Acute ophthalmic artery occlusion usually is encountered in elderly patients consequent to underlying cardiovascular disease1 and presents with acute and total loss of vision in the affected eye along with signs of choroidal and retinal ischemia. Only two cases have been reported in children, and in both, the condition was precipitated by intralesional corticosteroid injection into periocular tumors.2,3 This article presents a 7-year-old girl with spontaneous ophthalmic artery occlusion in the right eye.
A 7-year-old girl presented with blindness in the right eye, first noted 6 weeks prior to referral to our institution. The child awoke in the morning and was unable to see. An ophthalmologist who had seen her the same day had recorded absence of light perception, ischemic retinal whitening, attenuation of retinal arteries, and a pigmented lesion at the macula in the right eye. There was no history of headache, seizure, motor weakness, diplopia, trauma, febrile illness, or any other precipitating event. Ocular and medical history were unremarkable, and no family history of migraine or blood disorders including hereditary coagulopathies was elicited.
Figure 1 : Fundus photograph of the right eye shows primary optic atrophy, attenuated arteries, pallor of the posterior pole, and reddish-brown discoloration with yellow deposits at the macula.
Ophthalmic examination disclosed abnormalities limited to the right eye. Visual acuity continued to be at die level of no light perception with a relative afferent pupillary defect. There was no deviation of visual axis or abnormality in ocular movements, and external and anterior segment examinations were unremarkable. Ophthalmoscopy revealed primary optic atrophy, arterial attenuation, mild pallor of the retina in the posterior pole, and a reddish-brown discoloration approximately two-third disc-diameter in size with glistening yellow deposits in the region of the macula (Figure 1).
Fundus fluorescein angiogram of the right eye demonstrated normal filling of the choroidal and retinal vessels. However, irregular enlargement of the foveal avascular zone with nonperfusion of the peri foveal capillary net and mottled hyperfluorescence in the posterior pole were observed (Figure 2). Visual evoked response was absent in the right eye and normal in the left.
Physical examination was normal with no evidence of hypertension, cardiovascular, or neurologic disease. Serum and urine chemistry (including amino acid profile), total blood cell count, and erythrocyte sedimentation rate were normal. Tests for hypercoagulability (prothrombin time, partial thromboplastin time, lupus anticoagulant, anticardiolipin antibodies, factor VIII activity, von Willebrand factor, antithrombin III, plasminogen, protein C, free protein S, and activated protein C resistance ratio), investigations for systemic vasculitis (serum antinuclear antibodies and other autoantibodies), and serology for toxoplasmosis and syphilis were negative. Electrocardiography, echocardiography, color Doppler imaging of the carotids, abdominal ultrasound, computed tomography of the orbits and brain, and magnetic resonance angiography of the head and neck vessels did not reveal any abnormality.
Figure 2: Fundus fluorescein angiogram of the right eye shows irregular enlargement of the foveal avascular zone with nonperfusion of the perifoveal capillary net and mottled hyperfluorescence in the posterior pole.
Acute ophthalmic artery occlusion is a rare entity and comprises only 2% of retinal arterial occlusions. The majority of affected patients are >40 years with underlying carotid artery disease. Younger patients, when affected, have unusual associations such as Sneddon's syndrome, neurofibromatosis, trauma, and atrial myxoma embolus as well as intralesional steroid injection.1'3,5"13
The ophthalmic artery is the first branch of internal carotid and enters the orbit through the optic canal, accompanying the optic nerve and lying inferolateral to it. The artery then crosses over to the medial side of the optic nerve, bending at a right angle; this sharp turn predisposes to lodgement of emboli.14 While crossing the optic nerve, the ophthalmic artery gives rise to the central retinal artery, which supplies the inner retina and the surface layer of the optic nerve head, and the lateral and medial posterior ciliary arteries, which supply the outer retina, choroid, and remaining portion of the optic nerve head.14
Acute ischemia of the retina and choroid may result from obstruction of the ophthalmic artery or simultaneous interruption of the retinal and ciliary circulations due to embolism, thrombosis, vasospasm, or vascular compression. The obstructed vessels usually reopen with time; however, irreversible ischemic damage to the optic nerve and sensory retina develops within 1-2 hours of complete occlusion.15 Clinically, acute ophthalmic artery occlusion mimics central retinal artery occlusion. However, ophthalmic artery occlusion causes choroidal circulatory compromise in addition to retinal ischemia, giving rise to distinct features that help differentiate it from central retinal artery occlusion.1
Our patient had complete loss of vision with primary optic atrophy, narrowed retinal arteries, and ischemic changes in the posterior pole (retinal pallor and macular infarction) of the right eye, suggesting occlusion of the ophthalmic artery. Mottled hyperfluorescence in the posterior pole due to window defects on fluorescein angiography indicated retinal pigment epithelial drop out in the region, and absent visual evoked response substantiated the clinical impression of severe optic atrophy. These features further supported the diagnosis of ophthamic artery occlusion. Angiographic abnormalities of choroidal perfusion and retinal arterial filling defects characteristic of the acute stage could not be documented as the patient presented late in the illness.
Acute stage management of ophthalmic artery occlusion is similar to that of central retinal artery occlusion and includes firm ocular massage, intravenous acetazolamide and hyperosmotic agents, inhalation therapy with carbogen, and anterior chamber paracentesis. However, the results of treatment are disappointing, visual prognosis is poor, and total blindness occurs in most eyes.1 Injection of tissue plasminogen activator into the ophthalmic artery, which is claimed to benefit patients with central retinal artery occlusion,16 has not been studied in ophthalmic artery occlusion.
Subsequent management is directed toward identification and treatment of any underlying disease, with etiologic considerations being similar to those for central retinal artery occlusion. A thorough and aggressive search for a potential cause is mandatory in view of the increased incidence of cerebrovascular accidents associated with retinal vascular occlusions and risk of involvement of the fellow eye. However, despite extensive investigations, the underlying etiology remains undetermined in a portion of patients with central retinal artery occlusion.17
The causes of retinal arterial obstruction in children and in adults <40 years are different from those in the elderly.18 Atheromatous vascular disease is rare. On the other hand, conditions such as migraine, coagulation abnormalities, sickle-cell hemoglobinopathies, trauma, cardiac disorders, oral contraceptive use, pregnancy, systemic lupus erythematous, intravenous drug abuse, and ocular anomalies (eg, optic nerve head drusen and prepapillary arterial loops) are more commonly encountered. Fibromuscular dysplasia appears to play a role in the cause of retinal arterial occlusions and should be included in the differential diagnosis of predisposing conditions.19 This entity usually involves the renal arteries, and although its presence may be suspected on clinical grounds (unexplained hypertension or renal insufficiency), specific diagnostic maneuvers that include ultrasonography, renal arteriography, and isotopie blood flow scans are necessary for establishing diagnosis. Abdominal ultrasound in our patient did not reveal any abnormality. Renal angiography was not performed as the procedure is associated with a low but significant morbidity and is indicated only when there is a strong clinical suspicion of a stenotic lesion.20
Our patient did not give any history suggestive of migraine or trauma. General and ocular examination as well as investigations failed to reveal any predisposing abnormality. Coagulopathies, both inherited and acquired, heart disease, and carotid artery disease were ruled out. Clinical evaluation along with results of fundus fluorescein angiography and visual evoked response indicated a compromised choroidal circulation in addition to retinal ischemia in the tight eye, implying occlusion of the ophdialmic artery. Spontaneous ophthalmic artery occlusion in the pediatric age group has not been reported previously.
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