COMBINED VENOUS LYMPHATIC MALFORMATIONS OF THE ORBIT (SO-CALLED LYMPHANGIOMAS) - ASSOCIATION WITH NONCONTIGUOUS INTRACRANIAL VASCULAR ANOMALIES

Objective: The authors present seven cases of orbital combined venous lymphatic vascular malformations (CVLVM) (lymphangioma) with evidence of noncontiguous intracranial vascular anomalies. Design: The study de sign was a review. Main Outcome Measures: Radiologic studies of 25 pat ients with combined venous lymphatic vascular malformations were evalu ated for noncontiguous intracranial vascular anomalies. Features of th e intracranial anomalies and orbital lesions, their clinical presentat ion, and prognosis are described. Results: Seven patients (28%) had as sociated noncontiguous intracranial vascular anomalies. Intracranial h emorrhage occurred in one of these patients. The intracranial anomalie s had radiologic characteristics of developmental venous anomalies (DV As). Diffuse orbital lesions with superficial and deep components (7/7 ), orbital bony expansion (7/7), and intraconal and extraconal compone nts (4/7) were most common. They involved the inferior orbital fissure and extended into the pterygopalatine fossa in five patients. Involve ment of the superior orbital fissure was noted in all seven patients w ith extension into the middle cranial fossa in three patients. At birt h, these patients generally had a visible superficial component and th en had episodes of sudden proptosis associated with deep orbital hemor rhages. Visual outcome was poor (20/200 or less) in four (57%) of seve n cases. Anterior extension into soft tissues of the face and forehead and other associated vascular lesions, such as palatal involvement, w ere relatively common. In contrast, CVLVMs (lymphangiomas) without non contiguous intracranial vascular anomalies were more anterior, less di ffuse, less likely to extend into the soft tissues of the face, have a ssociated vascular lesions, or have a poor visual outcome. Conclusions : 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 v asculature should be evaluated prospectively in these lesions, especia lly if they are diffuse.