Acute presentation of vascular disease within the orbit-a descriptive synopsis of mechanisms

Abstract

Vascular events in the orbit can present with an acute onset of symptoms, and, if untreated, raised orbital pressure and reduced arterial perfusion can lead to loss of orbital functions. Such events are commonly due to haemorrhage, but can also be due to arterio-venous shunts and very rarely arise from intraorbital vascular occlusion. The likely diagnosis is often evident on taking a thorough history and examination and in most cases, after appropriate imaging, the process can be monitored for progression. Visual failure is usually due to high pressure at the orbital apex, resulting in ischaemic optic neuropathy, and any evidence of a persistent significant visual impairment should prompt intervention.

Figure 1

(a) Patient in whom periocular bruising appeared 3 days after overnight onset of left orbital pain and minor proptosis. (b) Chronic right episcleral venous congestion due to an orbital mass encroaching on the superior orbital fissure, (c) Leakage of fluid into the right orbit due to raised intravascular pressure, secondary to a low-flow dural shunt. (d) Massive orbital haemorrhage and conjunctival bleeding due to acute high-pressure vascular shunting in the left orbit.

Figure 2

(a) Child with gross left proptosis due to orbital haemorrhage after a blow to his left frontal region during a fall; CT shows a large subperiosteal haemorrhage, arising from a frontal plate fracture, with the mass causing major distortion of the globe (b). (c) Elderly diabetic patient with overnight onset of right periocular swelling and minimal change in ocular motility, this being due to a spontaneous orbital haemorrhage; such vasculopathic haemorrhages typically have a ‘beached whale' configuration and occupy the inferior-temporal quadrant (d). (e) Massive loculated left retrobulbar haemorrhage in a 2-year-old child with lymphangioma. (f) Patient with acute onset of right blindness and orbital pain, eyelid bruising appearing 2 days after onset of symptoms; imaging (g) showed a diffuse apical haemorrhage scattered amongst a pre-existing occult venous malformation. (h) Rapid onset of left orbital inflammation secondary to necrosis within an unknown intraocular melanoma.

Figure 3

(a) Persistent left hypoglobus, proptosis and lateral displacement, with marked restriction of eye movements (b and c), at 8 months after onset of retrobulbar haemorrhage. At anterior orbitotomy a well-defined encysted orbital haemorrhage was (d) exposed, (e) drained and the fibrotic, blood-stained ‘ochre' membrane excised (f).

Figure 4

(a and b) Gross left proptosis due to a longstanding intraconal lymphangioma surrounding the optic nerve. The mass, of 17-ml volume, was excised intact (c) with an acuity of 6/5 and good ocular motility after surgery (d).

Figure 5

(a) Mild proptosis and enlargement of the left superior ophthalmic vein due to low-flow dural shunt, the enlarged superior ophthalmic vein showing a reversal of flow-direction (from the normal anterior-to-posterior direction) and arterial wave-form on Doppler ultrasonography (b). Spontaneous low-flow dural arterio–venous shunts, typically arising in patients with chronic vascular disease such as diabetes or hypertension, present with acute engorgement of episcleral veins (c); low-flow shunts, in many cases, resolve spontaneously after many months—in this case at 15 months after onset (d), (e) where impairing ophthalmic functions, persistent high-flow shunts into the cavernous sinus can be treated by cannular placement into the deeper extent of the engorged superior ophthalmic vein (asterisk), with retrograde placement of platinum coils (f) to induce thrombosis within the central venous system.

Figure 6

Localised post-traumatic arterio–venous shunt in the right upper lid sulcus, viewed during (a) the systolic phase and (b) diastolic phase of the cardiac cycle. (c) Young adult with left orbital high-flow arterio–venous malformation that has increased in size and effect for 11 years after onset of symptoms. (d) Marked left proptosis, orbital congestion and optic neuropathy due to acute carotico–cavernous fistula after gunshot injury. Carotid angiography (e) demonstrates intense filling of the cavernous sinus (large arrows) during the arterial phase and inappropriate retrograde filling of the superior ophthalmic vein (small arrow); after balloon embolisation (f) there has been closure of the fistula between the carotid siphon and cavernous sinus.

Figure 7

(a) Acute severe proptosis and ocular surface exposure in a 2-year old with known right mixed venous–lymphatic malformation, the child being referred for orbital exenteration. (b) Imaging showed widespread vascular anomalies throughout the orbit and thrombosis within several parts of the lesion—the cause for the acute severe deterioration of signs—facilitated extensive resection of the mass (c). (d) The child at 18 months after resection of the mass, with an amblyopic eye and some residual vascular anomaly in the superotemporal quadrant of the right orbit.

Figure 8

Orbital ischaemic syndrome arising in an intravenous drug user who slept overnight resting continuously on his right orbit. (a) A day after injury, the right eye was blind and he had no movements on the affected side (attempted upgaze). A few months later there was complete optic atrophy (b), but good recovery of eye movements (c and d).