lesions with superficial and deep components (7/7), orbital bony expansion (7/7), and intraconal and extraconal components (4/7) were most common. They involved the inferior orbital fissure and extended into the pterygopalatine fossa in five patients. Involvement of the superior orbital fissure was noted in all seven patients with extension into the middle cranial fossa in three patients. At birth, these patients generally had a visible superficial component and then had episodes of sudden proptosis associated with deep orbital hemorrhages. Visual outcome was poor (20/200 or less) in four (57%) of seven cases. Anterior extension into soft tissues of the face and forehead and other associated vascular lesions, such as palatal involvement, were relatively common. In contrast, CVLVMs (lymphangiomas) without noncontiguous intracranial vascular anomalies were more anterior, less diffuse, less likely to extend into the soft tissues of the face, have associated vascular lesions, or have a poor visual outcome. Orbital CVLVMs (lymphangiomas) may be associated with noncontiguous intracranial vascular anomalies that may bleed. This association with intracranial DVAs has not been reported previously. The intracranial vasculature should be evaluated prospectively in these lesions, especially if they are diffuse.