title: Fluctuating nature of an orbital venous-lymphatic anomaly in association with intracranial vascular malformations: a classical presentation.
abstract: Venous-lymphatic anomalies (VLA) are rare and benign congenital lesions of the lymphatic system, composed of endothelial-lined lymphatic cysts. They are most frequently located in the region of the head and neck, and represent 4% of all orbital masses. In those patients with extensive orbital VLAs, a strong association with intracranial vascular anomalies has been reported. Factors known to suddenly increase the size of these lesions include upper respiratory tract infections or intralesional haemorrhage; however, complete spontaneous regression is rare. We report on the classic presentation of a patient with a fluctuating right orbital VLA in association with an intracranial cavernous malformation and intracranial developmental venous anomaly.
authors.affiliation: Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Sivashakthi
authors.initials: S
authors.lastname: Kanagalingam
authors.affiliation: Johns Hopkins Hospital, Baltimore, Maryland, USA.
authors.forename: Emily
authors.initials: E
authors.lastname: Wyse
authors.affiliation: Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Shannath L
authors.initials: SL
authors.lastname: Merbs
authors.affiliation: Department of Radiology, Johns Hopkins Hospital, Baltimore, Maryland, USA Department of Radiology, Children's National Medical Center, Washington DC, USA.
authors.forename: Monica Smith
authors.initials: MS
authors.lastname: Pearl
mesh_terms: D000293:Adolescent; D001921:Brain / Q000473:pathology*; D005260:Female; D005901:Glaucoma / Q000209:etiology; D006801:Humans; D044148:Lymphatic Abnormalities / Q000473:pathology* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D014057:Tomography, X-Ray Computed; D054079:Vascular Malformations / Q000473:pathology* / Q000601:surgery